微血管减压术治疗面肌痉挛并耳聋、耳鸣

目的探讨微血管减压术治疗面肌痉挛并耳聋、耳呜的临床疗效。方法我科自2000年6月至2006年6月在局麻下对17例面肌痉挛并耳鸣、耳聋的患者施行了微血管减压术。术中分别松解动脉对第Ⅶ、Ⅷ颅神经的压迫，并以Teflon片分隔神经与血管。结果所有患者面肌痉挛均治愈。9例耳聋症状完全缓解，8例耳聋症状明显改善；15例耳呜症状完全缓解，2例耳鸣症状明显改善。结论微血管减压术对由血管压迫引起的面肌痉挛并耳聋、耳鸣患者疗效明确。     

A case of Tolosa-Hunt syndrome accompanied by facial and vestibular nerve damage]

A 37-year old man, who had repeatedly suffered from transient ophthalmoplegia in his left eye at the age of 29 and 36, developed left painful ophthalmoplegia accompanied by ipsilateral facial nerve palsy in August, 1991. Neurological examination revealed involvement of the left oculomotor, trochlear, ophthalmic division of the trigeminal, abducens, facial and vestibular nerves. Gadolinium-enhanced MRI which was taken at the acute phase of the illness demonstrated markedly enhanced left cavernous sinus and adjacent thickened dura mater in the middle cranial fossa. At the remission phase after starting corticosteroid therapy, these enhanced lesions were no longer observed even in enhanced MRI studies. We diagnosed him as suffering from Tolosa-Hunt syndrome presently accompanied by facial and vestibular nerve damage because of his history of illness, confined lesion in the left cavernous sinus and steroid-induced remission. We concluded that Tolosa-Hunt syndrome may be accompanied by damage of other cranial nerves in its course and that repeated gadolinium-enhanced MRIs are necessary for diagnosis and observation of the patients.     

An unusual case of paroxysmal facial pain

Summary Paroxysmal facial pain is a rare sequel to brainstem infarction. This is a report of a patient who developed paroxysmal facial pain with the exceptional features of a trigger zone and relative refractory pain-free periods in an area previously anesthetic after infarction of the brainstem. The facial pain responded favorably to carbamazepine. The characteristic of this patient's pain is compared with the pain of trigeminal neuralgia and possible similar pathogenetic mechanisms.

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Zusammenfassung Anfallsartige Gesichtsschmerzen stellen eine seltene Folge eines Hirnstamminsultes dar. Es wird ein Patient beschrieben, bei welchem sich in einem Bereiche, welcher infolge eines Hirnstamminfarktes zunächst anästhetisch war, ein paroxysmaler Gesichtsschmerz entwickelt. Dieser war von einer Triggerzone aus auslösbar, und es bestand anschließend eine auf Schmerzen refraktäre Periode. Der Schmerz sprach günstig auf Carbamazepin an. Die Charakteristika dieses Falles werden mit den Gesichtsschmerzen der Trigeminusneuralgie verglichen, und es werden die pathogenetischen Mechanismen besprochen.

     

A case of facial hemiatrophy associated with linear scleroderma and homolateral masseter spasm

The rare combination of progressive facial hemiatrophy, linear scleroderma and spasm of the homolateral masseter muscle was observed in a 39 year old woman. The possible pathogenetic meaning of the association is discussed on the electrophysiological evidence and in the light of published cases.

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Sommario In una donna di 39 anni è stata osservata la rara associazione di emiatrofia facciale progressiva, sclerodermia lineare e spasmo del muscolo massetere omolaterale. Sulla base dello studio elettrofisiologico nonché dalla revisione della letteratura, gli Autori discutono sul possibile significanto di tale associazione da un punto di vista patogenetico.

     

Woman with facial spasm induced exclusively by sound.

A woman is described in whom facial spasm occurs solely and predictably in the presence of certain noises. The symptoms resolve promptly as soon as the auditory stimulus stops.     

A型肉毒毒素肌肉注射疗法在特发性眼睑、面肌痉挛中的应用

目的探讨A型肉毒毒素注射治疗特发性眼睑、面肌痉挛的疗效。方法应用A型肉毒毒素对按Cohen、Albert等分级为Ⅱ～Ⅳ级特发性眼睑、面肌痉挛的患者72例进行眼轮匝肌、口轮匝肌及面部肌肉多点注射治疗，对治疗前后的病情分级进行对比。结果应用A型肉毒毒素治疗特发性眼睑、面肌痉挛的短期有效率为97．2％，丰年的复发率为58．3％，未见过敏反应及严重全身不良反应。结论A型肉毒毒素为治疗特发性眼睑、面肌痉挛首选药物，尽管其疗效半年左右，但仍不失为一种简单有效的治疗方法。     

The electromyogram in facial myokymia and hemifacial spasm

Electromyographic findings in 5 patients with facial myokymia due to multiple sclerosis and in 10 patients with hemifacial spasm are described and compared.Many of the spontaneously discharging motor units in myokymia are not under voluntary control.In hemifacial spasm the high frequencies of discharge reached in blink bursts are similar to those recorded during normal involuntary blinking.The possible sites of origin of the discharges are briefly discussed.     

Hemifacial spasm and facial myokymia: electrophysiological findings

In two patients, one with clinical hemifacial spasm (HFS) and one with clinical facial myokymia, the spontaneous electromyographic findings consisted of regular burst activity: each burst composed of high frequency repetitive motor unit potentials. These bursts fired synchronously in multiple ipsilateral facial muscles in both patients and from a contralateral facial muscle in the patient with HFS. In addition, the patient with HFS exhibited crossed evoked responses on the affected side with stimulation of the facial and supraorbital nerves on the unaffected side. It is concluded that the current electrophysiological distinctions between HFS and facial myokymia may be unsupportable and that facial nuclear hyperactivity with activation of bilateral facial nuclear connections plays a role in the pathophysiology of HFS.     

Electrodiagnostic studies of the facial nerve in peripheral facial palsy and hemifacial spasm

Electrodiagnostic (EDX) assessment is one of the most important aspects in the evaluation of the two most common disorders of the facial nerve: facial palsy and hemifacial spasm. Facial palsy is usually an acute disorder that resolves in a few weeks but, in a number of cases, leads to a postparalytic facial syndrome featuring muscle synkinesis, myokymia, and involuntary mass contractions of muscles on the affected side. Hemifacial spasm is usually a chronic disorder characterized by paroxysms of involuntary, clonic, and synchronous twitching of all facial muscles on the affected side. EDX studies provide information on lesion location and severity, pathophysiology underlying the two disorders, and differential diagnosis between syndromes presenting with abnormal facial muscle activity. This monograph is intended to describe the most relevant EDX findings in the two disorders and the most appropriate timing for the examinations in order to provide useful information for prognosis and therapeutic decision-making.     

A case of Klippel-Trenaunay-Weber syndrome accompanied by congenital hydrocephalus and micropolygyria]

We report a patient with the Klippel-Trenaunay-Weber syndrome accompanied by congenital hydrocephalus, which was slowly progressive and an Ommaya's reservoir was set up. However, the hydrocephalus remained stable even when the shunt was removed due to infection. Generalized tonic clonic convulsions had appeared from six months after birth and were treated with valproic acid. The electroencephalogram showed hypsarrhythmia. He died at eight months of age. Autopsy revealed extensive micropolygyria of the bilateral cerebral hemispheres and hydrocephalus. To our knowledge, it is rare for the Klippel-Trenaunay-Weber syndrome to be accompanied by congenital hydrocephalus, and there has been no previous report of its occurrence with micropolygyria.     

小剂量A型肉毒毒素治疗颜面部痉挛的临床分析

目的 探讨小剂量A型肉毒毒素(BTX-A)多点注射治疗颜面部痉挛的临床疗效及安全性.方法 回顾性分析BTX-A注射治疗颜面部痉挛患者的资料,评估疗效起止时间、症状缓解程度、不良反应发生率及其严重程度.结果 80例患者接受BTX-A注射治疗.平均起效时间(3.2±1.6)d,达高峰时间(7.2±2.2)d,疗效维持时间(19.6±6.1)周.达到完全缓解50例(62.5％)、明显缓解26例(32.5％)、部分缓解4例(5％).14例(17.5％)患者出现轻度不良反应.结论 小剂量BTX-A多点注射能够明显改善颜面部痉挛患者的症状,不良反应轻.     

A case of paraneoplastic syndrome accompanied by two types of cancer

A case of paraneoplastic syndrome accompanied by two types of cancer is reported. The patient was a 62 year old man who progressively developed cerebellar ataxia, especially an abnormal gait. The anti-Hu antibody titre was high. A small tumour was detected in the middle lobe of the right lung and was surgically treated. The histology was adenocarcinoma. After lobectomy, however, the ataxia deteriorated, and plasma exchange, 250 ml/kg/day, was conducted for 6 days. After plasma exchange, the anti-Hu antibody titre decreased and the ataxia temporarily ceased to progress. A week after the last plasma exchange, a mass appeared in the anterior cervical region and rapidly increased in size. The biopsy of the neck tumour disclosed a small cell carcinoma. Five months later small cell carcinoma appeared in the left lung. This case shows the importance of searching for small cell carcinoma when anti-Hu antibodies are detected. It is assumed that plasma exchange removed not only a pathogenic factor of ataxia but also a factor which inhibited the growth of the small cell carcinoma. It is recommended that plasmapheresis should be performed with caution in paraneoplastic syndrome when the origin of a tumour is obscure.