Rinsho Shinkeigaku (Clinical Neurology)

Brief Clinical Note

Callosal disconnection syndrome due to acute disseminated enchephalomyelitis

Yuji Tanaka, M.D.1), Hiroshi Nishida, M.D.2), Rumiko Hayashi, M.D.3), Takashi Inuzuka, M.D.1) and Mika Otsuki, M.D.4)

1)Department of Neurology and Geriatrics, Division of Neuroscience, Graduate School of Medicine, Gifu University
2)Department of Neurology, Gifu Prefectural Gifu Hospital
3)Division of Speech Therapy, Gifu Prefectural Gifu Hospital
4)School of Psychological Science, Health Sciences University of Hokkaido

We have reported a very rare case of a patient with callosal disconnection syndrome due to acute disseminated enchephalomyelitis (ADEM). A 54-year-old right-handed woman developed sudden consciousness disturbance and fever after 2 weeks when she had common cold. She did not have a history of stuttering. On admission, the neurological finding showed consciousness disturbance, and exaggerated muscle stretch reflex in four extremities without meningeal irritation. The cerebrospinal fluid examination revealed the cell counts of 273/mm3, the protein of 348 mg/dl and the myelin basic protein 18.3 pg/ml. The brain MRI demonstrated a focal high signal intensity in the right cerebellar peduncle, right temporal lobe, left occipital lobe and corpus callosum on the T2-weighted and FLAIR images. The lesion of corpus callosum was the trunk and splenium, sparing the dorsal side of splenium. Her diagnosis was ADEM. The steroid pulse therapy (metylpredinisolone 1 g/day for 3days) improved the consciousness disturbance. After 6 weeks from the onset, the neuropsycological examination was performed. The remarkable findings included pure alexia without color naming defects, the acquired stuttering and one-way disturbance of somesthetic transfer from the left hand to the right. This patient may contribute to the study of functional localization of the corpus callosum.

(CLINICA NEUROL, 46: 50|54, 2006)
key words: acute disseminated encephalomyelitis, callosal apraxia, callosal disconnection syndrome, pure alexia, stuttering

(Received: 20-Oct-04)