Varicella with delayed hemiplegia

doi:10.1016/0887-8994(90)90124-J

Pediatric Neurology

Volume 6, Issue 4, July–August 1990, Pages 279-281

https://doi.org/10.1016/0887-8994(90)90124-J Get rights and content

Abstract

We report 4 children who developed acute hemiplegia 7 weeks to 4 months after varicella infection. In 2 patients, carotid angiography demonstrated segmental narrowing and occlusion of the middle cerebral artery. Their clinical and angiographic features were similar to those associated with contralateral hemiplegia after herpes zoster ophthalmicus, the pathogenesis of which comprises cerebral angiitis due to varicella zoster viral infection. We believe that our patients had the same pathogenesis. In a survey of infectious diseases in our region, the frequency of varicella with delayed hemiparesis was roughly 1:6,500 varicella patients.

References (13)

S Cope et al.
Hemiplegia complicating ophthalmic zoster
Lancet
(1954)
I Eda et al.
Acute hemiplegia with lacunar infarct after varicella infection in childhood
Brain Dev
(1983)
DN Bourdette et al.
Herpes zoster ophthalmicus and delayed ipsilateral cerebral infarction
Neurology
(1983)
DC Hilt et al.
Herpes zoster ophthalmicus and delayed contralateral hemiparesis caused by cerebral angiitis: Diagnosis and management approaches
Ann Neurol
(1983)
D Eidelberg et al.
Thrombotic cerebral vasculopathy associated with herpes zoster
Ann Neurol
(1986)
CC Linnemann et al.
Pathogenesis of varicella-zoster angiitis in the CNS
Arch Neurol
(1980)

There are more references available in the full text version of this article.

Cited by (46)

Post-varicella Arterial Ischemic Stroke in Denmark 2010 to 2016
2018, Pediatric Neurology
Citation Excerpt :
Post-varicella AIS has been described in several case series,2,18-22 but comparable population-based incidence estimates of post-varicella AIS are lacking in the literature. Ichiyama et al. estimates one of 6,500 children with varicella based on a report of four children,22 whereas Askalan et al. mentions an absolute risk of one of 15,000 children with varicella.6 In the International Pediatric Stroke Study, PVA was found in 3.6% of children with AIS, but some of the enrolled children were from countries with a varicella vaccination program in place and the study populations might not be comparable.23
Varicella, most often a benign disease of childhood, is associated with an increased risk of arterial ischemic stroke in children. The aim of the present study was to estimate the incidence of post-varicella arterial ischemic stroke in the Danish child population and describe clinical characteristics of children admitted with post-varicella arterial ischemic stroke.
In the Danish National Patient Register, we identified inpatients 28 days to 16 years of age with a discharge diagnosis of stroke or cerebrovascular disease from 2010 to 2016. Medical files were reviewed, and children with arterial ischemic stroke and varicella infection less than 12 months before onset of symptoms were included.
We identified 15 children with arterial ischemic stroke and varicella less than 12 months before. In nine children, the diagnosis was confirmed by detection of varicella zoster virus DNA or varicella zoster virus immunoglobulin G in the cerebrospinal fluid. All children were previously healthy, the mean age was four years, and 67% were male. The median time from varicella rash to arterial ischemic stroke was 4.6 months. The most common location of arterial ischemic stroke was the basal ganglia, and affected vessels were most often in the anterior circulation. Fifty-three percent experienced neurological sequelae of varying degree.
In Denmark, where varicella vaccination is not part of the childhood vaccination program, the estimated risk of post-varicella arterial ischemic stroke was one case (including possible cases) per 26,000 children with varicella.
Mechanisms of pediatric cerebral arteriopathy: An inflammatory debate
2013, Pediatric Neurology
Citation Excerpt :
A relationship between stroke and Varicella zoster has been recognized for many years [36]. Many case reports have implied an association between recent Varicella zoster infection and focal arteriopathy [37-44]. Pleocytosis [42,43,45] or Varicella zoster antigens [37,40,41,43] in the cerebrospinal fluid of patients with stroke and recent infection with Varicella zoster have been described.
Arteriopathy is the leading cause of childhood arterial ischemic stroke, but its mechanisms are poorly understood. This review explores the possible role of inflammatory mechanisms and evidence for inflammatory pathophysiology in specific pediatric cerebral arteriopathies. Pathologically proven small-vessel central nervous system vasculitis provides a definitive inflammatory model where available treatments are likely improving outcomes. In contrast, a common large-vessel arteriopathy presents many features suggestive of inflammation, but definitive proof remains elusive. Recent advances and future research directions, including biomarker, neuroimaging, and pathologic approaches and how they might address these important clinical questions, are discussed.
A case of intracranial saccular aneurysm after primary varicella zoster virus infection
2012, Brain and Development
Citation Excerpt :
The frequency of varicella with delayed hemiplegia, a common clinical feature of PVA, is estimated roughly as 1:6500 varicella patients in Japan. Therefore, PVA associated with primary VZV infection is apparently rare in immunocompetent children [6]. In fact, PVA is thought to result from direct infection of the vessels with varicella, predisposition to inflammatory changes, or from a postviral immune-mediated reaction [3].
We report a case of intracranial saccular aneurysm that developed 3 years after post-varicella ischemic stroke. A 6-year-old girl without apparent immunologic defects presented with right hemiparesis and expressive aphasia 1 month after chickenpox. Her magnetic resonance imaging scans revealed left basal ganglia infarction because of left lenticulostriate artery occlusion. Although her neurologic symptoms improved gradually, segmental irregular narrowing remained in the A1 and M1 segments of the left anterior and middle cerebral arteries, respectively. Three years later, the follow-up magnetic resonance angiography indicated saccular aneurysm in the anterior communicating artery and the anti-VZV IgG antibody index in the cerebrospinal fluid was elevated. Subclinical reactivation of VZV and the segmental vascular narrowing might cause intracranial aneurysm, even in immunocompetent children.
Acquired, induced and secondary malformations of the developing central nervous system
2007, Handbook of Clinical Neurology
Citation Excerpt :
In addition to the vascular lesions, trans‐synaptic spread of the varicella/zoster virus from infected neurons in the visual system has been demonstrated (Rostad et al., 1989), but this type of spread in the fetus is not well documented. Even in older children with postnatally acquired varicella, acute or delayed onset of hemiparesis may occur secondary to narrowing and occlusion of the internal carotid or middle cerebral arteries (Liu and Holmes, 1990; Ichiyama et al., 1990), because of the endothelial vascular lesions induced by the virus, just as in the fetus. Neurosensory hearing loss is the most frequent clinical manifestation of congenital rubella but a diffuse congenital meningoencephalitis, multicystic degeneration of white matter and dystrophic calcifications may occur, particularly if the time of the infection is early in gestation (Reef et al., 2000).
Most malformations of the brain are genetic disorders, but cerebral dysgeneses can also be acquired in vitro as secondary impairments of developmental processes. The most frequent causes of these acquired malformations are fetal hypoxia, ischemia or hypoperfusion, and congenital infections. The principal mechanism is congenital infection. Cytomegalovirus (CMV) induces cerebral dysgenesis less by direct viral invasion of neurons and glia than by viral involvement of the endothelial cells of cerebral blood vessels, leading to impaired perfusion and multiple infarcts. In the fetal brain, infarcts may damage radial glial fibers that guide migratory neuroblasts and glioblasts. Other damage to the developing brain may be because of toxins, including alcohol and teratogenic drugs, maternal malnutrition, trauma to the fetus, and fetal exposure to ionizing radiation. When fetal or infant brains are demonstrated by neuroimaging to exhibit pachygyria, lissencephaly, schizencephaly, and subcortical heterotopia, the cause is not necessarily a genetic mutation; hence, the identification of nongenetic causes is of great importance in genetic counseling.
Cerebrovascular disease and varicella in children
2006, Brain and Development
Varicella-associated stroke has been reported with increasing frequency in recent years. In many cases, diagnosis is difficult because of the late onset of manifestations after the acute infectious episode. Four cases of cerebrovascular disease after varicella infection were observed. Three children presented hemiparesis and one facial paresis. The neuroradiological findings comprised stenosis/occlusion of middle cerebral artery or nucleo capsular signal alteration. Because, several pathogenetic mechanisms have been proposed as the cause of stroke, the relationship between prothrombotic conditions, antipospholipid antibodies and stroke in these patients is discussed. The difficulty in defining the pathogenesis of the ischemic episode is related to problems in the choice of antithrombotic treatment, which is still not standardized and must be decided on individual basis. In the event of rapid onset of stroke after exanthem high dose antiviral therapy seems to be justified. On the basis of our experience and of literature data on varicella-associated stroke, we recommend that VZV infection be taken into account in every episode of stroke in children.
Pediatric stroke: The child is not merely a small adult
2005, Neuroimaging Clinics of North America

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Case reportVaricella with delayed hemiplegia

Abstract

Lancet

Brain Dev

Herpes zoster ophthalmicus and delayed ipsilateral cerebral infarction

Neurology

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

Ann Neurol

Thrombotic cerebral vasculopathy associated with herpes zoster

Ann Neurol

Pathogenesis of varicella-zoster angiitis in the CNS

Arch Neurol

Case report
Varicella with delayed hemiplegia