Rasmussen's encephalitis and Behcet's disease: autoimmune disorders in first degree relatives.
We report a patient with adolescent-onset, Rasmussen's encephalitis, presenting with intractable focal seizures, mild hemiparesis, cognitive impairment, dystonia, and severe hemiballism. His father had Behcet's disease, considered to be an autoimmune disorder. Recent reports have directly implicated the role of cytotoxic T lymphocytes in the pathogenesis of both Rasmussen's encephalitis and Behcet's disease. The occurrence of Behcet's disease and Rasmussen's encephalitis in the same family suggests involvement of common genetic factors such as HLA haplotypes in both autoimmune disorders. It is possible that members of this family are genetically susceptible to developing autoimmune conditions that have been precipitated by separate environmental triggers.
Kinay
Demet
D
Bakirkoy State Hospital for Research and Training in Neurology, Neurosurgery, and Psychiatry, Istanbul. demet.kinay@gmail.com
Bebek
Nerses
N
Vanli
Ebru
E
Gurses
Candan
C
Gokyigit
Ayşen
A
Andermann
Frederick
F
D000293:Adolescent; D000927:Anticonvulsants / Q000627:therapeutic use; D001479:Basal Ganglia / Q000473:pathology; D001528:Behcet Syndrome / Q000000981:diagnostic imaging / Q000235:genetics* / Q000473:pathology; D003072:Cognition Disorders / Q000209:etiology; D004351:Drug Resistance; D020820:Dyskinesias / Q000209:etiology; D004421:Dystonia / Q000209:etiology; D004660:Encephalitis / Q000000981:diagnostic imaging / Q000235:genetics* / Q000473:pathology; D019788:Fluorodeoxyglucose F18; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D010291:Paresis / Q000209:etiology; D049268:Positron-Emission Tomography; D019275:Radiopharmaceuticals; D012640:Seizures / Q000188:drug therapy / Q000209:etiology
D000927:Anticonvulsants; D019275:Radiopharmaceuticals; D019788:Fluorodeoxyglucose F18