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Autoimmune Epilepsies. Azhar Daoud , MD, FRCP Professor and Consultant in Child Neurology, Specialty Hospital, Amman, Jordan. Anticonvulsant activity of steroids Fever and infection exacerbate seizures Seizures frequency in autoimmune diseases

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autoimmune epilepsies

Autoimmune Epilepsies

AzharDaoud, MD, FRCP

Professor and Consultant in Child Neurology, Specialty Hospital,

Amman, Jordan

indications for the role of immunity in epilepsies

Anticonvulsant activity of steroids

  • Fever and infection exacerbate seizures
  • Seizures frequency in autoimmune diseases
  • Detection of autoantibodies in certain epilepsies
  • Seizures aggravated by vaccinations
Indications for the role of immunityin epilepsies
autoimmune epilepsies1

ACTH and steroid in infantile spasm

  • Inflammation appear to have a central mechanism in certain experimental modules of epilepsy.
  • Inflammation produce cascade of molecules against exogenous pathogen
  • Autoantibodies also have a role in inflammation, hashimotos encephalopathy
Autoimmune epilepsies
possible aetiology

Antibodies directed against molecules on the neural surface, e.g. N-methyl-D-aspertate receptor (NMDA-R) or the voltage gated potassium channel(VGKC) complex

  • Underlying mechanism of Rasmussen syndrome is chronic and progressive inflammation
  • Acute inflammation and cytokine cascade activation is important in status epileptics
Possible aetiology
examples of autoimmune epilepsies

Dravet syndrome where fever trigger long duration of seizures

  • In some children with sever epilepsies, fever induces periods of seizures freedom
  • Idiopathic hemiconvulsion-hemiplegia syndrome (IHHS) and FIRES have clear relation to infection.
Examples of autoimmune epilepsies
epilepsies caused by autoantibodies related conditions

Limbic encephalopathy and temporal lobe seizures (subacute onset of sever memory impairment and mood disturbance with recurrent temporal lobe seizures)

  • Onconeural antibodies directed against the intracelullar protein Hu,Ma ½,amphiphysin,or CV2 and can be associated with tumors
  • Antibodies against VGKC
Epilepsies caused by autoantibodies related conditions
limbic encephalopathies

Some time associated or preceded by fasiobrachial dystonic seizures

  • MRI, medial temporal changes
  • Elevated serum VKGC antibody titer confirm the diagnosis
  • Respond to intravenous immunoglobulin or corticosteroids
Limbic Encephalopathies
encephalopathy with nmdar antibodies

Acute psychiatric disorders(delirium, visual or auditory delusions, aggression and irritability) and epileptic seizures, usually of extratemporal origin, initial phase followed by

  • Sever phase choreoathetoid movement disorders, dysautonomia and impaired consciousness
  • Autoantibodies against NMDARs
Encephalopathy with NMDAR antibodies
epileptic encephalopathy

Recent data show up to 45% associated with tumors

  • Brain MRI are normal in 50%
  • CSF show inflammation in 90% of case with or without oligoclonal bands
  • EEG abnormal in 90% of case
  • Immunotherapy is effective in 50%
Epileptic Encephalopathy
epilepsies with inflammation chronic inflammation

Rasmussen’s encephalitis, hemispheric brain inflammation leading to unilateral brain atrophy, viral infections

  • Pharmacoresistant focal seizures, progressive unilateral motor deficit and cognitive decline
  • Cell mediated immunity of cytotoxic T cells
  • Immunosuppressive before surgery
Epilepsies with inflammationChronic inflammation
epilepsies with possible acute inflammation

IHHS, febrile seizures followed by hemiplesia started as flaccid before becoming progressively spastic

  • First 2 year of life
  • In preexisting brain disorder, SW, TS, corpus callosum agenesis but some time in normal healthy children
  • CSF normal nor oligoclonal banding
Epilepsies with possible Acute inflammation
slide13

Fever trigger status epileptics, clonic jerks or head and eye deviation, predominant in one side and last for several hours

  • EEG high amplitude 2-3Hz rhythmic slow wave activity contra lateral to hemiclonic jerks.
  • ¾ of them have persistent epilepsy
  • MRI, unilateral edematous swelling followed by hemiatrophy
IHHS
conclusion

The finding of inflammatory markers and especially autoantibodies in several epileptic disorders of unknown etiology has open up a group of possible causes and investigations in epilepsies.

  • New immunosuppressive and immunomodulatoey treatment for certain epilepsies
Conclusion
concern

This finding raise many questions

  • How to classify these disorders
  • The relationship of antibodies and specific clinical phenotypes
  • The mechanism of generation of these autoantibodies
  • Optimum treatment strategies
  • Ketogenic diet efficacy
Concern