DOCSLIB.ORG

Myoclonic Atonic Epilepsy Another Generalized Epilepsy Syndrome That Is “Not So” Generalized

Total Page：16

File Type：pdf, Size：1020Kb

Download full-text PDF Read full-text
Myoclonic Atonic Epilepsy Another Generalized Epilepsy Syndrome That Is “Not So” Generalized EDITORIAL Myoclonic atonic epilepsy Another generalized epilepsy syndrome that is “not so” generalized John M. Zempel, MD, Myoclonic atonic/astatic epilepsy (MAE), first described have shown predominant thalamic activation PhD well by Doose1 (pronounced dough sah: http://www. and default mode network deactivation.6–8 Even Tadaaki Mano, MD, PhD youtube.com/watch?v5hNNiWXV2wF0), is a general- Lennox-Gastaut syndrome, a devastating epileptic ized electroclinical syndrome with early onset charac- encephalopathy with EEG findings of runs of slow terized by myoclonic, atonic/astatic, generalized spike and wave and paroxysmal higher frequency Correspondence to tonic-clonic, and absence seizures (but not tonic activity, has fMRI correlates that are more focal than Dr. Zempel: [email protected] seizures) in association with generalized spike-wave expected in a syndrome with widespread EEG (GSW) discharges. Thought to have a genetic com- abnormalities.9,10 Neurology® 2014;82:1486–1487 ponent that has proven to be complicated,2 MAE EEG-fMRI is maturing as a research and clinical sometimes occurs in children who have otherwise technique. Recording scalp EEG in an electrically been developing normally and has variable outcome. hostile environment is not an easy task. Substantial MAE is typically treated with antiseizure medications technical artifacts, such as changing imaging gradients that are used for generalized epilepsy syndromes, with and ballistocardiogram (ECG-linked artifact observed perhaps a best response to valproate, felbamate, or the in the scalp electrodes), contaminate the EEG signal. ketogenic diet.3,4 However, the relatively distinctive EEG discharges in In this issue of Neurology®, Moeller et al.5 report patients with epilepsy have partially circumvented on the fMRI correlates of GSW discharges as mea- this problem. EEG-fMRI even has applicability to sured with simultaneous EEG during sleep in chil- young pediatric populations6–8 and was performed dren with a clinical diagnosis of MAE. They were by Moeller et al.5 with chloral hydrate–induced able to obtain blood oxygen level–dependent sleep in mostly young children (ages 3–13 years, (BOLD) fMRI signal changes in most individual but mostly 3–5). participants. This study, like other EEG-fMRI stud- Clinically, EEG-fMRI is advancing in both tech- ies of the generalized epilepsies, shows that fMRI nology and utilization across many types of epilepsy. activations involve selective brain networks that are EEG-fMRI localization is used increasingly in focal more localized than the widespread EEG discharges epilepsies, where more precise localization may lead associated with generalized epilepsies. Group fMRI to successful surgical treatment.11 EEG-fMRI may analysis showed BOLD signal increases in the thala- eventually have clinical utility in the generalized epi- mus, putamen, and premotor cortex, with BOLD lepsies, for both treatment and diagnosis. The signal decreases in nodes of the default mode response to treatment with valproate, for example, network (posterior cingulate/precuneus and lateral is related to the generator of the GSW discharge.12 parietal cortex/inferior parietal lobule). In addition EEG-fMRI may be particularly helpful diagnostically to the common brain networks highlighted in other by providing a biomarker for distinguishing subtypes generalized epilepsy syndromes with thalamic of these often overlapping generalized epilepsy syn- activation and posterior cingulate/precuneus deacti- dromes. The BOLD signal fingerprint may provide vation, children with MAE have prominent activa- both syndrome- and patient-specific information as tion of nodes of the motor network, consistent the underlying causes of the many related generalized with the motor features of the common myoclonic syndromes are better understood.13 atonic/astatic seizures seen in this distinctive electro- clinical syndrome. AUTHOR CONTRIBUTIONS More generally, the results of this study are in John M. Zempel: drafting/revising the manuscript, study concept or design. Tadaaki Mano: study concept or design, study supervision. line with a broadly developing theme in the study of childhood generalized epilepsy syndromes. STUDY FUNDING EEG-fMRI studies of childhood absence epilepsy No targeted funding reported. See page 1508 From the Department of Neurology and Pediatrics (J.M.Z.), Washington University School of Medicine, St. Louis, MO; and Gifu University of Medical Science (T.M.), Seki, Gifu, Japan. Go to Neurology.org for full disclosures. Funding information and disclosures deemed relevant by the authors, if any, are provided at the end of the editorial. 1486 © 2014 American Academy of Neurology DISCLOSURE 8. Carney PW, Masterton RA, Harvey AS, Scheffer IE, The authors report no disclosures relevant to the manuscript. Go to Berkovic SF, Jackson GD. The core network in absence Neurology.org for full disclosures. epilepsy: differences in cortical and thalamic BOLD response. Neurology 2010;75:904–911. REFERENCES 9. Pillay N, Archer JS, Badawy RA, Flanagan DF, 1. Doose H. Myoclonic-astatic epilepsy. Epilepsy Res Suppl Berkovic SF, Jackson G. Networks underlying paroxysmal 1992;6:163–168. fast activity and slow spike and wave in Lennox-Gastaut 2. Tang S, Pal DK. Dissecting the genetic basis of myoclonic- syndrome. Neurology 2013;81:665–673. astatic epilepsy. Epilepsia 2012;53:1303–1313. 10. Siniatchkin M, Coropceanu D, Moeller F, Boor R, 3. Zupanc ML, Roell Werner R, Schwabe MS, et al. Efficacy Stephani U. EEG-fMRI reveals activation of brainstem of felbamate in the treatment of intractable pediatric epi- and thalamus in patients with Lennox-Gastaut syndrome. lepsy. Pediatr Neurol 2010;42:396–403. – 4. Kelley SA, Kossoff EH. Doose syndrome (myoclonic- Epilepsia 2011;52:766 774. astatic epilepsy): 40 years of progress. Dev Med Child 11. An D, Fahoum F, Hall J, Olivier A, Gotman J, Dubeau F. Neurol 2010;52:988–993. Electroencephalography/functional magnetic resonance 5. Moeller F, Groening K, Moehring J, et al. EEG-fMRI in imaging responses help predict surgical outcome in focal – myoclonic astatic epilepsy (Doose syndrome). Neurology epilepsy. Epilepsia 2013;54:2184 2194. 2014;82:1508–1513. 12. Szaflarski JP, Kay B, Gotman J, Privitera MD, 6. Moeller F, Siebner HR, Wolff S, et al. Simultaneous EEG- Holland SK. The relationship between the localization fMRI in drug-naive children with newly diagnosed of the generalized spike and wave discharge generators absence epilepsy. Epilepsia 2008;49:1510–1519. and the response to valproate. Epilepsia 2013;54: 7. Bai X, Vestal M, Berman R, et al. Dynamic time course of 471–480. typical childhood absence seizures: EEG, behavior, and 13. Moeller F, Stephani U, Siniatchkin M. Simultaneous EEG functional magnetic resonance imaging. J Neurosci 2010; and fMRI recordings (EEG-fMRI) in children with epi- 30:5884–5893. lepsy. Epilepsia 2013;54:971–982. Neurology 82 April 29, 2014 1487.
Load more

Recommended publications
  • Status Epilepticus Clinical Pathway
    JOHNS HOPKINS ALL CHILDREN’S HOSPITAL Status Epilepticus Clinical Pathway 1 Johns Hopkins All Children's Hospital Status Epilepticus Clinical Pathway Table of Contents 1. Rationale 2. Background 3. Diagnosis 4. Labs 5. Radiologic Studies 6. General Management 7. Status Epilepticus Pathway 8. Pharmacologic Management 9. Therapeutic Drug Monitoring 10. Inpatient Status Admission Criteria a. Admission Pathway 11. Outcome Measures 12. References Last updated: July 7, 2019 Owners: Danielle Hirsch, MD, Emergency Medicine; Jennifer Avallone, DO, Neurology This pathway is intended as a guide for physicians, physician assistants, nurse practitioners and other healthcare providers. It should be adapted to the care of specific patient based on the patient’s individualized circumstances and the practitioner’s professional judgment. 2 Johns Hopkins All Children's Hospital Status Epilepticus Clinical Pathway Rationale This clinical pathway was developed by a consensus group of JHACH neurologists/epileptologists, emergency physicians, advanced practice providers, hospitalists, intensivists, nurses, and pharmacists to standardize the management of children treated for status epilepticus. The following clinical issues are addressed: ● When to evaluate for status epilepticus ● When to consider admission for further evaluation and treatment of status epilepticus ● When to consult Neurology, Hospitalists, or Critical Care Team for further management of status epilepticus ● When to obtain further neuroimaging for status epilepticus ● What ongoing therapy patients should receive for status epilepticus Background: Status epilepticus (SE) is the most common neurological emergency in children1 and has the potential to cause substantial morbidity and mortality. Incidence among children ranges from 17 to 23 per 100,000 annually.2 Prevalence is highest in pediatric patients from zero to four years of age.3 Ng3 acknowledges the most current definition of SE as a continuous seizure lasting more than five minutes or two or more distinct seizures without regaining awareness in between.
    [Show full text]
  • Myoclonic Status Epilepticus in Juvenile Myoclonic Epilepsy
    Original article Epileptic Disord 2009; 11 (4): 309-14 Myoclonic status epilepticus in juvenile myoclonic epilepsy Julia Larch, Iris Unterberger, Gerhard Bauer, Johannes Reichsoellner, Giorgi Kuchukhidze, Eugen Trinka Department of Neurology, Medical University of Innsbruck, Austria Received April 9, 2009; Accepted November 18, 2009 ABSTRACT – Background. Myoclonic status epilepticus (MSE) is rarely found in juvenile myoclonic epilepsy (JME) and its clinical features are not well described. We aimed to analyze MSE incidence, precipitating factors and clini- cal course by studying patients with JME from a large outpatient epilepsy clinic. Methods. We retrospectively screened all patients with JME treated at the Department of Neurology, Medical University of Innsbruck, Austria between 1970 and 2007 for a history of MSE. We analyzed age, sex, age at seizure onset, seizure types, EEG, MRI/CT findings and response to antiepileptic drugs. Results. Seven patients (five women, two men; median age at time of MSE 31 years; range 17-73) with MSE out of a total of 247 patients with JME were identi- fied. The median follow-up time was seven years (range 0-35), the incidence was 3.2/1,000 patient years. Median duration of epilepsy before MSE was 26 years (range 10-58). We identified three subtypes: 1) MSE with myoclonic seizures only in two patients, 2) MSE with generalized tonic clonic seizures in three, and 3) generalized tonic clonic seizures with myoclonic absence status in two patients. All patients responded promptly to benzodiazepines. One patient had repeated episodes of MSE. Precipitating events were identified in all but one patient. Drug withdrawal was identified in four patients, one of whom had additional sleep deprivation and alcohol intake.
    [Show full text]
  • ILAE Classification and Definition of Epilepsy Syndromes with Onset in Childhood: Position Paper by the ILAE Task Force on Nosology and Definitions
    ILAE Classification and Definition of Epilepsy Syndromes with Onset in Childhood: Position Paper by the ILAE Task Force on Nosology and Definitions N Specchio1, EC Wirrell2*, IE Scheffer3, R Nabbout4, K Riney5, P Samia6, SM Zuberi7, JM Wilmshurst8, E Yozawitz9, R Pressler10, E Hirsch11, S Wiebe12, JH Cross13, P Tinuper14, S Auvin15 1. Rare and Complex Epilepsy Unit, Department of Neuroscience, Bambino Gesu’ Children’s Hospital, IRCCS, Member of European Reference Network EpiCARE, Rome, Italy 2. Divisions of Child and Adolescent Neurology and Epilepsy, Department of Neurology, Mayo Clinic, Rochester MN, USA. 3. University of Melbourne, Austin Health and Royal Children’s Hospital, Florey Institute, Murdoch Children’s Research Institute, Melbourne, Australia. 4. Reference Centre for Rare Epilepsies, Department of Pediatric Neurology, Necker–Enfants Malades Hospital, APHP, Member of European Reference Network EpiCARE, Institut Imagine, INSERM, UMR 1163, Université de Paris, Paris, France. 5. Neurosciences Unit, Queensland Children's Hospital, South Brisbane, Queensland, Australia. Faculty of Medicine, University of Queensland, Queensland, Australia. 6. Department of Paediatrics and Child Health, Aga Khan University, East Africa. 7. Paediatric Neurosciences Research Group, Royal Hospital for Children & Institute of Health & Wellbeing, University of Glasgow, Member of European Refence Network EpiCARE, Glasgow, UK. 8. Department of Paediatric Neurology, Red Cross War Memorial Children’s Hospital, Neuroscience Institute, University of Cape Town, South Africa. 9. Isabelle Rapin Division of Child Neurology of the Saul R Korey Department of Neurology, Montefiore Medical Center, Bronx, NY USA. 10. Programme of Developmental Neurosciences, UCL NIHR BRC Great Ormond Street Institute of Child Health, Department of Clinical Neurophysiology, Great Ormond Street Hospital for Children, London, UK 11.
    [Show full text]
  • “Seizures (Epilepsy)” a Seizure: Partial – Start in a Specific Part of the Brain, Not in the Whole Is a Symptom of an Electrical Disturbance in the Brain Brain
    “Seizures (Epilepsy)” A seizure: Partial – start in a specific part of the brain, not in the whole Is a symptom of an electrical disturbance in the brain brain. Unlike generalized seizures, partial seizures can have a Is a rare event warning before they occur (aura). Auras are actually a kind of Has a typical beginning (best clue for accurate diagnosis) seizure. There are several different kinds of partial seizures: Is involuntary simple (motor, sensory or psychological), complex, partial Lasts only a short time (90% complete in 90 seconds) seizure with secondary generalization. May cause post seizure impairments. Most seizures do not involve convulsions. Accurate seizure diagnosis by the health care provider is very The most common type of seizure is one mostly involving important because the medications used to treat seizures often vary loss of awareness. depending on the type. There are 20 types of seizures in the Seizures can be very subtle and hard to notice. International Classification of Seizures (over 2,000 types reported in the literature). Examples of post-seizure impairments: A detailed description of the seizure by the person observing the Post ictal confusion (length is individual) seizure is necessary for accurate diagnosing. Having a seizure while in Initial difficulty speaking the doctor’s office is very rare. Confusion about when, where, or what was just happening Memory disturbance which can last a while (behaving Seizure observation: - 3 important ones to make (in order of usual normally but can’t retain/absorb information) importance): Headache with some kinds of seizures What happened right as the seizure was beginning? What is epilepsy? What happened after the seizure was over? What happened during the seizure? Epilepsy is a condition where a person has “recurrent, unprovoked” seizures.
    [Show full text]
  • Managing Children with Epilepsy School Nurse Guide
    MANAGING CHILDREN WITH EPILEPSY SCHOOL NURSE GUIDE ACKNOWLEDGEMENTS TO THOSE WHO HAVE CONTRIBUTED TO THE NOTEBOOK Children’s Hospital of Orange County Melodie Balsbaugh, RN Sue Nagel, RN Giana Nguyen, CHOC Institutes Fullerton School District Jane Bockhacker, RN Orange Unified School District Andrea Bautista, RN Martha Boughen, RN Karen Hanson, RN TABLE OF CONTENTS I. EPILEPSY What is epilepsy? Facts about epilepsy Basic neuroanatomy overview Classification of epileptic seizures Diagnostic Tests II. TREATMENT Medications Vagus Nerve Stimulation Ketogenic Diet Surgery III. SAFETY First Aid IV. SPECIAL CONCERNS MedicAlert Helmets Driving Employment and the law V. EPILEPSY AT SCHOOL School epilepsy assessment tool Seizure record Teaching children about epilepsy lesson plan Creating your own individualized health care plan VI. RESOURCES/SUPPORT GROUPS VII. ACCESS TO HEALTHCARE CHOC Epilepsy Center After-Hours Care After Hours Health Care Advice Healthy Families California Kids MediCal CHOC Clinics Healthy Tomorrows VIII. REFERENCES EPILEPSY WHAT IS EPILEPSY? Epilepsy is a neurological disorder. The brain contains millions of nerve cells called neurons that send electrical charges to each other. A seizure occurs when there is a sudden and brief excess surge of electrical activity in the brain between nerve cells. This results in an alteration in sensation, behavior, and consciousness. Seizures may be caused by developmental problems before birth, trauma at birth, head injury, tumor, structural problems, vascular problems (i.e. stroke, abnormal blood vessels), metabolic conditions (i.e. low blood sugar, low calcium), infections (i.e. meningitis, encephalitis) and idiopathic causes. Children who have idiopathic seizures are most likely to respond to medications and outgrow seizures.
    [Show full text]
  • Language Dysfunction in Pediatric Epilepsy
    THE JOURNAL OF PEDIATRICS • www.jpeds.com MEDICAL PROGRESS Language Dysfunction in Pediatric Epilepsy Fiona M. Baumer, MD, Aaron L. Cardon, MD, MSc, and Brenda E. Porter, MD, PhD pilepsy is one of the most common and severe neu- assessment of language extremely difficult; this review will there- rologic diseases in children, affecting 0.9%-2% of the fore focus on studies of children with normal or near-normal E pediatric population.1,2 Children and adolescents with intelligence. This paper will first review studies characteriz- epilepsy and their parents indicate that quality of life is driven ing language deficits in pediatric epilepsy from the most severe as much or more by cognitive comorbidities as by seizure forms with total loss of language to the more common forms control.3-5 Surveys of these families found that cognitive prob- of language impairment found in the inappropriately termed lems were second only to medication side effects in terms of “benign” epilepsies of childhood. Next, we will describe what decreasing quality of life.6 The new International League Against is known about the structural and electrophysiologic changes Epilepsy classification considers the cognitive comorbidities seen associated with language dysfunction, reviewing the in epilepsy to be part of the condition.7 Of the cognitive prob- neuroimaging, electroencephalogram (EEG), and genetic studies lems seen in epilepsy, language disorders (Table) are particu- related to language dysfunction. Epilepsy surgery planning and larly important to identify and address, as language dysfunction resection of epileptic foci provide additional tools to under- can contribute to academic underachievement and long- stand the impact of focal epilepsy on language network de- term social, professional, and psychological problems.10 velopment and interaction with overall cognition in children The impact of epilepsy on language is relevant not only from with epilepsy.
    [Show full text]
  • Evaluation of Behavior and Cognitive Function in Children with Myoclonic
    & The ics ra tr pe ia u Niimi et al., Pediat Therapeut 2013, 3:3 t i d c e s P DOI: 10.4172/2161-0665.1000159 Pediatrics & Therapeutics ISSN: 2161-0665 Case Report Open Access Evaluation of Behavior and Cognitive Function in Children with Myoclonic Astatic Epilepsy Taemi Niimi1, Yuji Inaba1, Mitsuo Motobayashi1, Takafumi Nishimura1, Naoko Shiba1, Tetsuhiro Fukuyama2, Tsukasa Higuchi3, and Kenichi Koike1 1Department of Pediatrics, Shinshu University School of Medicine, Matsumoto, Japan 2Department of Pediatric Neurology, Nagano Children’s Hospital, Azumino, Japan 3Department of General Pediatrics, Nagano Children’s Hospital, Azumino, Japan Abstract Background: Myoclonic astatic epilepsy (MAE) is an idiopathic and generalized childhood epileptic syndrome. Although several studies have reported on cognitive function and intellectual outcome in MAE, little is known about the behavioral problems associated with this disease. The aim of this study was to clarify behavior and cognitive function in children with MAE. Methods: Four children who were diagnosed as having MAE using the proposed criteria of the International Classification of Epilepsies were retrospectively analyzed using patient records with regard to clinical and neuropsychological findings such as age of seizure onset, semiology and severity of seizures, treatment course, behavioral problems, EEG findings, and WISC-III and Pervasive Developmental Disorders Autism Society Japan Rating Scale (PARS) scores that indicate the tendency of autistic behaviors. Results: Disease onset in our cohort ranged from 9 to 35 months of age. Seizures were controlled within 3-8 months over a follow-up period of 4-12 years. All patients had borderline normal intelligence (mean IQ: 75.8 at 5-7 years of age) and exhibited impaired coordination, clumsiness, hyperactivity or impulsivity, and impaired social interaction after improvement of seizures.
    [Show full text]
  • Understanding Seizures and Epilepsy
    Understanding Sei zures & Epilepsy Selim R. Benbadis, MD Leanne Heriaud, RN Comprehensive Epilepsy Program Table of Contents * What is a seizure and what is epilepsy?....................................... 3 * Who is affected by epilepsy? ......................................................... 3 * Types of seizures ............................................................................. 3 * Types of epilepsy ............................................................................. 6 * How is epilepsy diagnosed? .......................................................... 9 * How is epilepsy treated? .............................................................. 10 Drug therapy ......................................................................... 10 How medication is prescribed ............................................ 12 Will treatment work?............................................................ 12 How long will treatment last?............................................. 12 Other treatment options....................................................... 13 * First aid for a person having a seizure ....................................... 13 * Safety and epilepsy ....................................................................... 14 * Epilepsy and driving..................................................................... 15 * Epilepsy and pregnancy ............................................................... 15 * More Information .......................................................................... 16 Comprehensive
    [Show full text]
  • Juvenile Myoclonic Epilepsy: Under-Appreciated and Under-Diagnosed R Renganathan, N Delanty
    78 Postgrad Med J: first published as 10.1136/pmj.79.928.78 on 1 February 2003. Downloaded from REVIEW Juvenile myoclonic epilepsy: under-appreciated and under-diagnosed R Renganathan, N Delanty ............................................................................................................................. Postgrad Med J 2003;79:78–80 Juvenile myoclonic epilepsy (JME) is a hereditary, AETIOPATHOGENESIS idiopathic, generalised epilepsy and is found in JME is an inherited disorder, but the exact mode of inheritance is not clear. There is a positive fam- 5%–11% of patients with epilepsy. It is characterised by ily history in 50% of cases or less. One study has myoclonic jerks, occasional generalised tonic-clonic confirmed a causative role of EJM1 in the patho- seizures, and sometimes absence seizures. JME genesis of idiopathic generalised seizures in the majority of German families of JME patients. This continues to be under-appreciated and study has refined a candidate region of 10.1 cM in under-diagnosed. Accurate diagnosis is important as it the chromosomal region 6p21 between the flank- usually responds well to treatment with appropriate ing loci HLA-DQ and D6s1019.3 However, some studies have revealed controversial results and anticonvulsants and misdiagnosis often results in genetic heterogeneity is suspected. Linkage to unnecessary morbidity. In addition lifelong therapy is chromosome 15 has at most a minor role in usually indicated as the natural history is one of relapse JME.4 Few studies have focused on pathological find-
    [Show full text]
  • Idiopathic Intracranial Hypertension: Consensus Guidelines On
    General Neurology J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp-2017-317440 on 14 June 2018. Downloaded from REVIEW Idiopathic intracranial hypertension: consensus guidelines on management Susan P Mollan,1,2 Brendan Davies,3 Nick C Silver,4 Simon Shaw,5 Conor L Mallucci,6,7 Benjamin R Wakerley,8,9 Anita Krishnan,4 Swarupsinh V Chavda,10 Satheesh Ramalingam,10 Julie Edwards,11,12 Krystal Hemmings,13 Michelle Williamson,13 Michael A Burdon,2 Ghaniah Hassan-Smith,1,12 Kathleen Digre,14 Grant T Liu,15 Rigmor Højland Jensen,16 Alexandra J Sinclair1,2,12,17 ► Additional material is ABSTRact contains information that will be of interest to those published online only. To view The aim was to capture interdisciplinary expertise from a in primary care and other healthcare professionals. please visit the journal online large group of clinicians, reflecting practice from across The increasing economic burden of IIH has been (http:// dx. doi. org/ 10. 1136/ 1 2 jnnp- 2017- 317440). the UK and further, to inform subsequent development of highlighted by a number of groups. Clear guid- a national consensus guidance for optimal management ance will help educate the attending doctors to For numbered affiliations see of idiopathic intracranial hypertension (IIH). manage these patients appropriately. This will help end of article. Methods Between September 2015 and October reduce the repeat unsolicited emergency hospital 2017, a specialist interest group including neurology, attendances and reduce IIH-related disability. Correspondence to There are a number of ongoing clinical trials in IIH Dr Alexandra J Sinclair, neurosurgery, neuroradiology, ophthalmology, nursing, Metabolic Neurology, Institute primary care doctors and patient representatives (https://www.
    [Show full text]
  • European Headache Federation Guideline on Idiopathic Intracranial
    Hoffmann et al. The Journal of Headache and Pain (2018) 19:93 The Journal of Headache https://doi.org/10.1186/s10194-018-0919-2 and Pain CONSENSUSARTICLE Open Access European Headache Federation guideline on idiopathic intracranial hypertension Jan Hoffmann1* , Susan P Mollan2, Koen Paemeleire3, Christian Lampl4, Rigmor H Jensen5 and Alexandra J Sinclair6 Abstract Background: Idiopathic Intracranial Hypertension (IIH) is characterized by an elevation of intracranial pressure (ICP no identifiable cause. The aetiology remains largely unknown, however observations made in a number of recent clinical studies are increasing the understanding of the disease and now provide the basis for evidence-based treatment strategies. Methods: The Embase, CDSR, CENTRAL, DARE and MEDLINE databases were searched up to 1st June 2018. We analyzed randomized controlled trials and systematic reviews that investigate IIH. Results: Diagnostic uncertainty, headache morbidity and visual loss are among the highest concerns of clinicians and patients in this disease area. Research in this field is infrequent due to the rarity of the disease and the lack of understanding of the underlying pathology. Conclusions: This European Headache Federation consensus paper provides evidence-based recommendations and practical advice on the investigation and management of IIH. Objective Headache Society (IHS) [3] (Table 1). The term ‘pseudo- Idiopathic Intracranial Hypertension (IIH) is character- tumor cerebri’, in the past commonly used as a synonym ized by an elevation of intracranial pressure (ICP) with for IIH, is now used as an umbrella term that describes no identifiable cause [1]. Despite the fact that its aeti- the chronic elevation of ICP regardless of its aetiology ology remains largely unknown, the observations made and further subdivides in the primary (IIH) and second- in a significant number of recent clinical studies and the ary forms [2].
    [Show full text]
  • Outpatient Neurology Quick Reference Guide
    OCTOBER 1, 2015 Outpatient Neurology Quick Reference Guide Objective: Ensure your ICD-10 success by documenting at the required level on future orders. Limit the use of unspecified diagnosis codes; drive for specificity when anatomy, etiology, or severity is known. ICD-9 Codes ICD-10 Code Requirements for ICD-10 Success Produced from Order Equivalents of ICD-9 Codes Not Specific Specific 780.39: Convulsions R56.9: Unspecified convulsions Type of Convulsions: • Epileptic (coded as epilepsy) • Febrile • Jacksonian (coded as epilepsy) • Myoclonic • Neonatal • Obstetrical • Reflex • Other (coded as seizure) 345.90: Epilepsy, unspecified, without G40.909: Epilepsy, unspecified, not Type of Epilepsy: intractable epilepsy intractable, without status • Due to syphilis epileptus • Related to (e.g., alcohol, drugs, stress, etc.) • Localization-related • Generalized • Other Localization-related Epilepsy • Idiopathic or Symptomatic? • With seizures of localized onset? (idiopathic) • Complex partial seizures or simple partial seizures? (symptomatic) • Intractable? • With status epilepticus? Generalized Epilepsy • Idiopathic? • Intractable? • With status epilepticus? Please submit ICD-10 questions to [email protected]. continued 11372 08/15 Copyright © 2015 Munson Healthcare, Traverse City, MI. All rights reserved. OCTOBER 1, 2015 Outpatient Neurology Quick Reference Guide Specific ICD-10-CM Codes Code Definition Convulsions R56.01 Complex febrile convulsions R53.00 Simple febrile convulsions G25.3 Myoclonus R25.8 Other abnormal involuntary movements
    [Show full text]