Cerebellar Mutism and Reversible Cytotoxic Edema in Influenza B–Associated Encephalopathy

doi:10.1016/j.pediatrneurol.2013.06.014

Pediatric Neurology

Volume 49, Issue 6, December 2013, Pages 489-492

https://doi.org/10.1016/j.pediatrneurol.2013.06.014 Get rights and content

Abstract

Background

Influenza virus–associated neurological complications are rare, though well-documented, especially in children. Encephalopathy and seizures are the most common complications and are typically associated with influenza A infection. Cerebellar mutism has been rarely reported in association with influenza B infection.

Patient

We describe a 3-year-old boy who presented with cough, fever, altered mental status, seizure, hypotonia, and mutism. He tested positive for influenza B virus. His brain magnetic resonance imaging showed reversible cytotoxic edema limited to the middle cerebellar peduncles and the dentate nuclei. Other viral, vascular, toxic, and metabolic causes were ruled out.

Conclusion

Our patient represents a case of cerebellar mutism associated with influenza B encephalopathy in which the brain magnetic resonance imaging scan showed reversible cytotoxic edema limited to the middle cerebellar peduncles and the dentate nuclei. This clinicoradiological correlation supports other reports in which the dentate nuclei play a major rule in the pathogenesis of cerebellar mutism.

Introduction

During the 2012-2013 influenza season, many areas of the United States experienced a high rate of influenza virus activity.¹ Children younger than 5 years of age and with chronic diseases including neurological and neurodevelopmental disorders are at higher risk. Influenza infection–associated neurological complications are rare, though well-documented, especially in children. In most instances, these neurological complications are associated with influenza A infection.2, 3 However, influenza B has also been linked to encephalopathy.⁴ We describe a child with influenza B encephalopathy who developed acute cerebellar mutism. His brain magnetic resonance imaging (MRI) showed a reversible cytotoxic edema of the middle cerebellar peduncles and the dentate nuclei. This presentation has not been reported with influenza B infection in this context. Influenza virus–associated neurological complications, the reversibility of the cytotoxic edema, and the pathogenesis of the cerebellar mutism and its association with influenza virus are discussed.

Section snippets

Case Report

A 3-year-old previously healthy boy presented with a 5 day history of fever, cough, and fatigue. He was initially off balance with increasing weakness and refused to talk 2 days later. He was diagnosed with otitis media and began amoxicillin. The next day, he had three episodes of rhythmic jerking of his arms while sleeping. Subsequently, he was transferred to our pediatric intensive care unit with acute encephalopathy with ataxia and seizures. His vaccinations were up-to-date except for a the

Discussion

The most common neurological complications associated with influenza infection are seizures and encephalopathy. Less common neurological complications include Guillain-Barré syndrome, transverse myelitis,⁵ posterior reversible encephalopathy syndrome, intracranial vasculopathy,⁶ acute disseminated encephalomyelitis, and cerebellar mutism.7, 4 Encephalopathy could range from mild confusion and altered mental status to severe coma. Acute necrotizing encephalopathy is a severe form of

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Cited by (12)

Cerebellar mutism syndrome
2018, Handbook of Clinical Neurology
Citation Excerpt :
Mutism was not described initially as part of the CCAS spectrum (Schmahmann and Sherman, 1998; Levisohn et al., 2000); however, the cognitive and behavioral findings in the acute stage and at follow-up strongly suggest that CMS is actually an extreme and hyperacute form of the CCAS spectrum (Bodranghien et al., 2016; Catsman-Berrevoets, 2017). The development of CMS is not exclusive to children who have undergone surgical procedures for cerebellar tumors (Coplin et al., 1997; Nishikawa et al., 1998; Papavasiliou et al., 2004; Baillieux et al., 2007; Frassanito et al., 2009; Siffert et al., 2000; Al-Anazi et al., 2001; Parrish et al., 2010; Ellis et al., 2011; Muthappan et al., 2012; (Marien et al., 2013; Thabet et al., 2013; Erol et al., 2014; McAndrew et al., 2014; Kariyattil et al., 2015), but it most commonly develops in the context of surgery for pediatric midline intraventricular and cerebellar tumors in the posterior fossa, the most common such entity being medulloblastoma. Therefore, most of our current knowledge about the mechanism of CMS is derived from studies performed on cohorts of children receiving surgery for medulloblastoma.
Cerebellar mutism most commonly, but not exclusively, develops in children after surgery for midline cerebellar or intraventricular tumors in the posterior fossa, typically medulloblastoma. Cerebellar mutism syndrome (CMS) comprises a complex set of neurologic and neurocognitive signs and symptoms, the cardinal and central component of which is an initially profound but usually reversible speech disorder. As such, CMS is currently recognized as an extreme form of the so-called cerebellar cognitive affective syndrome (Schmahmann syndrome). The putative cause of CMS is a substantial surgical injury to the proximal components of the bilateral efferent cerebellar pathways, disrupting cerebellar input to the supratentorial brain. The resultant cerebellocerebral diaschisis may lead to supratentorial cortical perfusion depression with frontal predominance. The speech disorder is, therefore, likely an apraxia. As our understanding of the mechanism and the clinical spectrum of CMS evolves, clinically useful preoperative risk stratification schemes, adjustments to surgical strategies and techniques, and possible early therapeutic-rehabilitative measures are being sought and developed to reduce the burden of this severe and particularly handicapping chronic morbidity on affected individuals and their families.
MR imaging of adult acute infectious encephalitis
2017, Medecine et Maladies Infectieuses
Citation Excerpt :
Brain MRI may be normal or show non-specific changes within the cortex, white matter, basal ganglia, brain stem and cerebellum. Patients present with acute necrotizing encephalopathy [80–84]. Reversible splenial lesions have been reported [83,85].
Imaging is a key tool for the diagnosis of acute encephalitis. Brain CT scan must be urgently performed to rule out a brain lesion with mass effect that would contraindicate lumbar puncture. Brain MRI is less accessible than CT scan, but can provide crucial information with patients presenting with acute encephalitis.
We performed a literature review on PubMed on April 1, 2015 with the search terms “MRI” and “encephalitis”.
We first described the various brain MRI abnormalities associated with each pathogen of acute encephalitis (HSV, VZV, other viral agents targeting immunocompromised patients or travelers; tuberculosis, listeriosis, other less frequent bacterial agents). Then, we identified specific patterns of brain MRI abnomalies that may suggest a particular pathogen. Limbic encephalitis is highly suggestive of HSV; it also occurs less frequently in encephalitis due to HHV6, syphillis, Whipple's disease and HIV primary infection. Rhombencephalitis is suggestive of tuberculosis and listeriosis. Acute ischemic lesions can occur in patients presenting with severe bacterial encephalitis, tuberculosis, VZV encephalitis, syphilis, and fungal infections.
Brain MRI plays a crucial role in the diagnosis of acute encephalitis. It detects brain signal changes that reinforce the clinical suspicion of encephalitis, especially when the causative agent is not identified by lumbar puncture; it can suggest a particular pathogen based on the pattern of brain abnormalities and it rules out important differential diagnosis (vascular, tumoral or inflammatory causes).
L’imagerie cérébrale est essentielle au diagnostic d’encéphalite aiguë. Le scanner cérébral doit être réalisé en urgence afin d’exclure une lésion cérébrale avec effet de masse qui contre-indiquerait la ponction lombaire. L’IRM cérébrale est moins accessible que le scanner, mais peut apporter des informations importantes en cas d’encéphalite aiguë.
Revue de la littérature sur PubMed le 1^er avril 2015 avec les mots clés « IRM » et « encéphalite ».
Nous décrivons les différentes anomalies IRM associées à chaque agent pathogène de l’encéphalite aiguë (HSV, VZV, autres virus ciblant les patients immunodéprimés ou voyageurs ; tuberculose, listériose, autres agents bactériens moins fréquents). Nous identifions des patterns spécifiques d’anomalies IRM qui peuvent orienter vers un pathogène particulier. L’encéphalite limbique est très évocatrice de l’HSV ; elle survient aussi moins fréquemment dans les encéphalites dues au HHV6, à la syphilis, à la maladie de Whipple et à la primo-infection par le VIH. Une rhombencéphalite doit fait rechercher la tuberculose et la listériose. Des lésions ischémiques aiguës peuvent survenir chez des patients atteints d’encéphalite bactérienne grave, de tuberculose, d’encéphalite à VZV, de syphilis et d’infections fongiques.
L’IRM cérébrale est cruciale dans le diagnostic des encéphalites aiguës : elle permet de détecter des anomalies de signal cérébrales qui renforcent la suspicion clinique d’encéphalite, notamment lorsque l’agent causal n’est pas identifié par ponction lombaire ; elle peut suggérer un pathogène particulier en fonction du pattern des anomalies cérébrales et elle permet d’éliminer des diagnostics différentiels importants (causes vasculaires, tumorales ou inflammatoires).
Posterior Fossa Syndrome (PFS) and Cerebellar Mutism
2016, The Linguistic Cerebellum
During the past few decades, the role of the cerebellum in behavioral, cognitive, and affective processes has evolved from a mere afterthought to an exciting area of innovative research across disciplines. Advances in the understanding of the neuroanatomy of the cerebellum and its reciprocal connections with the supratentorial brain regions subserving cognition, behavior, and affect combined with evidence from functional neuroimaging studies, neurophysiological research data, and clinical neuropsychological findings have radically redefined the traditional view on the cerebellum as a sole coordinator of sensorimotor function to that of a universal modulator of cognitive and affective processes.
Brain Pathology in COVID-19: Clinical Manifestations and Potential Mechanisms
2024, Neuroscience Bulletin
Cerebellar mutism syndrome of non-tumour surgical aetiology—a case report and literature review
2023, Child's Nervous System
Cerebellar Mutism Syndrome in Children and Adults
2021, Handbook of the Cerebellum and Cerebellar Disorders: Second Edition: Volume 3

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Clinical ObservationsCerebellar Mutism and Reversible Cytotoxic Edema in Influenza B–Associated Encephalopathy

Abstract

Background

Patient

Conclusion

Introduction

Section snippets

Case Report

Discussion

Semin Pediatr Neurol

Pediatr Neurol

Eur J Radiol

J Neuroimaging

National survey of pandemic influenza A (H1N1) 2009-associated encephalopathy in Japanese children

J Med Virol

Neurologic complications associated with influenza A in children during 2003−2004 influenza season in Houston, Texas

Pediatrics

Encephalitis associated with influenza B virus infection in 2 children and a review of the literature

Clin Infect Dis

Clinical Observations
Cerebellar Mutism and Reversible Cytotoxic Edema in Influenza B–Associated Encephalopathy