Diagnosis of Neoplastic and Paraneoplastic Cerebellar Ataxia
Acquired cerebellar ataxia encompasses various diseases. Neoplastic causes have specific characteristics. First, because of the limited space in the posterior fossa, these tumors are considered as critical brain lesions. Clinically, patients with cerebell
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Genevie`ve Demarquay and Je´roˆme Honnorat
Abstract
Acquired cerebellar ataxia encompasses various diseases. Neoplastic causes have specific characteristics. First, because of the limited space in the posterior fossa, these tumors are considered as critical brain lesions. Clinically, patients with cerebellar tumors frequently present with signs of intracranial pressure, such as headache, vomiting, or decreased level of consciousness. Second, the incidence and the type of cerebellar tumors vary considerably with age. Pilocytic astrocytomas and medulloblastoma are common cerebellar neoplasms in children. Conversely, the most frequent cerebellar tumors in adults are metastases. However, in a context of a systemic cancer and in absence of brain metastase, paraneoplastic cerebellar ataxia (PCA) must be considered. In the past 20 years, several antibodies directed against neuronal and tumoral antigens have been described in association with PCA, leading to the description of different subtypes of PCA based on the associated antibodies, the clinical evolution, and the type of associated tumor. The purpose of this chapter is to present the main etiologies of neoplastic and paraneoplastic causes of acute cerebellar ataxia in children and adults.
G. Demarquay Centre de Re´fe´rence, de Diagnostic et de Traitement des Syndromes Neurologiques Parane´oplasiques, Hospices Civils de Lyon, Lyon, France e-mail: [email protected] J. Honnorat (*) Centre de Re´fe´rence, de Diagnostic et de Traitement des Syndromes Neurologiques Parane´oplasiques, Hospices Civils de Lyon, Lyon, France and Universite´ Claude Bernard Lyon 1, UMR_S Inserm 842, Lyon, France and Neuro–Oncologie, Hoˆpital Neurologique, 59 Bd Pinel, 69677 BRON Cedex, France e-mail: [email protected] M. Manto, D.L. Gruol, J.D. Schmahmann, N. Koibuchi, F. Rossi (eds.), 2039 Handbook of the Cerebellum and Cerebellar Disorders, DOI 10.1007/978-94-007-1333-8_94, # Springer Science+Business Media Dordrecht 2013
2040
G. Demarquay and J. Honnorat
Introduction Sporadic and acquired cerebellar ataxia encompasses various diseases, such as vascular, toxic, demyelinating, inflammatory, infectious, autoimmune, and neoplastic disorders. Neoplastic causes of acute cerebellar ataxia include cerebellar tumors, both primary and metastatic as well as paraneoplastic disorders. These disorders have specific characteristics. Because of the limited space in the posterior fossa, cerebellar tumors are considered as critical brain lesions. Clinically, patients with cerebellar neoplasm present with progressive cerebellar ataxia and frequently with signs of increased intracranial pressure (headache, vomiting) related to obstructive hydrocephalus due to the compression of the fourth ventricle. Regarding histopathological diagnosis, the incidence and the type of cerebellar tumors vary considerably with age. Pilocytic astrocytomas and medulloblastomas are the most frequent cerebellar tumors in childhood. Conversely, the most frequent tumors observed in adults are metastases
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