Marfan Syndrome: neuropsychological aspects.

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Marfan Syndrome: neuropsychological aspects.

Hofman KJ, Bernhardt BA, Pyeritz RE.

Departments of Pediatrics, s Hopkins Medical Institutions, Baltimore,

land.

We evaluated the neurodevelopmental status and cognitive ability of 30

school-age children with Marfan syndrome. We found average intellectual

(mean full

scale IQ = 109.3) and gross motor development. Fifty percent had one or

more neuropsychologic deficits: learning disability (LD) (13%), attention

deficit

disorder with or without hyperactivity (ADD +/- H) (17%), neuromaturational

immaturity (NMI) (10%), and a performance IQ score different from their

verbal

score by more than 20 points (30%). Patients with a large

verbal-performance discrepancy, with one exception, scored lower on tests

of performance, and

particularly low on two subtests, object assembly and coding, compared with

the rest of the patients (P less than 0.01). The factor most highly

correlated

with verbal-performance discrepancy was the severity of joint hypermobility

(P = 0.02), which suggests that the depressed performance score was due, in

part, to motor incoordination. Children with Marfan syndrome may be prone

to neuropsychologic problems; the role of routine screening remains to be

tested.

Those with hand-wrist hypermobility seem at particular risk for

difficulties with tasks that involve writing and should receive careful

evaluation in the

classroom. Due to the high risk of aortic dilatation in these patients, if

stimulant medications are considered for treatment of ADD +/- H, the risk of

adverse sympathomimetic effect should be weighed against the potential

benefit.

PMID: 3232699 [PubMed - indexed for MEDLINE]