Myoclonic cerebellar dyssynergia (Ramsay Hunt). Description of 2 cases

The authors report two cases of dyssynergia cerebellaris myoclonica in siblings. Apart from typical clinical manifestations attention is called to the results of computerized tomography of the brain which demonstrated cerebellar atrophy. This helped in verification of the diagnosis. Comparing these cases with other ones described earlier in the literature the authors conclude that dyssynergia cerebellaris myoclonica is not a separate nosological entity but a syndrome associated with myoclonia.     

肌阵挛性小脑协调障碍一例报告及文献复习

患者女,32岁,因"四肢抖动5年,发作性四肢抽搐伴青语障碍1年"于2008年6月30号入上海交通大学医学院附属仁济医院神经内科诊治.患者于5年前无明显诱因出现四肢不对称、急速、短时的不自主抽动,常因体位改变、做精细动作、声光刺激、睡眠差、情绪改变时诱发或加剧,曾在外院诊治,服用中药及抗免疫药物(具体不详)无好转.     

Sleep abnormalities in four cases of dyssynergia cerebellaris myoclonica of Ramsay-Hunt

The nocturnal sleep of four patients with dyssynergia cerebellaris myoclonica (DCM) of Ramsay-Hunt was recorded with a polygraph. The following features were observed: a reduction of spindles, K complexes and vertex spikes; frequent arousals; rare rapid eye movements with a modification of their morphology and pattern; change in sleep stage percentages. In addition, myoclonus and polyspike-and-wave complexes appeared less frequently during sleep than during wake fulness. Three generalized convulsive and sixteen clonic seizures were recorded during stage 3/4 or on arousal. The clinical and physiopathological implications of these data are discussed.

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Sommario Il sonno notturno di quattro pazienti affetti da Dissinergia cerebellare mioclonica di Ramsay-Hunt è stato registrato tramite poligrafo. Sono state osservate le seguenti caratteristiche: riduzione dei fusi del sonno, dei complessi K e delle punte al vertice; frequenti risvegli; riduzione notevole dei movimenti oculari rapidi con modificazioni della loro morfologia; modificazioni della percentuale dei vari stadi del sonno. Inoltre le mioclonie e le corrispondenti polipunte-onda comparivano meno frequentemente durante il sonno rispetto ai periodi di veglia. Sono state registrate tre crisi convulsive generalizzate e sedici crisi cloniche durante lo stadio 3–4 del sonno o al risveglio. Vengono discusse le implicazioni cliniche e fisiopatologiche di questi dati.

     

Atypical case of the Ramsay-Hunt syndrome

The authors report a case of Ramsay-Hunt syndrome during cervical zoster in the C2--C4 area. The case reserves attention in view of the fact that facial nerve paralysis and paralysis of both parts of the right acoustic nerve occurred as a second exacerbation of the disease 16 days after the onset. Permanent hearing impairment in the right ear after regression of facial nerve paralysis and paralysis of the vestibular part of the acoustic nerve were also exceptionally rare signs.     

Normal muscle mitochondrial function in Ramsay-Hunt syndrome

Mitochondrial encephalomyopathies may dispaly clinical features similar to Ramsay-Hunt syndrome (RHS). We studied muscles mitochondrial function in 2 patients with RHS. Histochemical and ultrastructural studies of muscle biopsies and biochemical analysis of muscle mitochondrial enzymes were normal. There is no evidence for a disturbance of muscle mitochondrial function in RHS.

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Sommario Le encefalomiopatie mitocondriali possono presentare caratteristiche cliniche simili alla sindrome di Ramsay-Hunt (SRH). Abbiamo studiato la funzione mitocondriale muscolare in 2 pazienti con SRH. Lo studio ultrastrutturale, istochimico e biochimico eseguito su materiale muscolare ottenuto tramite biopsia del tricipite è risultato normale. Non sembra perciò esistere evidenza di un disturbo mitocondriale muscolare nella SRH.

     

30例Ramsay-Hunt综合征临床分析

目的探讨Ramsay-Hunt综合征的药物治疗以及影响预后的因素。方法回顾性分析2015-01—2019-01在我科诊治的30例Ramsay-Hunt综合征患者的临床资料,面神经功能评估采用House-Brackman分级。结果所有患者于发病1周内就诊,可见耳甲腔或外耳道疱疹,同侧面瘫均Ⅲ级及以上,其中伴声嘶2例,伴眩晕5例,伴面部疱疹4例。经过2周药物治疗后,2例伴声嘶患者面神经功能为Ⅲ级,1例治疗后2周复发,其余患者面神经功能均恢复正常。结论Ramsay-Hunt综合征早期药物治疗面瘫恢复较好,但累及迷走神经者预后较差。