Marfan's syndrome, recurrent complex partial status epilepticus and myoclonus: a case report.
A 26 year-old mentally retarded woman suffering from Marfan's syndrome and epileptic seizures presented weekly recurrent complex partial status epilepticus for two years, and myoclonus at rest. A marked increase of the myoclonic jerks and transient signs of cerebellar dysfunction followed each occurrence of the status epilepticus. Myoclonus could be interpreted as: 1) a permanent neurologic consequence of primary CNS disorders or secondary to disorders of the connective tissue, 2) a persistent post-ictal sequela sustained by recurrence of complex partial status, or 3) an additional consequence of both brain disorders due to Marfan's syndrome and recurrence of complex partial status epilepticus. The case report raises the possibility that recurrence of complex partial status for a long period may be a risk factor of permanent neurologic deficit, especially in already brain damaged patients.
Ambrosetto
G
G
Institute of Neurology, University of Bologna, Italy.
Tinuper
P
P
Tassinari
C A
CA
D000328:Adult; D004569:Electroencephalography; D005260:Female; D006801:Humans; D008382:Marfan Syndrome / Q000150:complications*; D009207:Myoclonus / Q000150:complications*; D012008:Recurrence; D013226:Status Epilepticus / Q000150:complications* / Q000175:diagnosis