نتایج جستجو برای: hypomyelinating leukoencephalopathy

تعداد نتایج: 5021  

Journal: :iranian journal of child neurology 0
parvaneh karimzadeh 1. pediatric neurology research center, shahid behesti university of medical sciences, tehran, iran 2. department of pediatric neurology, pediatric neurology center of excellence, faculty of medicine, mofid children hospital, shahid behesti university of medical sciences, tehran, iran

how to cite this article: karimzadeh p. pelizaeus-merzbacher- disease (pmd) and pelizaeus-merzbacher-like disease (pmld). iran j child neurol autumn 2014;8:4 (suppl.1):9-10.   pls see pdf.

Journal: :Archives of neurology 2012
Marjan E Steenweg Nicole I Wolf Jolanda H Schieving Mahmoud Fawzi Elsaid Richard L Friederich John R Ostergaard Frederik Barkhof Petra J W Pouwels Marjo S van der Knaap

OBJECTIVE To describe 4 children with a novel hypomyelinating leukoencephalopathy, defined by a distinct pattern of magnetic resonance imaging (MRI) abnormalities. DESIGN In our ongoing study on leukoencephalopathies of unknown origin, MRIs of patients are rated in a standardized manner. Patients are grouped according to their MRI abnormalities. The clinical and laboratory data are retrospect...

Journal: :iranian journal of radiology 0
parvaneh karimzadeh pediatric neurology research center, shahid beheshti university of medical sciences, tehran, iran; pediatric neurology research center, shahid beheshti university of medical sciences, tehran, iran. tel: +98-2122909559, fax: +98-2122909559 farzad ahmadabadi ardabil university of medical sciences, ardabil, iran omid aryani special medical center, tehran, iran massoud houshmand department of human genetics, national institute for genetic engineering and biotechnology, tehran, iran alireza khatami pediatric neurology research center, shahid beheshti university of medical sciences, tehran, iran

pelizaeus­-merzbacher-like disease (pmld) is a hypomyelinating leukoencephalopathy disorder with a genetically heterogeneous pattern. mutations in the gja12/gjc2 gene cause one form of autosomal recessive pelizaeus­-merzbacher-like disease. here, we report a new mutation in a ­10-month-old girl with nystagmus, psychomotor delay, hypotonicity, head nodding and dysmyelination from healthy second ...

2014
Parvaneh Karimzadeh Farzad Ahmadabadi Omid Aryani Massoud Houshmand Alireza Khatami

Pelizaeus--Merzbacher-like disease (PMLD) is a hypomyelinating leukoencephalopathy disorder with a genetically heterogeneous pattern. Mutations in the GJA12/GJC2 gene cause one form of autosomal recessive Pelizaeus--Merzbacher-like disease. Here, we report a new mutation in a -10-month-old girl with nystagmus, psychomotor delay, hypotonicity, head nodding and dysmyelination from healthy second ...

2018
Haoran Ji Dongxiao Li Ye Wu Quanli Zhang Qiang Gu Han Xie Taoyun Ji Huifang Wang Lu Zhao Haijuan Zhao Yanling Yang Hongchun Feng Hui Xiong Jinhua Ji Zhixian Yang Liping Kou Ming Li Xinhua Bao Xingzhi Chang Yuehua Zhang Li Li Huijuan Li Zhengping Niu Xiru Wu Jiangxi Xiao Yuwu Jiang Jingmin Wang

OBJECTIVE Hypomyelinating disorders are a group of clinically and genetically heterogeneous diseases characterized by neurological deterioration with hypomyelination visible on brain MRI scans. This study was aimed to clarify the clinical and genetic features of HMDs in Chinese population. METHODS 119 patients with hypomyelinating disorders in Chinese population were enrolled and evaluated ba...

Journal: :Journal of neurology, neurosurgery, and psychiatry 1985
Y Harati I J Butler

Two patients with congenital hypomyelinating neuropathy are reported with details of sural nerve pathology. The resemblance of this condition to the hypomyelinating neuropathy of Trembler mice is discussed and the pertinent medical literature reviewed.

Journal: :American journal of human genetics 2011
Hirotomo Saitsu Hitoshi Osaka Masayuki Sasaki Jun-Ichi Takanashi Keisuke Hamada Akio Yamashita Hidehiro Shibayama Masaaki Shiina Yukiko Kondo Kiyomi Nishiyama Yoshinori Tsurusaki Noriko Miyake Hiroshi Doi Kazuhiro Ogata Ken Inoue Naomichi Matsumoto

Congenital hypomyelinating disorders are a heterogeneous group of inherited leukoencephalopathies characterized by abnormal myelin formation. We have recently reported a hypomyelinating syndrome characterized by diffuse cerebral hypomyelination with cerebellar atrophy and hypoplasia of the corpus callosum (HCAHC). We performed whole-exome sequencing of three unrelated individuals with HCAHC and...

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