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Epileptic encephalopathy

This document discusses epileptic encephalopathies, which are a group of heterogeneous brain disorders occurring during brain development where epileptiform activity contributes to cognitive and behavioral regression beyond the underlying pathology. It defines and classifies several early-onset epileptic encephalopathy syndromes according to age of onset such as Ohtahara syndrome, early myoclonic encephalopathy, West syndrome, Dravet syndrome, Lennox-Gastaut syndrome, and myoclonic-astatic epilepsy. It provides details on clinical presentation, EEG features, causes, and treatment approaches for each condition.

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EPILEPTIC ENCEPHALOPATHIES DR SOORAJ PATIL SR NEUROLOGY IMS BHU Moderator- Prof Deepika Joshi
LEARNING OBJECTIVES • Definition of epileptic encephalopathy • Classification according to age • Brief overview of each epileptic encephalopathy syndromes • EEG features and treatment
DEFINITION • Epileptic Encephalopathy is defined as condition in which Epileptiform activity itself contributes to severe cognitive and behavior impairments and beyond what might be expected from the underlying pathology alone ( eg. Cortical Dysplasia ) ILAE classification of the epilepsies: Position paper of the ILAE Commission for Classification and Terminology Ingrid E. Scheffer- 2017
DEFINITION • A group of heterogeneous brain disorders occurring at a critical period of brain development, where frequent abnormal ictal and/or interictal EEG epileptiform activity is mainly responsible for behavioural, cognitive and motor regression • (1) Electrographic EEG paroxysmal activity that is often aggressive • (2) Seizures that are usually multi-form and intractable • (3) Cognitive, behavioural, and neurological deficits that may be relentless, sometimes early death EPILEPSY FOUNDATION 2017
Ohtahara Syndrome Early Myoclonic Encephalopathy West Syndrome Dravet Syndrome Myoclonic Status In Non Progressive Encephalopathies Lennox -Gastaut Syndrome Landau -Kleffner Syndrome Epilepsy With CSWS Rassmussen encephalitis Neonatal Infantile Childhood
“Age-related” epileptic encephalopathy Progressive maturation of the various areas of the brain Early and late childhood Late- onset epilepti c spasms in clusters Lennox– Gastaut syndrome infancy West syndrom e neonatal period Early epileptic encephalopathy
EARLY INFANTILE EPILEPTIC ENCEPHALOPATHY/OHTAHARA SYNDROME • Neonatal onset 10 days to 3 months of age, some times intrauterine seizures • Static structural brain damage most common • Tonic spasm > Generalized /Lateralized, tonic clonic, myoclonic, atonic, absences, partial, gelastic, and Jacksonians • Disorder progressively deteriorates with increasing frequency of seizures and severe Psychomotor Retardation Diagnosis and Management of Epileptic Encephalopathies in Children Puneet Jain Suvasini Sharma and Manjari Tripathi- EPILEPSY REASEARCH & TREATMENT 2013
CAUSES • Structural- Hemimegancephaly and Cortical Dysplasia • Metabolic- Non ketotic Hyperglycemia, Pyridoxine Deficiency , and a variety of disorders implicating the Mitochondrial Respiratory Chain. • Genetic Causes -ARX, CDKL5, SLC25A22, STXBP1, SPTAN1, KCNQ2, ARHGEF9, PCDH19, PNKP, SCN2A, PLCB1, SCN8A, S3GAL3, TBC1D24, and BRAT1.
EIEE • EEG – Burst Suppression pattern with high voltage Paroxysmal spike/ poly-spike discharges for Up to 3-6 seconds followed by prolonged periods of nearly flat tracing for up to 2-5 seconds. • This pattern remains unchanged during wakefulness and sleep
EIEE- EEG
TREATMENT • Prognosis Poor • Variety of antiepileptic drugs-clobazam , clonazepam , vigabatrin , topiramate , zonisamide , Phenobarbital, valproate, or felbamate . • Ketogenic diet • Most patients -Adverse Neurocognitive outcome , early death / may progress to WEST/ LGS • Minority specific treatment – Hemispherectomy / Pyridoxine supplement
EARLY MYOCLONIC ENCEPHALOPATHY • Neonatal period up to 3 months • Focal myoclonia of the face or extremities with random migration from one part to another • IEM -Most common causes( Amino Acidopathies), NKH, Zellweger syndrome, menkes ds, pyridoxin deficiency • Less often – structural abnormality ( cortical dysplasia) Review Article- Pediatric Neurol 2012 Early-Onset Epileptic Encephalopathies: Ohtahara Syndrome and Early Myoclonic Encephalopathy
EME EEG – Suppression Burst Pattern • Back ground- Suppression Burst Pattern • Burst suppression pattern more prominent during the sleep • The bursts last for 1–5 seconds with longer periods of suppression (3–10 seconds)
EIEE- EEG
EIEE VS EME
TREATMENT • AEDS, Pyridoxin trial • Ketogenic Diet • Epilepsy Surgery • Correcting Metabolic Problem • Many children progress to WEST /LGS
Epilepsy of Infancy with Migratory Focal seizures • First few months of life • Focal seizures with multiple topographical origins • Seizures are typically prolonged and often fulfill criteria for status epilepticus • Genetic - mostly
Epilepsy of Infancy with Migratory Focal seizures • Mostly genetic mutations- SCNA1 , SCN2A , PLCB1 , TBC1D24 • EEG- prominent slowing , disorganization and multifocal independent epileptiform discharges • The area of ictal onset shifts from one region to another and from one hemisphere to the other, with occasional overlapping of consecutive seizures
TREATMENT • Usually drug refractory • There are some reports of Stiripentol and bromides • Ketogenic diet • Vagal nerve stimulation • Surgical options- FOCAL LESIONS
INFANTILE SPASMS (WEST SYNDROME)
William James West, 1841 • Dr West's original letter gives a vivid clinical description of what was to become known, over a century later as West's syndrome • This letter, that was a call for help about his son to the medical profession, appeared in The Lancet 180 years ago
Duncan. Infantile spasms: the original description of Dr West. Epileptic Disorders. 2001;3:47-8. Article historique
Infantile spasms (IS) • An epilepsy syndrome with onset usually in infancy • Rarely an onset >2 years • Main clinical manifestation: clinical spasms • Clusters • Most characteristic EEG finding is hypsarrhythmia • Not always, not throughout • Often associated with developmental arrest or regression
West syndrome (WS) Clustered spasms Hypsarrhy -thmia on EEG West syndrome
INFANTILE SPASMS (WEST SYNDROME) • Spasm -Flexor / Extensor • Individual spasm generally last between 0.5 and 1.0 seconds and repeat every 5 to 30 seconds in clusters approximately 2 to 20 minutes
Infantile spasm-single spasm variant (ISSV) ISSV=Single spasms + hypsarrhythmia WS= clustered spasms + hypsarrhythmia hypsarrhythmia without IS= no spasms + hypsarrhythmia (HWIS)
CAUSES STRUCTURAL • Pre, Peri And Post Natal Cerebral Ischemia • Cerebral Malformations • Neuro Infections Sequel • Neurocutaneous Hamartoma Metabolic- Biotidinase Deficiency , PKU, Mitochondrial Disorders , Menkes Disease ,NKH GENETIC • CDKL-5 , MeCP 2, ARX, STXBP-1 , SPTAN 1and PLC-B1 • Chromosomal Disorders : Down Syndrome , Ip 36 deletion and Pallister – Killan syndrome Unknown
3 clinical states and corresponding EEG types • Clinical state 1 • Weeks to months • Clinically silent phase • Type 1 EEG: presents with (multi-)focal epileptic discharges <50% of NREM EEG recording time • Clinical state 2 • Several weeks • Beginning mental deterioration • Type 2 EEG: with bihemispheric epileptic discharges >50% of the non- NREM EEG recording time within abnormal background activity (imminent hypsarrhythmia)
• Clinical state 3 • Mental deterioration • Characterized by hypsarrhythmia Conclusions: Infants with WS could be reliably identified several weeks before the occurrence of hypsarrhythmia by a typical EEG pattern (type 2), thereby opening the way for early intervention studies
Type 1 EEG (clinical state 1) • Focal or multifocal sharp-wave activity • Occupying <50% of the non-REM EEG recording time • Background activity is normal or mildly abnormal
Type 2 EEG (clinical state 2) bihemispheric sharp-wave activity Occupying 50%–90% of the non-REM EEG Background activity is definitely abnormal. Type 2 EEG appears ~ modified hypsarrhythmia.
Type 3 EEG (clinical state 3) 90%–100% non-REM EEG Irregular bihemispheric sharp-waves hypsarrhythmia
TREATMENT • ACTH High dose, Low dose • Corticosteroids • AEDS - Clobazam, Clonazepam , Valproic Acid • VIGABATRIN- Most effective in Tuberous Sclerosis Complex • Use associated with Irreversible Visual Loss • Resectable surgeries for focal etiologies like cortical dysplasia
TREATMENT
TREATMENT • Are other forms of corticosteroids as effective as ACTH for short-term treatment of infantile spasms? • Are low-dose ACTH regimens effective for short-term treatment of infantile spasms? • Is ACTH more effective than VGB for short-term treatment of infantile spasms? • Is there a role for the ketogenic diet or for AEDs other than VGB in managing infantile spasms? • Does the successful short-term treatment of infantile spasms lead to long-term improvement of neurodevelopmental outcomes or a decreased epilepsy incidence?
TREATMENT The evidence is insufficient to recommend the use of prednisolone, dexamethasone, and methylprednisolone as being as effective as ACTH for short-term treatment of infantile spasms (class 3 & 4) Class I study showed similar efficacy between low-dose (20–30 IU) and high-dose (150 IU/m2) natural ACTH Two Class III studies (1 from the 2004 parameter and a later study) demonstrated that ACTH is more effective than VGB for short-term treatment of children with infantile spasms (excluding those with TSC) A shorter lag time to treatment of infantile spasms with either hormonal therapy or VGB may be considered to improve long-term cognitive outcomes (Level C)
Treat for 14 days Monitor ADR: infection, irritability, HTN Start low-dose ACTH at 20- 30 IU/m2 Increase rapidly to max doses as rapidly Repeat EEG after 2 weeks EEG s/o hyps but no clinical IS No clinical spasms and EEG resolved Neuro-developmental follow-up Continue Rx for 2-4 weeks
SEVERE MYOCLONIC EPILEPSY OF INFANCY/DRAVET SYNDROME • Early infantile febrile clonic seizures (before 1 yr of age ) • Myoclonic jerks, tonic- clonic, atypical absences, focal aware / impaired awareness • Vaccine seizures • Low grade fever, hot bath , warm environment • Cognitive and neurological detioration Retracing the natural history of Dravet syndrome: Report and review of literature Sachin Sureshbabu, Ivy Sebastian- Neurol india 2018
Age 4 -8 months(Feb rile stage) • Febrile Seizures • EEG normal Age 1- 4 yrs (worsening stage) • Other seizure types appear, regression of mile stones • EEG abnormal Age 5+ stabilizatio n stage • Seizures may become less frequent • Cognitive problems plateau Dravet Life Timeline
EEG Progressive increase of Paroxysmal Abnormalities • During wakefulness background activity remains normal in 50 % of the cases / Remaining background activity becomes slow and poorly organized. • Generalized Fast Spike – wave or Poly – spike appears in bursts or in isolation , sometimes with unilateral predominance/ induced by Eye Closure and intermittent Photic stimulation. • Focal and multi focal abnormalities such as Fast Spikes or poly – spikes involving Asynchronously the Fronto- Central or Centro-Temporal areas.
TREATMENT • Limited success AEDS- Levetiracetam, Clobazam, Clonazepam , Topiramate, ,Valproic Acid • Ketogenic Diet • Vagal Nerve Stimulation • Surgical Options • Cannabidiol –some patients • Avoid Na Channel Blockers
LENNOX- GASTAUT SYNDROME • LGS - triad of multiple drug-resistant seizure types, a specific interictal electroencephalographic (EEG) pattern showing bursts of slow spike-wave (SSW) complexes or generalized paroxysmal fast activity (GPFA) and intellectual disability (ID) • Multiple seizure types - Atypical absence , Tonic seizures (most common) and Drop seizures , NCSE in 2/3rd Asadi-Pooya AA. Lennox-Gastaut syndrome: a comprehensive review. Neurol Sci. 2018 Mar
LGS • Causes are genetic, structural in 60-70 % • 20 % may have west syndrome in past • LGS is considered as secondary epilepsy
EEG • The EEG background activity is probably never normal in LGS and shows a diffuse increase in slow waves • Characteristic EEG feature in LGS is slow (< 2.5 Hz) spike-and-wave complexes with an abnormally slow background activity • Hyperventilation may facilitate GSWD and Atypical Absences
EEG • Bursts of diffuse or bilateral fast (10–25 Hz) rhythm patterns, also called GPFA, are usually recorded during slow wave sleep
TREATMENT • Drop seizures most difficult to control • Topiramate/ Valproic acid / Felbamate / Lamotrigine, Benzodiazepines • Clobazam and Rufinamide to combat Drop Seizures. • Ketogenic diet in early age ~50% reduction in seizures, ~ 20% seizure free • Corpus callosotomy, VNS in refractory seizures Asadi-Pooya AA. Lennox-Gastaut syndrome: a comprehensive review. Neurol Sci. 2018 Mar
MYOCLONIC – ASTATIC SEIZURES (DOOSE SYNDROME) • Normal development, Male predominant in 70-90 % • Onset of Myoclonic , Myoclonic Atonic or Atonic seizures between the age group of 1 and 6 yrs • Part of GEFS + • No tonic seizures • Normal EEG background with generalized spike / Poly spike discharges at 3- 7Hz with Photosensitivity
EMAS • Initial seizures can be Febrile or Afebrile Generalized Tonic Clonic Seizures • Followed by characteristic seizure the Myoclonic- Atonic which combines a symmetrical Myoclonic jerk immediately followed by an Atonic seizure causing a Drop attack • Episodes of NCSE lasting hours / days occur in some children.
EMAS • EEG- Background activity is normal at the age • Rhythmic theta activity in the parasagittal region may be the only significant abnormality. • Frequent paroxysms of GSWD at 3 Hz or more appear once patient develops drop attacks
Features LGS EMAS Type of seizures Tonic, Atonic , Atypical Absences Myoclonic,Atonic and Myoclonic- Atonic Tonic seizures Common and characteristic, diurnal and nocturnal Not Seen Developmental abnormalities before onset of seizures Common Exceptional if any Etiology Symptomatic ( Most Cases) Idiopathic Genetic Predisposition None Common Progression from West Syndrome Common Incompatible Prognosis Commonly Bad Relatively Good EEG Background Abnormal By Rule Usually Normal Paroxysmal Fast Activity Common and Characteristic Not Seen EEG GSWS Slow (< 2.5 Hz) Fast (> 3Hz)
LANDAU KLEFFNER SYNDROME • Rapidly Progressive Linguistic Deterioration following seizure episode • Onset between 2 and 8 yrs of life • Atypical absence (50%), oral (33%), secondarily generalized tonic–clonic (SGTC) (33%), atonic (16%), and hemiconvulsive (16%) attacks • Diagnosis is often delayed as children are often thought to have acquired deafness , Autistic Regression or Mutism • Seizures ( Mostly Nocturnal ) occur in 2/3rd and easy to control • Behavioral and Cognitive disturbances are commonly observed
LKS-EEG • Background activity remains normal in most cases • High amplitude , Epileptiform discharges are seen in Temporo Parietal areas/ Centro Parietal areas ( spikes, sharp waves /spike and sharp wave) • EEG abnormality is enhanced by sleep deprivation • ICTAL EEG – Focal seizures , Focal Ictal pattern from Temporo- Parietal / Posterior Temporal areas
TREATMENT • AEDS- BZD and VAL (does not effect language) • High dose corticosteroids – Stabilization of Language Problems • The prognosis is Favorable • 17 % can have complete recovery, 63 % can develop GDD • Poor prognosis in <4 years of age, duration > 1 years
CONTINUOUS SPIKE WAVE EPILEPSY • Classical presentation is a child with around 5 years of age, typically between age 4 and 7 years • TRIAD consists of 1. Intractable seizures 2. Interictal Epileptiform Activity that becomes prominent during sleep , leading to EEG pattern of Electrical Status Epilepticus in sleep (ESES) or CSWS 3. Neurocognitive Regression after 2-3 years of seizures
CONTINUOUS SPIKE WAVE EPILEPSY • EEG abnormalities became continuous with sleep onset and ceased to be continuous upon arousal, occupying at least 85% of slow sleep tracing • neurocognitive impairments are the presenting symptoms in the absence of clinical seizures but can be seen in up to 20% of cases
CSWS- EEG Active stage • Typical CSWS pattern appears on EEG 1-2 years after the first seizures • Children with Frontal CSWS – Aggressiveness/ Disinhibition/ Inattention • Children with Temporal CSWS- Expressive Aphasia with Non fluent speech Remission stage • After 3- 8 years of age seizures remit in all cases • EEG gradually remit to normal state
CSWS LKS Clinical Seizures All patients 40- 70 % Primary language impairment Expressive aphasia, motor, cognitive decline Verbal auditory agnosia Behavioral deficit Nearly all patients 40- 50 % EEG CSWS 100% of patients 80% of patients Spike location Frontal , centro –temporal Posterior temporal , centro - parietal
TREATMENT • High dose Benzodiazepines • Corticosteroids • AEDS= Valproate / Ethosuximide / Levetiracetam/ Lamotrigine Continuous Spike-Wave during Slow Wave Sleep and Related Conditions Nilika Shah Singhal* and Joseph E. Sullivan ISRN-2014
RASSMUSSENS ENCEPHALITIS • Drug-resistant focal epilepsy, progressive hemiplegia, and cognitive decline, with unihemispheric brain • Affected brain shows inflammation on histology and Autoimmune pathology (GLUR3 antibody). • Age of onset between 6- 8 yrs and affected children have previous normal cognition and development. Rasmussen’s encephalitis: clinical features, pathobiology, and treatment advances-Lancet Neurol 2015
STAGES - RASSMUSSENS ENCEPHALITIS Acute stage- • EPC, Progressive hemiparesis , hemianopia , cognitive deterioration and aphasias. • Median duration is 8- 12 months. Residual stage- • Permanent and stable neurological deficits and frequent seizures , although less frequent seizures than in acute stage Rasmussen’s encephalitis: clinical features, pathobiology, and treatment advances-Lancet Neurol 2015
DIAGNOSTIC CRITERIA Rasmussen’s encephalitis: clinical features, pathobiology, and treatment advances-Lancet Neurol 2015
EEG • Background EEG may be normal. Subsequently unilateral focal theta- delta range slowing appears , which often becomes synchronized . • INTER –ICTAL Discharges- Multiple independent spike/ sharp – wave discharges are seen in the affected hemisphere • In 30 % the discharges are bilateral. • Activation – EEG abnormalities are enhanced by sleep deprivation as well in sleep Rasmussen’s encephalitis: clinical features, pathobiology, and treatment advances-Lancet Neurol 2015
Rasmussen’s encephalitis: clinical features, pathobiology, and treatment advances-Lancet Neurol 2015
TREATMENT • Steroids and other Immunomodulators are given early in the course of disease to reduce the inflammation , seizure activity and severity of deficit • Refractory seizures – Hemispherectomy • Prognosis below the age of 10 is good who are treated well. Rasmussen’s encephalitis: clinical features, pathobiology, and treatment advances-Lancet Neurol 2015
Immunomodulatory therapy versus surgery Takahashi Y et al. Immunomodulatory therapy versus surgery for Rasmussen syndrome in early childhood. Brain Dev. 2013
ALGORITHM < 3 Months <3 months – 1 year Burst Suppression Pattern Partial Seizures/ Erratic Myoclonus Ohtahara Syndrome Early Myoclonic Encephalopathy Infantile Spasm West Syndrome Tonic Spasms
> 1 YR -Atypical Febrile seizures -EEG- Normal background > 3 Hz Generalized Discharges -Myoclonic Status - Generalized Spike< Slow wave Background Slowing -Myoclonic Asatic Seizures -Parasagittal Slowing -Fast Generalized Spike/Polyspike Discharges Dravet Syndrome Myoclonic Status in Non Progressive Encephalopathy Doose Syndrome
-Drop Attacks -GSWD, Multifocal Discharges -Paroxysmal Fast Activity -Background Slowing Partial Seizures LGS Auditory Agnosia+/- ESES on EEG ESES on EEG -EPC -Progressive Neurological Deficit LKS CSWS Rasmussen Encephalitis >1 yr
TAKE HOME MESSAGES • Age of presentation and prior developmental status is most important to diagnose epileptic encephalopathy syndromes • EEG will help to early detection and differentiation of each encephalopathies • Proper treatment helps child to have a seizure free childhood and normal development
THANK YOU

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Epileptic encephalopathy

  • 1.
    EPILEPTIC ENCEPHALOPATHIES DR SOORAJ PATIL SRNEUROLOGY IMS BHU Moderator- Prof Deepika Joshi
  • 2.
    LEARNING OBJECTIVES • Definitionof epileptic encephalopathy • Classification according to age • Brief overview of each epileptic encephalopathy syndromes • EEG features and treatment
  • 3.
    DEFINITION • Epileptic Encephalopathyis defined as condition in which Epileptiform activity itself contributes to severe cognitive and behavior impairments and beyond what might be expected from the underlying pathology alone ( eg. Cortical Dysplasia ) ILAE classification of the epilepsies: Position paper of the ILAE Commission for Classification and Terminology Ingrid E. Scheffer- 2017
  • 4.
    DEFINITION • A groupof heterogeneous brain disorders occurring at a critical period of brain development, where frequent abnormal ictal and/or interictal EEG epileptiform activity is mainly responsible for behavioural, cognitive and motor regression • (1) Electrographic EEG paroxysmal activity that is often aggressive • (2) Seizures that are usually multi-form and intractable • (3) Cognitive, behavioural, and neurological deficits that may be relentless, sometimes early death EPILEPSY FOUNDATION 2017
  • 6.
    Ohtahara Syndrome Early Myoclonic Encephalopathy West Syndrome DravetSyndrome Myoclonic Status In Non Progressive Encephalopathies Lennox -Gastaut Syndrome Landau -Kleffner Syndrome Epilepsy With CSWS Rassmussen encephalitis Neonatal Infantile Childhood
  • 7.
    “Age-related” epileptic encephalopathy Progressivematuration of the various areas of the brain Early and late childhood Late- onset epilepti c spasms in clusters Lennox– Gastaut syndrome infancy West syndrom e neonatal period Early epileptic encephalopathy
  • 8.
    EARLY INFANTILE EPILEPTIC ENCEPHALOPATHY/OHTAHARASYNDROME • Neonatal onset 10 days to 3 months of age, some times intrauterine seizures • Static structural brain damage most common • Tonic spasm > Generalized /Lateralized, tonic clonic, myoclonic, atonic, absences, partial, gelastic, and Jacksonians • Disorder progressively deteriorates with increasing frequency of seizures and severe Psychomotor Retardation Diagnosis and Management of Epileptic Encephalopathies in Children Puneet Jain Suvasini Sharma and Manjari Tripathi- EPILEPSY REASEARCH & TREATMENT 2013
  • 9.
    CAUSES • Structural- Hemimegancephalyand Cortical Dysplasia • Metabolic- Non ketotic Hyperglycemia, Pyridoxine Deficiency , and a variety of disorders implicating the Mitochondrial Respiratory Chain. • Genetic Causes -ARX, CDKL5, SLC25A22, STXBP1, SPTAN1, KCNQ2, ARHGEF9, PCDH19, PNKP, SCN2A, PLCB1, SCN8A, S3GAL3, TBC1D24, and BRAT1.
  • 10.
    EIEE • EEG –Burst Suppression pattern with high voltage Paroxysmal spike/ poly-spike discharges for Up to 3-6 seconds followed by prolonged periods of nearly flat tracing for up to 2-5 seconds. • This pattern remains unchanged during wakefulness and sleep
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    TREATMENT • Prognosis Poor •Variety of antiepileptic drugs-clobazam , clonazepam , vigabatrin , topiramate , zonisamide , Phenobarbital, valproate, or felbamate . • Ketogenic diet • Most patients -Adverse Neurocognitive outcome , early death / may progress to WEST/ LGS • Minority specific treatment – Hemispherectomy / Pyridoxine supplement
  • 13.
    EARLY MYOCLONIC ENCEPHALOPATHY •Neonatal period up to 3 months • Focal myoclonia of the face or extremities with random migration from one part to another • IEM -Most common causes( Amino Acidopathies), NKH, Zellweger syndrome, menkes ds, pyridoxin deficiency • Less often – structural abnormality ( cortical dysplasia) Review Article- Pediatric Neurol 2012 Early-Onset Epileptic Encephalopathies: Ohtahara Syndrome and Early Myoclonic Encephalopathy
  • 14.
    EME EEG – SuppressionBurst Pattern • Back ground- Suppression Burst Pattern • Burst suppression pattern more prominent during the sleep • The bursts last for 1–5 seconds with longer periods of suppression (3–10 seconds)
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    TREATMENT • AEDS, Pyridoxintrial • Ketogenic Diet • Epilepsy Surgery • Correcting Metabolic Problem • Many children progress to WEST /LGS
  • 18.
    Epilepsy of Infancywith Migratory Focal seizures • First few months of life • Focal seizures with multiple topographical origins • Seizures are typically prolonged and often fulfill criteria for status epilepticus • Genetic - mostly
  • 19.
    Epilepsy of Infancywith Migratory Focal seizures • Mostly genetic mutations- SCNA1 , SCN2A , PLCB1 , TBC1D24 • EEG- prominent slowing , disorganization and multifocal independent epileptiform discharges • The area of ictal onset shifts from one region to another and from one hemisphere to the other, with occasional overlapping of consecutive seizures
  • 20.
    TREATMENT • Usually drugrefractory • There are some reports of Stiripentol and bromides • Ketogenic diet • Vagal nerve stimulation • Surgical options- FOCAL LESIONS
  • 21.
  • 22.
    William James West, 1841 •Dr West's original letter gives a vivid clinical description of what was to become known, over a century later as West's syndrome • This letter, that was a call for help about his son to the medical profession, appeared in The Lancet 180 years ago
  • 23.
    Duncan. Infantile spasms:the original description of Dr West. Epileptic Disorders. 2001;3:47-8. Article historique
  • 24.
    Infantile spasms (IS) •An epilepsy syndrome with onset usually in infancy • Rarely an onset >2 years • Main clinical manifestation: clinical spasms • Clusters • Most characteristic EEG finding is hypsarrhythmia • Not always, not throughout • Often associated with developmental arrest or regression
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    INFANTILE SPASMS (WESTSYNDROME) • Spasm -Flexor / Extensor • Individual spasm generally last between 0.5 and 1.0 seconds and repeat every 5 to 30 seconds in clusters approximately 2 to 20 minutes
  • 27.
    Infantile spasm-single spasmvariant (ISSV) ISSV=Single spasms + hypsarrhythmia WS= clustered spasms + hypsarrhythmia hypsarrhythmia without IS= no spasms + hypsarrhythmia (HWIS)
  • 28.
    CAUSES STRUCTURAL • Pre, PeriAnd Post Natal Cerebral Ischemia • Cerebral Malformations • Neuro Infections Sequel • Neurocutaneous Hamartoma Metabolic- Biotidinase Deficiency , PKU, Mitochondrial Disorders , Menkes Disease ,NKH GENETIC • CDKL-5 , MeCP 2, ARX, STXBP-1 , SPTAN 1and PLC-B1 • Chromosomal Disorders : Down Syndrome , Ip 36 deletion and Pallister – Killan syndrome Unknown
  • 29.
    3 clinical statesand corresponding EEG types • Clinical state 1 • Weeks to months • Clinically silent phase • Type 1 EEG: presents with (multi-)focal epileptic discharges <50% of NREM EEG recording time • Clinical state 2 • Several weeks • Beginning mental deterioration • Type 2 EEG: with bihemispheric epileptic discharges >50% of the non- NREM EEG recording time within abnormal background activity (imminent hypsarrhythmia)
  • 30.
    • Clinical state3 • Mental deterioration • Characterized by hypsarrhythmia Conclusions: Infants with WS could be reliably identified several weeks before the occurrence of hypsarrhythmia by a typical EEG pattern (type 2), thereby opening the way for early intervention studies
  • 31.
    Type 1 EEG(clinical state 1) • Focal or multifocal sharp-wave activity • Occupying <50% of the non-REM EEG recording time • Background activity is normal or mildly abnormal
  • 32.
    Type 2 EEG(clinical state 2) bihemispheric sharp-wave activity Occupying 50%–90% of the non-REM EEG Background activity is definitely abnormal. Type 2 EEG appears ~ modified hypsarrhythmia.
  • 33.
    Type 3 EEG(clinical state 3) 90%–100% non-REM EEG Irregular bihemispheric sharp-waves hypsarrhythmia
  • 34.
    TREATMENT • ACTH Highdose, Low dose • Corticosteroids • AEDS - Clobazam, Clonazepam , Valproic Acid • VIGABATRIN- Most effective in Tuberous Sclerosis Complex • Use associated with Irreversible Visual Loss • Resectable surgeries for focal etiologies like cortical dysplasia
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  • 36.
    TREATMENT • Are otherforms of corticosteroids as effective as ACTH for short-term treatment of infantile spasms? • Are low-dose ACTH regimens effective for short-term treatment of infantile spasms? • Is ACTH more effective than VGB for short-term treatment of infantile spasms? • Is there a role for the ketogenic diet or for AEDs other than VGB in managing infantile spasms? • Does the successful short-term treatment of infantile spasms lead to long-term improvement of neurodevelopmental outcomes or a decreased epilepsy incidence?
  • 37.
    TREATMENT The evidence isinsufficient to recommend the use of prednisolone, dexamethasone, and methylprednisolone as being as effective as ACTH for short-term treatment of infantile spasms (class 3 & 4) Class I study showed similar efficacy between low-dose (20–30 IU) and high-dose (150 IU/m2) natural ACTH Two Class III studies (1 from the 2004 parameter and a later study) demonstrated that ACTH is more effective than VGB for short-term treatment of children with infantile spasms (excluding those with TSC) A shorter lag time to treatment of infantile spasms with either hormonal therapy or VGB may be considered to improve long-term cognitive outcomes (Level C)
  • 38.
    Treat for 14days Monitor ADR: infection, irritability, HTN Start low-dose ACTH at 20- 30 IU/m2 Increase rapidly to max doses as rapidly Repeat EEG after 2 weeks EEG s/o hyps but no clinical IS No clinical spasms and EEG resolved Neuro-developmental follow-up Continue Rx for 2-4 weeks
  • 39.
    SEVERE MYOCLONIC EPILEPSYOF INFANCY/DRAVET SYNDROME • Early infantile febrile clonic seizures (before 1 yr of age ) • Myoclonic jerks, tonic- clonic, atypical absences, focal aware / impaired awareness • Vaccine seizures • Low grade fever, hot bath , warm environment • Cognitive and neurological detioration Retracing the natural history of Dravet syndrome: Report and review of literature Sachin Sureshbabu, Ivy Sebastian- Neurol india 2018
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    Age 4 -8 months(Feb rilestage) • Febrile Seizures • EEG normal Age 1- 4 yrs (worsening stage) • Other seizure types appear, regression of mile stones • EEG abnormal Age 5+ stabilizatio n stage • Seizures may become less frequent • Cognitive problems plateau Dravet Life Timeline
  • 41.
    EEG Progressive increase ofParoxysmal Abnormalities • During wakefulness background activity remains normal in 50 % of the cases / Remaining background activity becomes slow and poorly organized. • Generalized Fast Spike – wave or Poly – spike appears in bursts or in isolation , sometimes with unilateral predominance/ induced by Eye Closure and intermittent Photic stimulation. • Focal and multi focal abnormalities such as Fast Spikes or poly – spikes involving Asynchronously the Fronto- Central or Centro-Temporal areas.
  • 43.
    TREATMENT • Limited successAEDS- Levetiracetam, Clobazam, Clonazepam , Topiramate, ,Valproic Acid • Ketogenic Diet • Vagal Nerve Stimulation • Surgical Options • Cannabidiol –some patients • Avoid Na Channel Blockers
  • 44.
    LENNOX- GASTAUT SYNDROME •LGS - triad of multiple drug-resistant seizure types, a specific interictal electroencephalographic (EEG) pattern showing bursts of slow spike-wave (SSW) complexes or generalized paroxysmal fast activity (GPFA) and intellectual disability (ID) • Multiple seizure types - Atypical absence , Tonic seizures (most common) and Drop seizures , NCSE in 2/3rd Asadi-Pooya AA. Lennox-Gastaut syndrome: a comprehensive review. Neurol Sci. 2018 Mar
  • 45.
    LGS • Causes aregenetic, structural in 60-70 % • 20 % may have west syndrome in past • LGS is considered as secondary epilepsy
  • 46.
    EEG • The EEGbackground activity is probably never normal in LGS and shows a diffuse increase in slow waves • Characteristic EEG feature in LGS is slow (< 2.5 Hz) spike-and-wave complexes with an abnormally slow background activity • Hyperventilation may facilitate GSWD and Atypical Absences
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    EEG • Bursts ofdiffuse or bilateral fast (10–25 Hz) rhythm patterns, also called GPFA, are usually recorded during slow wave sleep
  • 50.
    TREATMENT • Drop seizuresmost difficult to control • Topiramate/ Valproic acid / Felbamate / Lamotrigine, Benzodiazepines • Clobazam and Rufinamide to combat Drop Seizures. • Ketogenic diet in early age ~50% reduction in seizures, ~ 20% seizure free • Corpus callosotomy, VNS in refractory seizures Asadi-Pooya AA. Lennox-Gastaut syndrome: a comprehensive review. Neurol Sci. 2018 Mar
  • 51.
    MYOCLONIC – ASTATICSEIZURES (DOOSE SYNDROME) • Normal development, Male predominant in 70-90 % • Onset of Myoclonic , Myoclonic Atonic or Atonic seizures between the age group of 1 and 6 yrs • Part of GEFS + • No tonic seizures • Normal EEG background with generalized spike / Poly spike discharges at 3- 7Hz with Photosensitivity
  • 52.
    EMAS • Initial seizurescan be Febrile or Afebrile Generalized Tonic Clonic Seizures • Followed by characteristic seizure the Myoclonic- Atonic which combines a symmetrical Myoclonic jerk immediately followed by an Atonic seizure causing a Drop attack • Episodes of NCSE lasting hours / days occur in some children.
  • 53.
    EMAS • EEG- Backgroundactivity is normal at the age • Rhythmic theta activity in the parasagittal region may be the only significant abnormality. • Frequent paroxysms of GSWD at 3 Hz or more appear once patient develops drop attacks
  • 55.
    Features LGS EMAS Typeof seizures Tonic, Atonic , Atypical Absences Myoclonic,Atonic and Myoclonic- Atonic Tonic seizures Common and characteristic, diurnal and nocturnal Not Seen Developmental abnormalities before onset of seizures Common Exceptional if any Etiology Symptomatic ( Most Cases) Idiopathic Genetic Predisposition None Common Progression from West Syndrome Common Incompatible Prognosis Commonly Bad Relatively Good EEG Background Abnormal By Rule Usually Normal Paroxysmal Fast Activity Common and Characteristic Not Seen EEG GSWS Slow (< 2.5 Hz) Fast (> 3Hz)
  • 56.
    LANDAU KLEFFNER SYNDROME •Rapidly Progressive Linguistic Deterioration following seizure episode • Onset between 2 and 8 yrs of life • Atypical absence (50%), oral (33%), secondarily generalized tonic–clonic (SGTC) (33%), atonic (16%), and hemiconvulsive (16%) attacks • Diagnosis is often delayed as children are often thought to have acquired deafness , Autistic Regression or Mutism • Seizures ( Mostly Nocturnal ) occur in 2/3rd and easy to control • Behavioral and Cognitive disturbances are commonly observed
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    LKS-EEG • Background activityremains normal in most cases • High amplitude , Epileptiform discharges are seen in Temporo Parietal areas/ Centro Parietal areas ( spikes, sharp waves /spike and sharp wave) • EEG abnormality is enhanced by sleep deprivation • ICTAL EEG – Focal seizures , Focal Ictal pattern from Temporo- Parietal / Posterior Temporal areas
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    TREATMENT • AEDS- BZDand VAL (does not effect language) • High dose corticosteroids – Stabilization of Language Problems • The prognosis is Favorable • 17 % can have complete recovery, 63 % can develop GDD • Poor prognosis in <4 years of age, duration > 1 years
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    CONTINUOUS SPIKE WAVEEPILEPSY • Classical presentation is a child with around 5 years of age, typically between age 4 and 7 years • TRIAD consists of 1. Intractable seizures 2. Interictal Epileptiform Activity that becomes prominent during sleep , leading to EEG pattern of Electrical Status Epilepticus in sleep (ESES) or CSWS 3. Neurocognitive Regression after 2-3 years of seizures
  • 62.
    CONTINUOUS SPIKE WAVEEPILEPSY • EEG abnormalities became continuous with sleep onset and ceased to be continuous upon arousal, occupying at least 85% of slow sleep tracing • neurocognitive impairments are the presenting symptoms in the absence of clinical seizures but can be seen in up to 20% of cases
  • 63.
    CSWS- EEG Active stage •Typical CSWS pattern appears on EEG 1-2 years after the first seizures • Children with Frontal CSWS – Aggressiveness/ Disinhibition/ Inattention • Children with Temporal CSWS- Expressive Aphasia with Non fluent speech Remission stage • After 3- 8 years of age seizures remit in all cases • EEG gradually remit to normal state
  • 65.
    CSWS LKS Clinical Seizures Allpatients 40- 70 % Primary language impairment Expressive aphasia, motor, cognitive decline Verbal auditory agnosia Behavioral deficit Nearly all patients 40- 50 % EEG CSWS 100% of patients 80% of patients Spike location Frontal , centro –temporal Posterior temporal , centro - parietal
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    TREATMENT • High doseBenzodiazepines • Corticosteroids • AEDS= Valproate / Ethosuximide / Levetiracetam/ Lamotrigine Continuous Spike-Wave during Slow Wave Sleep and Related Conditions Nilika Shah Singhal* and Joseph E. Sullivan ISRN-2014
  • 67.
    RASSMUSSENS ENCEPHALITIS • Drug-resistantfocal epilepsy, progressive hemiplegia, and cognitive decline, with unihemispheric brain • Affected brain shows inflammation on histology and Autoimmune pathology (GLUR3 antibody). • Age of onset between 6- 8 yrs and affected children have previous normal cognition and development. Rasmussen’s encephalitis: clinical features, pathobiology, and treatment advances-Lancet Neurol 2015
  • 68.
    STAGES - RASSMUSSENSENCEPHALITIS Acute stage- • EPC, Progressive hemiparesis , hemianopia , cognitive deterioration and aphasias. • Median duration is 8- 12 months. Residual stage- • Permanent and stable neurological deficits and frequent seizures , although less frequent seizures than in acute stage Rasmussen’s encephalitis: clinical features, pathobiology, and treatment advances-Lancet Neurol 2015
  • 69.
    DIAGNOSTIC CRITERIA Rasmussen’s encephalitis:clinical features, pathobiology, and treatment advances-Lancet Neurol 2015
  • 70.
    EEG • Background EEGmay be normal. Subsequently unilateral focal theta- delta range slowing appears , which often becomes synchronized . • INTER –ICTAL Discharges- Multiple independent spike/ sharp – wave discharges are seen in the affected hemisphere • In 30 % the discharges are bilateral. • Activation – EEG abnormalities are enhanced by sleep deprivation as well in sleep Rasmussen’s encephalitis: clinical features, pathobiology, and treatment advances-Lancet Neurol 2015
  • 71.
    Rasmussen’s encephalitis: clinicalfeatures, pathobiology, and treatment advances-Lancet Neurol 2015
  • 72.
    TREATMENT • Steroids andother Immunomodulators are given early in the course of disease to reduce the inflammation , seizure activity and severity of deficit • Refractory seizures – Hemispherectomy • Prognosis below the age of 10 is good who are treated well. Rasmussen’s encephalitis: clinical features, pathobiology, and treatment advances-Lancet Neurol 2015
  • 73.
    Immunomodulatory therapy versussurgery Takahashi Y et al. Immunomodulatory therapy versus surgery for Rasmussen syndrome in early childhood. Brain Dev. 2013
  • 74.
    ALGORITHM < 3 Months<3 months – 1 year Burst Suppression Pattern Partial Seizures/ Erratic Myoclonus Ohtahara Syndrome Early Myoclonic Encephalopathy Infantile Spasm West Syndrome Tonic Spasms
  • 75.
    > 1 YR -AtypicalFebrile seizures -EEG- Normal background > 3 Hz Generalized Discharges -Myoclonic Status - Generalized Spike< Slow wave Background Slowing -Myoclonic Asatic Seizures -Parasagittal Slowing -Fast Generalized Spike/Polyspike Discharges Dravet Syndrome Myoclonic Status in Non Progressive Encephalopathy Doose Syndrome
  • 76.
    -Drop Attacks -GSWD, Multifocal Discharges -ParoxysmalFast Activity -Background Slowing Partial Seizures LGS Auditory Agnosia+/- ESES on EEG ESES on EEG -EPC -Progressive Neurological Deficit LKS CSWS Rasmussen Encephalitis >1 yr
  • 77.
    TAKE HOME MESSAGES •Age of presentation and prior developmental status is most important to diagnose epileptic encephalopathy syndromes • EEG will help to early detection and differentiation of each encephalopathies • Proper treatment helps child to have a seizure free childhood and normal development
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Editor's Notes

  • #41 See NINDS Febrile seizures- Can be whole body, unilateral, and they tend to be prolonged. Switching side to the other side is hallmark of Dravet Syndrome MRI and EEG are normal * After age 1 is the active seizure phase: they are multiple seizure types. These tend to be frequent and prolonged, and resistant to treatment. EEG becomes slow and abnormal with abdundant signs of active epilepsy (spikes) Parellel Lines. Seizure types, Developmental delays