[Juvenile onset Creutzfeldt-Jakob disease with the history of neurosurgical operation].

ABSTRACT

Juvenile onset Creutzfeldt-Jakob disease with the history of neurosurgical operation a case report A 15-year-old boy gradually developed gait disturbance and dementia. After three months, his condition was deteriorated and he became a state of akinetic mutism. Then, he was transferred to our hospital. He had a history of neurosurgical operation for arterio-venous malformation in his right occipital lobe at the age of five. He showed myoclonus, periodic synchronous discharges in electroencephalogram, and a high cerebrospinal fluid level of neuron-specific enolase. He also revealed progressive brain atrophy by CT examination. He was diagnosed as having Creutzfeldt-Jakob disease (CJD). We could not detect gene mutation of prion protein. As far as we know, this is the youngest case of CJD in Japan. He is too young for the sporadic form of CJD. So the possibility of new variant form of CJD or iatrogenic CJD was considered, the former having been reported in England and the neighboring countries in Europe. However, new variant form of CJD was less likely because of the presence of definite PSD in EEG and the absence of the history of his stay in England. The relationship between the CJD onset and the neurosurgical operation was suggested, but no evidence such as frozen dura matter graft was proved.