Sclerosing vasculopathy of the central nervous system in nonelderly demented patients
M. L. Estes, M. I. Chimowitz, I. A. Awad, J. T. McMahon, A. J. Furlan and N. B. Ratliff
Department of Pathology, Cleveland Clinic Foundation, OH 44195.
Three nonelderly patients without hypertension whose clinical and
radiologic features otherwise resembled Binswanger's subcortical
arteriosclerotic encephalopathy underwent biopsy of the hyperintense
periventricular lesions seen on magnetic resonance imaging. The pathologic
findings of the periventricular lesions consisted of gliosis with mild
rarefaction and edema of the white matter. All patients had a sclerosing
vasculopathy of unknown cause, which involved numerous small vessels within
the periventricular lesions. The vessels stained negatively for amyloid,
amyloid precursors, desmin, vimentin, keratin, immunoglobulin, and
complement. On electron microscopy, small arteries, arterioles, venules,
and capillaries were characterized by swollen astrocytic foot processes
surrounding the vessels; dense, perivascular collagen packing; crystalline
arrays of filaments within basement membrane; giant lipid-laden lysosomes
within perivascular cells; and narrowing of the vascular lumina. Similar
changes were not seen in a control group of 19 patients. The pathologic
features of the vessels in these cases are distinct from the vasculopathy
associated with Binswanger's subcortical arteriosclerotic encephalopathy.
We suggest that a spectrum of vasculopathies may be associated with
dementia and periventricular hyperintense lesions on magnetic resonance
imaging.
