نتایج جستجو برای: mitochondrial cytopathies

تعداد نتایج: 132410  

Journal: :AJNR. American journal of neuroradiology 2005
Fernando Scaglia Lee-Jun C Wong Georgirene D Vladutiu Jill V Hunter

BACKGROUND AND PURPOSE Predominant cerebellar involvement has not been previously reported as a common neuroradiologic feature in pediatric mitochondrial cytopathies. Here we report the neuroradiologic findings of predominant cerebellar volume loss in children with various mitochondrial disorders. METHODS A retrospective analysis of the medical records of 400 consecutive patients referred for...

Journal: :Journal of neurology, neurosurgery, and psychiatry 2005
T Gioltzoglou C Cordivari P J Lee M G Hanna A J Lees

Botulinum toxin type A (BTXA) is widely used in neurological therapeutics for a variety of indications such as dystonia, spasticity, hyperhidrosis, and hypersalivation. It is relatively contraindicated in disorders of neuromuscular transmission, in individuals with known hypersensitivity or bleeding disorders, and during pregnancy. Two patients are presented with initially undetermined multisys...

Journal: :Irish medical journal 2006
E Ryan M D King P Rustin P D Mayne G K Brown A A Monavari R Walsh E P Treacy

Mitochondrial respiratory chain disorders account for significant and varied presentations in paediatric practice. The true prevalence of these disorders in the paediatric population is still not well documented with predicted geographic variation. We report a retrospective analysis over a seven year period of cases presenting to a tertiary care centre and associated clinical features. The over...

Journal: :Archives of neurology 2006
Ann Meulemans Sara Seneca Lieven Lagae Willy Lissens Boel De Paepe Joél Smet Rudy Van Coster Linda De Meirleir

BACKGROUND Mitochondrial cytopathies are a heterogeneous group of disorders with a broad spectrum of clinical symptoms. OBJECTIVE To characterize a novel mutation in the transfer RNA(Asn) (m.5728A>G) identified in a 13-year-old boy with multiorgan failure. DESIGN Biochemical and immunocytochemical studies were performed in combination with transmitochondrial cybrid analysis. SETTING A uni...

Journal: :Journal of neurology, neurosurgery, and psychiatry 1993
P R Barnes D J Taylor G J Kemp G K Radda

Skeletal muscle bioenergetics and control of intracellular pH have been investigated in 46 patients with chronic fatigue syndrome by phosphorus magnetic resonance spectroscopy. The results have been compared with those from healthy controls and from a group of patients with mitochondrial cytopathies affecting skeletal muscle. No consistent abnormalities of glycolysis, mitochondrial metabolism o...

Journal: :Brain : a journal of neurology 2008
Patrizia Amati-Bonneau Maria Lucia Valentino Pascal Reynier Maria Esther Gallardo Belén Bornstein Anne Boissière Yolanda Campos Henry Rivera Jesús González de la Aleja Rosanna Carroccia Luisa Iommarini Pierre Labauge Dominique Figarella-Branger Pascale Marcorelles Alain Furby Katell Beauvais Franck Letournel Rocco Liguori Chiara La Morgia Pasquale Montagna Maria Liguori Claudia Zanna Michela Rugolo Andrea Cossarizza Bernd Wissinger Christophe Verny Robert Schwarzenbacher Miguel Angel Martín Joaquín Arenas Carmen Ayuso Rafael Garesse Guy Lenaers Dominique Bonneau Valerio Carelli

Mutations in OPA1, a dynamin-related GTPase involved in mitochondrial fusion, cristae organization and control of apoptosis, have been linked to non-syndromic optic neuropathy transmitted as an autosomal-dominant trait (DOA). We here report on eight patients from six independent families showing that mutations in the OPA1 gene can also be responsible for a syndromic form of DOA associated with ...

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