Epileptic encephalopathies

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  1. 1. Dr. Rahi kiran.B Senior resident Dept. of Neurology GMC Kota
  2. 2. 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 behavioral, cognitive and motor regression. (1) electrographic EEG paroxysmal activity that is often aggressive, (2) seizures that are usually multi-form and intractable, (3) cognitive, behavioral, and neurological deficits that may be relentless, (4) sometimes early death.
  3. 3. Not fully understood, not same for all Aggressive ictal(seizure) and electrical(EEG) epileptogenic activity in developing brain leading to excessive excitability which varies acc to age and stage of brain maturity
  4. 4. In neonates burst-suppression Infancy hypsarrythmia Child GSWD As age advances, these also change
  5. 5. Epileptic Encephalopathy Syndromes in neonates
  6. 6. Onset < age 3 m Male = female Etiology - Inborn errors of metabolism - mc-nonketotic hyperglycinemia, methylmalonic acidemia.
  7. 7. Triad of intractable seizures Erratic myoclonus f/b simple focal seizures f/b Tonic seizures Erratic myoclonus - shift typically from one part of the body to another in a random, asynchronous fashion. simple focal seizures Tonic seizures - a diffuse tonic contraction, usually extending to the extremities. Psychomotor devt abnormal from onset
  8. 8. Interictal EEG - suppressionburst activity, evolves into atypical hypsarrhythmia after 3 to 5 months of life, accentuated during sleep Myoclonus no EEG expression, follows burst CT and MR - related to etiology, grossly normal or have asymmetrical enlargement of one hemisphere, dilatation of the corresponding lateral ventricle, or cortical atrophy the serum levels of amino acids should be determined, especially glycine and glycerol
  9. 9. The inter-ictal EEG of early myoclonic encephalopathy is a repetitive suppressionburst pattern without physiological rhythms
  10. 10. The prognosis is poor - >50% die within weeks or months. no effective therapy pyridoxine trial can be tried
  11. 11. ILAE Definition Very early onset, within the first few months of life, frequent tonic spasms and a suppressionburst EEG pattern in both the waking and sleeping states. Onset - 10 days to 3 months of age (occ. Intrauterine). Etiology mc malformations, no familial cases (hemimegaloencephaly, porencephaly, Aicardi syn, focal cortical dysplasia)
  12. 12. Tonic spasms - forward tonic flexion lasting 110 s that is singular or in long clusters 10300 times every 24 h. Alternating hemiconvulsions or GTCS are exceptional. Myoclonic seizures are rare.
  13. 13. Burst suppression with pseudorhythmic periodicity, disappears in 6 months - Most survivors evolve to West syndrome with hypsarrhythmia, or later to LGS.
  14. 14. prognosis is poor with severe psychomotor retardation Psychomotor development may be slightly better if the infants do not develop West or Lennox-Gastaut syndrome. Half of the children are likely to die in infancy or childhood
  15. 15. No effective treatment vigabatrin and zonisamide some benefit ACTH not useful invasive surgery, such as a partial resection or complete hemispherectomy.
  16. 16. Epileptic Encephalopathy Syndromes in Infancy
  17. 17. frequent partial seizures of multifocal onset, with autonomic or motor involvement The interictal EEG reveals multifocal epileptiform activity and slowing, diffuse slowing of the background activity. asymmetry between different recordings The ictal EEG confirms multifocal onsets, No burst-suppression difficult to control with standard AEDs. Bromides, stiripentol, and clonazepam may be helpful
  18. 18. Triad of infantile spasms, arrest of psychomotor development, and hypsarrhythmia. Onset -age of 3 and 12 months. Male(60%) > female Etiology symptomatic(80%), idiopathic cerebral malformations, infection, hemorrhage, hypoxicischemic injury, metabolic disorders, and genetic conditions,such as Down syndrome, TS
  19. 19. Epileptic spasms - Clusters of sudden, brief (0.22 s), bilateral tonic contractions of the axial and limb muscles involving extension and/or flexion Occurs in clusters 1-30 / day, each with 20 150 attacks Precipitating factors- twilight state,sudden loud noises or tactile stimulation,feeding. Spasms flexor extensor mc Flexor salaam spasms Extensor like moro reflex Psychomotor delay from the onset
  20. 20. interictal -Gibbs and Gibbs hypsarrhythmic EEG.(2/3 rds) Disorganized pattern with asynchronous, very high amplitude slowing and frequent multifocal spike and sharp wave discharges Sleep REM normal NREM more synchronous By 2-4yrs - LGS
  21. 21. Ictal EEG mc high voltage, gen. slow wave episodic,low amplitude slow wave diffuse attenuation (electrodecremental ictal EEG pattern).
  22. 22. Brief flexor spasm associated with high voltage slow wave discharge
  23. 23. Good if treated early Mortality - 5%. 60% - develop other seizure types LGS 50%-permanent motor disabilities 2/3rd -severe cognitive and psychological impairment. 512% - normal mental and motor development.
  24. 24. First line ACTH and vigabatrin (TS) No role of pyridoxine valproate, levetiracetam, topiramate, zonisamide, lamotrigine, and benzodiazepines ketogenic diet is helpful in most cases Hemispherectomy - medically intractable No specific AED has been shown to affect long-term developmental outcome
  25. 25. Onset day 1 to 5yrs Female : male 2: 1 Etiology 50 % - a/w angelman syn, 20% - birth asphyxia c/b fixed, non-progressive encephalopathy long-lasting recurrent episodic erratic myoclonic status epilepticus with atypical absence seizures.
  26. 26. interictal EEG-multifocal epileptiform discharges and background slowing Ictal EEG - continuous generalized slow spike and wave, or an absence pattern Myoclonic status improves but with residual disability Treatment myoclonic status BZDs Others - valproate with ethosuximide or clobazam
  27. 27. Onset - always female Triad- Polymorphic intractable seizure that are mainly tonic (80%), atonic (50%)and atypical absence seizures (70%), Myoclonic Jerks(1128%) sleep & awake Cognitive and behavioural abnormalities. EEG with paroxysms of fast activity and slow (less than 2.5 Hz) generalised spike- wave discharges (GSWD).
  28. 33. symptomatic - 7078% of patients with LGS encephalitis and/or meningitis, tuberous sclerosis, brain malformations (e.g., cortical dysplasias), Idiopathic normal psychomotor development occurred prior to the onset of symptoms, no underlying disorders or definite presumptive causes are present, no neurologic or neuroradiologic abnormalities are found. birth injury hypoxia-ischemia injury, frontal lobe lesions trauma.
  29. 34. ILAE inclusion criteria Atleast 2 seizure types tonic, atonic, atypical absence(mandatory) EEG GSWD with episodic fast activity Impaired intellectual functioning Not imp. onset age, imaging, etiology
  30. 35. Tonic seizures 80 100% mc & most characteristic, Symmetrical, brief, awake & NREM, not in REM Atonic 50% Atypical absence 2/3rd Myoclonic jerks 11-28% Drop attacks -
  31. 36. Inter-ictal EEG abnormal BG fragmented alpha, excess slow waves Ictal EEG a) tonic paroxysmal fast activity b) Atonic gen. polyspikes, slow GSWD c) Atypical absence - 50 % NREM > 85 % - poor prognosis < 85% - better performance
  32. 53. I stage is before the discoveryof CSWS The EEG shows multi-focal spikes and bisynchronous generalised sharp or spike-wavedischarges. First seizure is usually nocturnal II stage is when CSWS isfound Increase in seizures and the appearance or deterioration of neuropsychological symptoms (frontal& temporal)that prompt a sleep EEG. Tonic seizures never occur Continuous bilateral and diffuse slowspikes-waves of 1.52 Hz during NREM sleep is the defining EEG patternof ECSWS. III stage is after clinical and EEG remission starts 2-7yrs from onset, seizures remit in all pts, EEG normalises, left with residual deficits
  33. 54. Seizures remit in all by 10 15yrs age irrespective of etiology Good prognosis Cognition & behaviour incomplete recovery Early treatment - better
  34. 55. may or may not respond - benzodiazepines, valproate, ethosuximide, carbamazepine, phenytoin. Only benzodiazepines and adrenocorticotrophic hormone have been reported to suppress the electrical Status TOC - benzodiazepines + valproate long-lasting improvement of the language function - multiple subpial transections of the region of focal epileptic discharges
  35. 56. LKS CSWS Seizures 3/4th All Language Verbal auditory agnosia Expressive Bahavioral Common All CSWS onEEG 80% 100% Spike localisation Temporal Frontal Normal life 50% 25%
  36. 57. Other Severe Epileptic Encephalopathies
  37. 58. Both sexes are equally affected Etiology- chronic viral infection, immune response to infection,immune-mediated causes.
  38. 59. typically shows 1. Focal motor seizures or epilepsia partialis continua (EPC) 2. Generalized seizures contralateral to the ictal and interictal epileptiform activity 3. Unilateral hemispheric cortical brain atrophy on MRI (abnormal signal T2/FLAIR and atrophy or hyperintense signal of the ipsilateral caudate head)
  39. 60. MRI - to exclude other causes of focal seizures EEG - response to treatment is very poor
  40. 61. A previously normal child, aged 215 years, within a few days of developing a FOU, develops an acute prolonged perisylvian refractory convulsive status epilepticus, persisting for more than 1 month