Hereditary cerebral hemorrhage with amyloidosis-Dutch type. Clinical and computed tomographic analysis of 24 cases
J. Haan, P. R. Algra and R. A. Roos
Department of Neurology, University Hospital, Leiden, The Netherlands.
Clinical and computed tomographic findings in 24 patients with hereditary
cerebral hemorrhage with amyloidosis-Dutch type were reviewed. The common
initial symptoms were headache and vomiting. Computed tomographic scans
showed 50 hypodense and 49 hyperdense cortical lesions and in 20 patients
the scans also showed generalized white matter hypodensity. Impairment of
consciousness was related to the size of the hemorrhagic lesion. Dementia,
seen in 11 patients, was related to the duration of the disease and the
number of focal lesions on the computed tomographic scans, but not to the
presence of white matter hypodensity. It is concluded that in hereditary
cerebral hemorrhage with amyloidosis-Dutch type, lobar hemorrhages account
predominantly for the acute clinical syndromes. The hemorrhages often have
an irregular shape and are responsible for progression of the symptoms
after an acute onset. Furthermore, cerebral amyloid angiopathy leads to a
generalized abnormality of the white matter, probably due to chronic
hypoperfusion.
