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Familial spastic paraparesis syndrome associated with HTLV-1 infection

Article Abstract:

Human T-cell lymphotropic virus Type I (HTLV-I) has been identified as the agent that causes adult T-cell leukemia, a disorder in which abnormal T cells (a type of white blood cell) proliferate extensively. It is possible that HTLV-1 also causes chronic progressive myelopathies (diseases of the spinal cord), a group of disorders that includes tropical spastic paraparesis, partial paralysis of the lower limbs. An association between familial spastic paraparesis (hereditary spastic paraplegia) and infection with HTLV-I is described in members of three generations of a family from Paraguay. The case studies of the proband (the initial patient), the proband's father, grandmother, and other relatives are provided. The symptoms consisted of progressive weakness and spasticity (involuntary contractions) of the legs, moderate loss of sensation, and loss of sphincter control. Since antibodies against HTLV-I were found in affected family members, it seemed likely that the viral agent was the cause of the familial spastic paraparesis syndrome. HTLV-I is probably transmitted by sexual, perinatal, and parenteral (other than digestive tract) routes. Also, for three family members, transmission through breast milk seemed likely. (Consumer Summary produced by Reliance Medical Information, Inc.)

Author: Roos, Raymond P., Salazar-Grueso, Edgar F., Holzer, Timothy J., Gutierrez, Robin A., Casey, James M., Desai, S.M., Devare, Sushil G., Dawson, George
Publisher: Massachusetts Medical Society
Publication Name: The New England Journal of Medicine
Subject: Health
ISSN: 0028-4793
Year: 1990
Case studies, Complications and side effects, Demographic aspects, Genetic aspects, Disease transmission, HTLV-I infections, HTLV (Viruses), HTLV viruses, Paraparesis, Tropical spastic, Tropical spastic paraparesis

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New diagnostic tests for prion diseases

Article Abstract:

A new test for Creutzfeldt-Jakob disease appears to be fairly accurate. Creutzfeldt-Jakob disease belongs to a group of diseases called prion diseases. Prion diseases usually cause degeneration of nerve cells in the brain. This is believed to be due to the formation of an abnormal form of the prion protein, which occurs naturally in the brain. A 1996 study found that an assay for a brain protein called 14-3-3 protein could be used on cerebrospinal fluid. The protein appears to be specific for prion diseases but the test should only be used in patients with dementia.

Author: Collinge, John
Publisher: Massachusetts Medical Society
Publication Name: The New England Journal of Medicine
Subject: Health
ISSN: 0028-4793
Year: 1996
Analysis, Diagnosis, Spongiform encephalopathy, Cerebrospinal fluid

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Controlling new prion diseases

Article Abstract:

The US has implemented several measures to prevent prion diseases from spreading in the US. Prion diseases include scrapie in sheep and goats, bovine spongiform encephalopathy (BSE), or mad cow disease in cows, and Creutzfeldt-Jakob disease in humans.

Author: Roos, Raymond P.
Publisher: Massachusetts Medical Society
Publication Name: The New England Journal of Medicine
Subject: Health
ISSN: 0028-4793
Year: 2001
Prevention, Bovine spongiform encephalopathy

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Subjects list: Editorial, Creutzfeldt-Jakob disease, Prion diseases
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