February 1997

Cerebellar Volume in Adults With Down Syndrome

Author Affiliations

From the Divisions of Psychiatric Neuroimaging (Drs Aylward, Warren, Barta, and Pearlson, Ms Habbak, and Mr Jerram) and Medical Psychology (Dr Pulsifer), The Johns Hopkins University School of Medicine, Baltimore, Md.

Arch Neurol. 1997;54(2):209-212. doi:10.1001/archneur.1997.00550140077016

Objectives:  To determine the effects of aging on cerebellar volume in individuals with Down syndrome (DS). To determine whether volume of cerebellum is associated with dementia or with age-related decline in fine-motor control.

Design:  Case-control study involving comparison of cerebellar volumes in adults with DS and matched control subjects; survey study involving correlations between cerebellar volume and subjects' age and performance on a test of fine-motor control; and longitudinal study assessing change in cerebellar volume in adults with DS.

Setting:  The Johns Hopkins University School of Medicine, Baltimore, Md.

Patients and Other Participants:  Subjects included 40 adults with DS. Thirty of them were matched on age, sex, and race with cognitively normal subjects. A diagnosis of probable dementia was made for 5 of the subjects with DS. Longitudinal data were available for 23 of the 40 subjects with DS, with a mean interscan interval of 2 years.

Main Outcome Measures:  Volumes of cerebellum, total brain, and intracranial region were measured on magnetic resonance imaging scans. The Purdue Pegboard, a test of fine-motor control, was administered to 38 of the subjects with DS.

Results:  Subjects with DS had significantly smaller cerebellar volumes than matched controls, even after adjusting for total brain volume or total intracranial volume. Volume of cerebellum did not correlate significantly with age for either the subjects with DS or controls. Longitudinal change in cerebellar volume in subjects with DS was not significant. Volume of total brain, but not cerebellum, correlated significantly with performance on the Purdue Pegboard.

Conclusions:  Although cerebellar volumes are disproportionately small in individuals with DS, they do not diminish significantly with age and do not undergo agerelated atrophy that is different from that of normal controls. Volume reduction in the cerebellum does not appear to be specifically responsible for the age-related decline in finemotor control that is observed in adults with DS.