Molecular Imaging in Neurology Clinic 2019
Perfusion Glucose Dopamine Amyloid Molecular Imaging in CNS Diseases
Satoshi Minoshima, MD, PhD Department of Radiology and Imaging Sciences CBF SPECT FDG PET DAT SPECT Amyloid PET University of Utah
Current Clinical Applica ons FDGWhich PET patient shows hasmore Alzheimer’s marked abnormalities. disease? • FDG PET – Demen a: Alzheimer vs frontotemporal – Epilepsy: Pre-surgical localiza on – Brain tumor: Recurrence vs. necrosis • Amyloid PET – Possible Alzheimer; mild cogni ve impairment • Dopamine SPECT – Benign tremor vs. neurodegenera ve Parkinsonian – Demen a: Alzheimer vs. Demen a with Lewy bodies Alzheimer’s disease No dementia
FDG PET Case: 67 yrs, dementia, MMSE 23/30
J Nucl Med 1995;36:1238-1248
Case: 57 yrs female, impaired word comprehension, agnosia
Minoshima et al. 2002
Left Temporal Lobe Epilepsy
Left Temporal Lobe Epilepsy Intractable seizure: Pre-surgical FDG PET Statistical Mapping – Z-score Maps
Male, 38 years old, memory loss, generalized tonic/clonic seizure Limbic Encephalitis
Amyloid PET
Robin Smithuis, Radiology Department of the Rijnland Hospital, Leiderdorp, the Netherlands Alzheimer’s Disease Amyloid and Tau PET
Neurofibrillary tangle
Tau Senile Plaque
Amyloid
VA North Texas Health System
William Jagust et al., Neuron 2016; The Telegraph March 2, 2016
Negative Positive Dose and Imaging Timing
Radiotracer Recommended Waiting Period Acquisition Dose/Activity
18F-Florbetapir 370 MBq (10 30-50 minutes 10 minutes mCi) 18F-Florbetaben 300 MBq (8 mCi) 45-130 minutes 20 minutes
18F- 185 MBq (5 mCi) 90 Minutes 20 minutes Flutemetamol
Rowe CC and Villemagne VL J Nucl Med 2011;52:1733-1740 Chet Mathis, ALZForum
TI-weighted images from old MRI scanner? Is this amyloid PET positive or negative? Is this amyloid PET positive or negative? Nega ve amyloid PET
• Indicates sparse to no amyloid plaques • Inconsistent with a neuropathological diagnosis of AD and reduces the likelihood that a pa ent's cogni ve impairment is due to AD.
Is this amyloid PET positive or negative? Is this amyloid PET positive or negative?
Posi ve amyloid PET
• Indicates moderate to frequent amyloid plaques
• This amount of amyloid plaque is present in AD pa ents, but may also in pa ents with other neurologic condi ons & in older people with normal cogni on. • A posi ve scan DOES NOT establish a diagnosis of AD, or other cogni ve disorder. Appropriate Situations
1) Possible Alzheimer’s disease (AD) with an atypical clinical Imaging Dementia—Evidence for course or an etiologically mixed presentation Amyloid Scanning (IDEAS) Study 2) Unexplained Mild Cognitive Impairment (MCI), persistent and progressive A Coverage with Evidence Development 3) Atypically early age of onset (65 years or less in age) Longitudinal Cohort Study
Sponsored by: Alzheimer’s Association Managed by: American College of Radiology American College of Radiology Imaging Network Advisor: Centers for Medicare & Medicaid Services (CMS)
IDEAs Aims PET-MRI Scanner: Structure, Amyloid, Perfusion
• Aim 1: To assess the impact of Aβ PET imaging on the management of pa ents mee ng Appropriate Use Criteria (AUC) • Aim 2: To assess the impact of Aβ PET imaging on hospital admissions and emergency room visits in pa ents enrolled in the study cohort over 12 months
Drzezga, Barthel, Minoshima, Sabri, J Nucl Med 2014 FDA Approval Jan 2011
Dopamine Transporter SPECT
[F-18]DOPA
[C-11]DTBZ [F-18]FP-DTBZ
[C-11]MPH [I-123]β-CIT [I-123]FP-CIT [I-123]Altropane [Tc-99m]TRODAT
[C-11]RAC [I-123]IBF D2 [I-123]IBZM
DaTscan: Current Indica on Parkinsonian Syndrome • • Differen ate essen al tremor (ES) from Progressive supranuclear palsy (PSP) Parkinsonian syndrome (idiopathic Parkinson’s • Mul ple system atrophy (MSA) disease, mul -system atrophy, and • Demen a with Lewy body disease (DLB) progressive supranuclear palsy, etc) • Cor co-basal (ganglionic) degenera on (CBD) • Frontotemporal demen a (FTD) • Differen al diagnosis of DLB from AD (Europe) • A rigid variant of Hun ngton’s disease • Wilson’s disease • Others “Comma” “Dot”
www.fda.gov/downloads/AdvisoryCommittees/.../UCM186404.pdf www.fda.gov/downloads/AdvisoryCommittees/.../UCM186404.pdf
Case Most appropriate • findings 66 year old female • Tremor 1. Normal uptake
• Family history 2. Decreased uptake in the putamen • Essen al tremor versus Parkinson’s disease 3. Decreased uptake in the caudate nucleus 4. Significant asymmetry
Case Most appropriate findings • 65 year old male 1. Normal uptake • Current diagnosis of Parkinson’s disease
2. Decreased uptake • Fine tremor, both hands, gait slowing, rigidity in the putamen 3. Decreased uptake • Worsening motor symptoms in the caudate • MRI microvascular disease nucleus • Ques onable levodopa responsiveness 4. Significant asymmetry Most appropriate Most appropriate findings: findings:
1. Increased 1. Increased background background uptake uptake 2. Normal caudate 2. Normal caudate uptake uptake 3. Decreased 3. Decreased striatal uptake striatal uptake 4. Significant 4. Significant asymmetry asymmetry
Different Types of Region of Interest Specific Binding or Binging Potential (simplified) = (striatal uptake – background) / background
Morton et al, Nuc Med Commun 2005;26:1139 Morton et al, Nuc Med Commun 2005;26:1139
MR-guided High intensity Focused Ultrasound (HIFU) Summary
• Clinically available molecular imaging • Demen a, epilepsy, movement disorders • FDG PET and sta s cal mapping • Amyloid PET and Appropriate Use Criteria • DAT SPECT for movement disorders
Dobrakowski, et al., Interv Neuroradiol. 2014;20(3):275-82
New FDA-approved Non-invasive Therapy for Essen al Tremor Salt Lake City – UU, The Temple, Sundance Film Festival, Goldman Sachs, Adobe What the Neurosurgeon Needs to Know
• Presurgical Imaging Brain Tumor Imaging: What the Surgeon Needs to Know • Intraoperative Imaging • Postoperative Imaging Sarah T. Menacho, M.D. Assistant Professor of Neurosurgery and Critical Care Brain and Spine Imaging Conference 8/8/2019
Pre-Surgical Imaging Differential Diagnosis
• Tumor • Differential Diagnosis • Stroke • What further imaging is recommended • Demyelinating
• Imaging interpretation • Vascular Malformation
• Radiation Necrosis
• Pineal lesions
• LMD
• Lymphoma
When Not To Operate • Admitted to MICU, intubated, on pressors, GCS 11T
• 58 year old female presenting with altered mental status, fever, elevated wbc count following recent lumbar fusion surgery • 66 year old male with 1 month of headaches, recently • Pathology: Renal Cell Carcinoma treated for sinus infection without relief
• Taken to OR for transsphenoidal biopsy
Further Imaging Recommended MR Spectroscopy • fMRI
• MR spectroscopy
• CTA/DSA
• CT C/A/P
• Lab Values
• LP
Imaging Interpretation • 33 year old female with first-time seizure episode • Eloquent cortex
• Proximity to vasculature
• Extension into the ventricle
• Osseous Invasion
• All of the above affects my ability to obtain a GTR, and changes how I counsel the patient • fMRI obtained to locate Wernicke’s area • Pathology: AA Intraoperative Imaging • 17 year old female with progressively worsening headaches
• OR for right frontal craniotomy and resection of tumor in Postoperative Imaging intraoperative MRI suite
• Gross Total Resection?
• Complications?
• Radiology Humor
“Interval placement of right frontal approach ventriculostomy catheter” Some Imaging is Obvious… • 53 year old male found down on the ski slopes at Deer Valley Thank You! Questions?
• Karen Salzman, M.D. • Brad Wright, M.D.
• Anne Osborne, M.D. • Jeff Anderson, M.D., PhD
• Richard Wiggins, M.D. • Chris Davidson, M.D.
• Troy Hutchins, M.D. • Edward Quigley, M.D.
• Lubdha Shah, M.D.
Acute Pediatric CNS Disorders Disclosures
• Author – Elsevier, Wolters Kluwer, and CRC Press
• Honoraria for teaching
• Compensa on Gary L. Hedlund, D.O. – Medical-legal case review & tes mony University of Utah School of Medicine Department of Radiology Salt Lake City, Utah
Seizing 4-year-old male
Courtesy Dr. Nicholas Satovick
DDx Varicella-Zoster Cerebelli s
• Infectious / Autoimmune • Toxic / Metabolic • Neoplastic • Other Opioid Toxicity Neuroectodermal Tumor
Hemophagocy c Heat Stroke Lymphohis ocytosis (HLH)
Heat Stroke Overview Heat Stroke Risks in Pediatrics
• Core body temperature > 40°C • Children and infants are more vulnerable to • Thermoregulatory failure heat stroke! • Multi-organ failure – High surface area : body mass • Signs and symptoms: – Lower proximal to distal heat conductance – severe fatigue, diarrhea, erythematous hot dry skin, – Less robust total sweat produc on diaphoresis, nausea, emesis, dizziness, confusion, – Insufficient acclima on to high heat condi ons à seizure, weakness, tachycardia inadequate HSP synthesis
Heat Shock Proteins and Heat Stress Pathophysiology of Heat Stroke
• Heat stress ac vates HSP
• HSP ac va on: Protects cells from the • Mimics Sepsis – increased metabolic demand, splanchnic hypoperfusion effects of the inflammatory acute-phase hypotension, rhabdomyolysis, hepato-renal failure response • Cytokine eleva on
• Biologically adap ve • Coagula on cascade • Other roles of HSPs: • Endothelial-cell injury and diffuse vascular thrombosis – protein transloca on • DIC even a er core temperature normaliza on – apoptosis inhibi on • Ac va on of heat shock proteins (HSP)
Neuroimaging Spectrum Obtunded 6-year-old male • Several pathophysiological altera ons – Tropism for Purkinje cells • Parenchymal T2 prolonga on • Variable diffusivity altera ons • Occasional hemorrhage – Associated brain injury • Thalami (paramedian nuclei) • Hippocampi • Splenium of corpus callosum • Interarterial watershed zones
DDx
• ANE/H1N1 • MELAS / Stroke • Central herniation / DKA Acute Necro zing Encephali s MELAS
Pediatric Arterial Stroke Central Brain Hernia on Syndrome • Causes Diabe c Ketoacidosis (DKA) – Idiopathic – Trauma c • Rare • Pathophysiology – Cardiac • Significant M&M – Combina on of – Autoimmune osmo c changes • Clinical – Prothrombo c disorders cerebral edema • Protein C & S deficiency – DKA ischemia • Iron deficiency anemia – Increasing somnolence • Sickle cell • Presents before or – Ocular dysfunc on – Syndromes during treatment • Down syndrome – Moya Moya Elsevier – Cardio/Respiratory • Homocysteinuria collapse – Metabolic disorders • MELAS
Central Brain Hernia on Syndrome 5-month-old with fever and seizure Diabe c Ketoacidosis (DKA)
Shrier et al. AJNR 1999 20;1885-1888 Infant CSF Spaces DDx • Meningi s • Subdural hygroma v. cSDH • Leptomeningeal neoplasm – Leukemia – PNET – Melanoma Normal 6-month-old Our Pa ent
H. Flu Meningi s
H. Flu Meningi s
Common Causes of Meningitis Bacterial Meningi s • Clinical – Fever – Lethargy – Poor feeding – Seizure • Imaging – NECT • Expanded CSF spaces • Increased a enua on – MRI – Gold Standard • DWI, SWI, and + C Hedlund GL et al, Chapt 11, Pediatric Neuroimaging. 6th ed. by JA Barkovich, 2019 Elsevier Bacterial Meningi s Granulomatous TB Meningi s • Complica ons – Empyema – Cerebri s/Abscess – CSVT – Infarc on • Outcome – Developmental delay – Hearing loss – Seizures
Subdural hygroma Chronic SDH
Leptomeningeal Neoplasms Leptomeningeal PNET
AML Melanoma Couresy Dr. K Kedzierska Thank You [email protected] IMAGING ACUTE ACUTE ENCEPHALOPATHY ENCEPHALOPATHY Getting It Right When It Counts
• Conundrum in ER, acute care facilities • History, labs, neurologic exam often lacking • Terminology inconsistent • “Altered mental status” • “Delirium” • “Confusion” • “Acute brain dysfunction” • “Found down” • “Not acting right” Anne G. Osborn, M.D.
WORKING DEFINITION ALTERED MENTAL STATUS How Common Is It? Why Is It Important?
• Varying statistics “Altered consciousness with change in • 1-10% of ED patients cognition and/or with a perceptual • University of Utah Medical Center disturbance developing over hours or days • “Altered mental status” searched as imaging indication and that which was not better accounted • > 6000 cases since 1/1/2013 for by a pre-existing or evolving chronic • 3288 NECTs • 705 MRs dementia.” --American Psychiatric Association
“MUST MAKE” DIAGNOSES DISCUSS BY URGENCY Causes of Acute/Hyperacute Encephalopathy
• Trauma • Hyperacute (minutes count!) • Middle-aged/older adult “found down” • Treatable • Alcohol • Non-treatable • Acute alcohol poisoning • • Methanol poisoning Acute (hours to two days) • And …. • Treatable • Non-treatable • Drugs and toxins • Subacute (several days to a few weeks) • Metabolic derangements • Must recognize but not time-sensitive • Stroke • Can be going on for weeks ± exacerbation • “Weird” (unusual) strokes • Seizure (nonconvulsive) • Infection, post-infectious
Page 1 ACUTE ALCOHOL POISONING: ACUTE ALCOHOL POISONING Several Days of 1 Gallon Vodka/Whiskey
• Rare • Usually binge drinking • Blood EtOH above 80 mg% • Adolescents • Brain undergoing structural maturation • Uniquely sensitive to EtOH • Use of “hard liquor” in colleges • Alcohol • Reduces forebrain function • Can cause life-threatening brain swelling • Non-convulsive status epilepticus • Acute demyelination • White matter (especially CC splenium) • Visual tracts (especially optic nerves, chiasm)
38yF Ataxia, Delirium in ER after same- day screening colonoscopy ADDITIONAL HISTORY
Prolonged NPO; history of gastric bypass as teen NECT negative; initial labs negative so MRI obtained
Diagnosis???
WERNICKE ENCEPHALOPATHY “MUST MAKE” DIAGNOSES Causes of Acute/Hyperacute Encephalopathy • Can be EtOH, nonalcoholic; can happen in kids! • 50% nonalcoholic (check B1 levels!) • Trauma • Malnutrition, malabsorption • Middle-aged/older adult “found down” • Hyperemesis • Prolonged hyperalimentation • Alcohol • Classic triad in only 30%!! • Acute alcohol poisoning • Pathology • Methanol poisoning • Acute demyelination • And ….Wernicke encephalopathy • Petechial hemorrhages • Drugs and toxins • Location • Metabolic derangements • Medial thalami • Stroke • Mammillary bodies/hypothalamus • “Weird” (unusual) strokes • Tectum • Periaqueductal GM • Seizure (nonconvulsive)
Milone M et al: Wernicke encephalopathy following laparoscopic sleeve gastrectomy-a call to evaluate • Infection, post-infectious thiamine deficiencies after restrictive bariatric procedures Obes Surg 28: 852-853, 2018
Page 2 ACUTE METABOLIC ENCEPHALOPATHIES The “Must Make” Diagnoses HYPERTENSION AND THE BRAIN
• Blood pressure disorders • Acute hypertensive encephalopathy • Acute severe hypertension • Acute hypotensive encephalopathy • Posterior reversible encephalopathy (PRES) • Liver, renal disorders • Malignant hypertension • Glucose disorders • Cerebral hyperperfusion syndromes • Sodium (osmolality) disorders • Chronic hypertensive encephalopathy • Thyroid disorders • Seizure-related disorders
Miller JB et al: New developments in hypertensive encephalopathy. Curr Hypertens Rep 20: 13, 2018
PRES “Posterior Reversible Encephalopathy Syndrome” “ATYPICAL” PRES • Clinical • All ages (peak 20-40y), F > M • “Atypical” > “typical” PRES!! • 92% (not 100%) have BP • Seizure, headache • Not always hypertensive! • Dx often relies on exclusion of other etiologies • Imaging findings variable • Pathoetiology • Rarely just posterior • Failed autoregulation? • “Watershed” zones • BBB disruption • “Central variant” (BG, brainstem, • “Breakthrough” vasogenic edema cerebellum) • Hydrostatic leakage of fluid, blood • Vasculopathy? • May be ONLY areas involved • Diverse causes • Not always reversible (15%) • Eclampsia • Hemorrhage • Immunosuppresive agents • Infarcts • Some foods (e.g., licorice) • May restrict on DWI • Thrombotic microangiopathies • SMART Lee SW, Lee SJ: Central-variant Posterior reversible encephalopathy syndrome…Case Rep • Stroke-like migraine attacks after radiation therapy Neurol 10: 29-33, 2018
“ATYPICAL” PRES “MUST MAKE” DIAGNOSES Acute Metabolic Encephalopathies
• Blood pressure disorders • Liver, renal disorders • Glucose disorders • Osmolality disorders • Seizure-related disorders
Page 3 “MUST MAKE” DIAGNOSES ACUTE HEPATIC ENCEPHALOPATHY Acute Metabolic Encephalopathies • Hyperammonemia • Inherited, acquired • Hepatic, nonhepatic (renal) • Causes • Blood pressure disorders • Viral hepatitis • Drug toxicity • Liver, renal disorders • Valproate • Acetaminophen • Glucose disorders • Chemotherapy • Osmolality disorders • Ammonia, glutamate • Both cross BBB • Thyroid disorders • Pathology, imaging • Seizure-related disorders • Diffuse astrocytic swelling • Laminar cortical necrosis • DDx • Hypoglycemia, HIE • Status epilepticus
HYPOGLYCEMIC ENCEPHALOPATHY HYPOGLYCEMIA 21y Male “Found Down”
• Clinical • Most common endocrine-related AMS! • Etiology • Childhood • Usually type 1 DM • Inherited disorders rare • Adult • Type 2 DM • Relative insulin excess • Pathology • Reflects direct/indirect effects • Cortical necrosis • Parietooccipital • Other (BG, hippocampi) • Usually spares WM!!! Additional history: diabetic!
“MUST MAKE” DIAGNOSES OSMOTIC DEMYELINATION Acute Metabolic Encephalopathies SYNDROME (ODS)
• Blood pressure disorders • Hypoosmolar states • • Wide fluxes in serum Na++ Liver, renal disorders • Osmotic stress • Glucose disorders • Mostly affects oligodendrocytes • Osmolality disorders • Location • Isolated pontine (50%) • Both CPM + EPM (30%) • Exclusively extrapontine (20-25%) • Misc (BG, thalami, hemisphere WM)
Page 4 SEIZURE-RELATED NONCONVULSIVE SEIZURE 74yM in ER with AMS • Post-ictal • Typically resolves in < 1 hour • Non-convulsive seizure • Can be cause of AMS • No tonic-clonic activity • NECT usually normal; MR ±
Clue = cortex + thalamus!!
2d later
INFECTIOUS, PARA-INFECTIOUS, INFLAMMATORY CAUSES OF ACUTE AMS MENINGITIS
• Meningitis • Agents vary • Encephalitis • Pathology similar • Purulent exudate • Herpes (most common nonepidemic) • Predilection for basal cisterns • Autoimmune encephalitides (“brain in flame”) • Perivascular inflammation • Post-infectious/inflammatory • Potentially life-threatening • Cytotoxic injury • Clinical-laboratory diagnosis! • Acute disseminated encephalomyopathy (ADEM) • But “fast track” imaging • Acute hemorrhagic encephalomyelitis (AHLE) • Key to management
Shih RY, Koeller KK: Bacterial, fungal, and parasitic infections of the CNS. RadioGraphics 35: 1141-1169, 2015
INFECTIOUS, PARA-INFECTIOUS, MR OF MENINGITIS INFLAMMATORY CAUSES OF ACUTE AMS
• General features • Meningitis • “Dirty” CSF • Iso- on T1WI • Encephalitis • Hyperintense on T2WI • Autoimmune encephalitides (“brain in flame”) • Hyperintense sulci on FLAIR (nonspecific) • Other findings • Post-infectious/inflammatory • Foci of restricted diffusion in parenchyma • Cytotoxic injury • Exudates may restrict • Acute disseminated encephalomyopathy (ADEM) • Enhancement on T1C+ • Acute hemorrhagic encephalomyelitis (AHLE) • Pia/subarachnoid space most common • Dura-arachnoid pattern less common • Post-contrast FLAIR • More sensitive than T1C+FS
Page 5 CYTOTOXIC INJURY OF THE ACUTE HEMORRHAGIC CORPUS CALLOSUM LEUKOENCEPHALOPATHY
28yF with AMS following viral infection • A.K.A. Weston-Hurst disease • Etiology, epidemiology • ? Hyperacute, fulminant ADEM • After viral illness, vaccination • Rare • Usually fatal • Clinical • Fever, neck stiffness, Sz, MS • Pathology • Perivenular demyelination • Diffuse hemorrhagic necrosis • Focal, petechial WM hemorrhages Starkey J et al: Cytotoxic lesions of the corpus callosum that show restricted diffusion. Nabi S et al: Weston-Hurst syndrome: A rare fulminant form of ADEM. RadioGraphics 37: 562-76, 2017 BMJ Case Rep Oct 19, 2016
IMAGING OF AHLE: AMS, REPIRATORY 69yF, MS After Viral Prodrome FAILURE T2WI GRE SWI
55y male, sheet metal worker, 5y history of cognitive decline, last 2 months altered behavior and hallucinations. No history of alcohol or drug abuse. First MR (not shown) normal. One month later had acute respiratory failure and aspiration, intubated and taken to ICU. Became encephalopathic. Initial CT negative.
AMS, ENCEPHALOPATHIC MRI ICU, INTUBATED, REPIRATORY FAILURE SWI 4+ WM microbleeds
Case courtesy M. Jhaveri, M.D.
Page 6 CRITICAL ILLINESS-ASSOCIATED CEREBRAL MICROBLEEDS AMS SUMMARY • Clinical • If NECT normal, get MR! • Unexplained persistent cognitive impairment after ICU admission • FLAIR, DWI, T2* essential • Patients usually intubated, often have ARDS • Focus on the “must make” diagnoses • +/- on ECMO • Trauma • Pathology • Look for small SDH, disproportionate mass effect • Clusters of RBCs, surrounding WM degradation • Beware the elderly patient with repeated falls • Imaging • Think about alcohol, drugs and toxins • T2/FLAIR hyperintensities variable; can be absent • Acute hyper-, hypotensive encephalopathy • Remember: Atypical PRES > classical! • “Black dots” on T2* SWI (GRE may be subtle) • Can be normotensive! • Juxtacortical WM, corpus callosum; brainstem, peduncles • Acute metabolic encephalopathy • Deep WM, basal ganglia, thalami typically spared • Hyperammonemia (diffuse cortex, BG) • Take home message • Hypoglycemia (posterior cortex) • Microbleeds in critical illness may be hypoxemia-related • Osmotic derangements (not always pontine) • Not associated with specific underlying disease • Parainfectious/inflammatory • “Brain in flame” autoimmune encephalitis Fanou EM et al: Critical illness-associated cerebral microbleeds. Stroke 48: 1085-87, 2017 • AHLE (fulminant ADEM) • Critical illness-associated microbleeds
Page 7 Pediatric Neuroimaging for Adult Radiologists
Kevin R. Moore, MD Pediatric Neuroradiologist DISCLOSURE Intermountain Pediatric Imaging (IPI) Vice Chair for Medical Imaging Primary Children’s Hospital The content of this presentation Salt Lake City, UT 84113 does not relate to any product Kevin.moore @ imail.org of a commercial entity; therefore, I have no relationships to report.
Outline Topics that didn’t make the cut • Introduction Acute cerebellitis – Dr. Hedlund – Pediatric specific issues and concepts • – Differences between older vs. younger children • Abusive head trauma • DDx of big, fluid filled head • Pediatric specific topics Making sense of brain midline anomalies – Pediatric cervical spine imaging and injuries • • Injury patterns in toddlers and infants • Seizure imaging evaluation in children • Role of CT and MR • Pediatric posterior fossa brain tumors • Pearls and pitfalls • Pediatric back pain imaging – Imaging the immature (infant) brain • SCIWORA • Normal myelination AFM, ADEM, and transverse myelitis • Use of FLAIR MR in infants • • Pearls and pitfalls • NF-1 in pediatric versus adult patients • Parting thoughts • Fibromatosis coli – H&N!
Imaging children vs. adults Imaging children vs. adults
Children's Health and the Environment WHO Training Package for the Health Sector Children's Health and the Environment WHO Training Package for the Health Sector World Health Organization 2008 World Health Organization 2008 www.who.int/ceh www.who.int/ceh Potential pediatric imaging pitfalls Potential pediatric imaging pitfalls for adult imagers for adult imagers • Smaller patients (and parts) • Mainly though we are talking about little children (babies and toddlers) • Unfamiliar appearance of immature structures (ex: vertebral bodies/discs, brain – Imaging premature and term infants is the most myelination) challenging thing I do • Imaging of older school age children and • Different disease processes than adults adolescents (fortunately) looks a lot more • Different manifestations of same diseases like adult patients than little children do in children and adults (ex: NF-1) – Still need to be aware of pediatric specific • Higher prevalence of (multi-organ) diseases and injuries though developmental or genetic disorders
Pediatric cervical spine Cervical spine injury in children imaging and injuries • Approximately 1% to 4% of children admitted to major trauma centers (2007) • Motor vehicle accidents, falls, sports- related injuries, and auto–pedestrian accidents most common • M > F (especially older children)
Cervical spine injury in children Cervical spine biomechanics in younger children • Most common injury mechanisms by age Disproportionately larger head – Younger (0–9 years): falls, pedestrian • accidents • Weaker neck muscular support – Older : MVA, sports-related injuries • Other unique anatomic, biomechanical features • Most common injury patterns by age – Wedged anterior vertebral bodies Younger: CVJ/upper cervical > subaxial – Shallow, horizontal orientation of facet joints allows – greater motion spine injuries, ligamentous injuries > fractures, more severe/complete spinal cord – Uncinate process (limits lateral, rotational movement) absent < 10 years injuries, higher mortality – Expansile intervertebral disc and annulus fibrosis – Older: injury patterns similar adults - fractures > ligamentous injuries, subaxial – Ligaments and joint capsules can expand more without tearing spine > CVJ/ upper cervical spine Cervical spine injuries in children CVJ injuries in young children
• More malleable spine (compared to adults) • How do you approach imaging? – Better tolerance of mechanical deformation up – Plain radiographs first (usually) to a point – CT or MR if further imaging needed – Permits more forceful, exaggerated head and • MR better for cord, ligaments, soft tissues neck movement compared to adults • CT better for fractures, more readily available during – Observed injuries correlate well with spine initial trauma assessment response to deformational forces • CT seems to be modality of choice for • More ligamentous and upper cord injuries, cross-sectional imaging of pediatric cervical fewer fractures compared to adults trauma at non-pediatric trauma centers
CVJ injuries in young children Basion-axial interval (BAI) and Basion – dens interval (BDI) • How to assess the CVJ on imaging (CT) ? – Qualitative? – Quantitative (e.g. measure something)? • We evaluated this with a trauma service QI project (semi-willingly) to quantitatively measure parameters on ALL cervical CTs – Pre-project sentiment: Tedious, one size fits all busy work, probably not necessary – Post-project sentiment: Tedious, one size fits all busy Same limits reported for children; work, probably not necessary for older kids but helpful BAI < 12 nl BDI < 12 nl less reassuring because of unossified in younger patients spine elements
• We have moved from mostly qualitative to 1. Rojas et al. Reassessment of the craniocervical junction: normal values on CT. AJNR quantitative assessment on all trauma patients Am J Neuroradiol. 2007 Oct;28(9):1819-23 2. Bertozzi JC, Rojas CA, Martinez CR. Evaluation of the pediatric craniocervical junction on MDCT. AJR Am J Roentgenol. 2009 Jan;192(1):26-31
Basion-axial interval (BAI) and Intermountain Pediatric Imaging Basion – dens interval (BDI) CVJ normal values • Atlantooccipital Interval (normal< 2.5 mm) • Atlantoaxial Interval (normal< 3.9 mm) • Predental Space (normal < 2.6 mm)
Smith, Linscott LL et al. Normal Development and Measurements of the Occipital Condyle-C1 Interval in Children and Young Adults. AJNR Am J Neuroradiol. 2016 May;37(5):952-7 10 years 8 months 10 months Rojas et al. Evaluation of the C1-C2 articulation on MDCT in healthy children and young adults. AJR Am J Roentgenol. 2009 Nov;193(5):1388-92 Pang D et al. Atlanto-occipital dislocation: part 1—normal occipital condyle-C1 interval in 89 children. Neurosurgery. 2007 Sep;61(3):514-21 17 month old male in high 17 month old male in high velocity MVA velocity MVA
Atlantooccipital Interval (normal< 2.5 mm) Atlantoaxial Interval (normal< 3.9 mm) Predental Space(normal < 2.6 mm)
17 month old male in high 34 month old male in high velocity MVA speed MVA
What about this patient?
34 month old male in high 34 month old male in high speed MVA speed MVA
Atlantooccipital Interval (normal< 2.5 mm) Atlantoaxial Interval (normal< 3.9 mm) Predental Space(normal < 2.6 mm) 10 month male in high speed MVA Odontoid synchondrosis fx
OdontoidWhat issynchondrosis the injury? fx
Odontoid synchondrosis fx Summary of pediatric cervical spine injuries • CVJ injuries may be challenging to recognize (especially on CT) – Unossified cartilaginous elements – Ligamentous injuries without fracture • Measuring predental, C0-1, C1-2, especially in young patients, improves injury detection • Be aware of unique pediatric fractures – Odontoid synchondrosis fracture – Ossiculum terminale dislocation
Summary of pediatric cervical spine injuries Imaging the immature brain
• Younger children - predisposition to upper cervical injury, ligamentous > fractures – Big heads, weaker neck musculature, more pliable soft tissues – Spinal cord injuries more common and severe • Older children - injuries more similar to adults Neonate presents to community Normal myelination - CT hospital with seizures – which side is normal? • Unmyelinated WM is hypodense relative to GM, myelinated WM • Gray-white differentiation in neonate accentuated by high WM water content • Density ↑ with myelination relatively subtle – CT insensitive for detecting myelination delays
Hemimegalencephaly
4 month old infant with apnea spells Normal or abnormal myelination ?
6 days 2 weeks 6 weeks 4 months Normal or abnormal MR ? All normal for age
MR myelin evaluation MR evaluation of the immature brain • Normal myelination progress is predictable, • Gray-white differentiation accentuated in organized, and predetermined neonate 2 to high water content in – Myelin maturation proceeds (in general) caudal to rostral, ° central to peripheral, dorsal to ventral unmyelinated white matter • Begins in 5th fetal month → continues into adult life – Mostly finished at 2 years, but continues into 3rd decade • Myelin sheaths tighten and peri-axonal • Appearance is age dependent water is displaced → Increased T1WI, – Need to know corrected gestational age before assigning decreased T2WI signal myelination status • White matter (WM) tracts ↑ in size as • Myelin matures earlier on T1WI than T2WI myelination progresses (ex: corpus – Use T1 up to about 12 months callosum) – T2 more helpful after that Normal myelination – T1WI MR
• Neonate Normal myelination – T1WI MR – Dorsal brainstem – Dentate nucleus – Optic tracts – Anterior commissure – Posterior limb internal capsule – Rolandic and perirolandic gyri – Pyramidal tracts • 2 months – Splenium of corpus callosum – Anterior limb internal capsule – Early optic radiations • 4 months – Genu of corpus callosum – Optic radiations become more apparent – Peripheral rami in pyramidal tracts (perirolandic gyri) • 6 months – Genu and splenium equally hyperintense – Peripheral rami in parietal and occipital lobes become hyperintense 26 week premie term 4 month old 8 month old 2 year old • 8 months – All but most peripheral rami of frontal gyri are hyperintense • 10-12 months – Adult appearance of myelin achieved on T1WI
Normal myelination – T2WI MR • Neonate – Dorsal brainstem – Part of posterior limb of internal capsule Normal myelination – T2WI MR – Perirolandic gyri • 4 months – More hypointense signal in rolandic and perirolandic gyri – Splenium of corpus callosum – More anterior extension in internal capsule • 8 months – Genu and splenium of corpus callosum – Anterior limb of internal capsule – Decreasing signal in centrum semiovale and optic radiations – Decreasing signal in basal ganglia and thalamus • 12 months – External capsule hypointense signal becomes apparent – Expansion of centrum semiovale hypointensity – Clearly defined peripheral rami around central sulcus and in occipital poles • 16 months – Better definition of deep nuclei in brainstem and basal ganglia – Peripheral rami in parietal lobes become hypointense • 18 months 26 week premie term 4 month old 8 month old 2 year old – All but most peripheral frontal white matter rami are now hypointense – Some residual hyperintense signal around trigones of lateral ventricles; "terminal zones" • 36 months – Adult appearance of myelin achieved on T2WI
Normal 1 month old infant 4 month old infant with apnea spells
WM tracts are visible even before they are myelinated Normal ! FLAIR MR in the infant Normal FLAIR MR
• Triphasic appearance of the white matter in first two years of life • Therefore, using to assess brain parenchyma in children < 2 years is challenging
10 days 4 months 4 years
Subdural fluid on FLAIR MR Inflammatory extra-axial fluid on FLAIR MR
NAT 5 month old male E. Coli meningitis 7 month old female
Parting thoughts
• Cervical spine imaging in the very young – Significant biomechanical and anatomical differences between young and older children – Results in different injury patterns – Be attentive for CVJ and pure ligamentous injuries in young children – Consider quantitative craniometric assessment of trauma cervical CTs • Age related myelination maturation drives brain appearance on CT and MR – Need to know patients correct age to assess – Beware of myelin appearance on FLAIR MR before 2 years of age – FLAIR MR useful for evaluating extra-axial processes however Imaging Assessment of Sentinel No Disclosures Head and Neck Hemorrhage: Medical Advisory Board IschemiaView, Inc
Triage to Treatment
Michele H Johnson, MD, FACR, FASER Professor of Radiology and Biomedical Imaging and Neurosurgery Director, Interventional Neuroradiology Yale University School of Medicine
Yale Yale Radiology and Radiology and Biomedical Biomedical Imaging Imaging 2019 Utah Brain and Spine Imaging
Imaging Assessment of Sentinel Head and Sentinel Hemorrhage: Neck Hemorrhage: Triage to Treatment Goals of Non Invasive Imaging
• To identify head and neck lesions with risk • Assess tumor burden of vascular compromise and hemorrhage • Assess vascular structures • Identify types of non-invasive imaging for evaluation of postoperative bleeding, • Assess for extravasation or epistaxis and carotid blowout syndrome pseudoaneurysm • To understand the procedures, alternative, • Assess post-operative anatomy risks, benefits and potential complications of emergent therapy for head and neck bleeding
Yale Yale Radiology and Radiology and Biomedical Biomedical Imaging 2019 Utah Brain and Spine Imaging Imaging 2019 Utah Brain and Spine Imaging
Non Invasive Imaging Options DSA
Catheter angiography is • Non Contrast CT generally reserved for those cases where • Contrast CT significant neovascularity is • CTA expected, or where potential for major • MRI/MRA vessel compromise or vascular disruption is present, requiring endovascular test occlusion, embolization, or stent revascularization. Yale Yale Radiology and Radiology and Biomedical Biomedical Imaging 2019 Utah Brain and Spine Imaging Imaging 2019 Utah Brain and Spine Imaging Vascular Injury DSA Related Complications
Ann Surg 2011;253:444–450 • Dissection • 1% of all patients • Pseudoaneurysm • Major complications- 0.5% • Puncture site complications • Occlusion • Hematomas • Frank rupture • Pseudo aneurysm or A-V fistula • Fistula formation • Femoral occlusions • Iatrogenic injury-stroke • Allergic reactions to contrast • Renal insufficiency
Yale Yale Radiology and Radiology and Biomedical Biomedical Imaging 2019 Utah Brain and Spine Imaging Imaging 2019 Utah Brain and Spine Imaging
Benign and Malignant Tumors Vascular Compression
• Primary or metastatic tumor with vascular compression • Radiation Injury • Sentinel Bleeding • Tumoral bleeding • Pseudoaneurysm • Fistula Formation • Rupture = “Carotid Blow Out” Yale Yale Radiology and Radiology and Biomedical Biomedical Imaging 2019 Utah Brain and Spine Imaging Imaging 2019 Utah Brain and Spine Imaging
14 yo Male with Undifferentiated Head and Oral Bleeding Necessitating Carotid Sacrifice Neck Sarcoma presents with Oral Bleeding Returns with bleeding from right ear 2 m later
Yale Yale Radiology and Radiology and Biomedical Biomedical Imaging 2019 Utah Brain and Spine Imaging Imaging 2019 Utah Brain and Spine Imaging 50 yo Male with SCCA Left Tonsil Radiation Induced CCA Stenosis Presents with Sentinel Hemorrhage
Yale Yale Radiology and Radiology and Biomedical Biomedical Imaging 2019 Utah Brain and Spine Imaging Imaging 2019 Utah Brain and Spine Imaging
50 yo Male with SCCA Left Tonsil Sentinel Hemorrhage –Tonsilar CA Presents with Sentinel Hemorrhage
Yale Yale Radiology and Radiology and Biomedical Biomedical Imaging 2019 Utah Brain and Spine Imaging Imaging 2019 Utah Brain and Spine Imaging
45 yo Female with Base of Tongue SCCA Sentinel Hemorrhage –Tonsilar CA and Sentinel Oral Bleeding
Yale 6-7mm Wallgraft Covered Stent (Boston Scientific, Inc) across the Yale Radiology and Radiology and Biomedical diseased segment resulted in cessation of the bleeding episodes. Biomedical Imaging Imaging 2019 Utah Brain and Spine Imaging 2019 Utah Brain and Spine Imaging 45 yo Female with Base of Tongue SCCA Tongue SCCA with Sentinel Oral Bleeding and Sentinel Oral Bleeding Lingual Artery Pseudoaneurysm PRE EMBO
Yale Yale Radiology and Radiology and Biomedical Biomedical 2019 Utah Brain and Spine Imaging Imaging 2019 Utah Brain and Spine Imaging Imaging POST GLUE EMBO
Recurrent oral bleeding at 1 month post Sentinel Hemorrhage CTA RICA Occlusion
Known nasopharyngeal carcinoma treated with surgery and radiation presented with a sentinel hemorrhage, followed by massive oral bleeding. CTA demonstrated a large pseudoaneurysm on the right treated with carotid sacrifice. Yale Yale Radiology and Radiology and Biomedical Biomedical Imaging 2019 Utah Brain and Spine Imaging Imaging 2019 Utah Brain and Spine Imaging
Carotid rupture – Controlled Carotid Carotid Rupture at Angioplasty Occlusion
Yale Yale Radiology and Radiology and Biomedical Biomedical Imaging 2019 Utah Brain and Spine Imaging Imaging 2019 Utah Brain and Spine Imaging Carotid Rupture NP CA Intraoperative/perioperative Injury
• Direct Surgical Injury • Immediate vascular compromise • Delayed vascular compromise • Carotid Blow Out
Yale Yale Radiology and Radiology and Biomedical She survived, without neurologic deficit, 8 months following Biomedical 2019 Utah Brain and Spine Imaging 2019 Utah Brain and Spine Imaging Imaging the initial bleed. Imaging
Peri-tracheal Bleeding Sphenoid Sinus Surgery –Massive 7 days S/P RND Intraoperative Bleeding
Packed in OR and brought to Yale Yale Radiology and Radiology and angiography. Biomedical Biomedical Imaging 2019 Utah Brain and Spine Imaging Imaging 2019 Utah Brain and Spine Imaging
Pituitary Surgery – Massive Intraoperative Abscess Surgery – Arterial Intraoperative Bleeding Bleeding
Controlled in OR and brought to angiography.
Packed in OR and Yale Yale Radiology and brought to angiography. Radiology and Biomedical Biomedical Imaging 2019 Utah Brain and Spine Imaging Imaging 2019 Utah Brain and Spine Imaging 5 Months S/P Abscess Surgery – Massive 59 yo Male Pre Op CT Inverted Papilloma Epistaxis
f/u
Yale Yale Radiology and Initial Radiology and Biomedical Biomedical Imaging angio2019 Utah Brain and Spine Imaging Imaging 2019 Utah Brain and Spine Imaging
59 yo Male Bleeding Uncontrollably Following Cone Beam Rotational CT Endoscopic Removal of Inverted Papilloma
Yale Yale Radiology and Radiology and Biomedical Biomedical Imaging Post2019 NBCAUtah Brain andEmbolization Spine Imaging Imaging 2019 Utah Brain and Spine Imaging
Carotid Blow Out: Massive Oral, Nasal or Impending CBO and Pseudoaneurysm Peritracheal Bleeding
• Many Different • Treatment strategies Appearances • Carotid sacrifice • High Index of • Embolic agents coils, suspicion
Yale Yale Radiology and Radiology and Biomedical Biomedical Imaging 2019 Utah Brain and Spine Imaging Imaging 2019 Utah Brain and Spine Imaging Carotid Blow-Out Syndrome Massive Oral Bleeding
Yale Yale Radiology and Radiology and Biomedical Biomedical Imaging 2019 Utah Brain and Spine Imaging Imaging 2019 Utah Brain and Spine Imaging
Rupture with Pseudoaneurysm Covered Stent Placement
Yale Yale Radiology and Radiology and Biomedical Biomedical 2019 Utah Brain and Spine Imaging 2019 Utah Brain and Spine Imaging Imaging Imaging No demonstrable Circle of Willis collaterals.
52 yo Female with Tonsillar SCCA S/P Surgery, Recurrent bleeding at 24 hours post stent Radiation and Osteonecrosis presents with Sentinel Hemorrhage
Yale Yale Radiology and Radiology and Biomedical Balloon carotid occlusion Biomedical Imaging 2019 Utah Brain and Spine Imaging Imaging 2019 Utah Brain and Spine Imaging CAROTID BLOW-OUT CAROTID BLOW-OUT
Balloon Test
Yale Yale Occlusion Radiology and Radiology and Biomedical Biomedical Imaging 2019 Utah Brain and Spine Imaging Imaging 2019 Utah Brain and Spine Imaging
DELAYED STENT EXTRUSION
Yale Yale Radiology and Radiology and Biomedical Biomedical Imaging 2019 Utah Brain and Spine Imaging Imaging 2019 Utah Brain and Spine Imaging Traumatic Brain Injury
2019 Brain and Spine Conference n Major public health concern Traumatic Brain Injury n 1.7 M sustain a TBI a year n 1.3 M visits to ED annually Yoshimi Anzai, MD MPH n Causes: fall, motor vehicle University of Utah crash, bike accident, sports related injury, assault, penetrating injury
Traumatic Brain Injury CT is the imaging modality of n Classifications: choice for acute TBI n Mild TBI (GCS 13-15)
n Moderate (GCS 9-12)
n Severe (GCS < 8) n 75% of TBI are mTBI n Efficiency n GCS useful for predicting n Access prognosis for severe TBI n n Inaccurate due to sedation Monitoring TBI patients and intubation in field n Accuracy of detecting neurosugergical cases n No pathophysiological and mechanical information
New Orleans Head CT rule: Who needs CT in the setting of Only for GCS 15 mild TBI n 7 criteria for minor head trauma
n Diagnostic benefit n Headache n Radiation n Vomiting n Costs n Age > 60 n Alcohol or drug intoxication n Amnesia n Who can safely avoid imaging n Visible trauma above clavicle n Prediction Rules n Post traumatic seizure n Clinical Sx & risk factors to predict CT abnormality and surgical intervention n If none is present, safely avoid head CT (sensitivity 97-100%) for intracranial pathology
Haydel MJ, et al. N Engl J Med. 2000 Jul 13;343(2):100-5. Indications for computed tomography in patients with minor head injury. Canadian CT Head rule : GCS 13-15 NOC v.s CCHR for mTBI n 7 criteria for minor head trauma n GCS < 15 at 2 hour trauma n Suspected open or depressed skull fracture
n Any sign of skull base fracture n Age >= 65 n Amnesia for 30 min or more
n Two or more episodes of vomiting n Dangerous mechanism* n IF none of them is present, safely avoid imaging; sensitivity 98.4%
The Canadian CT Head Rule for patients with minor head injury. Stiell IG, et al. Lancet. 2001 May 5;357(9266):1391-6.
Types of Traumatic Brain Injury Epidural hematoma (EDH) - Classic
n Space btw inner table of Primary TBI Secondary TBI calvaria and dura n Skull fractures n Cerebral edema n Temporoparietal:75% n Extra-axial hematoma n Infarction/ischemia n Ass/w fractures: 85-95% (EDH, SDH, SAH) n Herniation n Injury to MMA n Contusion n (Hydrocephalus) n Biconvex shape n Hematoma n Cross the falx, but tend not n Diffuse axonal injury to cross suture n Early Dx and Rx critical n Better outcomes than SDH
Venous EDH Vertex EDH – rare
n Meningeal and diploic veins or dural sinus injury n Parietoccipital region n Vertex from SSS or sphenoparietal sinus • Linear or diastatic Fx n Trans-tentorial Cross midline & sagittal suture n CTV or MRV to • SSS can be lacerated, compressed, evaluate dural sinus or thrombosed injury • Coronal/sagittal key
Vertex EDH Anterior Middle Fossa EDH - benign
Venous – sphenoparietal sinus Anatomically limited Laterally by sphenotemporal suture Courtesy: Ann G. Osborn, MD Medially by orbital fissure Courtesy: Ann G. Osborn, MD
Small acute SDH – Coronal/Sagittal Subdural hematoma (SDH) n Btw meningeal layer of dura and arachnoid n Superficial cortical veins & bridging veins n Shearing injury 2 rotation or linear force n Crescentic shape Other injuries common n Elderly: atrophy Contusions n tSAH infant – shaken baby Excitotoxic brain injury syndrome Poor prognosis Lee JJ et al: Subdural hematoma as a major determinant of short-term outcomes in traumatic brain injury. J Neurosurg Feb 2017 ePub ahead of print
Heterogenous SDH Brain Herniation 1: Subfalcine: under falx, 1 cingulate gyrus, ACA 2: Descend transtentorial: 2 tentorial notch, 4 diencephalon, CN, BA 3: Ascend transtentorial: 3 vermis, cerebellum 5 4: Uncal: tentorial notch, uncus, brain stem, CN III, PCA 5: Tosniler, F Magnum Anzai Y, Fink K: Imaging of Unclotted blood indicating active extravasation: Swirl sign Traumatic brain injury Subfalcine/uncal herniation
Admission
Post-op day 2
10y pedestrian v.s. car Duret Hemorrhage
n Ass/w descending trans- tentorial herniation n Streching perforating vessels from basilar artery n Elongation of brainstem in AP direction n Hemorrhage in pons n Poor functional prognosis
Duret hemorrhage Delayed hemorrhage
Immediate Admission Post-op #2 post-op
30 % of patients – delayed hemorrhage Often areas already injured Usually within 24 hours Subarachnoid hemorrhage (tSAH) SAH, Contusion, SDH
n Space btw arachnoid membrane and pia matter n Hemorrhage from cortical veins Admission n Ass/w skull fx or brain contusions n Strong predictor for outcomes
2 months
Brain contusion Intracerebral hematoma
n Collection of blood (> 5mm) due to disruption of blood vessels n Hemorrhage into relatively normal brain
n Deformational forces n Subcortical white n Mottled hyperdense matter or BG/Thalamus lesions –mixed blood and injured brain
Diffuse Axonal Injury Diffuse Axonal Injury n White matter shearing injury by inertial force n Most common in HS MVA and concussion n Area with different tissue density or rigidity n Longer fibers more susceptible n Some favor TAI > DAI n Axonal injury interrupts axonal transport – neuronal death - link to neurodegenerative disease
DAI CT vs MR CT vs FLAIR vs GRE
GRE FLAIR
SDH, SAH and contusion Traumatic Brain “SWELLING”
n What about the brain under SDH?? n Secondary brain injury n Cascade of events n Traumatic cerebral edema n Perfusion disturbances n Glutamate release, â uptake n Excitotoxic brain injury n Neuroinflammation (inflammasomes, microglial polarization, glymphatic clearance) Courtesy R. Hewlett, MD Ann G. Osborn
Simon DW et al: The far-reaching scope of neuroinflammation after traumatic brain injury. Nat Rev Neurology Feb 10, 2017 ePub ahead of print
SDHs and Secondary Brain Injury When to consider MR for TBI
n Discrepancy between CT and clinical findings – poor neurological status and negative CT
n Persistent symptoms after TBI/concussion EXCITOTOXIC INJURY n Predicting which patients suffer long term psychological and cognitive sequelae (research) J Trauma 2009; 66:1008-1014 n National Institute of Neurological Disorders and n Prospective study in 123 TBI patients underwent Stroke –working scientific advisory panel both CT (1st) and MR in 48h from admission n Need to establish reliable TBI classification n 82/123 pts (67%) CT=MR n 41/123 pts MR showed more findings than CT n No agreement as to definition of mTBI n No changes in management n CT remains the modality of choice in acute TBI n “ more widespread use of acute MRI will be important to provide additional detail necessary for accurate pathoanatomic classification, particularly of TAI/DAI spectrum.”
n Prospective study of 36 adult pts w single mTBI underwent CT (2h) and 3T MR (2w) n 135 mTBI patients from 3 level I trauma centers n GCS 15 (n=24), GCS 14 (n=10), GCS 13 (n=2) n 27% of mTBI w negative CT had abnormalities n 18 pts + on CT (50%), 27 pts + on MR (75%) n SAH is a predictor for poor outcome (E-GCS) n SAH CT 8 MR 2 after adjust demog, clinical, SS factors (OR 3.5).
n EDH 4 3 n >1 contusion on MR (OR 4.5), >4 foci of n SDH 6 11 hemorrhagic axonal injury (OR 3.2) independent
n hTAI 8 17 poor prognostic factors after adjust head CT and n nh TAI 0 4 demog, clinical, & SE factors. n Contusion 13 21 Yuh EL, et al. TRACK –TBI study, Ann Neurol 2013:224-235
MR TBI protocol Susceptibility-Weighted Imaging • SWI initially developed to visualize veins based on BOLD effect n T1 sag n FLAIR Ax – Cor • Accentuates magnetic n DWI susceptibility effects of paramagnetic or n Ax SWI diamagnetic substances n T1 Ax such as hemorrhage, iron, calcium
Detection of blood Summary GRE << SWI GRE SWI n TBI is far from simple n CT remains the first line of test for acute TBI n Herniation and secondary brain injury n MR more sensitive to detect DAI/TAI-mTBI n MR for prognosis assessment n Prediction rules- who can safely avoid head CT
Hunter et al. Emerging Imaging Tools for Use with TBI Research. 2012
Thank you
Neuroimaging Signs of AHT 2019 Brain and Spine Conference Disclosures
• Author – Elsevier, Wolters Kluwer, and CRC Press
• Honoraria for teaching
Elsevier • Compensa on Gary L. Hedlund, D.O. – Medical-legal case review & tes mony University of Utah School of Medicine Department of Radiology Salt Lake City, Utah
Trauma c Acute Subdural Hematoma Signs of AHT An object, quality, or event whose presence or Elsevier occurrence indicates the probable presence or occurrence of something else.
Most characteris c head injury associated with AHT PK Kleinman, Diagnos c Imaging of Child Abuse, 3rd ed. 2016 SDH Prevalence in AHT, as high as 92% Bradford, et al, J Neurosurg Pediatr, 2013 Pali a L et al AJNR, 2015
Cranial Meninges Bridging Veins Newborn, Infant and Young Child
• 48 autopsies Dura – 3 AHT – 5 Accidental
Arachnoid • 3D mapping of BVs – Locations Pia – Total number – Size
Hansel 2008 EC Cheshire et al Int J Legal Med, 2018 Bridging Veins Bridging Vein – SSS Complex Newborn, Infant and Young Child • Number – Mean 54.1 / brain – 94 largest # – AHT mean 30 • Size – Mean diam. 0.93 mm – Range 0.05 – 3.07 mm – “Extremely delicate”
Heat map BVs Sca er plot Elsevier EC Cheshire et al Int J Legal Med, 2018
Source of Acute SDH
BV-SSS-Complex
Meningeal dura
Cor cal artery tear
Aneurysm rupture
Mixed A enua on SDH
Acute hemorrhage
Hyperacute + Acute
Hematohygroma “Acute SDH was evacuated; two avulsed bleeding bridging veins, and a torn Chronic + Acute bleeding vein of Labbe’were iden fied”
Sargent S et al. J Forensic Sciences, 1995 Adamsbaum C, Pediatr Radiol, 2012
Bridging Vein Thrombosis in AHT Venous Injury in AHT Tadpole Sign
628 pa ents (0-2yrs.)
Abnormality in 81 • 29 pa ents • SDH • 40% BV injury • 28 pa ents • AHT • 1 pa ent • Hahnemann et al Eur Radiol, 2015 AccT Choudary AK et al, Pediatr Radiol, 2015
Key Point Restrained 10-week-old Cerebral Vertex Clots SUV rollover
Imaging findings of ruptured bridging veins confirms the trauma c nature of the SDH
BV injury in the infant and young child, is more commonly associated with AHT
Kleinman PK, 3rd ed. Diagnos c Imaging of Child Abuse, 2016 4-Month-old Hemophiliac 4-Month-old Hemophiliac
Non-Trauma c SDH Parting Thoughts
• The most common cause of SDH in the young pediatric patient is trauma and the most common cause of head trauma in the child less than one year of age is AHT
• Inflicted vs. accidental injury is determined by a competent child protection investigation
Thank You [email protected] Demystifying Dementias & Disclosures Neurodegenerative Disorders • None Nicholas A. Koontz, MD Director of Fellowship Programs Dean D.T. Maglinte Scholar in Radiology Education Assistant Professor of Radiology, Otolaryngology-Head & Neck Surgery Department of Radiology and Imaging Sciences, Indiana University School of Medicine
Acknowledgments WARNING
• Edward P. Quigley, MD, PhD • BAD News • GOOD News • Alisha Capps, MD • Neurodegenerative disorders • Imaging does not occur in a & dementia are not easy vacuum (usually) • Priya Rajagopalan, MBBS cases • Answer is often already in • William Fisher, MD the medical record • Aaron Kamer, MD • You just have to go digging • Satisfying cases
Introduction Dementia in Context
• Diverse spectrum of disorders resulting in reduced • 50 million people worldwide living with dementia mental status, cognitive decline, impaired memory, • 10 million new cases each year worldwide psychological changes, & varied physical manifestations • Someone develops dementia every 3 seconds • Dementia = syndrome, not a disease • Global cost of dementia = 1 trillion USD • Impaired thinking, memory, and social abilities • Dementia would be the 18th largest economy in the world • Usually progressive • Giant treatment gap • Neurodegeneration = progressive loss of neuronal • In high income countries, 50-80% of cases unrecognized structure or function • In low income countries, 90% of cases undiagnosed • Occurs with aging
• Expedited (and often incurable) in some disease states Alzheimer’s Disease International. Dementia statistics. http://www.alz.co.uk/research/statistics Growing Dementia Epidemic Considerations for Radiologists
• Neurodegenerative disease may manifest as volume loss • Gray matter atrophy • White matter atrophy • OFTEN lobar predominant! • May show a constellation of imaging findings, including atrophy, signal abnormality, reduced diffusivity… • RARELY enhancement • Imaging findings are almost invariably contextual • Responsibility of the radiologist to review the medical record
Alzheimer’s Disease International. Dementia statistics. http://www.alz.co.uk/research/statistics
MRI Protocol Advice IU Radiology Dementia Protocol
• Volumetric (3D) imaging is key • Sagittal MPRAGE • Ability to perform multiplanar reconstructions • Axial, sagittal, & coronal reconstructions • Post-processing, volumetric analysis • Sagittal 3D FLAIR • Maximize the gray-white differentiation • Axial, sagittal, & coronal reconstructions • MPRAGE, MP2RAGE, SPGR, etc. • Axial DTI • DWI (or preferably DTI) • Axial SWI • T2* GRE (or preferably SWI) • Axial T2WI FS • 3T preferable to 1.5T (never open-sided) • +/- Axial MPRAGE C+ • Contrast is not usually necessary • +/- Coronal T1WI C+ FS (spin-echo)
Quantitative Atrophy Assessment Quantitative Atrophy Measurements
• FDA-approved volumetric MRI assessment software • Autosegmentation with comparison to age-matched normative data • Automatic image upload • Volumetric T1 data transferred via DICOM proxy to cloud- based off-site postprocessing server • Automatic data retrieval • Segmentation maps and atrophy reports returned to PACS • More sensitive & reproducible than human eye alone Quantitative Atrophy Measurements Quantitative Atrophy Measurements
Quantitative Atrophy Measurements Overview
• Introduction • Normal aging brain • Dementia: memory-predominant diseases • Neurodegenerative diseases • Treatable disorders: don’t miss!
Overview Normal Aging Brain
• Introduction • Recognizing abnormal • Normal aging brain requires an understanding of NORMAL aging brain • Dementia: memory-predominant diseases • Neurodegenerative diseases • Wide range of normal • Global volume loss • Treatable disorders: don’t miss! • White matter > gray matter • Thin rim of bright periventricular FLAIR signal • Few white matter “dots” • Mineralized basal ganglia Normal Aging Brain Overview
• Introduction • Normal aging brain • Dementia: memory-predominant diseases • Neurodegenerative diseases • Treatable disorders: don’t miss!
Sagittal T1 FLAIR Axial FLAIR Axial FLAIR
62 year-old man
Early Onset Dementia Prevalence Alzheimer Disease (AD)
• Most common neurodegenerative disease in the US • 5.4 million people affected, 6th leading cause of death Alzheimer Disease • Progressive memory loss, cognitive decline, and inability 19% Vascular Dementia 34% to perform activities of daily living 7% Frontotemporal Lobar Degeneration • Mild cognitive impairment = pre-stage to clinical AD 10% Alcoholic Dementia • Tau proteinopathy with extracellular β-amyloid deposition 12% 18% Dementia with Lewy Bodies & intracellular neurofibrillary tangles Other • Roles of imaging • Exclude other structural abnormalities, identify degree/location Rossor MN, et al. The diagnosis of young-onset dementia. Lancet. 2010;9(8):793-806 of atrophy, identify early in disease (future therapies)
Alzheimer Disease Alzheimer Disease
• Imaging findings • MRI more sensitive than CT • Disproportionate mesial temporal atrophy • Earliest finding • Temporoparietal atrophy • Dilatation of perihippocampal fissure • Posterior cingulate atrophy
Axial FLAIR Sajjadi SA, et al. Can MRI visual assessment differentiate the variants of primary-progressive aphasia?. AJNR Am J Neuroradiol. 2017;38(5): 954-60 Alzheimer Disease Alzheimer Disease
• Nuclear medicine • 18F-FDG PET/CT • Bilateral temporoparietal, precuneus, & posterior cingulate hypometabolism • Amyloid PET • Increased gray matter activity due to amyloid deposition – May been seen earlier than FDG • False positives (10-20%?) Coronal T2WI Axial T2WI FS Axial Fused 18F-FDG PET/CT • False negatives with gray matter volume loss Sagittal 18F-FDG PET
Alzheimer Disease Alzheimer Disease
Axial 18F-FDG PET Axial T1 SPGR Axial Fused 18F-FDG PET/MRI Axial 18F-FDG PET Axial T1 SPGR Axial Fused 18F-FDG PET/MRI
PET/MRI PET/MRI
Amyloid PET (Amyvid) Amyloid PET (Amyvid)
Axial FLAIR FS Axial FLAIR FS Axial 18Florbetapir PET Axial 3D FLAIR Axial 3D FLAIR Axial 18Florbetapir PET
Negative Scan Positive Scan Amyloid PET (Amyvid) Vascular Dementia
NORMAL ABNORMAL • Progressive dementia correlating chronologically with ischemic insults à “step-wise” decline • Men > women, increases with age • Most common subtypes • Multiple cortical infarctions • Subcortical leukoencephalopathy • Leukoaraiosis > 25% of white matter • Isolated strategic infarctions • 30% of large territory infarctions à dementia
Axial 18Florbetapir PET Axial 18Florbetapir PET • Thalamic infarctions
Vascular Dementia Vascular Dementia
• CT findings • MRI findings • Cortical and/or subcortical • Reduced diffusivity in acute hypodensities infarctions • Diffuse periventricular & • Encephalomalacia and deep white matter gliosis in chronic infarctions hypoattenuation • Confluent and patchy bright • Usually near-symmetric T2/FLAIR signal • Basal ganglia infarctions • Subcortical • Periventricular • Pontine Axial NECT Axial FLAIR
Frontotemporal Lobar Degeneration Vascular Dementia (FTLD) • Neurodegenerative syndrome with multiple variants characterized by frontal & temporal lobe degeneration • Behavioral variant FTLD • Apathy, disinhibition • Language variant FTLD • Loss of language use, difficulty producing speech, & repetition • Semantic, non-fluent, and logopenic variants • Younger onset than Alzheimer disease • 40-60 years at onset; 10% of dementia under age 65
Axial T1WI Sagittal FLAIR Language Variant FTLD FTLD
• Semantic variant primary progressive aphasia (PPA) • General imaging findings • Progressive loss of the meaning of words • CT/MRI • Difficulty identifying objects or faces (semantic dementia) • Disproportionate atrophy of frontal & temporal lobes • Non-fluent/agrammatic PPA – OFTEN asymmetric • Impairment of grammar – Dilated frontal & temporal sulci – “Knife-like” gyri • Speech apraxia (inability to form sounds) • 18F-FDG PET • Logopenic • Frontotemporal decreased • Difficulty finding words when speaking glucose metabolism • Narrow attention span • > 95% specificity in distinguishing FTLD from AD Axial CECT
FTLD Variants Primary Progressive Aphasia
• Atrophy pattern can help predict clinical subtype • NOT a perfect paradigm! • Hemispheric predominance • RIGHT frontotemporal atrophy à behavioral variant • LEFT frontotemporal atrophy à language variant • Regional atrophy in primary progressive aphasia (PPA) • Anterior inferior temporal à semantic variant • Perisylvian + frontoinsular à non-fluent variant • Middle temporal + posterior perisylvian à logopenic variant
Sajjadi SA, et al. Can MRI visual assessment differentiate the variants of primary-progressive aphasia?. AJNR Am J Neuroradiol. 2017;38(5): 954-60
Behavioral Variant FTLD Semantic Variant FTLD
Sagittal T1 SPGR Sagittal 18F-FDG PET Axial Fused 18F-FDG PET/CT Axial NECT Axial FLAIR FS Coronal FLAIR FS
Inappropriate touching, aggressive outbursts, perseveration Word finding difficulty, decreased word comprehension Non-Fluent Variant FTLD Alcoholic Encephalopathy
• EtOH use may result in acute, subacute, and chronic neurotoxic effects on the central nervous system (CNS) • Potential CNS manifestations • Cerebral & cerebellar atrophy • Diffuse toxic demyelination • Wernicke encephalopathy +/- Korsakoff syndrome • Marchiafava-Bignami disease
Axial FLAIR FS Axial Fused 18F-FDG PET/CT Axial 18F-FDG PET • Trauma (SDH, SAH, etc.) • Associated with cognitive problems, impaired memory Difficulty “putting words together”, slow speech
Alcoholic Encephalopathy Alcoholic Encephalopathy
• CT/MRI findings Atrophy Acute Alcohol Poisoning Acute Wernicke Encephalopathy • Highly variable! • Atrophy • Generalized cerebral • Superior vermian • Wernicke encephalopathy • Reduced diffusivity + bright T2 signal in mamillary bodies, periaqueductal, thalami Coronal T2WI FS Axial T2WI Axial FLAIR • Marchiafava-Bignami • Callosal signal abnormality
Dementia with Lewy Bodies Dementia with Lewy Bodies
• Atypical parkinsonian-type dementia • Challenging diagnosis to • Fluctuating cognitive impairment make on imaging! • Visuospatial impairment • MRI • Visual hallucinations • Parietal, temporal, and occipital lobe atrophy • Sleep disturbance • Spares the hippocampus • Onset between 50-70 years; men > women – Distinguishes from Alzheimer • Accumulation of α-synuclein protein (Lewy body) • Hypothalamic, putaminal, and substantia innominata atrophy inclusions in cortex, brainstem, and substantia nigra less easily identified • Neuronal loss and hypodopaminergic state Axial T1 MPRAGE Dementia with Lewy Bodies Cerebral Amyloid Angiopathy (CAA)
• Nuclear medicine may also • Cerebral amyloid-β accumulation in small arteries of the help differentiate from brain à fragility and subsequent hemorrhage Alzheimer disease • Older adults, typically > age 60 18 • F FDG-PET • Frequently (~80%) associated with Alzheimer dementia • Occipital hypometabolism • Clinical presentation • 123I Ioflupane (FP-CIT) SPECT (DaTSCAN) • Acute stroke-like symptoms relating to intracranial hemorrhage • Decreased uptake in striatum • Chronic dementia (caudate + putamen) • Spectrum of bleeds (microbleeds à lobar hematoma) • FDOPA-PET • May have a profound inflammatory component • Decreased striatal uptake Axial Fused 18F-FDG PET/CT
Chronic Hypertensive Encephalopathy Cerebral Amyloid Angiopathy (CHE) • MRI findings • Chronic hypertension à lipohyalinosis of small arteries • Disproportionate peripheral, • Impaired arterial autoregulation supratentorial distribution of • Decreased ability to compensate for regional hypoperfusion cortical & subcortical • Demyelination and infarcts microhemorrhages • Blooming “black dots” • Most common in elderly, diabetics, & African Americans • Relative sparing of basal • Clinical presentation ganglia and pons • Memory loss • Superficial siderosis from • Depression prior subarachnoid • Pseudobulbar affect hemorrhage Axial SWI
Chronic Hypertensive Encephalopathy Chronic Hypertensive Encephalopathy
• CT findings • Scattered or confluent white matter hypodensities • Lacunar infarcts • Microhemorrhage • MRI findings • T2/FLAIR bright white matter lesions • Focal gradient susceptibility • Infarctions (various ages) Axial NECT Axial FLAIR FS Axial GRE
CAA versus CHE Chronic Traumatic Encephalopathy
Cerebral Amyloid Angiopathy Chronic Hypertensive Encephalopathy • Neurodegenerative tauopathy resulting from repetitive head trauma • Increasingly recognized, particularly in contact sports athletes (e.g., American Football, boxers) • Clinical presentation • Cognitive dysfunction • Memory loss • Behavior disorders • Depression and anxiety Axial SWI Axial GRE
Chronic Traumatic Encephalopathy Chronic Traumatic Encephalopathy
• Nonspecific! • CT/MRI findings • Age-advanced volume loss • Increased ventricular volume • Periventricular low density and T2/FLAIR hyperintensity • Microhemorrhages (~10%) • 18F-FDG PET • Temporoparietal hypometabolism Axial NECT Axial 3D-FLAIR Axial SWI
CADASIL CADASIL
• Cerebral Autosomal Dominant Arteriopathy with • MRI findings Subcortical Infarcts and Leukoencephalopathy • Patchy and confluent T2/ • Hereditary small vessel disease of the CNS FLAIR white matter hyperintensities • NOTCH3 gene mutation • Anterior temporal lobes • Mean age of onset = 45; MRI findings may appear by 30 • External capsules • Clinical presentation • Paramedian superior frontal • Migraines with aura • Microhemorrhages (~50%) • Recurrent infarcts (often with seizures) • Reduced diffusivity in acute • Cognitive decline, behavioral disturbances, depression infarctions Axial T2WI
Overview Parkinson Disease (PD)
• Introduction • Progressive neurodegenerative disease with loss of • Normal aging brain dopaminergic neurons, leading to: • Dementia: memory-predominant diseases • Tremor • Bradykinesia • Neurodegenerative diseases • Rigidity • Treatable disorders: don’t miss! • Balance impairment • Dementia (50-80%) • Most common movement disorder • Age of onset ~60 years; slightly more common in men
Parkinson Disease Parkinson Disease
• MRI findings • Nuclear medicine findings • Easily over- (and under-) • 123I Ioflupane (DaTSCAN) called on MRI • Loss of dopaminergic neurons • Morphologic changes of the in posterior striatum substantia nigra – Putamen > caudate 18 • Loss of pars compacta • F-FDG PET • Blurring of the pars reticulata • Normal • Distinguishes from atypical • Blurring of red nucleus syndromes
Axial T2WI 123I Ioflupane (DaTSCAN)
123I Ioflupane (DatSCAN) Parkinson Plus Syndromes
NORMAL ABNORMAL • Clinical presentation similar to Parkinson disease, but dementia often precedes parkinsonism • May include • Dementia with Lewy bodies • Multiple system atrophy • Progressive supranuclear palsy • Corticobasal degeneration • Early features suggestive of atypical Parkinson disease • Treatment & prognosis differs 123I Ioflupane 123I Ioflupane Multiple System Atrophy (MSA) Multiple System Atrophy (MSA)
• Sporadic neurodegenerative disease • No effective treatment à fatal • Abnormalities of α-synuclein metabolism resulting in • Two unique clinical (and imaging) subtypes intracellular deposition in neurons and oligodendroglia • Cerebellar (MSA-C; sporadic olivopontocerebellar atrophy) • Typical age of onset 40-60 years • Extrapyramidal (MSA-P; striatonigral degeneration) • Variable presentation • Autonomic (Shy-Drager) removed from MSA • Autonomic dysfunction classification, per current consensus • Cerebellar symptoms • Parkinsonism • Cognitive dysfunction
Cerebellar MSA (MSA-C) Extrapyramidal MSA (MSA-P)
• MRI findings • MRI findings • Cerebellar volume loss and • Atrophy of the putamen increased T2 signal intensity • Dark T2/GRE signal intensity • Hemispheric in putamen • Vermis • “Hyperintense putaminal rim” • Middle cerebellar peduncles sign (T2WI) • Pontomedullary volume loss • May be normal, especially at • Flattening high field strength • Cruciform bright T2 signal intensity in the pons • “Hot-cross buns” sign Axial T2WI FS Axial T2WI
Progressive Supranuclear Palsy Progressive Supranuclear Palsy
• Neurodegenerative tauopathy resulting in deposition of • MRI findings phosphorylated tau protein in the brain • Low specificity! • Clinical presentation • Midbrain atrophy • Supranuclear (vertical gaze) palsy • “Hummingbird” or “Penguin” signs of midbrain atrophy • Postural instability disproportionate to pons • Dementia (sagittal sequences) • +/- Parkinsonian features (often with normal eye movements) • “Morning glory” or “Mickey Mouse” signs (axial) th • Typical onset = 7 decade • Periaqueductal T2 • Median survival 5-6 years hyperintensity Sagittal T2WI
Progressive Supranuclear Palsy Corticobasal Degeneration
• Neurodegenerative disorder with features of FTLD & PD • Due to abnormal accumulation of tau protein in the brain • Clinical presentation • Progressive unilateral or asymmetric parkinsonism • Dystonia • Tremor • Cognitive decline
Sagittal T1 SPGR Sagittal T1 SPGR Emperor Penguin • “Alien limb” • Unresponsive to levodopa therapy Sagittal midbrain-to-pons ratio < 0.15 • Typical age of onset 60-70 years; mean survival 8 years
Corticobasal Degeneration Corticobasal Degeneration
• CT/MRI findings • Asymmetric frontoparietal cortical atrophy • Typically perirolandic • Increased T2 signal in subcortical white matter • Dark T2 signal in putamen & globus pallidi • 18F-FDG PET • Decreased frontoparietal activity Axial 3D FLAIR Axial T1 SPGR Axial T1 SPGR
Huntington Disease Huntington Disease
• Autosomal dominant trinucleotide (CAG) repeat à loss • Imaging findings of GABAergic neurons in basal ganglia • MRI • Classic triad • Striatal atrophy (caudate > putamen) with enlarged frontal • Movement disorder horns of the lateral ventricles • Dementia • Increased striatal T2 signal • Psychosis (juvenile) due to gliosis • Decreased striatal T2 signal • Mean age of onset = 35-45 years (adult) due to iron deposition • Juvenile variant onset < 20 years • 18F-FDG PET • Survival = 15-20 years (adult onset); < 10 years • Decreased glucose (juvenile) metabolism in basal ganglia Coronal T1 SPGR
Huntington Disease (Juvenile) Creutzfeldt-Jakob Disease (CJD)
• Prion disease in which misfolded protein “infection” results in accumulation of the same misfolded proteins • Usually affects patients > 50 years of age • Median survival = 6 months • Typical clinical presentation • Progressive cognitive impairment • Personality changes • Ataxia, myoclonus, and visual problems • Diagnosis: Imaging, CSF (14-3-3 protein), EEG, biopsy Axial 3D-FLAIR Axial T2WI FS
Creutzfeldt-Jakob Disease Creutzfeldt-Jakob Disease
• MRI findings • 18F-FDG PET • Gray matter-predominant • Regional glucose • Frontal, parietal, and temporal hypometabolism in affected cortices (often asymmetric) cortices • Basal ganglia (caudate, • May precede MRI putamen > globus pallidus) abnormalities and thalamus • Heidenhain variant = occipital gray matter à visual problems • Progressive bright T2/FLAIR signal intensity • Reduced diffusivity Axial DTI Axial Fused 18F-FDG PET/CT
Creutzfeldt-Jakob Disease Amyotrophic Lateral Sclerosis
• Neurodegenerative disorder targeting somatic motor neurons in brainstem and spine, large pyramidal neurons of motor cortex, and corticospinal tract fibers • Classically, both upper and lower motor neurons involved • Usual onset in 4th – 7th decade; juvenile form exists • Typical clinical presentation • Upper motor neuron signs (Babinski, spasticity, hyperreflexia) • Lower motor neuron signs (weakness, fasciculations, atrophy) • Bulbar signs (dysphagia, dysphonia, aspiration) Axial FLAIR FS Axial DWI Amyotrophic Lateral Sclerosis Overview
• MRI findings • Introduction • T2/FLAIR bright • Normal aging brain corticospinal tract (minority) • Dementia: memory-predominant diseases • Decreased T2 signal in motor cortex • Neurodegenerative diseases • Reduced diffusivity and • Treatable disorders: don’t miss! fractional anisotropy in corticospinal tract
Axial 3D-FLAIR
Normal Pressure Hydrocephalus Normal Pressure Hydrocephalus (NPH)
• Controversial and incompletely understood entity • CT/MRI findings • Poor venous compliance at superior sagittal sinus à • Ventriculomegaly impaired CSF pulsation reduced resorption at arachnoid villi • Narrow callosal angle • Ventriculomegaly à distorted corticospinal tracts to the legs • Disproportionately enlarged • Clinical presentation subarachnoid space • Dementia (apathy, sleepiness) hydrocephalus (DESH) • Dilated ventricles • Gait disturbance • Enlarged lateral sulci and • Bladder dysfunction (urgency or incontinence) basal cisterns • Crowded convexity sulci • Normal CSF opening pressure (< 18 cm H2O) • Predicts shunt responsiveness Coronal T1 MPRAGE
DESH Pattern NPH Chronic Subdural Hematoma (SDH)
• Risk of traumatic SDH increases with age • Atrophy, coagulopathy, EtOH use increase risk • Acute SDH à Chronic SDH risk also increases with age • Variable clinical presentation • Asymptomatic à loss of consciousness • Associated with dementia, which may be reversible • Burr hole craniotomy and SDH evacuation
Axial FLAIR FS Axial FLAIR FS Frontotemporal Brain Chronic Subdural Hematoma Sagging Syndrome • CT/MRI findings • Uncommon, but likely underrecognized phenomenon • Crescent-shaped extraaxial • Intracranial hypotension à sagging of frontal and collection temporal lobes, transtentorial herniation • May cross suture lines, but not • Mechanical forces on frontotemporal circuits dural attachments • Septate with internal • Clinically, mimics behavioral-variant FTLD membranes • Disinhibition, apathy, somnolence • Enhancing dura & membranes • Commonly associated with headaches • Variable density/signal • May signify CSF leak, spontaneous intracranial intensity, depending on age HYPOtension, or overshunting (if VP shunt) Axial CECT
Frontotemporal Brain Acute Wernicke Encephalopathy Sagging Syndrome • MRI findings • Thiamine deficiency à potentially reversible • Sagging brainstem • 50% due to alcohol abuse • Downward displacement of • 50% non-alcoholic frontal & temporal lobes, • Children, malabsorption, hyperemesis, hyperalimentation brainstem, & tonsils • Acute demyelination with petechial hemorrhage • +/- Dural enhancement • Clinical presentation • +/- Subdural collections • Ataxia • 18F-FDG PET findings • Oculomotor abnormalities • Frontotemporal • Confusion +/- Korsakoff (amnestic) syndrome hypometabolism Axial T2WI FS
Acute Wernicke Encephalopathy Parting Thoughts
• Know the patterns of disease, but… • Recognize that specificity is often low • It takes a team • Neuroradiology + Nuclear Medicine + Neurology • Volumetric pulse sequences, DWI/DTI, and GRE/SWI • Don’t leave home without them! • Increased use of voxel-based morphometry • Take the time to read the patient’s chart • It will improve patient care…and make you look a lot smarter! Axial FLAIR Axial DWI Summary
• Overview of dementia and neurodegenerative disorders • Common and uncommon pathologies • Multimodality imaging appearance • Suggested imaging protocol • Recognize common imaging patterns of these entities • Generate appropriate differential diagnoses • Critical for radiologists to query the medical record Delicate Arch • Radiologists can correlate clinically! Arches National Park, UT • Look for reversible causes of dementia Email: [email protected] Twitter: @nakoontz
Demystifying Dementias & Neurodegenerative Disorders
Nicholas A. Koontz, MD Director of Fellowship Programs Dean D.T. Maglinte Scholar in Radiology Education Assistant Professor of Radiology, Otolaryngology-Head & Neck Surgery Department of Radiology and Imaging Sciences, Indiana University School of Medicine Introduction
• Seizure vs Epilepsy Imaging in Adult • Overview of Epilepsy • Imaging Modalities Epilepsy • Etiologies/Pathology Edward P. Quigley III, MD PhD • Imaging Work up Neuroradiology, University of Utah • Treatments
Seizure vs Epilepsy Seizure:
• “Seizures are the manifestation of abnormal hypersynchronous discharges of cortical neurons” • Uncontrolled electrical/neurochemical “storm” within the brain • Loss of cortical inhibition • Propagation of disordered neuronal activity • Seizure type determined by location of foci and propagation pattern
Epilepsy: Epilepsy Epidemiology
• Constellation of • 9-10% lifetime risk of seizure symptoms • 0.8% prevalence of active epilepsy cases • 2 or more seizures • 3% lifetime risk of epilepsy separated by 24 hours • Aging population may skew these • 1 seizure and interictal numbers EEG correlation • 38% Medically refractory • Recurrent behavioral seizures Epilepsy Functional Classification: Imaging Modalities
• Partial Onset: • CT Less sensitive, Good Screening – partial seizures (consciousness preserved) – complex partial seizures (loss of awareness, post • MRI Sensitive, Screening, MTS MR ictal) • SPECT Activity related to blood flow – secondarily generalized tonic-clonic seizures • PET/CT Activity related to metabolic demand • Generalized Onset: – absence seizures • MEG High temporal and spatial resolution – tonic seizures (extension) • fMRI Superb for localization, treatment – clonic seizures (rhythmic) planning, functional mapping – myoclonic seizures (non rhythmic) – primary generalized tonic-clonic seizures (grand mal) • DTI Assess fiber tracts, organization, – atonic seizures surgical planning
MRI SPECT
99m-Tc Ceretec (HMPAO) SPECT Early Ictal Injection
PET CT Pathology: • Temporal lobe epilepsy – Hippocampal pathology – 2/3 of TLE develop Mesial Temporal Sclerosis • Neocortical epilepsy: structural and aquired abnormalities – Ischemia – Infection – Neoplasm – Developmental – Vascular malformation – Trauma • New: Astrocyte or Metabolic Hypothesis – Astrocytes provide support machinery to maintain neuronal activity – Failure leads to excitotoxic injury 18-FDG PET Interictal Injection – Ca+-dependent glutamate release Temporal Lobe Epilepsy and Mesial Temporal Sclerosis NORMAL VARIANTS THAT SHOULD NOT BE • Hippocampal MISTAKEN FOR DISEASE Anatomy • Amygdala • CA1,2,3,4 • Unfused hippocampal commissure • Normal Variants • Choroid fissure cyst – Hippocampal commissure nonfusion • Prominent perivascular (Virchow- – Choroid fissure cyst Robin) spaces – Perivascular spaces
• Copyright Amirysys
Mesial Temporal Sclerosis Hippocampal Volume Loss
• Primary signs: – Hippocampal Volume Loss – Loss of Normal Cytoarchitecture – Abnormal FLAIR Signal • Secondary: – Amygdala Volume Loss – Forniceal Atrophy – Loss of Parahippocampal white matter
Loss of Cytoarchitecture Forniceal Volume Loss
• .
• Copyright Amirsys Image Courtesy of T. Hutchins Stroke Infection: Neurocysticercosis
• Small % of stroke • Neurocysticercosis presents with is world wide most seizure as primary common cause symptom • Dying larvae • However, venous ischemia has release higher propensity inflammatory for hemorrhage and cascade seizure
NEOPLASMS Infection: Herpes encephalitis • Most common • Temporal lobe and – Fibrillary astrocytoma cingulate tropism – Ganglioglioma – Oligodendroglioma • Hemorrhage, • Less common necrotizing – Dysembryoplastic neuroepithelial tumor (DNET) • Can restrict on DW – Pilocytic astrocytoma – Pleomorphic xanthoastrocytoma (PXA) • Edema on FLAIR – Angiocentric neuroepithelial tumor (ANET) • Can enhance • Acyclovir early
GANGLIOGLIOMA: FIBRILLARY ASTROCYTOMA Epidemiology, Pathology • 1% of brain tumors • 80% < 30y • Slow-growing • Variable malignancy • Predilection for – Low grade (WHO II) hemispheres – Anaplastic (WHO III) – 40% temporal lobe – GBM (WHO IV) – 30% parietal • White > gray matter • Most common cause of chronic TLE • Mature ganglion cells + dysplastic neurons GANGLIOGLIOMA: Imaging Neoplasm: Mixed Ganglioglial
• Spectrum – 52% cyst + nodule – 43% solid • Ca++ common • May remodel skull • Little/no edema • Variable enhancement
NEOPLASM: NEOPLASM: OLIGODENDROGLIOMA: OLIGODENDROGLIOMA:
• Well-differentiated • Well-differentiated • Slow growing • Slow growing • Cortical, subcortical • Cortical, subcortical • May remodel bone • May remodel bone • Often causes • Often causes seizures seizures
Neoplasm: Pilocytic Astrocytoma DYSEMBRYOPLASTIC • Cyst and enhancing NEUROEPITHELIAL TUMOR nodule • Cortical location • WHO I • Multinodular, locally
multifocal • “Mucinous” -appearing cysts • Remodeling of overlying bone • Location: 40%-60% temporal lobe NEOPLASM:FIBRILLARY Neoplasm: Pleomorphic ASTROCYTOMA Xanthoastrocytoma • Supratentorial • Temporal lobe most • Variable malignancy – Low grade (WHO II) common – Anaplastic (WHO III) • Dural tail – GBM (WHO IV) • White > gray matter • Calvarial remodelling • Cyst and nodule
ANET: Imaging Neoplasm: Metastases
• Some cortical hyperintensity on T1WI, FLAIR • Gray + white matter • Stalk-like extension towards ventricle • No enhancement
Neoplasm Mimic CONGENITAL
• Status epilepticus • EMBRYOLOGIC – Developmental “folding errors” • Post ictal edema or • HETEROTOPIC GRAY MATTER venous congestion – Migrational arrest can lead to – Genetic causes (LIS 1 Gene, XLIS, Filamin) enhancement. – Range from entire brain to focal anomalies • Mimic of neoplasm, • SYNDROMES: – Neurocutaneous Syndromes cerebritis, or – Tuberous Sclerosis vasculitis – Sturge Weber Syndrome Adapted from Neurology 57, Issue 12 (December 2001)
Adapted from Neurology 57, Issue 12 (December 2001)
HETEROTOPIC GRAY MATTER: NEURONAL MIGRATION DISORDERS General Features • Heterotopic gray matter – Subependymal – Laminar – Subcortical • Polymicrogyria • Hemimegalencephaly • Tuber cinereum hamartoma
HETEROTOPIC GRAY MATTER: HETEROTOPIC GRAY MATTER: Subependymal Laminar (Band) HETEROTOPIC GRAY MATTER: Congenital: Polymicrogyria Subcortical (Masslike)
SCHIZENCEPHALY: Congenital: Heterotopic gray matter Pathology
• TORCH infection • Typical periventricular Calcification • CMV can cause migrational arrest • Heterotopias • Cortical dyplasia • Germinolytic cysts
Congenital: Hippocampal Congenital: Hemimegalencephaly Malrotation HIMAL • Criteria: (Gamms et al AJNR 2009) • Hamartomatous – Unilateral involvement and incomplete rotation of a overgrowth of a hippocampus that is normal in size and signal intensity but hemisphere abnormally rounded in shape, with blurred inner structure. – Ipsilateral findings of an • Insult to migration atypical collateral sulcus angle and atypical position and size • Macrocrania of the fornix – Corpus callosum is normal, and the temporal lobe remains • Prominent primitive normal in size, though the temporal horn may veins appear enlarged. • Dysplasia Congenital: Tuberous Sclerosis Congenital: Sturge Weber Syndrome Complex • Inherited tumors with • 90% have seizures multiple hamartomae • Calcified subependymal • Failure of cortical nodules (30-80% veins enhance) • Encephalotrigeminal • Subependymal giant cell angiomatosis astrocytoma at Foramen Monro (15%) • Cortical Calcification • Cortical tubers hamartoma • Pial angiomatosis • White matter streaks along radial glial scaffold • Venous occlusion and ischemia
Congenital: Tuber cinereum hamartoma VASCULAR
• AVM • Cavernous Malformation • Post SAH • Superficial siderosis
VASCULAR MALFORMATION: Vascular: Superficial Siderosis Cavernous Malformation
• Uncommon cause of epilepsy • 50% present with seizures • Peak 40-60y but may present in childhood • Discrete lobulated mass – Locules of blood – Hemosiderin rim/”scar” Acquired: TBI DAI IMAGING WORK UP
• New pediatric seizure: Hx, HCT, MRI gad • New adult seizure: HCT, CECT, MRI gad • Medically refractory seizure: – MR seizure protocol without gadolinium – PET CT – EEG – MEG – Stereotactic Guided EEG, Grid, Strip, Surface, Depth Electrodes – Functional MRI – DTI – MR Perfusion?
PET CT TREATMENT
• MULTIDISCIPLINARY APPROACH • EPILEPSY SURGERY • MRI/PET/CT/MEG/EEG/WADA • INVASIVE MONITORING GRID/STRIP/DEPTH/AWAKE • RESECTION: – Tractotomy, Cortical Fenestration, Hippocampectomy, Amygdalectomy, Temporal Lobectomy
Summary Acknowledgments:
• All that seizes is not epilepsy • Anne Osborn, MD • Multiple causes of epilepsy • Anton Hasso, MD • Ric Harnsberger, MD • Advanced imaging required to evaluate • Lubdha Shah, MD MTS • Richard Wiggins, MD • Multimodal imaging guides management • Paul House, MD of refractory epilepsy • Gary Hedlund, DO • Kathryn Morton, MD • Richard Boyer, MD
CRANIOVERTEBRAL DISCLOSURE JUNCTION Relevant Financial Relationship None JEFFREY S. ROSS, M.D. Mayo Clinic Arizona [email protected] Off Label Usage None
JS Ross, Craniovertebral Junction
Amirsys/Elsevier ©2013 MFMER | slide-1
CRANIOVERTEBRAL JUNCTION 1
• MEASUREMENTS • NOMENCLATURE • CONGENITAL / SYNDROMIC • TRAUMA • TUMOR
2 3 MEASUREMENTS - LANDMARKS CHAMBERLAIN
< 2mm above line
McGREGOR McRAE
< 4 mm above line Dens below line
WACKENHEIM CLIVAL LINE WELCHER BASAL ANGLE CLIVAL CANAL ANGLE
ODONTOID ANTERIOR
129 ° ± 6 (MR) NORMAL > 150 DEGREES NOMENCLATURE BASILAR INVAGINATION • DEVELOPMENTAL ANOMALY - ODONTOID • BASILAR INVAGINATION HAS ABNORMAL RELATIONSHIP TO FORAMEN MAGNUM (PROLAPSE) • BASILAR IMPRESSION • UNDERLYING BONE NORMAL • CRANIAL SETTLING • MULTIPLE CONGENITAL / SYNDROMIC CAUSES • PLATYBASIA • CATEGORIZED BY • BASILAR KYPHOSIS • +/- CHIARI • +/- STABILITY
BASILAR INVAGINATION BASILAR INVAGINATION
BASILAR INVAGINATION BASILAR IMPRESSION
• CONGENITAL • ETIOLOGIES • ACQUIRED ABNORMAL RELATIONSHIP • BASIOCCIPITAL HYPOPLASIA OF ODONTOID TO THE FORAMEN • CONDYLAR HYPOPLASIA MAGNUM • ATLAS HYPOPLASIA • ALSO CALLED SECONDARY BASILAR • C1 RING ANOMALIES INVAGINATION • ACHONDROPLASIA • C1 ASSIMILATION • ABNORMAL BONE • ASSOCIATED • CHIARI 1 • SYRINGOMYELIA • HYDROCEPHALUS BASILAR IMPRESSION BASILAR IMPRESSION • ACQUIRED • ETIOLOGIES • PAGET • OSTEOMALACIA • OSTEOGENESIS IMPERFECTA • TUMOR • INFECTION • HYPERPARATHYROIDISM • RICKETS
REMEMBER
• BASILAR INVAGINATION = CONGENITAL • BASILAR IMPRESSION = ACQUIRED
O.I.
RHEUMATOID ARTHRITIS
• BASILAR IMPRESSION (ACQUIRED) • CRANIAL SETTLING • UPWARD MIGRATION OF ODONTOID INTO FORAMEN MAGNUM • COMPRESSION OF BRAINSTEM EXACERBATED BY PANNUS FORMATION • POOR PROGNOSIS • MYELOPATHY • LOWER CRANIAL NEUROPATHY (IX-XII) PLATYBASIA
• ABNORMAL FLATTENING OF SKULL BASE • OCCURS IN VARIOUS CONGENITAL DISORDERS BUT USUALLY SECONDARY FINDING • NO CLINICAL IMPACT • OFTEN ASSOCIATED WITH BASILAR INVAGINATION • MEASUREMENT • NASION TO DORSUM TO BASION • ADULTS 129 +/- 6 • CHILDREN 127 +/- 5
BASILAR KYPHOSIS BASILAR KYPHOSIS
• FLEXION OF SKULL BASE (NOT CVJ) • NORMAL > 105 ° • ANTERIOR CRANIAL FOSSA – DORSUM – CLIVUS • ASSOCIATED WITH MULTIPLE SYNDROMES • FIBROBLAST GROWTH FACTOR RECEPTOR RELATED CRANIOSYNOSTOSIS SYNDROMES (FGFR-2)
“ACROCEPHALOSYNDACTYLY” • CROUZON • APERT • PFEIFFER • BEARE-STEVENSON • MUENKE (FGFR-3)
FGFR-2 • APERT (1906) • AUTOSOMAL DOMINANT • CRANIOSYNOSTOSIS • MIDFACE HYPOPLASIA • SYNDACTYLY • CERVICAL VERTEBRAL FUSIONS COMMON (C5-6) FGFR-2 CRANIOVERTEBRAL JUNCTION • CROUZON (1912) • AUTOSOMAL DOMINANT • CHIARI I, II, III • CRANIAL SYNOSTOSIS • CONGENITAL ANOMALIES • HYPERTELORISM • BASIOCCIPITAL HYPOPLASIA • EXOPHTHALMOS • CONDYLAR HYPOPLASIA • PARROT-BEAK NOSE • C1 ASSIMILATION TO OCCIPUT • HYPOPLASTIC MAXILLA • C1 RING HYPOPLASIA / DYPLASIA • MANDIBULAR PROGNATHISM • SYNDACTYLY NOT TYPICAL • OS ODONTOIDEUM • FUSION OF C2-3 • KLIPPEL FEIL • SYNDROMES
CHIARI 1 CHIARI 1
• CAUDAL PROTRUSION OF PEG-SHAPED TONSILS BELOW FORAMEN MAGNUM • >5MM • POSTERIOR FOSSA VOLUME SMALL • KLAUS HEIGHT INDEX • SYRINGOMYELIA 30-50% • OSSEOUS ABNORMALITIES • BASIOCCIPITAL HYPOPLASIA • BASILAR INVAGINATION
COMPLEX CHIARI CHIARI 1.5
• TONSILLAR HERNIATION + ONE OR MORE • CERVICOMEDULLARY KINK • BRAINSTEM HERNIATION • RETROFLEXED ODONTOID • ABNORMAL CLIVAL-CANAL ANGLE • C1 ASSIMILATION • BASILAR INVAGINATION • SYRINX • HIGHER INCIDENCE OF CVJ FUSION AND ODONTOID RESECTION • J NEUROSURG PED. 2012: 10; 134-141 CONGENITAL AND VARIANTS
• SKULL BASE • CONDYLES • C0-C1 • C1 • C2
BASIOCCIPITAL HYPOPLASIA BASIOCCIPITAL HYPOPLASIA
• SHORT CLIVUS • BASILAR INVAGINATION • VIOLATION OF CHAMBERLAIN LINE • CLIVUS CANAL ANGLE DECREASED • LOOK FOR BOWSTRING DEFORMITY OF BRAINSTEM • ASSOCIATED • CHIARI I • CONDYLAR HYPOPLASIA • RETROFLEXED ODONTOID
BASIOCCIPITAL HYPOPLASIA
• CHARGE SYNDROME • COLOBOMA • HEART DEFECTS • CHOANAL ATRESIA • RETARDED GROWTH • GENITAL HYPOPLASIA • EAR ANOMALIES • CHD7 MUTATIONS • SEVERE BASIOCCIPITAL HYPOPLASIA • AJNR 2009; 30(3): 629 C1 ASSIMILATION C1 ASSIMILATION
• CONGENTIAL FUSION OF ATLAS TO OCCIPUT • “OCCIPITO-CERVICAL FUSION” • “OCCIPITALIZATION OF THE ATLAS” • 0.1-2.7%, M=F • PARTIAL OR COMPLETE • ANTERIOR, LATERAL, POSTERIOR • MOST COMMON IS FUSION OF ANTERIOR ARCH TO BASION • MAY SHOW AGENESIS OF POSTERIOR ARCH
C1 ASSIMILATION C1 ANTERIOR ARCH ANOMALY
• TYPICALLY OCCUR WITH POSTERIOR ARCH DEFECT (0.1%) • COMBINED ANTERIOR AND POSTERIOR DEFECTS CALLED “SPLIT ATLAS” • LARGE CORTICATED ANTERIOR ARCH IN MIDLINE IMAGE • FATTY MARROW SHOW INC T1 SIGNAL • LOW SIGNAL IF SAGITTAL VIEW THROUGH FIBROUS UNION
SPLIT ATLAS SPLIT ATLAS C1 HYPOPLASIA C1 HYPOPLASIA C2 ANOMALIES
• OSSICULUM TERMINALE • AGENESIS/HYPOPLASIA OF ODONTOID • OS ODONTOIDEUM
OS ODONTOIDEUM OS ODONTOIDEUM
• 1886 GIACOMINI • SYNDROMIC ASSOCIATIONS • INDEPENDENT OSSICLE WITH SMOOTH • DOWN CORTICAL MARGIN SEPARATED FROM • KLIPPEL-FEIL FORESHORTENED ODONTOID PEG • MORQUIO’S • ROUND, CONE-SHAPED, BLUNT TOOTH LIKE • MULTIPLE EPIPHYSEAL DYSPLASIA • ORTHOTOPIC – MOVES WITH C1 ARCH • ACHONDROPLASIA (REDUCIBLE) • LARSON • DYSTOPIC – MIGRATED TOWARD CLIVUS/ • WIDE RANGE OF SIGNS/SYMPTOMS FUSED TO BASION • STABLE OVER MANY YEARS • INCOMPETENT CRUCIATE LIGAMENT • ATLANTOAXIAL INSTABILITY • CATASTROPHIC CORD INJURY WITH MINOR TRAUMA
OS ODONTOIDEUM CHRONIC UN-UNITED C2 FRACTURE OS ODONTOIDEUM SYNDROMES KLIPPEL-FEIL
• KLIPPEL-FEIL • FIRST DESCRIBED 1912 • FAILURE OF NORMAL SEGMENTATION • DOWN OF CERVICAL SOMITES • ACHONDROPLASIA • TRIAD (<50%) • MORQUIO • LOW POSTERIOR HAIRLINE • SHORT NECK • LIMITATION OF NECK MOTION • NONSEGMENTATION OF 2 OR MORE CERVICAL VERTEBRAE
KLIPPEL-FEIL KLIPPEL-FEIL
TYPE INHERITANCE GENE CHROMOSOME
KF SYNDROME 1 AUTOSOMAL GDF6* 8q22 DOMINANT KF SYNDROME 2 AUTOSOMAL MEOX1 17q21 RECESSIVE KF SYNDROME 3 AUTOSOMAL GDF3 12p13 DOMINANT
*GDF6 = GROWTH/DIFFERENTIATION FACTOR-6 IS MEMBER OF TRANSFORMING GROWTH FACTOR-BETA SUPERFAMILY MEOX1 = MESENCHYME HOMEOBOX 1
OMIM.ORG
MORQUIO DISEASE MORQUIO MPS IV MUCOPOLYSACCHARIDOSIS IV
• LYSOSOMAL STORAGE DISEASE • AUTOSOMAL RECESSIVE • ACCUMULATION OF KERATAN SULFATE & CHONDROITIN-6 SULFATE • PERVASIVE SKELETAL DYSPLASIA • CERVICAL STENOSIS • CERVICAL INSTABILITY MORQUIO MORQUIO
• QUADRIPARESIS IS MOST COMMON CAUSE OF PREMATURE DEATH IN AFFECTED INDIVIDUALS • PROPHYLACTIC DECOMPRESSION AND FUSION OF OCCIPITAL CERVICAL JUNCTION AT AN EARLY AGE FOR PATIENTS WITH MPS IV • ASYMPTOMATIC PTS IF SCS IS <14 MM OR CERVICAL INSTABILITY IS >8 MM • 5-8 MM OF CERVICAL INSTABILITY WITH EVIDENCE OF SPINAL CORD IMPINGEMENT OR DAMAGE ON FLEX-EXT AND MR • SPINE 31:E169-174, 2006
COMPLEX CHIARI BASILAR KYPHOSIS - APERTS
BASIOCCIPITAL HYPOPLASIA
ORION NEBULA, RUNNING MAN NEBULA DISCLOSURES
MRI in SPINE TRAUMA • NOTHING TO DISCLOSE
JEFFREY S. ROSS, M.D. MAYO CLINIC PHOENIX, AZ [email protected]
©2013 MFMER | slide-1
OUTLINE CERVICAL SPINE TRAUMA
• NUMBERS • 12,000 NEW QUAD-PARAPLEGICS / YR • UTILITY OF MR IMAGING IN TRAUMA • 250,000 EXISTING VICTIMS • WHY MRI? • 60% INVOLVE CERVICAL SPINE • SOURCES OF ANXIETY • 800,000 C SPINES CLEARED EACH YR • MOST 15-35 YEARS OF AGE • M:F = 4:1
UTILITY OF IMAGING CERVICAL SPINE LITIGATION
• Litigation of Missed Cervical Spine Injuries in Patients Presenting With Blunt Trauma • 20 cases cervical injury missed / delayed • Awards averaged $2.9 million MAGNETIC RESONANCE • Neurosurgery, 2007 • Cervical Spine Malpractice MULTI-DETECTOR CT • 68 patients • 22 plaintiffs’ verdicts ave payout $4.0 million DYNAMIC EVALUATION • 26 settlements ave payout $2.4 million PLAIN FILMS • 7 of 78 suits against Radiologist • J Spinal Disord Tech, 2010 MULTI-DETECTOR CT VS. MRI MRI TO CLEAR C-SPINE IN OBTUNDED/ COMA PTS? • CONSCIOUS/ORIENTED PATIENTS WITHOUT NEURO DEFICIT, • NORMAL CT EXCLUDES SIGNIFICANT (UNSTABLE) • META-ANALYSIS, 464 PTS INJURIES • RADIOLOGY 2005; 237:106 • COULD NOT BE CLINICALLY EVAL • ARCH SURG 2005; 140:762 • NORMAL CT DOES NOT EXCLUDE CORD OR DISC INJURY • MRI WITHIN 72 HOURS IF PATIENT CANNOT BE FULLY EXAMINED • SENS 95%, SPEC 98.5% • MRI IS GOLD STANDARD IN THE OBTUNDED / UNEVALUABLE PATIENT • 21% WITH ABNORMAL MR NOT SEEN ON PLAIN • J TRAUMA 2008; 64:179-189 FILM OR CT • J TRAUMA 2008; 64(4): 898-903 • MR IS GOLD STANDARD IN UNEVALUABLE • 6% MR FINDINGS ALTERED MANAGEMENT PATIENTS • J TRAUMA 2010, JAN 68: 109-13 • MUCHOW. J TRAUMA 2008; 64:179-189
ROLE OF MR WHY MRI IN ACUTE TRAUMA?
• PERSISTENT NECK PAIN WITH NORMAL CT (NO NEURO DEFICIT) • CORD INJURY • FLEX-EXT • MRI • LIGAMENTS • OBTUNDED PATIENT • DISC HERNIATIONS • FLEX-EXT NOT RECOMMENDED • HEMATOMA • MRI EAST - J TRAUMA 67:651, 2009 • UNSUSPECTED INJURY AANS 2013 – NEUROSURGERY 2013 THE SPINE JOURNAL 15:398–404, 2015
WHY MRI IN ACUTE TRAUMA? CORD CONTUSION PATTERN
• CORD INJURY • NON-HEMORRHAGIC • LIGAMENTS • GOOD PROGNOSIS • DISC HERNIATIONS • HEMORRHAGIC • HEMATOMA • POOR PROGNOSIS • UNSUSPECTED INJURY • KULKARNI, RADIOLOGY 1987 MR FINDINGS VS. OUTCOME
• 100 PATIENTS WITH TRAUMATIC CERVICAL SCI • M:F 4:1 • EXTENT OF CORD HEMORRHAGE AND SWELLING CORRELATED WITH NEUROLOGIC RECOVERY • Radiology, 243 (3) 2007 ACUTE TRAUMATIC CENTRAL CORD SYNDROME (ATCCS) • 54-year-old female presents to ED following fall from standing height • Loss of consciousness for unknown period of time • When awoke, immediately felt weakness in upper extremities > lower extremities
• Initial physical exam • Severe distal weakness of bilateral upper extremities with no function of intrinsic hand muscles • Able to move arms but not hold them up antigravity • Distal right lower extremity weakness • Able to flex at the hip and knee PREOP POSTOP CENTRAL CORD SYNDROME
USED WITH PERMISSION
ACUTE TRAUMATIC CENTRAL CORD SYNDROME (ATCCS) WHY MRI IN ACUTE TRAUMA? • MOST COMMON INCOMPLETE INJURY TO C-SPINE • MOTOR WEAKNESS UE > LE • CORD INJURY • HYPEREXT WITH PRE-EXISTING SPONDYLOTIC CHANGES • LIGAMENTS • NO HEMORRHAGE • DISC HERNIATIONS • 50-60% NO FX • HEMATOMA • SPONTANEOUS RECOVERY IRRESPECTIVE OF TREATMENT • ACUTE SURGERY NOT USUALLY REQUIRED • UNSUSPECTED INJURY • CLASS III EVIDENCE – SURGERY SAFE/EFFECTIVE FOR FOCAL COMPRESSION
• NEUROSURGERY 72: 195-204, 2013
WHY MRI IN ACUTE TRAUMA?
• CORD INJURY • LIGAMENTS • DISC HERNIATIONS • HEMATOMA • UNSUSPECTED INJURY
Courtesy Dr. Lubdha Shah 45 year old trampoline injury
WHY MRI IN ACUTE TRAUMA?
• CORD INJURY • LIGAMENTS • DISC HERNIATIONS • HEMATOMA • UNSUSPECTED INJURY
WHY MRI IN ACUTE TRAUMA?
• CORD INJURY • LIGAMENTS • DISC HERNIATIONS • HEMATOMA • UNSUSPECTED INJURY ANTERIOR CERVICAL PSEUDOMENINGOCELE
• SYMPTOMATIC IN A DELAYED FASHION • DEVELOPED AFTER SEVERE UPPER CERVICAL OR CRANIOVERTEBRAL INJURIES • N=3 • ONE LESION EVACUATED ON AN EMERGENCY BASIS, AND THE OTHER TWO RESOLVED SPONTANEOUSLY • J Neurosurg Spine 5:254–258, 2006
SOURCES OF (MY) ANXIETY
• ATLANTO-OCCIPITAL DISLOCATION • ANKYLOSING SPONDYLITIS / DISH • MR FRACTURE DELINIATION
BDI SUMMED INTERCONDYLAR AOD <10.5mm is normal in <4.2 mm children by sagittal CT <8.5mm in adults • COMPLETE (DISASSOCIATION) • PARTIAL (SUBLUXATION) • LIGAMENTOUS DISRUPTION BETWEEN OCCIPUT AND C1 • ANTERIOR DISPLACEMENT OF CRANIUM MOST COMMON • HARRIS RULES OF TWELVE, POWER RATIO, LEE X LINE NOT SENS OR SPECIFIC IN CT ERA ©2013 MFMER | slide-49
SPINE FX IN ANKYLOSING SPONDYLITIS
• 122 SPINE FRACTURES IN 112 CONSECUTIVE PATIENTS WITH AS • MAJORITY WERE TRANSDISCAL EXTENSION INJURIES • MOST COMMONLY AFFECTING C6–C7. • 32% MORTALITY • 58% CORD INJURY • Spine 2010; 35:E458–E464 MRI POOR FRACTURE DELINIATION
• DO NOT DICTATE “NO FRACTURE” • ODONTOID FX WITHOUT STIR HYPERINTENSITY • LENSING ET AL., AJNR 2014 • EXTENSION MECHANISM WITHOUT T2 HYPERINTENSITY • BRINCKMAN ET AL., SPINE JOURNAL 2014
WWW.CELESTIALROSS.COM M51 WHIRLPOOL GALAXY Aneurysm treatment options
Imaging the Post-procedural n Observation n Small (<5-7mm), elderly Cerebrovascular Patient n Anterior, not Pcom/Acom n Neurosurgical n Clipping n Trapping Gary Nesbit, M.D. n Bypass n Neurointerventional n Coiling n Stent-supported Coiling n Flow diversion n Parent vessel occlusion n Endograft Professor, Dotter Department of Interventional n There are specific issues with imaging follow-up after each Radiology, Diagnostic Neurology, and Neurosurgery of these interventions, mainly related to the materials used. Oregon Health & Science University
Aneurysm observation imaging Aneurysm observation imaging
n Size n Aneurysm size, neck width n Image Resolution n Neck to dome ratio n DSA > CTA ≥ MRA n Parent vessel diameter n Measurement of change n Neck to parent vessel ratio n Detection of new aneurysms n Shape n Saccular, fusiform, daughters n Image artifacts n Lobulated vs multiple aneurysms n DSA > CTA > MRA n Site n Flow/pulsation n Posterior circulation carries higher risk n Invasiveness n Surgical/endovascular access, eloquence n MRA > CTA > DSA n Siblings n Other associated aneurysms n 3D TOF MRA n Branch vessel involvement n The best balance of detection of new aneurysms and n A change in the aneurysm, parent vessel, or new aneurysm follow-up of known aneurysms and carries little risk. The are the critical items to detect on follow-up. only risk is missing a critical change.
Aneurysm observation imaging Image artifacts
n Slow flow n Large and treated aneurysms n Mainly on 3D TOF MRA n Solution: Do MRI/MRA without and with Gadolinium n Pulsation artifacts n Motion and disordered flow phase artifacts on MRA may limit detection/size n Pulsation blurring on CTA may affect size to a minimal degree 3D TOF CTA Surf n Can lead to fusion of adjacent vessel/aneurysm and poor characterization of neck Image artifacts Slow flow artifact n Slow flow n Large and treated aneurysms n Mainly on 3D TOF MRA n Solution: Do MRI/MRA without and with Gadolinium n Pulsation artifacts n Motion and disordered flow phase artifacts on MRA may limit detection/size n Pulsation blurring on CTA may affect size to a minimal degree 3D TOF T1 SE T2 TSE n Can lead to fusion of adjacent vessel/aneurysm and poor characterization of neck
Post contrast CTA pulsation and fusion
CTA T1 SE + Gad
Aneurysm observation imaging Surgical clip artifact
n Stainless steel clips are not MR compatible n Titanium alloy clips n Yasargil FT, Sugita T2, PeterLazic n MRI conditional to 3T n Local susceptibility artifact limits MRA n Cobalt Chromium alloy clips n Sugita Elgiloy, Yasargil FE Phynox n MRI conditional to 3T n More MRI/A susceptibility artifact n More radio-dense, more CTA streak artifact 3D RA n Pure Titanium clips n Spetzler: no MR artifact and low radio-density on CT Surgical clip artifact Surgical clip artifact
T2 TSE 3D TOF
n Right ophthalmic artery clip, left Pipeline flow diverter
Surgical clip artifact Surgical clip artifact
CTA 5mm CTA 5mm CTA 1mm CTA 1mm
n PeterLazic Titanium Alloy Clip
Imaging post coil embolization Imaging post coil embolization n Platinum Tungsten coils n Stryker, Medtronic, Microvention, Penumbra, Balt n Variety of detachment mechanisms, some have effect n Coil is very radio dense but not ferromagnetic, minimal susceptibility n Dense streak artifacts, CTA useless n MRA signal void of the coils, can 3D TOF see residual through the interstices n 3D TOF +/- Gad (slow flow)
Imaging post coil embolization Imaging post coil embolization
3D TOF T2 TSE SWI 3D RA Surf 3D RA MIP
Imaging post coil embolization Imaging post coil embolization
3D TOF n FDA 3/12/2018 MR susceptibility artefact associated with the use of Barricade coils for treatment of intracranial aneurysms Gontu V and McConachie N Interv Neuroradiol. 2015 21(2):188-190.
Imaging post coil embolization Imaging post coil embolization Stent supported coil imaging Stent supported coil imaging n Neuroform, Atlas, Enterprise Lvis n Nickle Titanium Alloy with Platinum Tungsten markers n Nickle has weak susceptibillity artifact n Some blooming and decreased signal: pseudo- stenosis, pseudo-thrombosis n The stent has mild radio T2 TSE T2* GRE density, but associated coils make CTA useless
Stent supported coil imaging Flow diverter imaging n Pipeline, Surpass, Silk n Platinum-Tungsten and Cobalt-Chromium mesh n MR Conditional to 3T n Co/Cr: (remember clips) moderate susceptibility somewhat more the Ni/Ti n MRA: more blooming = more pseudo-stenosis, more pseudo-thrombosis n Dense Radio-opacity makes 3D TOF visualizing small lumen nearly impossible
Flow diverter imaging Flow diverter imaging
CTA Left ICA FD 3D TOF Right ICA FD 3D TOF Stent/flow diverter imaging Stent/flow diverter imaging
3D TOF TE 3.5 Gad
n A somewhat lower TE and 3D TOF adding gad does not help. It 3D TOF TE 6.9 may lower overall flow TE 6.9 signal in higher flow vessels
TE 3.5 Gad
Stent/flow diverter imaging Stent/flow diverter imaging
TE 1.8 Dyn Gad
3D TOF TE 3.5 Gad TE 2.0 n You need to create a specific 3D TOF Dyn Gad protocol to focus on the COW/ TE 6.9 Aneurysm for your dynamic Gad low TE MRA n Axial-oriented Dynamic Gad centered on the circle of Willis
Recommended imaging Complication evaluation n Surgical clips: CTA The Post treatment n Ischemic n Coils, Stents and flow diverter: MRI/MRA Protocol n Thrombo-embolic or perforator infarct Remember the FDA recommendations: Shortest echo time and high readout n May be acute or delayed (weeks to months) bandwidth n Anti-platelet (DAPT) non-responders, DAPT non- n MRI +/- Gad, 3DTOF MRA +/- Gad compliance, Poor wall apposition, ICA dissection (lowest TE, high readout bandwidth), Dynamic Gad MRA n Hemorrhagic n Modify Dynamic Gad “neck to COW” n Aneurysm rupture, distal parenchymal hemorrhage MRA to smaller field of view to include the aneurysm and stent, higher resolution, n Embolic stroke on DAPT, deployment wire issues lowest TE and high readout bandwidth (most already are) n Inflammatory n Clips and coils in the same or multiple n Unknown cause, “Thrombophelbitis”, Most are aneurysms: just do DSA asymptomatic Complication evaluation Complication evaluation
CTA
n In the peri-procedural timeframe a stroke protocol n In the peri-procedural timeframe a stroke protocol with CT, CTA, CTP is probably best with CT, CTA, CTP is probably best
Complication evaluation Complication evaluation
CTA 3D TOF DWI n Basilar apex aneurysms s/p Pipeline embolization, n If CT/CTA/CTP does not help, then MR/MRA/ Right hemiparesis 2 weeks after treatment Diffusion may help
Complication evaluation Complication evaluation
CT CTA n Vessel is patent, but is there thrombus or n Expanding hematoma 8 hours following procedure Pseudostenosis? Complication evaluation Complication evaluation
CT FLAIR
n Basilar trunk aneurysm partially treated n Small embolus on immediate post-implant angio with coil embolization
Complication evaluation Complication evaluation
T2 TSE T1 SE FLAIR
n 2 months s/p Pipeline treatment with n Progressive pontine edema and cystic complete thrombosis change despite steroid therapy
Complication evaluation Post procedure imaging
n Surgical clips: CTA, some streak artifacts n Complete occlusion: Follow up at 1-2 years, 5, and 10 years n Coils, Stents and flow diverter: Post treatment MRI/ MRA protocol: MRI/MRA +/- & C Dyn Gad n Clips and coils in the same or multiple aneurysms: just do DSA n Follow up: complete occlusion n DSA 6-12 months post treatment, then MRI/MRA +/- at 2 years, and MRA +/- at 5 and 10 years n Follow up: incomplete occlusion T1 SE + Gad n DSA 3-6 months post treatment, then MRI/MRA +/- at 1 and 2 years, and MRA +/- at 5 and 10 years
n Intense wall and thrombus enhancement