Rinsho Shinkeigaku (Clinical Neurology)

Case Report

Pilocytic astrocytoma and diencephalic syndrome in an adult with neurofibromatosis type 1

Ichiro Kohira, M.D.1), Yoshikazu Yoshimura, M.D.2), Yotaro Ninomiya, M.D.1), Komei Ikeda, M.D.2) and Jun Iwata, M.D.3)

1)Department of Neurology, 2)Neurosurgery, and 3)Pathology, Kochi Municipal Central Hospital

We report a rare case of pilocytic astrocytoma and diencephalic syndrome occurring together in a 30-year-old woman with neurofibromatosis type 1 (NF-1). Diencephalic syndrome included emaciation and somnolence. Gadolinium-enhanced magnetic resonance imaging revealed a brain tumor in the optic chiasmal-hypothalamic region, which invaded the thalamus, brain stem and cerebellum. A biopsy specimen from the chiasmal tumor was compatible with pilocytic astrocytoma. Although she received radiation therapy, she died 22 months after the onset of the disease.

(CLINICA NEUROL, 43: 327|329, 2003)
key words: neurofibromatosis type 1, von Recklinghausen's disease, pilocytic astrocytoma, diencephalic syndrome, adult

(Received: 13-Nov-02)