Varicella with delayed contralateral hemiparesis detected by MRI

We report a 3 1/2-year-old boy who developed a hemiparesis 4 weeks after the onset of a varicella infection. In previously described cases of varicella with delayed contralateral hemiparesis, computed tomography typically revealed infarcts in the basal ganglia and internal capsule. To our knowledge, our patient is the first studied by magnetic resonance imaging. The pathogenesis of this syndrome may be a varicella zoster virus-related vasculopathy similar to that observed in herpes zoster ophthalmicus with delayed contralateral hemiparesis.     

Cerebral vasculopathy associated with primary varicella infection

A previously healthy 5-year-old boy developed cerebral vasculopathy, presenting as two episodes of acute hemiparesis 3 and 9 months, respectively, after a primary varicella infection (chickenpox). This association has not been reported before, to our knowledge, although cerebral vasculopathy is a well-known complication of herpes zoster ophthalmicus. The diagnosis was based on the presence of oligoclonal varicella-specific IgG in the cerebrospinal fluid and angiographic findings. Clinical and angiographic follow-up, and serial thymidine kinase activity levels in the cerebrospinal fluid suggested a self-limiting course of the virus-induced vasculopathy. Varicella zoster virus seems to be another potential causative agent to be considered in acute childhood hemiplegia.     

Acute hemiplegia with lacunar infarct after varicella infection in childhood

We report 4 cases of acute hemiplegia and a small low-density lesion on computerized tomography (CT) after varicella infection. In 3 of them, CT in the acute hemiplegic stage, and later, reveals the development of lacunar infarct around the internal capsule. Focal low density may be caused by occlusive vascular lesions of the penetrating arteries. Varicella infection may play an important role as one of the causes of acute hemiplegia in childhood producing lacunar infarct, as well as delayed hemiplegia, reported previously in herpes zoster ophthalmicus.     

Herpes zoster ophthalmicus and delayed contralateral hemiparesis caused by cerebral angiitis: Diagnosis and management approaches

Four patients with herpes zoster ophthalmicus and delayed contralateral hemiparesis are described, and their findings are compared with those in patients previously reported in the English language literature. The current patients evidenced multifocal ipsilateral cerebral angiitis by angiography and multifocal infarcts in the distribution of the ipsilateral middle cerebral artery by computed tomographic scanning. Cerebrospinal fluid showed mononuclear pleocytosis, positive oligoclonal bands, and an elevated immunoglobulin G index. Two patients were treated with corticosteroids and acyclovir, and 1 with corticosteroids alone, all without apparent response. Necrotizing angiitis ipsilateral to the herpes zoster ophthalmicus was demonstrated postmortem in 1 patient with multifocal cerebral infarction and progressive leukoencephalopathy. Neither herpes varicella zoster immunocytochemical reactivity nor viral inclusions were seen. The leukoencephalopathy associated with herpes varicella zoster either may be caused by cerebral angiitis or, as previously reported, may be a temporally remote manifestation of persistent herpes varicella zoster infection. The cerebral angiitis associated with herpes varicella zoster is histologically similar to granulomatous angiitis, and both may be related to herpes varicella zoster infection of the cerebral vasculature.     

Acute hemiplegia associated with herpes zoster infection in children: report of one case

Herpes zoster infection has been rarely reported to cause angiitis of the central nervous system in children. We describe a 4-year, 8-month-old female with acute hemiplegia and central facial palsy 6 weeks after she had had zoster ophthalmicus. The findings of magnetic resonance angiography, the clinical picture, and a preceding history of herpes zoster ophthalmicus suggested zoster vasculitis. Herpes zoster vasculitis is thus another consideration when examining a child with acute hemiplegia and a recent herpes zoster infection.     

Postvaricella acute transverse myelitis in a previously vaccinated child

We describe a 14-year-old boy with acute transverse myelitis after breakthrough varicella infection, despite immunization with the Varicella zoster virus vaccine 8 years earlier. He recovered fully after treatment with intravenous corticosteroids and acyclovir. To our knowledge, there are no previously reported cases of postvaricella acute transverse myelitis in vaccinated individuals. Our report emphasizes that the Varicella zoster virus booster vaccine may be necessary to prevent not only acute varicella, but also its postinfectious neurologic complications.     

Granulomatous angiitis following primary varicella zoster infection

Granulomatous angiitis is an uncommon but well recognized complication of varicella zoster infection. The classic presentation is of a delayed hemiparesis in an often immunocompromised patient following zoster ophthalmicus. This report outlines the case of a young woman who developed a large left middle cerebral artery territory infarct secondary to granulomatous angiitis during primary varicella zoster infection.     

多发性硬化血清麻疹、风疹和水痘-带状疱疹病毒IgG抗体水平及临床意义

目的通过检测多发性硬化(Multiple sclerosis,MS)患者血清中麻疹病毒、风疹病毒和水痘-带状疱疹病毒的免疫球蛋白G(Immunoglobulin G,IgG)抗体浓度,并进行扩展残疾状态量表(Expanded disability status scale,EDSS)评分,探讨上述病毒在MS发病中的临床意义及其与临床神经功能缺失的相关性。方法收集2013年9月-2015年2月郑州大学第一附属医院神经内科收治的50例MS患者急性期(复发期)的血清标本,其中临床孤立综合征(CIS亚组)22例,复发-缓解型MS(RRMS亚组)28例,对照组收集同一时期我院体检结果正常的37例健康志愿者的血清标本。采用双抗体夹心酶联免疫吸附方法(Enzyme linked immunosorbent assay,ELISA)测定并比较血清中3种病毒IgG抗体的浓度,分析血清中各病毒抗体水平与EDSS评分的相关性。结果 (1)MS病例组麻疹病毒、风疹病毒、水痘-带状疱疹病毒IgG抗体的血清浓度高于对照组,差异具有统计学意义(P0.05);(2)CIS亚组患者血清中麻疹病毒、风疹病毒、水痘-带状疱疹病毒IgG抗体水平低于RRMS亚组,EDSS评分亦低于RRMS亚组,差异均具有统计学意义(P0.05);(3)MS病例组血清中麻疹病毒、风疹病毒、水痘-带状疱疹病毒IgG抗体与EDSS评分无相关性(P0.05)。结论 MS患者血清中的麻疹病毒、风疹病毒和水痘-带状疱疹病毒可能不仅与MS的发病相关,而且与疾病的复发有关。     

Reactivation of varicella presenting as pseudotumor cerebri: three cases and a review of the literature

The possible association of pseudotumor cerebri and varicella infection was previously mentioned in a few case reports. In those cases, the history and clinical features of active varicella were obvious, and signs were directly related to the varicella infection. We describe three immunocompetent children with pseudotumor cerebri as the only manifestation of Varicella zoster virus reactivation, with a review of the literature. We suggest considering Varicella zoster virus in children with pseudotumor cerebri, even in the absence of a history of recent varicella infection.     

Optic neuritis heralding varicella zoster virus retinitis in a patient with acquired immunodeficiency syndrome

We report on a 29-year-old severely compromised acquired immunodeficiency syndrome patient who developed retrobulbar optic neuritis 5 weeks after an episode of cutaneous herpes zoster infection. During the optic neuritis, varicella zoster virus could be demonstrated in the cerebrospinal fluid. The neuritis responded well to treatment with foscarnet, but, 3 weeks into therapy, varicella zoster retinitis developed. Additional treatment with intravenous acyclovir stopped progression of the retinitis and resulted in healing of the retinal lesions. This case suggests that retrobulbar optic neuritis can be regarded as a prodrome of imminent acute retinal necrosis. Early recognition and prompt therapy with combined antivirals may prevent the development of this devastating ocular complication of varicella zoster infection.     

Sympathetic contralateral vestibulopathy after unilateral zoster oticus

A unique case of initially right sided varicella zoster induced Ramsay-Hunt syndrome with complete vestibular loss is reported. The patient subsequently developed deficits of the left vestibule 5 months later. An autoimmune pathogenesis of the left vestibular failure rather than bilateral varicella zoster infection was suggested by the following data: (1) no evidence of vesicular eruptions on the left auricle and the virtual absence of antiviral antibodies after onset of bilateral vestibulopathy; (2) prompt response of the left vestibule to immunosuppressive therapy with corticosteroids; and (3) presence of atypical nervous tissue specific autoantibodies against a 45 kDa protein.     

Multiple ischemic infarcts in a child with AIDS, varicella zoster infection, and cerebral vasculitis

A 4 1/2-year-old girl with acquired immunodeficiency syndrome and prolonged varicella zoster virus skin infection developed multiple ischemic strokes and radiologic and histopathologic evidence of central nervous system vasculitis. Typical features of acquired immunodeficiency syndrome encephalitis were not present and there was no evidence of vasculitis outside the nervous system. Central nervous system vasculitis probably resulted from varicella zoster virus infection that persisted because of immunodeficiency. This acquired immunodeficiency syndrome complication has only rarely been described in adults and to our knowledge has not been described in children.     

The Pathophysiology of Essential Tremor and Parkinson’s Tremor

Varicella zoster virus (VZV) is an exclusively human neurotropic alphaherpesvirus. Primary infection causes varicella (chickenpox), after which the virus becomes latent in ganglionic neurons along the entire neuraxis. With advancing age or immunosuppression, cell-mediated immunity to VZV declines, and the virus reactivates to cause zoster (shingles), dermatomal distribution, pain, and rash. Zoster is often followed by chronic pain (postherpetic neuralgia), cranial nerve palsies, zoster paresis, vasculopathy, meningoencephalitis, and multiple ocular disorders. This review covers clinical, laboratory, and pathological features of neurological complications of VZV reactivation, including diagnostic testing to verify active VZV infection in the nervous system. Additional perspectives are provided by discussions of VZV latency, animal models to study varicella pathogenesis and immunity, and of the value of vaccination of elderly individuals to boost cell-mediated immunity to VZV and prevent VZV reactivation.     

Recurrent hemiplegia associated with cerebral vasculopathy following third trimester maternal herpes zoster infection

The chickenpox virus (varicella zoster virus; VZV) is known to cause large and small vessel central nervous system vasculopathies that may be associated with strokes in both adults and children. We present the case of a female aged 2 years 6 months who developed a chronic progressive small-vessel vasculopathy with radiological features of moyamoya disease as a manifestation of congenital varicella syndrome. Clinically, the condition was characterized by recurrent ischaemic strokes, which were brought under control using intravenous acyclovir. The case is unique in that it is the first report of congenital varicella syndrome to occur after a maternal herpes zoster infection. Furthermore, it is the first case of symptomatic VZV infection in a child to occur after a maternal infection occurring in the third trimester of pregnancy.     

Herpes zoster myelitis: Nervous system complications

Transverse myelitis after a zoster viral infection is an exceptional occurrence. In this case we documented an antibody activity against varicella, zoster (V-Z) in the cerebrospinal fluid in association with Coxsackie A enterovirus. The clinical course was atypical because of a second episode of myelitis 2 years after the first episode of neurologic viral complications. The positive outcome of therapy with Acyclovir is noteworthy. This case was retrieved in a review of neurologic complications observed in the last few years in patients affected by zoster in our hospital.

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Sommario In un caso di mielite trasversa sono stati documentati antigeni contro il virus varicella zoster (V-Z) e contemporaneamente è stata individuata la presenza di un enterovirus coxsackie A. nel liquor. Si segnala il decorso anomalo dell' affezione per un episodio omologo di mielite recidivante dopo 2 anni. Il trattamento con Acyclovir è risultato molto utile. Il caso viene inserito, in una review di complicanze neurologiche osservate in questi ultimi anni in pazienti ricoverati in Istituto per deficit neurologici da virus (V-2).

     

Case of zoster sine herpete presenting with dysphagia diagnosed by PCR analysis of VZV DNA in auricular skin exudates]

A 66-year-old woman was admitted to our hospital because of hoarseness and dysphagia after right earache and pharyngalgia. She showed right glossopharyngeal nerve and vagus nerve palsies, but no other neurological deficits. There was no skin rash within the regions of her ear, oral cavity, pharynx and larynx. Slight increase of mononuclear cells was noted in the cerebrospinal fluid. MR brain imaging was normal. We diagnosed her as zoster sine herpete (ZSH) and treated her with acyclovir, after which she almost completely recovered. The examination of antibodies and DNA of varicella zoster virus (VZV) in the serum and cerebrospinal fluid revealed a pattern of previous zoster infection without evidences of reactivation. However, VZV DNA was detected in auricular skin exudates with PCR. We conclude that PCR analysis of VZV DNA in auricular skin exudates can be a useful diagnostic tool for the diagnosis of zoster sine herpete presenting with painful glossopharyngeal nerve and vagus nerve palsies.     

Long-segment transverse myelitis in an immunocompetent patient due to varicella zoster virus reactivation

Transverse myelitis is a segmental, full-thickness inflammation of the spinal cord with various etiologies including varicella zoster virus infection. Symptoms can manifest as sensory deficits, severe lower back pain radiating down the legs, bowel/bladder incontinence, and extremity weakness. This case report is unique in that it highlights a patient who developed long-segment transverse myelitis after a varicella zoster virus infection which has not been previously reported in the literature.

     

Neurovirulence of varicella and the live attenuated varicella vaccine virus

Varicella-zoster virus (VZV) is a neurotropic herpesvirus, which can cause a variety of complications during varicella infections. These range from meningoencephalitis to polyneuritis to retinitis. After primary VZV infection, VZV enters the dorsal root ganglia in a latent state. Reactivation from latency leads to zoster. The velocity of VZV is 13 cm per day, as the virus travels from ganglion to skin. The live attenuated varicella vaccine virus is markedly less neurovirulent than the wild-type virus. Nevertheless, a few cases of herpes zoster due to the vaccine virus have been documented. Usually, herpes zoster occurs in the same arm as the vaccination, often 3 or more years after vaccination. Thus, herpes zoster in a vaccinee often represents a reactivation of vaccine virus that was carried to the cervical dorsal root ganglia from a site of local replication in the arm. Finally, the role of autophagy during VZV infection is discussed. Autophagosome formation is a prominent feature in the skin vesicles during both varicella and herpes zoster. Therefore, autophagy is one of the innate immune mechanisms associated with VZV infection in humans.     

Association of a history of varicella virus infection with multiple sclerosis

Objective

To analyze the association of a previous history of varicella virus infection with multiple sclerosis (MS) and its subtypes.

Material and methods

We performed a case–control study including 126 cases and 157 controls. Subjects were divided into subgroups according to MS subtype and the history of varicella virus infection along with other variables was assessed.

Results

History of varicella zoster virus (VZV) infection was positive in 42% of controls and 66% of MS cases (p ≤ 0.001). Patients with a history of VZV infection had a threefold risk increase of having MS. Regarding MS subtypes, relapsing-remitting (RR) MS had four times the risk and secondary progressive had a threefold increase in risk when compared with control patients.

Conclusions

An association between varicella infection history and MS was found, particularly in the RR subtype.     

Striatal encephalitis after varicella zoster infection complicated by Tourettism.

We describe a case of encephalitis after primary varicella zoster infection with localised basal ganglia imaging abnormalities. The patient subsequently developed a chronic tic disorder with attention deficit disorder. This case furthers the proposed association between Tourettism and the basal ganglia.