Acute cerebellitis is an inflammatory process involving the cerebellum. We report the clinical, CT and MRI features of four cases and a review of the literature. Bilateral diffuse hemispheric abnormalities represent the most common imaging presentations. Our observations demonstrate the various imaging appearances of acute cerebellitis. Simultaneous involvement of both hemispheres and the vermis has not been reported previously. The development of cerebellar atrophy following an initial normal MR imaging examination is also a new finding. In atypical clinical presentation, MR imaging can lead to the diagnosis. MR imaging findings have, however, no prognostic value.

MRI findings in acute cerebellitis

Acute cerebellar ataxia and acute cerebellitis represent a process characterized by parainfectious, postinfectious, or postvaccination cerebellar inflammation. There is considerable overlap between these entities. The mildest cases of acute cerebellar ataxia represent a benign condition that is characterized by acute truncal and gait ataxia, variably with appendicular ataxia, nystagmus, dysarthria, and hypotonia. It occurs mostly in young children, presents abruptly, and recovers over weeks. Neuroimaging is normal. Severe cases of cerebellitis represent the other end of the spectrum, presenting with acute cerebellar signs often overshadowed by alteration of consciousness, focal neurological deficits, raised intracranial pressure, hydrocephalus, and even herniation. Neuroimaging is abnormal and the prognosis is less favorable than in acute cerebellar ataxia. Acute disseminated encephalomyelitis may be confused with acute cerebellitis when the clinical findings are predominantly cerebellar, but lesions on neuroimaging are usually widespread. Paraneoplastic opsoclonus-myoclonus syndrome is often initially misdiagnosed as acute cerebellar ataxia, but has very specific features, course, and etiopathogensis.

Acute cerebellar ataxia, acute cerebellitis, and opsoclonus-myoclonus syndrome

Acute cerebellitis is postulated to result from viral and/or autoimmune etiologies. This disease has been reported to have a variable course. We report a case of sudden death from acute fulminant cerebellitis in a 13-year-old ballet dancer. Serial CT and MRI demonstrated rapid progression of the disease. Histopathologic correlation is provided. The etiologies, clinical course, therapeutic interventions and postmortem evaluation of this potentially life-threatening condition are briefly reviewed.

Sudden Death from Fulminant Acute Cerebellitis

Encephalitis is one of the manifestations of infection with Epstein-Barr virus with clinical outcome varying from complete recovery to death. A 16-year-old boy with Epstein-Barr virus encephalitis with global cortical and subcortical gray matter involvement and a full clinical recovery is reported. The case inspired a literature review which yielded 100 cases of Epstein-Barr virus encephalitis subjected to radiological investigation and published in 28 reports. Cerebellum and basal ganglia were reported to be equally involved by Epstein-Barr virus infection, next to cerebral hemisphere. Patients with isolated hemispheric gray or white matter involvement were reported to achieve good recovery while almost half of the patients with thalamic involvement developed sequelae. The highest mortality rate was among patients with isolated brain stem involvement. In conclusion, neuroanatomic distribution of the radiological abnormalities in Epstein-Barr virus encephalitis may be useful as a prognostic marker.

The neuroanatomic localization of Epstein-Barr virus encephalitis may be a predictive factor for its clinical outcome: a case report and review of 100 cases in 28 reports.

Many acutely ill children are not fully conscious. Most make a full neurological recovery as the underlying cause is treated, but considerable skill is required to distinguish the group at high risk of further deterioration, potentially leading either to death or to severe handicap. This article is an attempt to guide the worried paediatrician in casualty or on the ward faced with a child in non-traumatic coma who may need intensive care. The most effective method of deciding the order of priorities in this emergency situation is to ask oneself a series of questions.

This is the most important question of all and may well be the most difficult to answer. The Glasgow Coma Scale was designed to assess depth of coma after head injury in adults and has been used in paediatric non-traumatic coma.1-3 Although alternatives such as the Seshia scale have less interobserver variability, probably because there are fewer choices,4 the Glasgow scale is very familiar to nursing staff and casualty officers and works well down to the age of 5. In those below that age, the motor and eye opening scales may be used (except that children below the age of 9 months cannot localise pain), but a modification of the verbal scale is needed. The modified James scale (table 1) has been used successfully in several UK centres, can be consistently reproduced between observers,5 and has therefore been endorsed by the British Paediatric Neurology Association. The response to pain should be examined both with a supraocular stimulus (for localisation, flexion, and extension) and with nailbed pressure, for example with a pencil (for withdrawal). There may be a need for flexibility in terms of the overlap between the age groups. Thus, children of any age who are restless and talking unintelligibly have a verbal …

https://adc.bmj.com/content/archdischild/85/4/303.full.pdf

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Papers

Acute cerebellitis with near-fatal cerebellar swelling and benign outcome under conservative treatment with high dose steroids.