Abstract
We describe 2 normotensive sisters presenting slowly progressive dementia associated with acute or subacute focal neurological symptoms, unilateral or bilateral motor signs, and dysarthria. Their father, who died in the seventh decade, had a similar clinical picture. Computerized axial tomography (CT) scan of the head showed symmetrical hypodensities in the periventricular white matter and mild to moderate hydrocephalus. In these patients a diagnosis of Binswanger's disease was based on the clinical features supported by white matter changes on CT scan. Our study suggests that genetic factors may play a role in the etiology of Binswanger's disease.
Sommario
Descriviamo due giovani sorelle normotese affette da demenza lentamente progressiva associata a sintomi neurologici focali ad insorgenza sia acuta che subacuta. Un quadro clinico simile era presente nel padre deceduto all'età di 70 anni.
La TAC cranica mostrava nelle due pazienti ipodensità simmetriche della sostanza bianca perventricolare con idrocefalo moderato.
In questi pazienti una diagnosi di malattia di Binswanger fu fatta in base al quadro clinico ed alle lesioni riscontrate alla TAC. Il nostro studio dimostra la possibilità che fattori genetici siano responsabili di alcune forme di malattia di Binswanger soprattutto in pazienti normotesi, giovani e senza fattori di rischio per malattie cerebrovascolari.
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Salvi, F., Michelucci, R., Plasmati, R. et al. Slowly progressive familial dementia with recurrent strokes and white matter hypodensities on CT scan. Ital J Neuro Sci 13, 135–140 (1992). https://doi.org/10.1007/BF02226962
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DOI: https://doi.org/10.1007/BF02226962