[Amnesia and stiff-man syndrome. Manifestations disclosing paraneoplastic encephalomyelitis]

The authors report a case of hypertonia, the clinical and electrophysiological features of which were identical to those of the Stiff-man syndrome first described by Moersch and Woltman in 1956. This and the five other previously reported cases can be attributed to encephalomyelitis of the anterior horns of the spinal cord. All of these cases had severe but partial involvement of the anterior horns and the probable physiopathological mechanism of the resulting contractures was elective destruction of the small inhibitory neurones. In the present case, the association of a limbic encephalitis and secondary exteriorization of a carcinoma of the pharynx, strongly suggested a paraneoplasic encephalomyelitis. This is the first report indicating an association between the Stiff man syndrome and a paraneoplastic syndrome. Another point of interest was the spectacular response of the hypertonia to steroid therapy suggesting a central neuropharmacological effect quite apart from the non specific anti-inflammatory action on the encephalomyelitis.