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2023

ANUÁRIO DO HOSPITAL
DONA ESTEFÂNIA

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AUTOIMMUNE ACUTE CEREBELLITIS

Pinheiro1 Carolina, Mariana Cardoso Diogo, Carla Conceição3

1. Department of Neuroradiology, Hospital Dona Estefania, CHLC

Poster:  Oporto Neuroradiology 50th Anniversary International Symposium, 30 setembro – 1 outubro 2016, Porto, Portugal.

Introdução: Acute cerebellitis (AC) is a rare syndrome, characterized by cerebellar dysfunction (headache, vomiting and disturbances of consciousness), due to cerebellar inflammation, usually in a post-infectious or postvaccination context. In most cases a etiology remains undetermined. It is typically a benign and self-limiting disease, however, it may result in death or give rise to severe cerebellar atrophy. Neuroimaging with brain CT is useful in the acute phase, to detect acute hydrocephalus, cerebellar edema or brainstem compression. MRI is essential as children may not present with typical cerebellar signs. Characteristic MRI findings are bilateral diffuse bilateral cortical swelling and high intensity of the cerebellum on T2-WI. Pial enhancement may be observed on contrast-enhanced MRI. MRI findings have no prognostic value. Differential diagnosis of includes acute disseminated encephalomyelitis (ADEM), Lhermitte-Duclos disease, diffusely infiltrating glioma or lymphoma, vasculitis and drug related inflammatory processes. Treatment is symptomatic and anti-infectious; antibiotics or antiviral drugs, with corticosteroids, mannitol in cases of severe cerebellar swelling. Surgical decompression may be the only strategy in most severe cases.
Caso: A 5-year-old girl, previously healthy, presented behavior changes with refusal to play, gait imbalance, dysarthria and double vision. Latter she developed occipital/cervical aches with nausea and vomiting, and prostration.
Objetivos: a) What are the findings? b) Differential diagnosis c) If the patient was an adult, what would be the first diagnosis?
Conclusões: a) On brain-CT there is bilateral cerebellar hemispheric hypodensity. MRI T2-WI show symmetrical edematous changes of the cerebellar hemespheres with CSF spaces effacement. b) Lhermitte-Duclos disease, ADEM, diffusely infiltrating glioma or lymphoma vasculitis and drug related inflammatory processes. c) Cerebellar lymphoma

Palavras Chave: Autoimmune Acute Cerebellitis.