Cerebellar Disorders in Children – Chapter 34: Paraneoplastic Cerebellar Syndromes (ebook)


Chapter 34 – Paraneoplastic Cerebellar Syndromes: Neurodegeneration in Langerhans Cell Histiocytosis 

Andrea Poretti, Eugen Boltshauser, and Jeremy D. Schmahmann

Paraneoplastic neurological syndromes (PNS) represent remote effects of cancer on the nervous system. PNS in the paediatric age group are exceptionally rare and we focus on three underlying conditions, which are discussed in separate chapters: neurodegeneration in Langerhans histiocytosis (this chapter); paraneoplastic cerebellar degeneration in paediatric Hodgkin disease (Chapter 35); and opsoclonusmyoclonus syndrome (associated with neuroblastoma) (Part 8 Acute Ataxia, Chapter 38).

Please note that Chapters 32-35 are also available in Part 7: Vascular Disorders, Tumours, and Paraneoplastic Cerebellar Syndromes.

About the Complete Book:

This clinically orientated text by an international group of experts is the first definitive reference book on disorders of the cerebellum in children. It presents a wealth of practical clinical experience backed up by a strong scientific basis for the information and guidance given. The first part sets out the theoretical underpinnings of cerebellar disorders. This is followed by sections on clinical conditions grouped according to common characteristics such as aetiology and symptomatology. The descriptions of the clinical conditions each systematically cover, as appropriate, epidemiology, prevalence, diagnostic criteria, clinical features (including course and prognosis), pathophysiology, genetics, investigations, differential diagnosis, and management and treatment. This book will be an invaluable resource for all those caring for children affected by cerebellar disorders, including malformations, genetic and metabolic disorders, acquired cerebellar damage, vascular disorders and acute ataxias. This comprehensive reference text on cerebellar disorders in children includes chapters on cerebellar development, prenatal cerebellar imaging, imaging of the posterior fossa, with coverage of a broad range of malformations, genetic and metabolic disorders involving the cerebellum, prenatal cerebellar disruptions (as related to prematurity), vascular disorders, tumors and paraneoplastic syndromes, as well as acute ataxia and trauma to the posterior fossa. Numerous checklists are provided to assist in the differential diagnosis of clinical signs and neuroimaging findings.

Paediatric neurologists, paediatricians, neurologists, developmental paediatricians, neuroimaging specialists, geneticists, neonatologists

Clinics in Developmental Medicine No. 191-192