Abstracts

PURPOSE:
Rasmussen syndrome (RS) is a progressive neurological disorder characterized by intractable focal seizures, hemiparesis, cognitive decline and progressive unihemispheric atrophy on MRI. RS is regarded as an autoimmune disorder.Usually, it concerns children; some cases have been described in adulthood.We reported two new cases, each one with a different clinical course and response to treatment, underlying the likely heterogeneity of etiologic factors of RS .
CASES REPORT:
Patient 1 had her first nocturnal generalized tonic-clonic seizures at the age of 17. Nine years later, multifocal seizures within the left hemisphere occured; on average 10 seizures per year persisted despite treatment. CT was normal. After 30 years of relative stability of the disease an aggravation with several partial motor epilepticus status and then an epilepsia partialis continua arised. At the same time, right hemiparesis and cognitive decline appeared. Progressive atrophy and hypoperfusion of the left hemisphere were observed on MRI and SPECT. CSF examination revealed normal protein and cells; no oligoclonal bands.There were no anti-GluR3 antibodies detected in serum but other auto-antibodies (indeterminate nature) were present. Immunoglobulin(Ig) therapy was performed (7 years after the onset of aggravation) with efficacy: control of seizures and no worsening of neurological deterioration were obtained during two years of follow-up.
RESULTS: Patient 2 had her first left-side motor seizure at the age of 27. Right from the beginning, seizures were intractable and several partial epilepticus status occured. MRI showed right progressive parietal atrophy. Parieto-temporal cortectomy was performed 11 years later. Unfortunatly, pathologic examination was not informative. No seizures occured during 2 months. Then they reappeared and implied several different areas on the perimeter of cortectomy, they became intractable with several partial motor epilepticus status. Cognitive decline and worsening of post-operative hemiparesis progressively developped. Progressive atrophy and hypoperfusion on the perimeter of cortectomy were observed on MRI and SPECT. CSF examination revealed no abnormality. There were no autoantibodies detected in serum. Ig therapy was ineffective. Currently, she continues to worsen (8 years after cortectomy).
CONCLUSIONS: Clinical and MRI presentation of these two cases are suggestive of RS. However clinical course and response to Ig therapy are quite different. In the first case, RS seems to occur in a second time after a long period (30 years) of the evolution of a multifocal regional epilepsy, Ig therapy is effective; this suggests the appearance of a secondary immunologic disorder. In the second one, RS evolves in one piece (exepted transitory improvement after cortectomy) with a progressive and inexorable worsening during a period of 20 years without any arguments in favor of an immunological disorder. RS is a clinical and radiological syndrome which probably has various etiologic factors.
[Supported by: We received no funding for this study]