Infectious Diseases of the Posterior Fossa
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 loc
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93
Mario Manto and Patrice Jissendi
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 present with an acute or subacute cerebellar syndrome following an initial infection, often of viral origin, or a vaccination. Clinical picture may also manifest with combinations of cerebellar and brainstem deficits. Human prion diseases include in particular Creutfeldt– Jakob disease (CJD), variant CJD, and Gertsmann–Str€aussler–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 of a host-encoded glycoprotein (prion protein). 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 an urgent decompressive therapy. Early administration of steroids is recommended for the treatment of cerebellitis. There is still no cure for prion disorders.
M. Manto (*) Unite´ d’Etude du Mouvement (UEM), FNRS, Neurologie ULB Erasme, 808 Route de Lennik, 1070 Bruxelles, Belgium e-mail: [email protected] P. Jissendi Service de Neuroradiologie, ULB Erasme, 808 Route de Lennik, 1070 Bruxelles, Belgium e-mail: [email protected] M. Manto, D.L. Gruol, J.D. Schmahmann, N. Koibuchi, F. Rossi (eds.), 2027 Handbook of the Cerebellum and Cerebellar Disorders, DOI 10.1007/978-94-007-1333-8_93, # Springer Science+Business Media Dordrecht 2013
2028
M. Manto and P. Jissendi
Infections of the posterior fossa include bacterial infections, viral infections, and human prion diseases. The clinical presentation may be a pure cerebellar syndrome or various combinations of cerebellar and extra-cerebellar deficits, in particular signs of brainstem compression. Indeed, bacterial infections and cerebellitis carry a risk of obstructive hydrocephalus due to the small volume of the posterior fossa. Mass effect may develop very rapidly, requiring an urgent decompressive therapy with a ventricular drain. It is therefore of major importance to diagnose and treat quickly any infection of the posterior fossa.
Bacterial Infections Although the majority of cerebellar abscesses occur in supratentorial areas, they may develop exclusively in the posterior fossa (Manto 2010). It is estimated that about 8–18% of purulent brain
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