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Atypical Form of Amyotrophic Lateral Sclerosis JNNP 66:581-585, Sasaki,S.&Iwata,M., 1999 See this aricle in Pubmed Article Abstract All eight patients basically showed a similar distribution of muscular weakness and atrophy. Subluxation of the shoulder joints was found in all patients. Reflexes were absent in the upper limbs in all patients, but were almost normal in th e face and legs in most patients. Pathological reflexes could be elicited in only one patient. Electromyography showed typical neurogenic changes in the limbs of all patients. Cervical MRI disclosed moderate spondylotic changes in seven patients. Anti ganglioside antibodies were negative in six patients tested. Abnormal trinucleotide (CAG) repeat expansion of androgen receptor gene was not recognised in five patients examined. Bulbar involvement developed in three patients during the course of the di sease. At necropsy, one patient showed degeneration of the pyramidal tracts and motor cortex including Betz cells as well as loss of spinal anterior horn cells and brainstem motor neurons, which is consistent with ALS; in another patient there was neuron al loss of anterior horn cells at the spinal cord accompanied by astrolgliosis, whereas the motor cortex and brainstem motor nuclei were relatively well preserved. Intracytoplasmic inclusions such as Bunina bodies, skeinlike inclusions, and Lewy body-lik e inclusions were found in both patients. These patients with their peculiar pattern of muscular atrophy seem to have ALS or a subtype of ALS>

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(click to filter results - removes previous filter) amyotrophic lateral sclerosis amyotrophic lateral sclerosis,atypical motor neuron disease proximal muscle atrophy shoulder,subluxation

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