17 – Neurodegenerative Disorders of the Cerebellum

摘要: This chapter discusses the important diagnostic information about cerebellar neurodegenerative disorders. The disorders of central nervous system are characterized by progressive degeneration and subsequent loss neurons accompanied reactive gliosis, fibers from deteriorating neurons, clinical symptoms reflecting locations lost neurons. These occur sporadically, usually without known cause or genetic disease with either dominant recessive inheritance. Many affect cerebellum its pathways, resulting in deterioration function manifested increasing unsteadiness gait. An autosomal disorder, Friedreich's Ataxia, FA short, is one most common hereditary ataxias. was thought to begin childhood early adolescence, at times late adolescence only rarely above age 20. In these “typical” patients, disorder begins ataxia legs followed arms then progresses leg weakness, leading paraplegia, ataxic dysarthria, decreased peripheral sensation all modalities particularly severe involvement vibration sense position sense, areflexia, bilateral extensor plantar reflexes. patients have an increased prevalence impaired glucose tolerance diabetes mellitus comparison general population.