[Ramsay Hunt syndrome with hearing loss and vertigo]. / Sindrom Ramseia-Khanta s kokhleovestibulopatiei.

The article contains literature review on etiology, natural history and treatment of Ramsay Hunt syndrome. Differential diagnosis in case of 8th cranial nerve involvement is discussed. We present a case of the patient with Ramsay Hunt syndrome with hearing loss and vertigo is described. Clinical symptoms and diagnosis of sensorineural hearing loss and acute unilateral vestibulopathy are presented. The successful treatment of the patient resulted in complete facial nerve recovery, hearing improvement and partial recovery of vestibular function.

Magnetic Resonance Imaging Evidence of Varicella Zoster Virus Polyneuropathy: Involvement of the Glossopharyngeal and Vagus Nerves Associated With Ramsay Hunt Syndrome.

The involvement of lower cranial nerve palsies is less frequent in Ramsay Hunt syndrome caused by varicella zoster virus (VZV). The authors report 1 of extremely rare patients of radiologically proven polyneuropathy of VZV infection with magnetic resonance imaging findings of VII, IX, and X cranial nerve involvement is a 62-year-old female patient, who initially presented with Ramsay Hunt syndrome. Varicella zoster virus infection should be considered even in patients who show unilateral palsy of the lower cranial nerves associated with laryngeal paralysis. Thin-section T2W and T1W images with a contrast agent should be added to the imaging protocol to show the subtle involvement.

Benefits of High-Dose Corticosteroid and Antiviral Agent Combination Therapy in the Treatment of House-Brackman Grade VI Ramsay Hunt Syndrome.

OBJECTIVE: Few large-scale investigations have been conducted on treatment of House-Brackmann grade VI (HB grade VI) Ramsay Hunt syndrome (RHS) patients. We compared recovery rates among patients receiving a normal-dose corticosteroid (prednisolone [PSL] 60 mg/d) or high-dose corticosteroid (PSL 200 mg/d), both with or without an antiviral agents. Recovery rates were also examined based on the order of presentation of herpetic vesicles versus facial palsy. STUDY DESIGN: Retrospective case review. SETTING: Tertiary referral center. PATIENTS: A total of 128 patients with HB grade VI RHS were treated in our department between 1995 and 2017. These patients were divided into four treatment groups based on corticosteroid dosage and use of an antiviral agent. METHODS: We assessed treatment outcomes for HB grade VI patients together with logistic regression analysis to investigate factors that can impact treatment outcomes, that is, sex, age, days to start of treatment, PSL dosage, and antiviral agent administration. RESULTS: Recovery rates were best in the high-dose corticosteroid group with an antiviral agent (71.1%) in comparison with the normal-dose corticosteroid group with an antiviral agent (60.0%) or high-dose corticosteroid alone (57.1%). Significant factors for treatment outcomes were high-dose corticosteroid administration and early initiation of treatment. A better recovery rate was also found when the herpetic vesicles appeared before facial palsy. CONCLUSION: We showed that a combination of a high-dose corticosteroid and antiviral agent produced the best outcomes for patients with HB grade VI RHS. However, our results were not statistically significant because of small sample size.

For Whom the Bell's Toll: Recurrent Facial Nerve Paralysis, A Retrospective Study and Systematic Review of the Literature.

PURPOSE: To examine the etiology, clinical course, and management of recurrent peripheral facial nerve paralysis. METHODS: Retrospective review at a single tertiary academic center and systematic review of the literature. Clinical presentation, laboratory and imaging findings, treatment and outcome for all cases of recurrent ipsilateral, recurrent contralateral, and bilateral simultaneous cases of facial paralysis are reviewed. RESULTS: Between 2000 and 2017, 53 patients [41.5% men, 29 median age of onset (range 2.5 wk-75 yr)] were evaluated for recurrent facial nerve paralysis at the authors' institution. Twenty-two (41.5%) cases presented with ipsilateral recurrences only, while the remaining 31 patients (58.5%) had at least 1 episode of contralateral recurrent paralysis. No cases of bilateral simultaneous facial nerve paralysis were observed. The median number of paretic events for all patients was 3 (range 2-20). The median nadir House-Brackmann score was 4, with a median recovery to House-Brackmann grade 1.5 over a mean recovery time of 61.8 days (range 1-420 d). Diagnostic evaluation confirmed Melkersson-Rosenthal syndrome in four (7.5%) cases, neurosarcoidosis in two (3.7%), traumatic neuroma in one (1.9%), Ramsay Hunt syndrome in one (1.9%), granulomatosis with polyangiitis in one (1.9%), and neoplastic causes in three (5.7%) cases [facial nerve schwannoma (n = 2; 3.7%), metastatic squamous cell carcinoma to the deep lobe of the parotid gland (n = 1; 1.9%)]; ultimately, 77.4% (41) of cases were deemed idiopathic. Facial nerve decompression via a middle cranial fossa approach was performed in three (5.7%) cases without subsequent episodes of paralysis. CONCLUSION: Recurrent facial nerve paralysis is uncommon and few studies have evaluated this unique population. Recurrent ipsilateral and contralateral episodes are most commonly attributed to idiopathic facial nerve paralysis (i.e., Bell's palsy); however, a subset harbor neoplastic causes or local manifestations of underlying systemic disease. A comprehensive diagnostic evaluation is warranted in patients presenting with recurrent facial nerve paralysis and therapeutic considerations including facial nerve decompression can be considered in select cases.

[An Autopsy Case of Meningoencephalitis and Cerebral Infarction that Developed with Ramsay Hunt Syndrome and Disseminated Herpes Zoster].

We report here the clinical presentation and subsequent autopsy of a 90-year-old man who developed small papules with pain and swelling in his right ear. On admission, he exhibited right facial nerve paralysis, neck stiffness and Kernig's sign. The cell count was elevated and the varicella-zoster virus-PCR was positive in the CSF. Brain magnetic resonance imaging showed hyperintense lesions in the left pons and left temporal lobe, in FLAIR images. We diagnosed the patient with Ramsay Hunt syndrome and meningoencephalitis due to varicella-zoster virus. Although the symptoms of meningitis improved following treatment with intravenous acyclovir (750 mg/day initially, raised to 1,125 mg/day), 16 days after admission, he died suddenly due to gastrointestinal hemorrhage. The autopsy findings included lymphocytic infiltration of the leptomeninges and perivascular space of the cerebrum, and slight parenchyma in the left temporal lobe and insula, as the main histological features. Encephalitis due to varicella zoster virus has been recognized as a vasculopathy affecting large and small vessels. Pathological confirmation is rare in varicella zoster virus meningoencephalitis.

Delayed transmastoid facial nerve decompression surgery in patients with Ramsay-Hunt syndrome presenting with neurophysiologically complete paralysis.

OBJECTIVES: To determine the efficacy of delayed transmastoid facial nerve decompression in patients with Ramsay Hunt Syndrome (RHS) presenting with complete facial paralysis. METHODS: Twenty-five RHS patients with complete facial nerve paralysis presenting electroneuronographic (ENoG) degeneration ≥90% underwent transmastoid facial nerve decompression more than 3 weeks after the onset of paralysis. The principal features measured were 12 months pre- and post-operative House-Brackmann (HB) grades and the presence of a direct intraoperative neural response (INR) prior to decompression procedure. Correlations between these parameters, and the time between symptom onset and surgery (within or later than 30 and 50 d) were statistically analyzed. RESULTS: Of the 25 patients 13 (52%) exhibited good recovery (HB grade I or II) at 12 months-post-operatively. The timing of decompression generally did not significantly influence outcome but patients treated within 50 d of symptom onset enjoyed better outcomes than those treated later (p = .047). The presence of an INR significantly influenced outcomes (p = .0003). CONCLUSIONS: The success of delayed transmastoid facial nerve decompression in RHS patients was not affected between 25-30 and 30-40 d from symptom onset but was compromised when the delay was >50 d. The presence or absence of an INR was a good predictor of post-operative prognosis.

Ramsay Hunt Syndrome with Multiple Cranial Neuropathy in an Human Immunodeficiency Virus (HIV) Patient.

BACKGROUND Ramsay Hunt syndrome is a rare otologic complication resulting from varicella zoster virus reactivation that can present with a myriad of clinical presentations. Most common being triad of ear pain, vesicles at auricle, and ear canal with same side facial palsy. CASE REPORT We report a case of a 29-year-old male with a human immunodeficiency virus (HIV) infection who presented with left facial palsy, vesicles, pain in the left ear, dysphagia, dizziness, and headache resulting from multiple cranial nerves involvement such as cranial nerve V, VII, VIII, IX, and X. CONCLUSIONS This case report raises awareness among general practitioners to investigate for Ramsay Hunt syndrome in HIV patients presenting with ear pain with a thorough neurological exam and emphasize on the interplay of different specialties in managing these patients.

[A case report: Ramsay Hunt syndrome with throat as starting place merger ipsilateral cranial nerver involvement].

The clinical data of a case of Ramsay Hunt syndrome concurrent with ipsilateral Ⅴ, Ⅶ, Ⅷ, Ⅸ, Ⅹ, Ⅺ cranial nerves paralysis with throat as starting place was retrospectively analyzed, and the relevant literatures were also reviewed. The case is rare, so the relevant clinical reports are less. It is important to take the objective data as well as subjective symptoms of the patients into consideration to make a definite diagnosis, so that we can treat it as soon as possible to achieve better curative effect.

Symptomatic myoclonus.

A huge number of neurological disorders are associated with myoclonus. This paper describes these disorders whose diagnosis partly relies on the presence of myoclonus. The diagnostic approach is related to certain clinical features of myoclonus, which, after their integration in the clinical context, help orientate towards diagnosis. Myoclonus is frequent during dementia. Although its presence is well-known to take part in the diagnosis of Creutzfeldt-Jakob disease (CJD), myoclonus can also be present to a significant degree in Alzheimer's disease and Lewy body dementia (LBD), which raises a diagnostic issue. Both its clinical and electrophysiological features may help differential diagnosis, given that myoclonus with fast-evolving dementia and focal neurological signs should favor the diagnosis of CJD. Myoclonus in a context of progressive ataxia suggests one clinical form of the Ramsay-Hunt syndrome (progressive myoclonic ataxia, PMA), whose most frequent causes are: coeliac disease, mitochondriopathies, some spino-cerebellar degenerations, and some late metabolic disorders. In addition to ataxia and myoclonus, the presence of opsoclonus directs diagnosis toward the opsoclonus-myoclonus syndrome (OMS), whose origin, in adult, is idiopathic or paraneoplastic. Palatal tremor (myoclonus) with ataxia may represent either a sporadic pattern, which often reflects the evolution of degenerative or lesional disorders, or a familial pattern in some degenerative affections or metabolic diseases. Of more recent knowledge is the association of progressive ataxia, myoclonus, and renal failure, which corresponds to a recessive autosomic disease. In a context of encephalopathy, myoclonus is frequent in metabolic or hydro-electrolytic disorders, and in brain anoxia. One should distinguish these various forms of myoclonus which may occur in the acute post-anoxic phase, from those occurring as sequels at a later stage, i.e. the Lance and Adams syndrome whose clinical aspects are also multiple. Myoclonus is less frequent during toxic or drug exposures. Irrespective of its acute or insidious onset, Hashimoto's encephalopathy is accompanied by myoclonus and tremor. Myoclonus may also be present during encephalic and/or spinal infectious disorders. Myoclonus with focal neurological signs may be observed in thalamic lesions, responsible for unilateral asterixis or unilateral myoclonus superimposed on dystonic posture. Segmental spinal myoclonus or propriospinal myoclonus may be associated with several spinal-cord disorders. Myoclonus associated with peripheral nerve lesions is exceptional or even questionable for some of these.

CSF antigliadin antibodies and the Ramsay Hunt syndrome.

Although the association between celiac disease and progressive myoclonic ataxia is well recognized, in each of the reported cases the neurologic features began in middle adult life and usually in patients who had clinical or laboratory evidence of malabsorption. We report a case of progressive myoclonic ataxia and epilepsy (Ramsay Hunt syndrome) that began in childhood. In this patient there were no features suggestive of gluten intolerance. The presence of antigliadin antibodies in the serum and CSF suggested celiac disease was the cause of the patient's neurologic syndrome. Duodenal morphologic abnormalities reversed with treatment but no major changes were noted in the patient. Celiac disease should be considered in the differential diagnosis of myoclonic ataxia at any age, even in the absence of clinical evidence of gluten-sensitive enteropathy.

Acetazolamide therapy improves action myoclonus in Ramsay Hunt Syndrome.

Acetazolamide treatment significantly improves action myoclonus in Ramsay Hunt Syndrome. A family with two brothers and a sister, and a sporadic case with Ramsay Hunt Syndrome and uncontrollable action myoclonus, are described where addition of oral acetazolamide lead to marked improvement in their action myoclonus.

Acyclovir responsive brain stem disease after the Ramsay Hunt syndrome.

We report an immunocompetent patient with the Ramsay Hunt syndrome (RHS) followed days later by brainstem disease. Extensive virological studies proved that varicella zoster virus (VZV) was the causative agent. Treatment with intravenous acyclovir resulted in prompt resolution of all neurological deficits except peripheral facial palsy. This case demonstrates that after geniculate zoster, brainstem disease may develop even in an immunocompetent individual and effective antiviral therapy can be curative.

Acetazolamide improves action myoclonus in Ramsay Hunt syndrome.

The myoclonus of two patients with Ramsay Hunt syndrome was only partially controlled under treatment with clonazepam, sodium valproate, primidone, and piracetam. Acetazolamide (200 mg daily) was added to these drugs, resulting in a dramatic improvement. Placebo substitution (one patient) and withdrawal of acetazolamide in the other patient resulted in marked aggravation of the myoclonus. The mechanism of action of acetazolamide in myoclonus is unknown. Acetazolamide may be an additional therapeutic possibility for patients with severe action myoclonus.

Action myoclonus, Ramsay Hunt syndrome, and other cerebellar myoclonic syndromes.

Action myoclonus, reviewed in this chapter, is the term applied to arrhythmic muscular jerking induced by voluntary movement. It is made worse by attempts at precise or coordinated movement (intention myoclonus) and may also be provoked by certain sensory stimuli. The effective stimuli for action myoclonus is probably feedback from muscle afferents, although it may be initiated by corollary discharge from motor cortex to reticular formation before or at the onset of voluntary movement. The condition is usually associated with diffuse neuronal disease such as post-hypoxic encephalopathy, uremia, and the various forms of PME, although action myoclonus may be limited to one limb in some cases of focal cerebral damage. It is caused by hyperexcitability of the sensorimotor cortex (cortical reflex myoclonus) or reticular formation (reticular reflex myoclonus), or both. No consistent pathological change has been reported in autopsied cases of action myoclonus. The underlying disorder appears to be a loss of inhibitory mechanisms involving serotonin and possibly GABA as transmitter agents. The term PME is used for the association of myoclonus with degenerative changes in the nervous system which are commonly diffuse but may predominate in certain systems. There may or may not be associated tonic-clonic seizures, other manifestations of epilepsy, or dementia. Those cases of PME associated with Lafora inclusion bodies and cerebral storage diseases can be distinguished from the system degenerations. Systems which may be involved in the latter group include cerebellodentatorubral, pyramidal, extrapyramidal, optic, auditory, posterior columns and gracile and cuneate nuclei, spinocerebellar pathways, motor neurons of cranial nerves and anterior horns, and muscle fibers. Confronted with this diversity of pathological change, it seems unnecessary to make any clinical distinction between Ramsay Hunt syndrome and Unverricht-Lundborg syndrome (Baltic myoclonus) because cerebellar signs are found in patients described under both headings. Additional systems may be involved in individuals or families who are otherwise typical. All three names could well be joined in an eponymous title (Unverricht-Lundborg-Hunt disease) or the condition simply known as the systems degeneration type of PME, as Halliday (43) suggested. The cause of the condition (or spectrum of conditions) is at present unknown. Action myoclonus usually responds to sodium valproate or clonazepam, and some individuals, particularly those with posthypoxic myoclonus, improve with the administration of serotonin precursors.

[Herpes zoster oticus -- neuropathologic contribution to the genesis of concomitant facial paralysis (author's transl)]. / Herpes zoster oticus. Neuropathologischer Beitrag zur Genese der Begleitenden Facialisparese.

A woman of 71 years suffered from herpes zoster oticus, 7th and 10th nerve paralysis, vertigo and hearing loss; she died after 5 weeks. Neuropathologic examination revealed intensive inflammation in the pons and medulla oblongata and necrotizing arteritis in the cerebello-pontine angle, predominantly on the clinically affected side. The adjacent facial nerve was severely damaged. For the first time, necrotizing arteritis appears as important cause of facial paralysis in the Ramsey-Hunt syndrome.

[Interaction between erythromycin and carbamazepine]. / Een interactie tussen erytromycine en carbamazepine.

A six-month-old girl receiving erythromycin was given carbamazepine treatment. Two days later signs of carbamazepine intoxication developed and a serum carbamazepine concentration of 30 mg/l was found (4-9 mg/l being the desired therapeutical level). The interaction of erythromycin and carbamazepine by liver enzyme competition is clinically significant.

[Early stellate ganglion block failed to prevent progress of facial nerve palsy in a patient with Ramsay-Hunt syndrome--a case report].

A 73-year-old man complained of pain in his right ear with vesicular lesion for three days. He complained of no weakness of facial musculatures, but muscle test revealed slight weakness in orbital and oral muscles on admission. His hearing acuity of the left ear was intact. Vertigo with spontaneous nystagmus to left was complained. The patient was treated with stellate ganglion block four times a day, prednisolone 80 mg a day p.o. and acyclovir i.v. Mannitol solution 300 ml a day for eight days was given i.v. to reduce edema and to protect facial nerve. On his 3rd hospital day, his paralysis progressed and he could not close his eye or whistle with his mouth. Evoked myogram showed 91% impairment of the nerve. During recovery period there was discrepancy between facial palsy score of clinical signs and degree of nerve impairment by evoked myogram. On his 12th hospital day his facial nerve score improved 9 to 12 (0 complete paralysis, 40 no paralysis) but evoked myogram showed further progress of nerve impairment from 86% to 91% (Evoked wave heights were 14% and 9% of normal site respectively). The discrepancy is probably because facial palsy score reflects also neurapraxia of inflammatory nerve, and stellate ganglion block has no effect on changing course of facial nerve injury.

[Soft palate myoclonus and opsoclonus in disorders of cerebrovascular circulation in the vertebrobasilar system]. / Miokloniia miagkogo neba i opsoklonus pri narusheniiakh mozgovogo krovoobrashcheniia v vertebra'lno-baziliarnoi sisteme.

In 4 patients with residual cerebral circulation disorders in the vertebrobasilar vascular bed, the myoclonus of soft palate, pharynx, larynx, tongue (in 2 cases comprising opsoclonus) developed among other signs of brainstem or cerebellar lesions. Myoclonic hyperkinesia emerged after 1.5 to 4 months of the disease, not in its acute period, and was not related to second stroke. With focal neurological signs analyzed, the syndrome of the soft palate myoclonus++ plus opsoclonus appeared in connection with the lesions of upper cerebellar crus where dento olivary++ pathway and the fibers connecting the cerebellar flocculus with oculomotor nuclei run. Soft palate myoclonus++ without opsoclonus emerges with lesions of cerebellar dental nucleus and central pathway of the brainstem tegmentum.