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Progressive neurodegenerative disease in presumed spinal cord injury:
Case report of a patient with prion disease.
BACKGROUND: Prion diseases or transmissible spongiform encephalopathies (TSEs) are neurodegenerative syndromes caused by proteinaceous infectious particles (or "prions"), are fatally progressive, and affect humans and animals. Human prion disease may be familial, sporadic, or due to iatrogenic causes. The signs and symptoms include dementia, ataxia, myoclonus, dysautonomia, pyramidal and extrapyramidal tract signs, and akinesia. The incubation period of iatrogenic TSE ranges from 15 months to 30 years, and clinical presentations may be atypical. DESIGN: Case report. FINDINGS: This article presents the case study of a 39-year-old man who fell at work and subsequently complained of subjective lower extremity weakness, followed by onset of ataxia, bowel and bladder incontinence, and progressive decline in ambulation over 6 months. In the absence of a unifying diagnosis, the patient was presumed to have had a spinal cord injury (SCI). Because neuro-axis imaging studies failed to explain his symptoms, the patient's complaints were thought to have a large psychologic component. The patient then developed neurologic abnormalities proximal to the presumed SCI. Somatosensory evoked potentials were suggestive of a thoracic or lumbar cord myelopathy and cerebrospinal fluid analysis was suggestive of prion disease. Family members eventually revealed that the patient had had injections of growth hormones derived from cadaveric human pituitary glands as a child. Postmortem brain examination later revealed definitive Creutzfeldt-Jakob disease.
J Spinal Cord Med. 2003 Fall;26(3):259-61. Grant D, Loomis S. Department of Rehabilitation Medicine, University of Washington, Seattle, Washington, USA. PMID: 14997969