Alteration of cortical excitability and its modulation by Miglustat in Niemann-Pick disease type C

被引：5
|
作者：
Hassan, Shady Safwat [1 ,2 ]
Trenado, Carlos [1 ,3 ]
Elben, Saskia [1 ,3 ]
Schnitzler, Alfons [1 ,3 ]
Groiss, Stefan Jun [1 ,3 ]
机构：
[1] Heinrich Heine Univ, Fac Med, Inst Clin Neurosci & Med Psychol, Dusseldorf, Germany
[2] Assiut Univ Hosp, Dept Neurol, Assiut, Egypt
[3] Heinrich Heine Univ, Fac Med, Dept Neurol, Dusseldorf, Germany
来源：
JOURNAL OF CLINICAL NEUROSCIENCE | 2018年 / 47卷
关键词：
Niemann-Pick disease type C (NP-C); Transcranial magnetic stimulation (TMS); Cerebellar inhibition (CBI); Short interval intracortical facilitation (SICF); Short-latency afferent inhibition (SAI); TRANSCRANIAL MAGNETIC STIMULATION; HUMAN MOTOR CORTEX; INHIBITION; FACILITATION; CEREBELLUM; HUMANS;
D O I：
10.1016/j.jocn.2017.10.011
中图分类号：
R74 [神经病学与精神病学];
学科分类号：
摘要：
Niemann-Pick type C (NP-C) is a rare, neurodegenerative, lysosomal storage disease. Cortical excitability using different transcranial magnetic stimulation (TMS) protocols together with clinical and neuropsychological testing was longitudinally assessed in a patient with NP-C. Cerebellar inhibition, a measure for the integrity of the cerebello-thalamo-cortical network, was impaired. Short-latency afferent inhibition, a measure for cholinergic transmission, and cognitive functions were also impaired and improved under Miglustat treatment. Short interval intracortical facilitation, a marker for glutamatergic neuro-transmission, was absent initially but increased after treatment with Miglustat. Our results provide new insights into pathophysiological mechanisms of NP-C and the response to Miglustat treatment. (C) 2017 Elsevier Ltd. All rights reserved.
引用
收藏
页码：214 / 217
页数：4
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