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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.
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Diseases Transmitted Primarily by Arthropod Vectors 71. Rossi CA, Lewis TE, Drabick J, et al. Laboratory diagnosis of acute dengue fever in military troops during the United Nations Mission in Haiti, 1995-1996. J Trop Med. 1998; 59: 275278. Kalayanorooj S, Vaughn DW, Nimmannitya S, et al. Early clinical and laboratory indicators of acute dengue illness. J Infect Dis. 1997; 176: 313321. Kouri GP, Guzman MG, Bravo JR, Triana C. Dengue haemorrhagic fever dengue shock syndrome: Lessons from the Cuban epidemic, 1981. Bull World Health Organ. 1989; 67: 375380. Kanesa-thasan N, Hoke CH Jr. Dengue and related syndromes. In: Schlossberg D, ed. Current Therapy of Infectious Disease. 2nd ed. Chicago: Mosby-Year Book; 2001. World Health Organization. Dengue Hemorrhagic Fever: Diagnosis, Treatment and Control. 2nd ed. Geneva: WHO; 1997: 6. Kanesa-thasan N, Putnak JR, Hoke CH Jr. New and improved vaccines for dengue, Japanese encephalitis, and yellow fever viruses. In: Levine MM, Woodrow GC, Kaper JB, Cobon GS eds. New Generation Vaccines. 2nd ed. New York: Marcel Dekker; 1997: 587606. Pinheiro FP, Travassos da Rosa AP, Travassos da Rosa JF, et al. Oropouche virus, I: A review of clinical, epidemiological, and ecological findings. J Trop Med Hyg. 1981; 30: 149160. Leduc JW, Pinheiro FP. Oropouche fever. In: Monath TP, ed. The Arboviruses: Epidemiology and Ecology. Vol 4. Boca Raton, Fla: CRC Press; 1989: 114. Tesh RB. The emerging epidemiology of Venezuelan hemorrhagic fever and Oropouche fever in tropical South America. Ann N Y Acad Sci. 1994; 740: 129137. Baisley KJ, Watts DM, Munstermann LE, Wilson ML. Epidemiology of endemic Oropouche virus transmission in upper Amazonian Peru. J Trop Med Hyg. 1998; 59: 710716. Meegan JM, Bailey CL. Rift Valley fever. In: Monath TP, ed. The Arboviruses: Epidemiology and Ecology. Vol 4. Boca Raton, Fla: CRC Press; 1989: 5176. Shope R, Peters CJ, Walker JS. Serological relation between Rift Valley fever virus and viruses of Phlebotomus fever serogroup. Lancet. 1980; 1: 886887. McIntosh BM, Jupp PG, Dos Santos I, Barnard BJ. Vector studies on Rift Valley Fever virus in South Africa. S Afr Med J. 1980; 58: 127132. World Health Organization Scientific Group. Arthropod-borne and Rodent-borne Viral Diseases. Geneva: World Health Organization; 1985. Technical Report Series 719. Sall AA, Zanotto PM, Vialat P, Sene OK, Bouloy, MB. Origin of 199798 Rift Valley fever outbreak in East Africa. Lancet. 1998; 352: 15961597. Watts DM, el-Tigani A, Botros BA, et al. Arthropod-borne viral infections associated with a fever outbreak in the northern province of Sudan. J Trop Med Hyg. 1994; 97: 228230. Corwin A, Habib M, Watts D, et al. Community-based prevalence profile of arboviral, rickettsial, and hantaanlike viral antibody in the Nile River Delta of Egypt. J Trop Med Hyg. 1993; 48: 776783. Richards AL, Malone JD, Sheris S, et al. Arbovirus and rickettsial infections among combat troops during Operation Desert Shield Desert Storm. J Infect Dis. 1993; 168: 10801081. Gordon SW, Tammariello RF, Linthicum KJ, Dohm DJ, Digoutte JP, Calvo-Wilson MA. Arbovirus isolations from mosquitoes collected during 1988 in the Senegal River basin. J Trop Med Hyg. 1992; 47: 742748 and accutane and zestril, for example, zsetril 40 mg.
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