We reported a case of
opsoclonus-myoclonus syndrome. A 63-year-old man was admitted to Kenwakai Hospital with rapidly progressing symptoms, including
lumbago, whole body
pain,
vertigo,
nausea, and
anorexia. He became bed-ridden because of severe
vertigo and
truncal ataxia. Five days after admission, he developed
opsoclonus followed by
myoclonus and mild disturbance of consciousness, but he showed no
appendicular ataxia or pyramidal tract sign. He was treated with
prednisolone, 40 mg/day, which was effective for disturbance of consciousness, but
opsoclonus and
myoclonus persisted. He died of
liver dysfunction and
ventricular fibrillation 3 weeks after onset. Blood examination revealed high LDH (1,106 IU/l), Al-P, and gamma-
GTP titers.
Tumor markers were normal except for increase NSE activity (129 ng/ml). The cerebrospinal fluid showed normal cell count, 63.9 mg/dl of
protein, 7.3 mg/dl of
IgG, and normal
glucose. A cranial CT scan showed an old lacune only. Chest rentgenogram and CT scan revealed mediastinal and hilar lymph node enlargement. An abdominal CT scan showed multiple low density masses in the liver.
Small cell lung cancer associated with
opsoclonus-myoclonus syndrome was suspected. Western blot analysis revealed that his serum reacted with
protein in the cerebellum, cerebrum, and dorsal root ganglion with a molecular weight of 77 kDa. This is the first time such an antibody was ever been detected in patients with
opsoclonus-myoclonus syndrome. The molecular weights of the
antigens previously found by the serum of patients with this syndrome, were 55 kDa and 80 kDa in patients with
breast cancer, and 210 kDa in patients with
neuroblastoma.(ABSTRACT TRUNCATED AT 250 WORDS)