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Infectious Diseases of the Posterior Fossa

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Handbook of the Cerebellum and Cerebellar Disorders

Abstract

Infections of the posterior fossa include bacterial infections, viral infections, and human prion diseases. Bacterial infections can present as abscesses involving the cerebellum and/or brainstem structures. About 8–18% of purulent brain abscesses are located in the cerebellum. Cerebellar abscesses are mainly associated with otogenic infections, cyanotic heart disease, congenital dermal sinus, trauma, and postoperative complications. Cerebellitis often presents with an acute or subacute cerebellar syndrome following an initial infection, often of viral origin, or a vaccination. The clinical picture may also manifest with combinations of cerebellar and brainstem deficits. Human prion diseases include Creutzfeldt-Jakob disease (CJD), variant CJD, and Gertsmann-Sträussler-Scheinker disease which is transmitted as an autosomal dominant disease. Sporadic CJD may have an iatrogenic origin following contamination. The main clinical presentation of sporadic CJD is a rapidly progressive dementia developing between the age of 60 and 65 years. The association of dementia, myoclonic jerks, parkinsonism, cerebellar ataxia, and seizures is suggestive. Prions are mainly composed of abnormal isoforms (PrPsc) of a host-encoded glycoprotein (prion protein PrPc). Infections of the posterior fossa (abscesses and cerebellitis) carry the risk of brainstem compression with tonsillar herniation and obstructive hydrocephalus. Antibiotics are used for abscesses but raised intracranial pressure may require urgent decompressive therapy. Early administration of steroids is recommended for the treatment of cerebellitis. Plasmapheresis and immunoglobulins are used in some immune-mediated forms of acute ataxia. There is still no cure for prion disorders.

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Manto, M.U., Jissendi, P. (2021). Infectious Diseases of the Posterior Fossa. In: Manto, M., Gruol, D., Schmahmann, J., Koibuchi, N., Sillitoe, R. (eds) Handbook of the Cerebellum and Cerebellar Disorders. Springer, Cham. https://doi.org/10.1007/978-3-319-97911-3_93-2

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