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世界核心医学期刊文摘（神经病学分册） 2005年第7期1卷 42-43页

作者： Dichgans M. Herzog j. Freilinger T. 雷翀 Marchioninistrasse 15 81377 Muenchen Germany Dr.

Background: About 20%of patients with familial hemiplegic migraine (FHM) deve lop progressive cerebellar signs. Genetic studies have established an associatio n with mutations in the CACNA1A gene. However, the mechanisms underlying cerebel lar involvement are largely unknown. Objective: To use proton MR spectroscopy (1 H-MRs) to investigate metabolic alterations in the cerebellum as well as cortic al regions known to be involved in the propagation of migraine aura. Methods:Fif teen CACNA1A mutation carriers from three FHM families and 17 healthy control su bjects were studied. Eleven patients had clinical signs of cerebellar involvemen t. LCModel fits were used to estimate absolute concentrations of N-acetylaspart ate (NAA), myo-inositol (ml), glutamate (Glu), choline containing compounds, to tal creatine, and lactate in the superior cerebellar vermis (sCV), parietal cort ex, and occipital *** control for atrophy effects, automated image segment ation was performed using sPM99. The brain parenchyma fraction(BPF) was determin ed for all three regions. Results: Compared with controls, the brain parenchyma fraction (BPF), NAA,and Glu were significantly reduced and mI was significantly elevated in the sCV of patients with FHM. In contrast, no metabolite alterations were found in supratentorial regions. BPF and NAA in the sCV significantly corr elated with cerebellar scores, in particular, gait ataxia. Conclusions: The find ings suggest that there is a regionally distinct neuronal impairment in the superio r cerebellar vermis that exceeds macroscopic tissue loss. Correlations with clin ical scores emphasize the functional relevance of localized atrophy (brain paren chyma fraction) and N-acetyl aspartate levels. These measures may be useful to monitor disease progression. The observed reductionin glutamate may in part refl ect impaired glutamatergic neurotransmission.

关键词：小脑体 migraine 小脑蚓部 cerebellar 步态共济失调大脑皮质谷氨酸能神经元 glutamate neuronal cortex

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先天性全盲患者舌部电触觉刺激显示交叉式可塑性

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世界核心医学期刊文摘（神经病学分册） 2005年第8期1卷 13-14页

作者： Ptito M. Moesgaard s.M. Gjedde A. Kupers R. 郭俊 school of Optometry Université de Montréal CP 6128Montreal Que. H3C 3J7 Canada

In sensory substitution, information acquired with one sensory modality is use d to accomplish a task which is normally subserved primarily by another sensory modality. We used PET to study cross-modal plasticity in the congenitally blind , using electrotactile stimulation of the tongue. Blind (n=6) and sighted blindfolded controls (n=5) were scanned before and after they were trained to use their tongue in a snellen orientation detect ion task. Results showed that both groups of subjects learned the discrimination orientation task after seven 1 h training sessions. Before training, no signifi cant changes in regional cerebral blood flow (rCBF) were observed in the occipit al cortex in either group. In sharp contrast, activity in the occipital cortex i ncreased after practice for the blind, but not for the sighted, providing eviden ce for training-induced plasticity in the blind. An inter-regional correlation analysis showed that task-related rCBF changes in left posterior parietal cort ex were positively correlated with rCBF changes in the occipital area of the tra ined blind. These data reveal that cross-modal plasticity in the blind develops rapidly and that the occipital cortex is part of a functional neural network fo r tactile discrimination in conjunction with the posterior parietal cortex. Our data further show that the tongue can act as a portal to convey somatosensory in formation to visual cortex.

关键词：触觉刺激全盲交叉式可塑性变化 rCBF 局部脑血流量顶叶皮层检测任务视觉皮层枕区

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Tourette综合征和深部脑刺激

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世界核心医学期刊文摘（神经病学分册） 2005年第10期1卷 30-30页

作者： Houeto j.L. Karachi C. Mallet L. Y. Agid 邓剑平 Centre d’Investigation Clinique Hpital de la Sal pêtrière 47 boulevard de l’ Hpital 750 13 Paris France Dr.

In this prospective double blind randomised “ N of 1” study, a patient wit h a severe form of Tourette’ s syndrome was treated with bilateral high frequen cy stimulation of the centromedianparafascicular complex (Ce- Pf) of the thalam us, the internal part of the globus pallidus (GPi), or both. stimulation of eith er target improved tic severity by 70% , markedly ameliorated coprolalia, and e liminated self injuries. severe forms of Tourette’ s syndrome may benefit from stimulation of neuronal circuits within the basal ganglia, thus confirming the r ole of the dysfunction of limbic striatopallido- thalamo- cort- ical systems in this disorder.

关键词：深部脑刺激 Tourette综合征苍白球秽语症内侧部纹状体严重型电刺激肌张力边缘系统

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