title: Longitudinal cerebral blood flow changes during speech in hereditary ataxia.
abstract: The hereditary ataxias constitute a group of degenerative diseases that progress over years or decades. With principal pathology involving the cerebellum, dysarthria is an early feature of many of the ataxias. Positron emission tomography was used to study regional cerebral blood flow changes during speech production over a 21 month period in a group of seven right-handed subjects with hereditary ataxia (6 females and 1 male, 3 SCA1 and 4 SCA5, aged 38.3+/-18.9 years). The decline in blood flow was greatest in cerebellar regions. In contrast, blood flow actually increased during speech production in the classic speech area (Broca's area) but not in its right-hemisphere homologue at the second evaluation. This increase in cortical flow may have been compensatory for cerebellar degeneration as speech intelligibility did not decline significantly during this period. Compensation was not complete, though, as syllable timing shifted in the direction of equal syllable duration, one of the characteristics of ataxic dysarthria. These results are consistent with previous functional imaging studies of ataxia demonstrating a pattern of brain activity that reflects both loss of function and relative compensation when clinical signs and symptoms are still mild. The combination of disease-relevant tasks, behavioral measurement, and functional imaging may provide insight into the early changes associated with neurodegenerative disease.
authors.affiliation: Brain and Behavior Laboratory, Geriatrics Division, Nathan Kline Institute, 140 Old Orangeburg Road, Orangeburg, NY 10962, United States. john.sidtis@nyu.edu
authors.forename: John J
authors.initials: JJ
authors.lastname: Sidtis
authors.forename: Stephen C
authors.initials: SC
authors.lastname: Strother
authors.forename: Ansam
authors.initials: A
authors.lastname: Groshong
authors.forename: Ansam
authors.initials: A
authors.lastname: Naoum
authors.forename: David A
authors.initials: DA
authors.lastname: Rottenberg
authors.forename: Christopher
authors.initials: C
authors.lastname: Gomez
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D001921:Brain / Q000000981:diagnostic imaging / Q000503:physiopathology*; D001931:Brain Mapping; D002560:Cerebrovascular Circulation / Q000502:physiology*; D018450:Disease Progression; D005260:Female; D006801:Humans; D008137:Longitudinal Studies; D008297:Male; D008875:Middle Aged; D009460:Neurologic Examination; D049268:Positron-Emission Tomography; D013060:Speech / Q000502:physiology*; D013132:Spinocerebellar Degenerations / Q000000981:diagnostic imaging / Q000503:physiopathology*; D055815:Young Adult