نتایج جستجو برای: pick disease

تعداد نتایج: 1499420  

2012
Johannes Skorpen Ingrid B Helland Bjørn Tennøe

UNLABELLED INTRODUCTION Niemann-Pick disease type C is a rare genetic lysosomal storage disease associated with impaired intracellular lipid trafficking and a range of progressive neurological manifestations. The influence of seizure activity on disease course and response to miglustat therapy is not currently clear. CASE PRESENTATION Niemann-Pick disease type C homozygous for NPC1 mutatio...

Journal: :The Israel Medical Association journal : IMAJ 2008
Netta Levin Dov Soffer Iftah Biran John M Gomori Moshe Bocher Sergieu C Blumen Oded Abramsky Ricardo Segal Alexander Lossos

Journal: :Brain : a journal of neurology 2005
I F Bronner B C ter Meulen A Azmani L A Severijnen R Willemsen W Kamphorst R Ravid P Heutink J C van Swieten

Frontotemporal dementia and parkinsonism linked to chromosome 17 have been associated with mutations in the microtubule associated protein tau (MAPT or tau) gene. This disorder is characterized by a large spectrum of neuronal and glial tau lesions in different brain regions. Pick bodies were found in a family with hereditary Pick's disease with the G272V mutation and in several families with ot...

2016
Jean-Marie Cuisset S. Sukno A. Trauffler P. Latour D. Dobbelaere L. Michaud L. Vallée

BACKGROUND Niemann-Pick disease type C is a rare inherited neurodegenerative disease involving impaired intracellular lipid trafficking and accumulation of glycolipids in various tissues, including the brain. Miglustat, a reversible inhibitor of glucosylceramide synthase, has been shown to be effective in the treatment of progressive neurological manifestations in pediatric and adult patients w...

Journal: :Journal of Neuropathology & Experimental Neurology 2019

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