Characteristic clinical features in a case of fulminant subacute sclerosing panencephalitis.
A 12-year-old girl with fulminant subacute sclerosing panencephalitis (SSPE) without myoclonic jerk and periodic EEG complexes is reported. She presented with blurred vision, fell into a coma after 2 weeks and died within 4 months. Magnetic resonance image (MRI) disclosed high intensity lesions in the lateral geniculate bodies, when the patient initially complained of blurred vision. Reviewing the cases of fulminant SSPE in the literature, more than half of the patients showed blurred vision or visual agnosia as an initial symptom. Most of those patients were female and rapidly deteriorated to a comatose state within 2 weeks. Based on the MRI study of the present patient, we speculate that the initial visual impairment in such patients can be attributed to the involvement of the lateral geniculate body.
Takayama
S
S
Division of Child Neurology, National Center Hospital for Mental, Nervous and Muscular Disorders, Kodaira, Japan.
Iwasaki
Y
Y
Yamanouchi
H
H
Sugai
K
K
Takashima
S
S
Iwasaki
A
A
D002648:Child; D004569:Electroencephalography; D005260:Female; D005829:Geniculate Bodies / Q000473:pathology; D006801:Humans; D008279:Magnetic Resonance Imaging; D013344:Subacute Sclerosing Panencephalitis / Q000473:pathology / Q000503:physiopathology*; D014786:Vision Disorders / Q000503:physiopathology