نتایج جستجو برای: ataxia with ocular motor apraxia

تعداد نتایج: 9275574  

2007
Tetsuya Takahashi Masayoshi Tada Shuichi Igarashi Akihide Koyama Hidetoshi Date Akio Yokoseki Atsushi Shiga Yutaka Yoshida Shoji Tsuji Masatoyo Nishizawa Osamu Onodera

Aprataxin is the causative gene product for early-onset ataxia with ocular motor apraxia and hypoalbuminemia/ataxia with oculomotor apraxia type 1 (EAOH/AOA1), the clinical symptoms of which are predominantly neurological. Although aprataxin has been suggested to be related to DNA single-strand break repair (SSBR), the physiological function of aprataxin remains to be elucidated. DNA single-str...

Journal: :Chang Gung medical journal 2002
Hung-Na Hsu Meng-Ling Yang Huei-Chun Lai

Congenital ocular motor apraxia (COMA) is a unique ocular motor disorder which is characterized by a deficit in initiation of voluntary horizontal eye movement with reserved reflex eye movement. Although a portion of cases with COMA were found to be associated with other abnormalities, COMA in most patients is an isolated disorder. The most characteristic appearance of these patients is compens...

Journal: :Frontiers in Neurology 2023

Ataxia-telangiectasia-like disorder 1 (ATLD1) is a rare neurodegenerative associated with early onset ataxia and oculomotor apraxia. The genetic determination of ATLD1 mutation in the MRE11 gene (meiotic recombination 11 gene), which causes DNA-double strand break repair deficits. Clinical features patients resemble those telangiectasia (AT), slower progression milder presentation. Main symptom...

2013
Paola Michieletto Andrea Martinuzzi Stefano Pensiero

Purpose: To disclose the association between spinocerebellar ataxia with oculomotor apraxia and high grade (7 diopters) congenital astigmatism. Methods: Single observational case report. A 39-year-old patient affected by spinocerebellar ataxia from the age of 20 was submitted to genetic and ophthalmic investigations to reach a diagnosis. Results: Genetic testing did not lead to a sure diagnosis...

Journal: :Journal of neurology, neurosurgery, and psychiatry 1983
J M Ferro J M Bravo-Marques A Castro-Caldas L Antunes

An unusual combination of disconnective syndromes is reported: transcortical motor aphasia, left arm apraxia and optic ataxia. Neuropathological examination showed a left parieto-occipital and a subcortical frontal infarct and a lesion of the dorsal part of the posterior two-fifths of the callosum. The frontal lesion caused the transcortical motor aphasia and produced the left arm apraxia. Visu...

ژورنال: توانبخشی 2012
A'zimian, Mojtaba, Biglarian, Akbar, Rahgozar, Mehdi, Yadegari, Fariba,

Objective: Oral and verbal apraxias represent motor programming deficits of nonverbal and verbal movements respectively. Studying their properties may shed light on speech motor control processes. This study was focused on identifying cases with oral or verbal apraxia, their co–occurrences and severities. Materials & Methods: In this non-experimental study, 55 left adult subjects with left b...

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