We reported clinical and neuropathological observations of a 41-year-old man with
Degos disease. He first noted painless skin lesions over the upper extremities in January, 1982. Three years later he was diagnosed as
Degos disease by skin biopsy, and treatment with
aspirin was started. In September, 1985, he complained of
paresthesia on his right arm, followed by a series of new
neurological manifestations suggesting multifocal spinal cord lesions. On October 28, examination of admission showed papules with central umblication over the whole body except the head, face, palms, soles and scrotum. Neurological examination revealed no weakness, diminished right biceps reflex, exaggerated patellar reflexes and Achilles reflexes, left extensor plantar reflex,
hypesthesia and hypalgesia to the level of Th8, mild left
spastic gait, and retention of urine. In November, he had
paraparesis, loss of vibration sense of lower extremities,
hypesthesia and hypalgesia to the level of TH4, and weakness of right upper extremity. In December, he showed
tetraplegia, left-sided
facial palsy, and
hypesthesia and hypalgesia to the level of C5. In January, 1986, he showed right
facial palsy, left facial
hypesthesia,
pseudobulbar palsy. In February, he had bilateral
abducens nerve palsy and
hiccups. On February 18, he died of
intracranial hemorrhages. He had episodic
abdominal pain several times during admission. His condition deteriorated progressively in four months after the first manifestation of neurological symptoms, despite the
therapy with
heparin,
urokinase,
ticlopidine,
dipyridamole, and
prednisolone. Laboratory studies showed gradual increase of CSF
proteins (from 156 mg/dl to 602 mg/dl) and extremely increased platelet aggregation.(ABSTRACT TRUNCATED AT 250 WORDS)