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Referenz 97d Neurologie, 11. Auflage ; Bigio EH, Weiner MF, Bonte FJ, White CL. Familial dementia due to adult polyglucosan body disease. Clin Neuropathol 16; 227-234, 1997. Department of Pathology, University of Texas Southwestern Medical School, Dallas, USA. BACKGROUND: Adult polyglucosan body disease APBD ; is a rare disorder, presenting with varying combinations and severity of upper and lower motor neuron dysfunction, sensory deficits, dementia, and urinary incontinence. Onset is in the 40s or 50s. The diagnosis is made by finding polyglucosan bodies PB ; in histologic sections of brain or spinal cord, peripheral nerve, or dermal sweat glands. Although 2 pairs of the 22 previously reported cases were siblings, the familial nature of the disease has not been emphasized. METHODS: We report 2 adult siblings, a male and a female, each of whom had the clinical diagnosis of vascular dementia. Both were confirmed at autopsy to have APBD. We characterized the lesions in autopsy tissues using a battery of histological stains, lectin histochemistry, and electron microscopy. RESULTS: Innumerable PB were distributed throughout brain, heart, skeletal muscle, liver, and dermal sweat glands. PB were highlighted by periodic acid Schiff stain and concanavalin A lectin. Ultrastructurally, PB were composed of aggregates of filaments within axons and astrocytic processes, and lying free in the neuropil, but not within neuronal perikarya. CONCLUSIONS: It is important to consider APBD in cases of familial dementia of unknown etiology. Ante-mortem biopsy of axillary skin may be diagnostic and accutane.
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Kalso E, Edwards J, McQuay HJ, Moore RA. Five easy pieces of evidence based medicine 5 ; . Trading benefits against harm-pain relief vs. adverse effects. European Journal of Pain 2002; 6: 409-412. Collins R, MacMahon S. Reliable assessment of the effects of treatment on mortality and major morbidity, I: clinical trials. Lancet 2001; 357 9253 ; : 373-380. Wright JM. Why don't we initiate more large simple randomized controlled trials? CMAJ 2003; 169 11 ; : 1170-1171. Warlow C. Advanced issues in the design and conduct of randomized clinical trials: the bigger the better? Stat Med 2002; 21 19 ; : 2797-2805. Sandercock P. What question can large, simple trials answer? Cerebrovascular Diseases 1998; 8 Suppl 2 ; : 30-36. Hotopf M, Churchill R, Lewis G. Pragmatic randomised controlled trials in psychiatry. British Journal of Psychiatry 1999; 175: 217-223. Holbrook A, Goldsmith C, Grootendorst P, Willison D, Levine M, Gaebel K et al. optimal pharmacosurveillance? What information is required for Pharmacoepidemiol.Drug Saf 12 Supp 1 ; , S138-S139. 2003. Tonks A. A clinical trials register for Europe. BMJ 2002; 325 7376 ; : 1314-1315. McCray AT. Better access to information about clinical trials. Ann Intern Med 2000; 133 8 ; : 609-614.
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