Re: Anti-NMDA receptor encephalitis and other emerging autoimmune brain diseases
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http://rms.medhyg.ch/article_p.php?ID_ARTICLE=RMS_247_0925
Margitta Seeck, André Zacharia, Andrea O. Rossetti
Epilepsies d’origine auto-immune
Rev Med Suisse 2010;6:925-929
The diagnosis of drug-resistant epilepsy of autoimmune origin are becoming more frequent. It is reported more and more of limbic encephalopathy paraneoplastic origin not from the five to ten years.
Three antibodies are now described: antibody anticanaux dependent potassium voltage (voltage-gated potassium channels (VGKC)), the antacid glutamic decarboxylase (GAD) and antirécepteur N-methyl-D-aspartate (NMDA). We review the clinical syndromes in association with the imaging findings and the laboratory. Note that most reported cases involve the adult population, although some cases are also seen in adolescents and children
. Early recognition of such a syndrome is very important as an early immunomodulatory therapy appears to favorably modify the clinical course.
One third of patients with epilepsy will develop into a drug resistance (that is: a lack of adequate clinical response after the trial of two or more antiépilepeptiques). There are several reasons for this. Surgical treatment can be proposed that some of these patients. The origins of autoimmune increasingly recognized since the last five to ten years and should not be missed, since their detection may potentially lead to effective treatment.
The limbic encephalitis is typically associated with temporal lobe seizures and is the most common cause of late-onset temporal lobe seizures (after twenty years) .1
Conclusion
There are more and more cases of epilepsy known as autoimmune although their study is still in its infancy. Most studies including a large group of patients have been published over the past five years and are mainly adults but also children.
Given the large number and rate of discovery of different antibodies
, we expect the fact that more and more patients are diagnosed with epilepsy of autoimmune origin, in the coming years. Other autoantibodies are still discovered. For example, anti-AMPA were found in ten patients with limbic encephalitis (nine women, including seven with a tumor) .24 At the same time, new treatments have become available immunomodulators in the last decade as rituximab.
Diagnostic tools show significant progress but it still lacks major systematic studies of different immunological treatments.
The evidence is increasingly clear that a good symptomatic recovery is related to early treatment. This is all the more important it may be young or very young patients.
Practical implications
> An autoimmune balance should be considered for epilepsy late, severe epileptic encephalopathy or to the presence of other neurological symptoms such as memory impairment, ataxia, rigidity, involuntary movements, psychiatric disorders, coma and rarely as status epilepticus
> There are painting and not paraneoplastic paraneoplastic
> The assessment includes a review of neuropsychological and neurological, EEG, MRI, lumbar puncture (LP), antibody
> A normal PL does not exclude the presence of an array of encephalopathy with autoimmune
> The treatment is based on an antiepileptic and immunomodulatory therapy
> A report in a specialized center is recommended
