Varicella-zoster virus-specific cell-mediated immunity in Ramsay Hunt syndrome.

OBJECTIVES/HYPOTHESIS: The etiology of Ramsay Hunt syndrome (Hunt syndrome) is reactivation of latent varicella-zoster virus (VZV) in the geniculate ganglion of the facial nerve, leading to neuritis. Although the mechanism of the VZV reactivation is unclear, one possibility is that the reactivation involves a low level of VZV-specific cell-mediated immunity (CMI). The aim of this study was to clarify the characteristics of the VZV-specific CMI in Hunt syndrome compared to that in Bell's palsy, and to obtain clues to its role in the development of Hunt syndrome. STUDY DESIGN: Prospective study. METHODS: We determined the median spot numbers and examined VZV-specific CMI in patients with Hunt syndrome and with Bell's palsy using interferon-γ enzyme-linked immunospot (ELISPOT) assays. We analyzed the relationship between the value of VZV-specific CMI and days from disease onset. RESULTS: The median spot number in Hunt syndrome (87.3 spot-forming cells [SFCs]/4 × 10(5) peripheral blood mononuclear cells [PBMCs]) was higher than that in Bell's palsy (62.3 SFCs/4 × 10(5) PBMCs). Hunt syndrome showed a strong relationship between the ELISPOT count and days from onset (r = 0.65). Within the first 5 days from onset, no ELISPOT counts higher than 80 SFCs/4 × 10(5) PBMCs were observed. On the other hand, no correlation was observed between the ELISPOT count and days from onset in patients with Bell's palsy (r = -0.19). CONCLUSIONS: These results suggest that VZV-specific CMI in Hunt syndrome is low at disease onset and increases rapidly thereafter. Consequently, reduced VZV-specific CMI may play an important role in the reactivation of VZV in the facial nerve, leading to Hunt syndrome.

A rare case of Ramsay Hunt syndrome following temporomandibular joint surgery.

Surgical approaches to the temporomandibular joint (TMJ) have been designed specifically to minimize injury to the temporal branch of the facial nerve. In spite of this, facial nerve dysfunction occurs in 1-32% of patients undergoing TMJ surgery. Ramsay Hunt syndrome is characterized by peripheral facial paralysis that often involves other cranial nerves, mostly cranial nerve VIII. The pathology is attributed to the reactivation of latent varicella zoster virus in the geniculate ganglion. The diagnosis is based mostly on history and physical findings. Surgical procedures have been known to reactivate varicella zoster virus, but Ramsay Hunt syndrome subsequent to TMJ surgery has not been described yet. This report describes a case of Ramsay Hunt syndrome associated with TMJ surgery. Because of the relatively high incidence of facial nerve dysfunction associated with TMJ surgery, patients with varicella zoster virus reactivation may initially be misdiagnosed with iatrogenic facial palsy, or vice versa.

Atypical Ramsay Hunt syndrome.

Ramsay Hunt syndrome (RHS) is the reactivation of herpes zoster in the geniculate ganglion and typically presents the triad of ipsilateral peripheral type facial paralysis, ear pain, and erythematous vesicles in the external auditory canal and auricle. However, some unusual variants may occur. Here we present a patient of atypical RHS with uncommonly extensive dermatomal involvement of cranial nerve (CN) V2 and V3 and cervical roots, C2, C3 in addition to CN VII and VIII involvement.

Atrofia cerebelosa secundaria a cerebelitis aguda por Mycoplasma pneumoniae / Cerebellar atrophy following acute mycoplasma pneumoniae cerebellitis

Introducción. La cerebelitis aguda es una complicación infrecuente de la infección por Mycoplasma pneumoniae. Los pocos pacientes descritos han seguido un curso benigno y autolimitado de forma similar al de las cerebelitis relacionadas con otros gérmenes. Casos clínicos. Describimos dos pacientes con una cerebelitisaguda por M. pneumoniae que evoluciona en pocos meses hacia una marcada atrofia cerebelosa. Los pacientes presentaron un síndrome cerebeloso caracterizado por ataxia, hipotonía, disartria y dismetría de las cuatro extremidades, precedido por signos de infección respiratoria. La resonancia magnética (RM) cerebral practicada durante la fase aguda en el caso 1 resultó normal y e nel caso 2 demostró un llamativo edema del parénquima cerebeloso, con IV ventrículo pequeño y ventriculomegalia supratentorial, que se autolimitó y que no requirió de intervención neuroquirúrgica. Los estudios serológicos permitieron confirmar la infección reciente por M. pneumoniae. En el caso 1 se ha observado la persistencia de signos de disfunción cerebelosa, mientras que el caso 2 está asintomático. Ambos pacientes muestran una llamativa atrofia cerebelosa en las RM cerebrales practicadas a lo largo de su control clínico. Conclusiones. Debe considerarse la infección por M. pneumoniae en pacientes con cerebelitis aguda que presenten resolución incompleta de la disfunción cerebelosa o que evolucionen de forma precoz hacia la atrofia cerebelosa. Las manifestaciones neurorradiológicas iniciales no permiten predecir el pronóstico neurológico final (AU)

Introduction. Acute cerebellitis is an uncommon complication following Mycoplasma pneumoniae infection. A benign and self-limited course has been described in the few reports found of this association. Case reports. We report twopatients with an apparently M. pneumoniae-induced acute cerebellitis that resulted in cerebellar atrophy. Patients presented with a cerebellar syndrome including ataxia, hypotonia, dysarthric speech and dysmetria, which were preceded by signs of respiratory infection. Initial brain magnetic resonance imaging (MRI) in case 1 was normal but in case 2 it displayed striking cerebellar swelling, small fourth ventricle and supratentorial ventriculomegaly which was self-limited and did not requiren euro surgical intervention. Serological studies confirmed a recent M. pneumoniae infection. Case 1 has followed an unfavourable clinical course, with incomplete resolution of cerebellar dysfunction, while case 2 has remained asymptomatic. Follow-up brain MRI have demonstrated prominent cerebellar atrophy in both patients. Conclusions. M. pneumoniae infection should be considered in those patients with acute cerebellitis showing an incomplete resolution of cerebellar dysfunction or those who develop early cerebellar atrophy. The presenting MRI findings do not seem to predict final neurological outcome (AU)

Early-onset cerebellar ataxia, myoclonus and hypogonadism in a case of mitochondrial complex III deficiency treated with vitamins K3 and C.

A 16-year-old girl presented with early-onset cerebellar ataxia, myoclonus, elevated lactic acidosis and hypogonadotropic hypogonadism. Muscle biopsy specimens revealed fibres with a "ragged" appearance with increased mitochondria and lipid droplets. Biochemical investigation revealed a deficiency of complex bc1 (complex III) of the mitochondrial respiratory chain. Genetic analysis did not show either deletions or known mutations of mitochondrial DNA (mtDNA). Phosphorus magnetic resonance spectroscopy (31P-MRS) showed defective energy metabolism in brain and gastrocnemius muscle. A decreased phosphocreatine (PCr) content was found in the occipital lobes accompanied by normal inorganic phosphate (Pi) and cytosolic pH. These findings represented evidence of a high cytosolic adenosine diphosphate concentration and a relatively high rate of metabolism accompanied by a low phosphorylation potential. Muscle 31P-MRS showed a high Pi content at rest, abnormal exercise transfer pattern and a low rate of PCr post-exercise recovery. These findings suggested a deficit of mitochondrial function. Therapy with vitamins K3 and C normalized brain 31P-MRS indices, whereas it did not affect muscle bioenergetic metabolism. In this patient, the endocrinological disorder is putatively due to a mitochondrial cytopathy. Although an unknown mtDNA mutation cannot be ruled out, the genetic defect may lie in the nuclear genome.

Preserved simple and impaired compound movement after infarction in the territory of the superior cerebellar artery.

A patient with an infarct in the distribution of the right superior cerebellar artery was studied with regard to his ability to make simple movements (visually triggered, self-terminated ballistic wrist movements), and compound movements (reaching to a visual target and precision pinch of a seen object). Movements on the right side of the body alone were affected. Control movements were made by the normal left upper extremity. Wrist movement on the right side was normal in reaction time, direction, peak velocity, and end-point position control as compared to the left. By contrast, both reaching and pinching movements on the right were impaired. Reaching movements showed marked decomposition of the compound elbow-shoulder movement into seriatim simple movements made alternately at elbow and shoulder. Pinching movements were not made, and instead winkling movements (a movement of index alone) were substituted. These results are compared to similar results of controlled inactivation of the cerebellar dentate nucleus in monkeys. We conclude that one function of the cerebellum may be to combine elements in the movement repertoires of downstream movement generators. When that ability is lost, a strategy may be voluntarily adopted of using the preserved simple movements in place of the impaired compound movements.

Cerebellorubral degeneration after resection of cerebellar dentate nucleus neoplasms: evaluation with MR imaging.

The authors describe the magnetic resonance (MR) findings in seven patients who developed severe cerebellar symptoms and atrophy of the contralateral red nucleus following removal of unilateral neoplasms in the deep nuclei of the cerebellum. For most patients, pre- and postoperative spin-echo MR images were obtained with long repetition times (TRs) at 1.5 T. The long TR images obtained before surgery demonstrated unilateral masses involving the dentate nucleus. Long TR images obtained after surgical resection of the dentate nucleus showed increased signal intensity in all of seven contralateral red nuclei, three of seven ipsilateral superior cerebellar peduncles, and two of seven contralateral inferior olivary nuclei. Three other patients who underwent surgery for cerebellar neoplasms without resection of the dentate nuclei showed no postoperative brain stem changes on MR images. The authors speculate that the changes in the contralateral red nuclei are due to cerebellorubral degeneration (since well-described neural tracts interconnect the dentate nucleus and the contralateral red nucleus). Injury of the dentate nucleus may result in degeneration of distant neural connections.

[Equilibrium disorders and ataxia as dominating symptoms in a case of Alzheimer's disease]. / Zaburzenia równowagi i niezbornosc jako dominujace objawy w przypadku choroby Alzheimera.

A case of a 66-year-old female woman with Alzheimer's disease (confirmed histologically) is reported. In the case cerebellar symptoms predominated without evidence of dementia. The patient died after duodenal haemorrhage.

Importance of anticomplement immunofluorescence antibody titration for diagnosing varicella-zoster virus infection in Bell's palsy.

Anticomplement Immunofluorescence was used for antibody titration against varicella-zoster virus (VZV) in 43 patients with peripheral facial palsy. Nine of 31 patients (29%) with Bell's palsy and eight of 12 patients (75%) with Ramsey-Hunt syndrome had anticomplement immunofluorescence antibody titres of greater than or equal to 1/10. On the other hand, none of 14 patients with herpes simplex virus (HSV) infection and 51 healthy adults showed anticomplement immunofluorescence antibody titres of greater than or equal to 1/10. The anticomplement immunofluorescence antibody titre in two patients with Ramsey-Hunt syndrome increased later and decreased sooner than the indirect immunofluorescence antibody titre, becoming undetectable at 66 and 104 days, respectively, after onset of the disease. There was no cross reaction between anti-VZV and anti-HSV antibodies in the patients who showed a positive antibody rise for VZV. As the acute stage of VZV infection is obscure in the patients with peripheral facial palsy without herpes the screening of anticomplement immunofluorescence antibody to VZV at titres greater than or equal to 1/10 may be useful for the diagnosis of VZV infection in patients with peripheral facial palsy.

Herpes zoster: complications pathogenesis and pathology. The specialist view.

The term herpes zoster is derived from the Greek herpes: to creep and zoster: a belt or girdle. Shingles is from the Latin cingere: to gird, which was corrupted to mean a belt of girdle for the human form. A typical attack of herpes zoster is usually not difficult to recognise, but it is important to be aware of uncommon manifestations and complications.

[Tick-borne encephalitis and epilepsy]. / Kleshchevoi éntsefalit i épilepsiia.

In patients suffering from tick-borne encephalitis development of epileptic seizures, Kozhevnikov's epilepsy, Jackson's epilepsy, myoclonus-epilepsy, chorea-epilepsy Hunt's myoclonic dyssynergy and various hyperkineses without general convulsions was observed. It was found that a particular place in the structure of the convulsive syndrome was occupied by Kozhevnikov's epilepsy which was not so frequently encountered in other diseases. This is, probably, due to a particular vulnerability of the motor structures, mainly, at the spinal level, at which the final motor response is formed.