Varicella infection-induced cases, once the most common single Acute cerebellar ataxia, acute cerebellitis, and opsoclonus-myoclonus syndrome. J Child Department With Acute Ataxia in the Post-Varicella Vaccine Era. Acute post-infectious cerebellar ataxia is the most common cause of About 20 % of cases have been linked to varicella (chicken pox), but it. Post-viral cerebellar ataxia also known as acute cerebellitis and acute cerebellar ataxia (ACA) Viral infections that may cause it include the following: chickenpox, Coxsackie disease (viral infection also called hand-foot-and-mouth disease).

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Acute cerebellitis AC is the most common neurological complication of cerrbelitis. Nevertheless, it has been scarcely studied. The objective of this study were varicfla asses the occurrence of AC among children hospitalized for varicella and to analyze its specific clinical picture and outcome. We retrospectively reviewed the medical records of children admitted to the hospital for varicella between 1 st October and 1 st June and we compared our results with literature.

Children were all unvaccinated for varicella. In our case series, AC was found out in 48 out of patients The highest frequency of AC was observed in children from 1 to 5 years of age The most characteristic symptom of AC was a broad-based gait disturbance that progressed gradually over the course cerebeitis a varricela days Other common symptoms included slurred speech After a long hospitalization median of 11 daysall but one children were dismissed without invalidating sequelae.

Data from this study may help to better address the problem of varicella cerebellar complications in hospitalized children and to monitor changes over time caused by an increase in vaccination coverage.

Post viral cerebellar ataxia

Varicella is an acute, exanthematous, and highly infectious disease affecting virtually every child in the absence of vaccination programs. Varicella has mostly an uncomplicated course in early childhood. Nevertheless, it may result in severe complications [ 1 ]. A common measure of varicella complications is derived pist the hospitalization of patients. In a year study of varicella hospitalizations, we previously assessed the occurence of central nervous system complications, comparing our results with those from the literature.

Among neurological complications, acute cerebellitis AC was the most frequent manifestation [ 2 ]. However, the occurrence of AC complicating varicella in hospitalized children and its detailed clinical characteristics are unknown. The objectives of this varicelq were to assess the occurrence of AC out of neurological complications of varicella in hospitalized children and to define its specific clinical picture and final cerebeliris.

Patients over 18 years of age as well as children with immunodeficiency disorders were excluded.

Acute cerebellitis in varicella: a ten year case series and systematic review of the literature

According to the literature, the diagnosis of varicella is based on clinical evidence of characteristic skin lesions in varying stages of development and resolution.

We defined a neurological complication as an unfavorable neurological evolution occurring within three weeks of varicella onset [ 3 ]. AC was defined by clinical findings ataxia, unsteady gait or fine motor movement, trembling of the head and trunk in an upright position and the extremities when attempting to move against gravity [ 4 – 6 ]. The diagnosis was clinical as the onset of ataxia following the appearance of typical chickenpox rash requires no further diagnostic testing [ 7 ].

Real time PCR for varicella zoster virus was positive in all cerebrospinal fluid samples. The results were limited to publications written in English, concerning the pediatric age 0—18 years and published during the period between June and June We scanned the references of all included articles for additional studies.


Inclusion criteria for our systemic review of the literature were: From each article analyzed we extrapolated the total number of varicella cases, the number of cases with neurological complications and the number of cases with cerebellar involvement. Out of these, 17 studies were eligible for inclusion in the meta-analysis and were analyzed to ;ost a pooled rate of cerebellar complications in the pediatric age [ 1468 – 21 ].

We excluded articles for the following reasons: Finally, two studies referred to the same group of patients. Data were analyzed using Metanalysis 3 and a pooled estimate of the occurrence of varicella complications was calculated by using a random-effects model with inverse-variance weighting using the Der-Simonian and Laird method.

Statistical heterogeneity was measured by using the chi square test for heterogeneity. We used the Chi square test or the Fisher exact test for comparing proportions. We used the Standard T test for comparing continuous variables. We reviewed charts of children with varicella ppost during the study period. Neurological complications were identified in 92 out of patients The mean age of patients affected by neurological complications was 5.

The most frequent complication among neurological complications was AC The mean age of patients affected by cegebelitis complications was 5. The highest frequency of cases was observed in children aged 1 to 5 vadicela Children aged 5—10 years were They were all unvaccinated for varicella.

The most characteristic symptom of AC was cerwbelitis broad-based gait disturbance that progressed gradually over the course of the first two days recovery In a few cases, irritability 8. Tone, deep tendon reflexes and plantar responses were normal in all patients.

The mean time of onset for cerebellar symptoms was 7. In four patients neurological symptoms developed in the first two days. Only two children presented with late-onset complications after 14 days and within 21 days of varicella exanthema appearance. In no case did cerebellar symptoms precede skin lesions.

The median length of the hospital stay was 11 days range 2—23 days. Treatment was decided independently case by case. In three cases, antiviral therapy was not prescribed as these children previously received oral acyclovir at home for seven days from the onset of varicella.

Moreover, 16 patients CT was normal in all patients; MRI showed a hyperintense signal of the cerebellar gray matter in T2-weighted sequences in five cases. The mean time of follow up was 3. Only one patient presented invalidating sequelae. No cases of developmental retardation, dysarthria, hemiparesis, epilepsy, blindness, deafness or coordination disorders were reported.

Diffuse high signal intensity of both cerebellar hemispheres with prevalence on the right lobe black arrows. AC, the most common neurological complication of varicella, occurs about once in varicella cases among children. In our case series, the proportion of AC out of the total varicella cases was Reviewing the occurence of varicella AC complications in scientific published reports, the proportion of complications is slightly higher than those described by other authors, as shown by the metanalysis.

Therefore, the results that we obtained reflect a scenario not affected by immunization. Indeed, none of the patients included had been vaccinated against varicella. In our case series, the median age of children affected by AC was about 5. In the literature, the median age of children affected by AC was 4. Moreover, in our case series, children with AC were significantly older than the other children hospitalized for varicella median age 3.


They were also older than children affected by other neurological complications median age 5. Instead in a previous report in which children affected by AC median age 4.

This is in line with the literature, in which the median time between the onset of exanthema and hospital admission was 7 days. The hospitalization of our patients was longer than those reported in the literature 6. This may be due to the fact that four children had a complicated disease course, which required steroids for more than 14 days.

At admission, ataxia was the most frequent symptom, with wide-based gait Neurological presentation was also often characterized by dysmetry and difficult speech.

Clinical Practice Guidelines : Ataxia

Vomiting and cephalea were frequent, while nystagmus or other involuntary eye movements were rare. Moreover, non cerebellar symptoms, such as headache, were frequently referred by patients. In the literature reviewed, we did not find any description of the clinical presentation for cerebellitis, which would have been useful to compare with our data [ 5 ]. In our case-series, diagnosis was made based on patient history of varicella infection and physical examination. In fact, the onset of ataxia following the appearance of a typical chickenpox rash requires no further diagnostic testing.

Anyway, the result of these tests did not change the treatment. Brain imaging is not necessary for most cases of AC.

In fact, CT is of limited value given the difficulty of imaging the posterior fossa with this modality. Moreover, when obtained, CT is most often normal [ 26 ]. At MRI, bilateral diffuse abnormalities poet the cerebellar hemispheres are the most common imaging presentations but are not patognomonic and with a no evident prognostic value [ 27 ]. The role of antiviral therapy is controversial.

Some authors reported that acyclovir is indicated because of disease severity, while others varicel not recommend it, based on the strength of evidence regarding autoimmune pathogenesis [ 22 – 252829 ].

The real utility of steroids is controversial as well [ 28 ]. In our case-series, forty-five patients We prescribed antiviral therapy in order to reduce disease severity; in fact, in a recent article on varicella, treatment with antivirals was considered mandatory not only for patients at risk for severe disease, but also for any subject with varicella-zoster virus infection with virally mediated complications, such as AC [ 29 ].

In the revised literature, we found just two papers reporting the frequency of antiviral therapy and only one on the steroids [ 14 ]. Out of 67 children, Rack et al. Finally, as well as in our case-series, other authors generally did not refer invalidating problems at the follow-up [ 114 ]. AC is frequent during varicella in childhood and is associated with prolonged hospitalization. Neurological presentation is mostly characterized by ataxia, difficult speech, vomiting, headache and dysmetry.

BE provided medical assistance to the patient and collected medical information, BM and CV revised the literature, LD supervised the neuroradiological examination included in the case report, TAE supervised the examination examination included in the case report, KA was involved in the clinical follow-up of the patient, VA supervised the patient treatment plan. All authors read and approved the final manuscript. National Center for Biotechnology InformationU.

Journal List Ital J Pediatr v.