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Marjolijn Bornebroek, MD; Joost Haan, MD, PhD; Mark A. van Buchem, MD, PhD; Jan B. K. Lanser, PhD; M. A. C. Simone de Vries-vd Weerd, MD; Moniek Zoeteweij, PhD; Raymund A. C. Roos, MD, PhD

Arch Neurol. 1996;53(1):43-48.

Abstract
Objective
To determine early manifestations of hereditary cerebral hemorrhage with amyloidosis (Dutch).

Design
Survey.

Setting
Neurologic outpatient department of the University Hospital Leiden in the Netherlands.

Participants
Ten presymptomatic carriers of the amyloid precursor protein gene codon 693 mutation.

Main Outcome Measurements
Extensive neuropsychological examination and cerebral magnetic resonance imaging.

Results
Six subjects older than 40 years showed white matter hyperintensities on magnetic resonance imaging. Three of these six individuals had signs of cognitive deterioration. The four younger subjects (age, <31 years) showed no abnormalities on magnetic resonance imaging or on neuropsychological examination.

Conclusions
We suggest that white matter hyperintensities in hereditary cerebral hemorrhage with amyloidosis (Dutch) are probably caused by chronic ischemia due to stenosis of the meningocortical arterioles, which becomes visible on magnetic resonance imaging scans in individuals who are between the ages of 30 and 40 years. The finding of cognitive deterioration in three of 10 presymptomatic mutation carriers supports the finding that in hereditary cerebral hemorrhage with amyloidosis (Dutch), deterioration can occur without stroke. A direct relation between cognitive deterioration and white matter hyperintensities is unlikely, because only half of the individuals with white matter hyperintensities showed signs of deterioration.

Author Affiliations
From the Departments of Neurology (Drs Bornebroek, Haan, Lanser, and Roos) and Diagnostic Radiology (Dr van Buchem) and the Clinical Genetics Centre (Drs de Vries-vd Weerd and Zoeteweij), University Hospital Leiden, Leiden, the Netherlands, and the Department of Neurology, Rijnland Hospital, Leiderdorp (Dr Haan), the Netherlands.

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