Brain, Vol 121, Issue 5 923-929, Copyright © 1998 by Oxford University Press
S Webb and M Hutchinson
In the course of a study of a large family with pure autosomal dominant
hereditary spastic paraparesis (AD-HSP), mild cognitive impairment was
found in older family members. In order to determine if cognitive
impairment occurred more frequently in families with pure AD-HSP than
normally expected, a case control study of cognitive function in HSP was
undertaken. Thirty-one patients, from 12 kindreds with pure AD-HSP, matched
with 31 healthy control subjects for age, sex and years of education, were
assessed for evidence of cognitive impairment using the Cambridge Cognitive
Examination (CAMCOG). Twenty unaffected siblings matched with twenty
healthy control subjects were similarly assessed. The total CAMCOG score in
the affected group (mean 89.26/107, SD 11.08, 95% confidence interval
85.2-94.49) compared with the control group (mean 96.52/107, SD 5.52, 95%
confidence interval 94.49-98.54) was significantly reduced (P = 0.0003).
There were also significant abnormalities in three out of the nine subsets
including memory (P = 0.0002), language comprehension (P = 0.0166) and
language expression (P = 0.0025). The differences between the groups were
due to cognitive impairment appearing after the age of 50 years in patients
with AD-HSP; CAMCOG scores before this age were similar to control scores.
There was also a minor non-significant difference in total CAMCOG score for
the unaffected siblings (mean 93.7/107, SD 8.54, 95% confidence interval
89.70-97.70) compared with the control group (mean 97.9/107, SD 4.61, 95%
confidence interval 95.7-100.1) (P < 0.02). This study demonstrates that
mild cognitive impairment develops after the age of 50 years in patients
with pure AD-HSP and is further evidence of degeneration in other systems
in this disorder.
ARTICLES
Cognitive impairment in families with pure autosomal dominant hereditary spastic paraparesis
Department of Neurology, St Vincent's Hospital, Dublin, Ireland.
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