Spinal neuroaxonal dystrophy and angioneuromatosis.
A man of 75 years of age had had lightning pains in the legs for 8 years. Clinical examination demonstrated mild pyramidal signs and involvement of the posterior columns. A morphine pump was placed in the epidural space in the lumbar region to treat the pain. He died two weeks later from a massive pulmonary embolism. The nervous system was examined using the classical techniques along with the use of poly- and monoclonal antibodies against nervous system specific proteins. The following features were demonstrated: neuroaxonal dystrophy of the posterior and anterior horns, and the posterior columns and corticospinal pathways in decreasing order of importance; angioneuromatosis of the grey matter of the lumbosacral spinal cord and loss of neurons of the dorsal spinal root ganglia and bilateral degeneration of the fasciculus gracilis. Previously, we have only found such amounts of spheroids in the spinal cord as measured here in cases of Seitelberger's disease. The angioneuromatosis was isolated and did not result from previous trauma nor was it associated with a known phacomatosis. This combination of features is very unusual and may explain the clinical features including the lightning pains.
D000368:Aged; D000798:Angiomatosis / Q000150:complications* / Q000473:pathology / Q000503:physiopathology; D006801:Humans; D008161:Lumbosacral Region; D008297:Male; D009456:Neurofibromatosis 1 / Q000150:complications* / Q000473:pathology / Q000503:physiopathology; D010146:Pain; D013118:Spinal Cord Diseases / Q000150:complications* / Q000473:pathology; D013120:Spinal Cord Neoplasms / Q000150:complications* / Q000473:pathology / Q000503:physiopathology