近端/远端复合肌肉动作电位振幅比率在术后面神经功能评估上的价值

目的 探讨近端/远端复合肌肉动作电位振幅比率在术后面神经肿瘤侵犯段功能评估上的价值和对早期面神经修复的指导意义。方法 比较术末面神经近端/远端复合肌肉动作电位振幅比率和术后2周、6个月患侧面神经功能的H-B分级之间的关系。结果 术后6个月面神经H-B分级恢复到Ⅰ-Ⅱ级者,术末近端/远端复合肌肉动作电位振幅比率均>0.3。结论 术末面神经近端/远端复合肌肉动作电位振幅比率是判断术后面神经远期功能,特别是肿瘤侵犯段功能的重要指标,为术中直接修复面神经提供了指导依据。     

Novel C59T leader peptide mutation in the MPZ gene associated with late-onset, axonal, sensorimotor polyneuropathy

The objective of this study was to report a novel exon-1 mutation in the myelin protein zero (MPZ) gene, resulting in axonal Charcot–Marie–Tooth neuropathy with recurrent hyper-CK-emia. In a 64-year-old woman slowly progressive distal lower limb weakness, muscle cramps in the lower limb muscles, and stocking-type numbness had developed from the age of 61. Neurologic examination revealed discrete hip flexor weakness, weakness for foot extension, diffuse wasting of the distal lower limb muscles, reduced patella tendon reflexes, and absent Achilles tendon reflexes. There was recurrently elevated creatine kinase with a maximum of 607 U/l ( n , <145 U/l). Stimulation of the peroneal and tibial nerves did not evoke a muscular response. Electromyography was neurogenic. Biopsy of the right sural nerve showed diffuse axonal degeneration and loss of axons of all diameters. Muscle biopsy showed increased fiber-size variability, angulated fibers, internalized nuclei, accumulations of nuclei, grouped atrophic muscle fibers, and fiber splitting. Molecular genetic analysis by PCR and direct nucleotide sequencing revealed the heterozygous C59T exon-1 MPZ gene mutation, resulting in the amino acid exchange S20F of the MPZ signal protein domain (leader peptide). The novel C59T mutation in the leader peptide of the MPZ gene is pathogenic and manifests as severe, late-onset, axonal, symmetric sensorimotor polyneuropathy (CMT2) and hyper-CK-emia.     

一种新的单侧听觉气传导持续阻滞大鼠模型

用外耳道外沿皮下缝合法制备了一种新的单侧听觉气传导持续阻滞大鼠模型。它具有制作简便、损伤较小、效果持续的优点。     

Sural nerve biopsies in Guillain-Barre syndrome: axonal degeneration and macrophage-associated demyelination and absence of cytomegalovirus genome.

T-cell infiltration was detected by immunohistochemistry in only 2 of 10 sural nerve biopsies from patients with Guillain-Barré syndrome (GBS). The number of endoneurial macrophages, identified by the monoclonal antibody MAC 387, was increased, compared with the number in 10 cases of axonal neuropathy. Macrophage-associated demyelination was identified in 7 and axonal degeneration in 8 cases. Cytomegalovirus (CMV) genome was not detected with the polymerase chain reaction.     

Oculomotor nerve palsy due to thrombosis of a posterior communicating artery aneurysm following diagnostic angiography

We present a patient who developed a painful third nerve galsy two days after angiography had demonstrated a large aneurysm on the P1 segment of the left posterior cerebral artery. CT at this stage demonstrated extensive thrombus within the previously uncomplicated aneurysm. The haemodynamics of this aneurysm resulted in incomplete clearance of contrast medium from its fundus and we posit that this may have promoted thrombus formation. Six months later the aneurysm was shown angiographically to be completely occluded.     

Abnormal sensorimotor integration is related to disease severity in Parkinson's disease: a TMS study.

Hyperexcitability of the motor system has been reported in Parkinson's disease (PD). We evaluate how cutaneous afferents modulate motor excitability in PD patients and whether abnormal modulation is correlated to parkinsonian symptoms. Digital stimulation causes abnormal enhancement of motor responses in patients. This effect may be one of the features of motor hyperexcitability in PD. Cutaneomotor hyperexcitability correlates with clinical scores, suggesting that abnormal processing of cutaneous inputs might contribute to the pathogenesis of parkinsonian symptoms.     

腰神经通道卡压综合征病因的探讨

为了探讨腰神经通道狭窄的病因，我们对２２４例腰椎间盘突出及腰椎管狭窄症患者进行了腰神经通道探查手术，结果发现其病因以腰部组织变性为主。除外伤及反复慢性腰部损伤引起腰部病变外，活动少，或很少进行腰部锻炼，体形肥胖也是引起腰神经通道狭窄日趋加重的致病因素。在治疗时强调不只满足于切除突出的椎间盘，应沿神经通道探查，清除一切卡压神经根的病变组织才能获得较好的治疗效果。我们随访１９７例，平均随访３ａ，优良率９３５％。     

Possible injury to the branches of the peroneal nerve during fibular osteotomies

Conclusions The peroneal nerves and their blood supply are at risk during high tibial osteotomies. Fixation of nerves by fibrous tissues, compression by tendinous arcades of the peroneus longus tendon, and narrow passages for nerves crossing dense fibrous septa are all factors which favor the development of peroneal nerve lesions. Intraoperative soft tissue retraction and pull by retractors may damage nerves and vessels. The muscle branch for the extensor hallucis longus muscle is particularly at risk during the fibular osteotomy since it runs directly on the bone.     

Auditory and vestibular system findings in patients with vertebrobasilar dolichoectasia

Impairment of the auditory-vestibular system has been reported in patients with vertebrobasilar dolichoectasia (VBD), but little is known about the underlying cause of the symptoms. Auditory testing (pure tone audiometry, auditory brain stem response and stapedius reflex) and vestibular tests (assessment of nystagmus, eye tracking tests, caloric test and rotational test) were performed in 23 patients with auditory-vestibular symptoms and/or cranial nerve impairment associated with VBD. Specific evidence of auditory and/or vestibular system impairment was observed in 19 cases (83%). Among patients with abnormal test findings, 47% had evidence suggesting peripheral impairment, 16% evidence suggesting central dysfunction, and 37% evidence suggesting both peripheral and central dysfunction. Although compression of the vestibulocochlear nerve plays an important role in the genesis of the auditory-vestibular dysfunction in patients with VBD, mechanism such as brain stem-cerebellar ischemia and impaired blood supply to the vestibular labyrinth may be just as important.     

Respiratory Sinus Arrhythmia in Cluster Headache Syndrome

SYNOPSIS
Respiratory sinus arrhythmia is regarded as indicative of cardiac vagal integrity. A ratio of the longest R-R interval to the shortest R-R interval during deep breathing test (E:I ratio) was calculated in controls (n=49), cluster headache (n=33) and CPH (n=4) patients, E:I ratio decreased with age but was not dependent upon sex or upon smoking habits. Furthermore, there were no significant differences as regards E:I ratio between cluster headache patients in and outside a bout, or between patients with right-sided and left-sided headache. However, the E:I ratio was found to be significantly lower in the cluster headache group as such, when compared with controls, but the number of patients disclosing pathological or borderline results was small, 2 and 2, respectively. This may indicate that a putative vagal dysfunction in cluster headache is usually less marked than in patients with e.g. diabetic autonomic neuropathy. Significant attack-related change in the E:I ratio were detected in all individual patients though these changes were not of a uniform nature from individual to individual. E:I ratios were rather high in 3 out of 4 CPH patients examined. However, the number of patients in this group is too small to allow definite statements about the difference between CPH end cluster headache with regard to E:I ratios. There was no significant difference between E:I ratios outside and during a mild, short, mechanically precipitated attack in a single CPH patient.