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ASPNR INTERESTING CASE SESSION ASNR 54Th Annual Meeting May 24,23, 2016

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ASPNR INTERESTING CASE SESSION ASNR 54Th Annual Meeting May 24,23, 2016 ASPNR INTERESTING CASE SESSION ASNR 54th Annual Meeting May 24,23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy, MD, Secretary Arastoo Vossough, MD, PhD, Treasurer and Laurence Eckel, MD, Treasurer IN WHAT YEAR WAS THE ASPNR FOUNDED? A.1992 B. 1993 C.1994 D.1995 E. 1996 IN WHAT YEAR WAS THE ASPNR FOUNDED? A.1992 B. 1993 C.1994 D.1995 E. 1996 ESS CASE 1 11 year old girl “progressively obtunded and vomiting” WHAT IS THE DIAGNOSIS? A. Subdural hygromas B. Subarachnoid hemorrhage C. Anemia D. Iatrogenic effect WHAT IS THE DIAGNOSIS? A. Subdural hygromas B. Subarachnoid hemorrhage C. Anemia D. Iatrogenic effect Additional history we found in the medical record: Patient with seizures and rapidly declining mental status after local anesthetic (mepivicaine) injected for dental procedure INTRAVASCULAR LIPID ADMINISTRATION • Lipid infusions increasingly being used to treat local anesthetic systemic toxicity (LAST) • Possible antidotal effect of intravenous lipid emulsion on action of lipophilic drugs, including local anesthetics, first discovered in 1962 • First case reports of success in human in 2006 • Controversial, however, as efficacy and safety not clearly proven Lipid Rescue - Efficacy and Safety Still Unproven. Höjer J, Jacobsen D, Neuvonen PJ, Rosenberg PH. Basic Clin Pharmacol Toxicol. 2016 May 2. doi: 10.1111/bcpt.12607. [Epub] PMID: 27136445 SP CASE 1 • 7 month old previously healthy full term male • Found unresponsive in daycare • Caregiver stated that the baby choked while taking the bottle Non C+ CT + Retinal hemorrhages Negative skeletal survey 3D Volume rendering T2 FSE T2 FSE FS TOF MRA TOF MRV WHAT IS THE DIAGNOSIS? A. Abusive head trauma B. Vitamin K deficiency C. Osler-Weber-Rendu syndrome D. Menkes disease WHAT IS THE DIAGNOSIS? A. Abusive head trauma B. Vitamin K deficiency C. Osler-Weber-Rendu syndrome D. Menkes disease OSLER-WEBER-RENDU SYNDROME • Osler–Weber–Rendu syndrome (Hereditary hemorrhagic telangiectasia (HHT)) • Autosomal dominant genetic disorder that leads to abnormal blood vessel formation in the skin, mucous membranes, and organs OSLER-WEBER-RENDU SYNDROME Digital Subtraction Angiography ABUSIVE HEAD TRAUMA SUBDURALS • Mixed intensity SDH • Hematohygromas T2 FSE VITAMIN K DEFICIENCY • Early (within 24 hrs), Classic (1-7 days of life), and Late (2-24 weeks) • Neonates and infants at risk because Vit K transfer through the placenta is limited T2 T1 MENKES DISEASE • X-linked recessive disorder of transmembrane copper (Cu) transport • Rapid CNS degeneration • Elastin collagen formation • Fragile, tortuous vessels • Predisposition to ischemia • Rapid brain atrophy leads to SDH WHO WAS THE FIRST ASPNR PRESIDENT? A.Thomas P Naidich B. Robert A Zimmerman C.James A Brunberg D.A James Barkovich E. Patrick D Barnes WHO WAS THE FIRST ASPNR PRESIDENT? A.Thomas P Naidich B. Robert A Zimmerman C.James A Brunberg D.A James Barkovich E. Patrick D Barnes AP CASE 1 SECOND SIBLING WHAT IS THE MOST LIKELY DIAGNOSIS? A. TORCH Infection B. Zika Infection C. Aicardi-Goutieres Syndrome D. Chorioamnionitis WHAT IS THE MOST LIKELY DIAGNOSIS? A. TORCH Infection B. Zika Infection C. Aicardi-Goutieres Syndrome D. Chorioamnionitis AUTOSOMAL RECESSIVE PSEUDO-TORCH SYNDROME • Phenotypic overlap with AGS (Aicardi-Goutieres syndrome) • Failure to determine causative intrauterine infectious agent • CSF leukocytosis with elevated interferon-alpha, neuropterins • Hepatic transaminases and thrombocytopenia AV CASE 1 Child with right leg weakness WHAT IS THE DIAGNOSIS? A. RF213 mutation (inherited moyamoya) B. COL3A1 mutation C. ACTA2 mutation D. PHACES (unknown mutation) WHAT IS THE DIAGNOSIS? A. RF213 mutation (inherited moyamoya) B. COL3A1 mutation C. ACTA2 mutation D. PHACES (unknown mutation) ACTA2 MUTATION Multisystem smooth muscle dysfunction Straight and narrow intracranial vessels – occlusions and ischemia No moyamoya collaterals Small corkscrew distal vessels with tiny aneurysms HX of gastric dysmotility and congenital mydriasis. Several weeks of intermittent R hand numbness, progressively worsening slurred speech and expressive aphasia Courtesy of Michelle Silvera PHACES Posterior fossa malformations Segmental hemangiomas Arterial anomalies (narrowing, occlusions, primitive connections, dilatation, collaterals, anomalous course, later aneurysms, etc.) Cardiac defects Eye abnormalities Sternal or ventral defects WHO WAS THE FIRST FEMALE ASPNR PRESIDENT? A.Tina Young Poussaint B. Jill V Hunter C.Nancy K Rollins D.Patricia Davis E. Mary K Edwards-Brown WHO WAS THE FIRST FEMALE ASPNR PRESIDENT? A.Tina Young Poussaint B. Jill V Hunter C.Nancy K Rollins D.Patricia Davis E. Mary K Edwards-Brown ESS CASE 2 12 year old obese female, headache for one week, nausea/vomiting for one day WHAT IS THE DIAGNOSIS? A. Pseudotumor cerebri B. Normal MRI C. Acute infarction D. Venous Sinus Thrombosis WHAT IS THE DIAGNOSIS? A. Pseudotumor cerebri B. Normal MRI C. Acute infarction D. Venous Sinus Thrombosis PSEUDOTUMOR CEREBRI • Lumbar puncture: • Opening pressure: >60cm H20 • Headaches improved • Discharged on: Methylprednisone taper, Acetazolamide, Gabapentin, Metoclopramide prn EXCEPT… • One week after discharge, patient abruptly worsened • 10/10 occipital headache, blurry vision, confusion, weakness, emesis, enuresis, encopresis, fall and seizure • Sluggish 3mm pupils, bilateral papilledema, right CN VI palsy, hyporeflexia, unsteady gait, psychomotor slowing, and delayed, slurred speech • Lumbar puncture: • Opening pressure: >36cm H20 • Repeat 5 days later, opening pressure: 48cm H20 • All viral, toxicology, and rheumatological laboratory tests normal, aside from mild anemia Raised papules on extremities Progressive deterioration with bulbar signs, urinary retention, acutely decreased responsiveness 40lb weight loss since onset of illness (two months) Over one week, mental status substantially declined Minimally responsive to sternal rub, only able to communicate by blinking, progressed to complete unresponsiveness WHAT IS THE DIAGNOSIS? A. Inborn error of metabolism B. Vasculitis C. Pseudotumor cerebri D. Toxic exposure WHAT IS THE DIAGNOSIS? A. Inborn error of metabolism B. Vasculitis C. Pseudotumor cerebri D. Toxic exposure p-Dichlorobenzene: 2.8mcg/mL (normal = 0, reporting limit 0.020) 2,5-Dichlorophenol (DCB metabolite): 200mg/L (normal = 0, reporting limit 5.0) LEUKOENCEPHALOPATHY SECONDARY TO PARADICHLOROBENZENE TOXICITY • Paradichlorobenzene in many forms of mothballs • Patient kept many boxes in her poorly ventilated room for >3 years, “reminded her of her grandmother” • Excessive inhalation or ingestion can cause a progressive leukoencephalopathy with ichthyotic rash and anemia, reports of intracranial hypertension • Toxin stored & released from fat, acute worsening during catabolic stress (patient decompensated during stress & when NPO) Twin Girls with Neurocutaneous Symptoms Caused by Mothball Intoxication. http://www.nejm.org/doi/full/10.1056/NEJMc060329 Hernandez SH, Wiener SW, Smith SW. Case files of the New York City poison center: paradichlorobenzene-induced leukoencephalopathy. J Med Toxicol. 2010 Jun;6(2):217-29 SP CASE 2 • 3 yr old female with intermittent vomiting for 2.5 weeks • Headache • No fever • Diagnosed with abusive head trauma at 5mos of age T2 at 5mos 3 years later Cor Non C+ CT Ax Non C+ CT Ax T2 FSE Cor T1 C+ Ax T1 C+ DWI ADC WHAT IS THE DIAGNOSIS? A. Post-traumatic encephalomalacia B. Intracranial epidermoid cysts C. Malignant myxoid glial tumors D. Abscesses WHAT IS THE DIAGNOSIS? A. Post-traumatic encephalomalacia B. Intracranial epidermoid cysts C. Malignant myxoid glial tumors D. Abscesses MALIGNANT MYXOID GLIAL TUMORS • Very rare • Meningeal glial rests • Low grade and high grade histology BRAIN ABSCESS T2 T1 C+ DWI ADC INTRACRANIAL EPIDERMOID CYST ADC T1 C+ DWI WHAT IS THE NAME OF THE ASPNR BEST PAPER PRESENTATION AWARD? A. The Really Great Paper Award B. The Golden Brain Award C. The Derek Harwood-Nash Award D. The ASPNR Gold Medal Award WHAT IS THE NAME OF THE ASPNR BEST PAPER PRESENTATION AWARD? A. The Really Great Paper Award B. The Golden Brain Award C. The Derek Harwood-Nash Award D. The ASPNR Gold Medal Award Derek Harwood-Nash was one of the fathers of pediatric neuroradiology and a former President of the ASNR. In addition to his many tremendous contributions to our field, he was instrumental in establishing the ABR CAQ in Neuroradiology. The ASPNR is proud to honor his memory with this award. AP CASE 2 Ophthalmoplegia (non-painful) INTRAOPERATIVE ULTRASOUND WHAT IS THE MOST LIKELY DIAGNOSIS? A. Meningioma B. Hemangiopericytoma C. Hemangioendothelioma D. Schwannoma WHAT IS THE MOST LIKELY DIAGNOSIS? A. Meningioma B. Hemangiopericytoma C. Hemangioendothelioma D. Schwannoma HEMANGIOENDOTHELIOMA (VASCULAR TUMORS) • Differentiation from hemangioma based on infiltrative growth pattern and predominant Kaposi sarcoma-like content (fascicles of spindle cells) • Tumor can also have fibrogenic thrombi in the capillaries, hemorrhage, areas of lymphangiomatosis AV CASE 2 5 year old with progressive mental decline, swallowing problems, and weakness MRS, TE=135ms WHAT IS THE DIAGNOSIS? A. Leigh syndrome B. LBSL C. Listeria with rhombencephalitis D. Late-onset Alexander disease WHAT IS THE DIAGNOSIS? A. Leigh syndrome B. LBSL C. Listeria with rhombencephalitis D. Late-onset Alexander disease LEUKOENCEPHALOPATHY WITH BRAINSTEM AND SPINAL CORD INVOLVEMENT AND HIGH LACTATE (LBSL) • Childhood or adolescent onset and slow disease course. Patients develop insidious
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