Amyotrophy in prion diseases.
نویسندگان
چکیده
Amyotrophic lateral sclerosis was once thought to be caused by persistent viral infection, partly because some patients with transmissible Creutzfeldt-Jakob disease showed prominent amyotrophy. However, in the past 15 years there has been little interest in the amyotrophy in prion diseases, and the possible link to amyotrophic lateral sclerosis has been eschewed. We analyzed case reports of prion disease published after 1968 for evidence of amyotrophy. We defined amyotrophy as clinically evident fasciculation buttressed by electromyographic results in some cases. We sought evidence of motor neuron degeneration at autopsy. Prion disease was proved by transmissibility, immunohistochemistry demonstration of protease-resistant prion protein, or finding a mutation in the prion protein gene. Amyotrophy was noted in 27 patients: 13 with sporadic Creutzfeldt-Jakob disease, 2 with familial Creutzfeldt-Jakob disease, and 12 with Gerstmann-Sträussler-Scheinker disease. Of the 27, 23 showed clinical fasciculation and 10 had electromyographic evidence of denervation. The spinal cord was examined in 8 patients: 6 showed loss of motor neurons, 1 showed vacuolation of motor neurons, and 1 reported no abnormalities. Another 23 patients had typical histopathological characteristics but lacked molecular or biochemical proof of prion disease. The total number of patients with amyotrophy and proven prion disease that we identified was 50. This case review supports the belief that amyotrophy is occasionally a prominent feature of Creutzfeldt-Jakob disease and underscores the importance of documenting lower motor neuron function and the crucial role of examining the spinal cord at autopsy in cases of prion disease.
منابع مشابه
A patient with Creutzfeldt-Jakob disease presenting with amyotrophy: a case report
INTRODUCTION Creutzfeldt-Jakob disease (CJD) is an ultimately fatal, neurodegenerative disease caused by misfolded prion protein aggregation and accumulation. The development of amyotrophic features has been described in CJD, though rarely as an early or prominent feature. Consequently, the significance of amyotrophy in prion disease etiology remains unclear. CASE PRESENTATION Our patient, a ...
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OBJECTIVE To report the clinical and neuropathological features in a patient with Creutzfeldt-Jakob disease with amyotrophy and demyelinating polyneuropathy. DESIGN Case report. PATIENT AND RESULTS A 62-year-old man had progressive numbness of the left foot, unsteady gait, diminished deep reflexes, fasciculations, and tactile hypesthesia on the feet. Cerebrospinal fluid, electroneurography,...
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Combined Scottish Study (1961). Poliomyelitis-like disease in 1959. British Medical Journal, 2, 597-605. Danta, G. (1975). Electrophysiological study of amyotrophy associated with acute asthma (asthmatic amyotrophy). Journal of Neurology, Neurosurgery and Psychiatry, 38, 1016-1021. Gathier, J. C., and Bruyn, G. W. (1960). Neuralgic amyotrophy. Handbook of Clinical Neurology, Vol. 8, pp. 77-85. ...
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ورودعنوان ژورنال:
- Archives of neurology
دوره 57 1 شماره
صفحات -
تاریخ انتشار 2000