منابع مشابه
A Case of Adult Polyglucosan Body Disease
Adult polyglucosan body disease (APBD) is a rare neurological disease, characterized by adult onset (fifth to seventh decades), progressive sensorimotor or pure motor peripheral neuropathy, upper motor neuron symptoms, neurogenic bladder, and cognitive impairment. APBD is confirmed by a sural nerve biopsy that shows the widespread presence of polyglucosan bodies in the nerve. We report a 70 yea...
متن کاملImmunohistochemical and ultrastructural changes in the brain in probable adult glycogenosis type IV: adult polyglucosan body disease.
Glycogenosis type IV is caused by a deficiency of glycogen branching enzyme (alpha-1,4 glucan 6-transglucosylase). Adult polyglucosan body disease (APBD) may represent a neuropathological hallmark of the adult form of this storage disease of the central nervous system. We analysed a case of a 45-year-old unconscious woman who died three days after admission to the hospital. Neuropathological ex...
متن کاملAdult polyglucosan body disease associated with lewy bodies and tremor.
BACKGROUND Adult polyglucosan body disease (PGBD) is rare and typically presents with upper and lower motor neuron involvement and neurogenic bladder. Extrapyramidal features are unusual in PGBD and are presumed secondary to widespread pathology that includes the basal ganglia. There are no prior reports of Lewy bodies in PGBD. OBJECTIVE To report a unique finding of Lewy bodies in a patient ...
متن کاملAdult polyglucosan body disease associated with an extrapyramidal syndrome.
A 50 year old patient is described who presented with parkinsonism, frontal dementia, peripheral neuropathy, neurogenic bladder, and upper motor neuron signs. No improvement in objective measurements of extrapyramidal dysfunction were seen with an incremental apomorphine test or more prolonged oral dopamine challenge. Neurophysiology disclosed changes compatible with a diffuse axonal neuropathy...
متن کاملAdult polyglucosan body disease: clinical and histological heterogeneity of an Italian family
Adult polyglucosan body disease: clinical and histological heterogeneity of an Italian family I. Colombo1, S. Pagliarani1, S. Testolin1, E. Salsano2, L. Napoli1, A. Bordoni1, S. Salani1, E. D’Adda3, L. Morandi4, L. Farina5, M. Riva6, A. Prelle3, M. Sciacco1, G. Comi1, M. Moggio1 1University of Milan, Dino Ferrari Center, Fondazione Cà Granda, IRCCS Ospedale Maggiore Policlinico, Milano, 2Clinic...
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ژورنال
عنوان ژورنال: Mayo Clinic Proceedings
سال: 2000
ISSN: 0025-6196
DOI: 10.4065/75.12.1327