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TWO CASES OF ATYPICAL SPINAL PROGRESSIVE MUSCULAR ATROPHY (Lumbosacral Leg‐Thigh Type) A Clinicopathological Study with the Survey Of the Relevant Literature
Author(s) -
Okumura Nikichi,
Terao Akira
Publication year - 1965
Publication title -
psychiatry and clinical neurosciences
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.609
H-Index - 74
eISSN - 1440-1819
pISSN - 1323-1316
DOI - 10.1111/j.1440-1819.1965.tb00039.x
Subject(s) - medicine , lumbosacral joint , spinal muscular atrophy , thigh , pathological , anterior horn cell , atrophy , anatomy , anterior compartment of thigh , pathology , amyotrophic lateral sclerosis , disease
S ummary The case histories and anatomic alterations of two patients with the lumbosacral leg‐thigh type of spinal progressive muscular atrophy are presented. The course of the diseese was very rapid, and the patients died of bulbar palsy in 7 months (first case) and 18 months (second case), respectively. Cutaneous sensation was normal and pyramidal signs were not elicited in both cases. Electromyogram and biopsy specimens showed characteristic changes of lower motor neuron damage. Pathological examination revealed degeneration of the anterior horn cells with marked decrease in their numbers and demyelination of anterior roots. The giant pyramydal cells of Betz and pyramydal pathways were, nevertheless, intact. The second case generally demonstrated myasthenic syndrome clinically, and dystrophic changes of muscle fibers on pathological studies. There have been three clinical pictures simulating the lumbosacral leg‐thigh type of spinal progressive muscular atrophy. They are all atypical amyotrophic lateral sclerosis; the leg‐thigh form without spastic phenomena (Kahler, 1884), the pseudo‐polyneuritic form (Marie et Patrikios, 1918), and the peroneal form (Hemmer, 1951). Their characteristic findings were compared clinicopathologically with our two cases.