[Acute encephalitis/encephalopathy associated with transient involuntary movement and limbic dysfunction in the recovery phase].
We report 3 patients aged 3 - 4 years who presented with impaired consciousness and status epilepticus or a cluster of seizures, followed by transient involuntary movements and limbic dysfunction in the recovery phase. In all the patients, the involuntary movements were observed mainly on the left side and consisted of dystonia, athetosis, chorea, facial myoclonus, and oral dyskinesia. The patients also showed stereotypic movement, oral tendency, visual agnosia, and emotional disturbance, which suggested limbic dysfunction resembling Klüver-Bucy syndrome. Single-photon emission computed tomography (SPECT) revealed hypoperfusion of the unilateral basal ganglia and the adjacent frontal and temporal lobes. No obvious lesions were observed on brain MRI in the acute phase in 2 patients, who recovered completely during the follow-up period. However, the last patient, who had abnormalities with regard to the limbic system and subcortical white matter on diffusion-weighted image in the acute phase, exhibited mental retardation, epilepsy, and persistent oral tendency during the follow-up period.
Maruyama
Koichi
K
Department of Pediatric Neurology, Central Hospital, Aichi Welfare Center for Persons with Developmental Disabilities, Kasugai, Aichi. maruyama@aichi-colony.jp
Itomi
Seiko
S
Sofue
Ayako
A
Natsume
Jun
J
D000208:Acute Disease; D002675:Child, Preschool; D020820:Dyskinesias / Q000209:etiology*; D004660:Encephalitis / Q000150:complications*; D005260:Female; D006801:Humans; D020232:Kluver-Bucy Syndrome / Q000209:etiology*; D008032:Limbic System / Q000473:pathology; D008279:Magnetic Resonance Imaging; D008297:Male; D011379:Prognosis; D013226:Status Epilepticus / Q000209:etiology