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CADASIL or CADVaSIL?
Author(s) -
Rafalowska Janina,
Fidzianska Anna,
Dziewulska Dorota,
Podlecka Aleksandra,
Szpak Grazyna Maria,
Kwiecinski Hubert
Publication year - 2004
Publication title -
neuropathology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.701
H-Index - 61
eISSN - 1440-1789
pISSN - 0919-6544
DOI - 10.1111/j.1440-1789.2003.00527.x
Subject(s) - cadasil , leukoencephalopathy , angiopathy , pathology , cerebral amyloid angiopathy , pathological , medicine , anatomy , disease , dementia , diabetes mellitus , endocrinology
In the present study, morphological examination of patients from two unrelated Polish families with CADASIL was performed. Using light microscopy, there were evident changes characteristic to the disease. On electron microscopy, deposits of granular osmiophillic material (GOM) were found not only in cerebral arteries and veins but also in cerebral capillaries and vessels of the internal organs. These findings indicate that pathological process in CADASIL is generalized and involves also small vessels devoid of smooth muscle cells. Therefore, we propose to consider a replacement for the name CADASIL that better reflects the morphological picture of the disease like, for example, cerebral autosomal dominant vasculopathy with subcortical infarcts and leukoencephalopathy (CADVaSIL) or, to preserve the commonly known acronym, cerebral autosomal dominant angiopathy with subcortical infarcts and leukoencephalopathy.