Mohammed T. Abou-Saleh

248 PRINCIPLES AND PRACTICE OF GERIATRIC PSYCHIATRYcommissure and internal capsules. The walls of white matterpenetrating arteries are hyalinized but the vessels are patent. Thiscondition is probably caused by hypoxic–ischaemic damage to thedistal watershed periventricular white matter, secondary tonarrowing of arterioles 23,27,28 .A closely related cause of dementia due to diffuse white matterdamage is CADASIL (cerebral autosomal dominant arteriopathywith subcortical infarcts and leukoencephalopathy) 29 . This is adisease that usually presents in the fifth decade with strokes ordementia, and is an inherited vascular disorder due to mutationaffecting the Notch3 gene on chromosome 19. Pathologically, thedisease is very similar to Binswanger’s disease, with widespreadlacunar infarcts and diffuse cerebral white matter degeneration.The distinguishing feature is the presence of non-hypertensivearteriolosclerosis, characterized by deposition of granular osmiophilicmaterial in relation to vascular smooth muscle cells, withdegeneration of these cells and thickening of the vessel walls. Thisis a systemic vascular disorder, although other tissues are lessseverely affected than the brain, and the diagnosis is thereforepossible on skin or muscle biopsy, although genetic analysis isrequired for confirmation 30 .In the experience of the author, vascular dementia is much lesscommon than AD. However, it is given a significance disproportionateto its incidence by the fact that some causes of vasculardementia—such as giant-cell arteritis 31 and thromboangiitisobliterans 32 —are potentially treatable, and that vascular dementiaas a whole is theoretically preventable by addressing risk factorsfor cerebrovascular disease 33 .REFERENCES1. Tomlinson BE, Blessed G, Roth M. Observations on the brains ofdemented old people. J Neurol Sci 1970; 11: 205–42.2. Jellinger K, Danielczyk W, Fischer P, Gabriel E. Clinicopathologicalanalysis of dementia disorders in the elderly. J Neurol Sci 1990; 95:239–58.3. Nolan KA, Lino MM, Seligmann AW, Blass JP. Absence of vasculardementia in an autopsy series from a dementia clinic. J Am Geriat Soc1998; 46: 597–604.4. Seno H, Ishino H, Inagaki T et al. A neuropathological study ofdementia in nursing homes over a 17 year period in ShimanePrefecture, Japan. Gerontology 1999; 45: 44–8.5. Skoog I, Nilsson L, Palmertz B. A population-based study ofdementia in 85 year-olds. N Engl J Med 1993; 328: 153–8.6. Alafuzoff I. The pathology of dementias: an overview. Acta NeurolScand 1992; (suppl 139): 8–15.7. Thomas BM, Starr JM, Whalley LJ. Death certification in treatedcases of presenile Alzheimer’s disease and vascular dementia inScotland. Age Ageing 1997; 26: 401–6.8. Hershey LA. Dementia associated with stroke. Stroke 1990; 21 (supplII): 9–11.9. Amar K, Wilcock G. Vascular dementia. Br Med J 1996; 312: 227–31.10. Hulette C, Nochlin D, McKeel D et al. Clinical–neuropathologicfindings in multi-infarct dementia: a report of six autopsied cases.Neurology 1997; 48: 668–72.11. del Ser T, Bermejo F, Portera A et al. Vascular dementia. Aclinicopathological study. J Neurol Sci 1990; 96: 1–17.12. Wolfe N, Babikian VL, Linn RT. Are multiple cerebral infarctssynergistic? Arch Neurol 1994; 51: 211–15.13. Loeb C. Dementia due to lacunar infarctions: a misnomer or a clinicalentity? Eur Neurol 1995; 35: 187–92.14. Forette F, Boller F. Hypertension and the risk of dementia in theelderly. Am J Med 1991; 90: 14–19S.15. Garcia JH, Brown GG. Vascular dementia: neuropathologicalterations and metabolic brain changes. J Neurol Sci 1992; 109:121–31.16. Benson FD, Cummings JL, Tsai SY. Angular gyrus syndromesimulating Alzheimer’s disease. Arch Neurol 1982; 39: 616–20.17. Esiri MM, Wilcock GK, Morris JH. Neuropathological assessment ofthe lesions of significance in vascular dementia. J Neurol NeurosurgPsychiat 1997; 63: 749–53.18. Roman GC. New insight into Binswanger’s disease. Arch Neurol1999; 56: 1061–2.19. Bogousslavsky J. Binswanger’s disease: does it exist? Cerebrovasc Dis1996; 6: 255–63.20. Hachinski VC, Potter P, Merskey H. Leukoaraiosis. Arch Neurol1987; 44: 21–3.21. Ferrer I, Bella R, Serrano MT et al. Arterioscleroticleucoencephalopathy in the elderly and its relation to white matterlesions in Binswanger’s disease, multi-infarct encephalopathy andAlzheimer’s disease. J Neurol Sci 1990; 98: 37–50.22. Meyer JS, Kawamura J, Terayama Y. White matter lesions in theelderly. J Neurol Sci 1992; 110: 1–7.23. Pantoni L, Garcia JH. The significance of cerebral white matterabnormalities 100 years after Binswanger’s report. A review. Stroke1995; 26: 1293–301.24. van Gijn J. Leukoaraiosis and vascular dementia. Neurology 1998; 51(suppl 3): S3–8.25. Babikian V, Ropper AH. Binswanger’s disease: a review. Stroke 1987;18: 2–12.26. Fisher CM. Binswanger’s encephalopathy: a review. J Neurol 1989;236: 65–79.27. Pantoni L, Garcia JH. Cognitive impairment and cellular/vascularchanges in the cerebral white matter. Ann N Y Acad Sci 1997; 826:92–102.28. van Swieten JC, van den Hout JHW, van Ketel BA et al.Periventricular lesions in the white matter on magnetic resonanceimaging in the elderly: a morphometric correlation witharteriosclerosis and dilated perivascular spaces. Brain 1991; 114:761–71.29. Ruchoix M-M, Maurage C-A. CADASIL: cerebral autosomaldominant arteriopathy with subcortical infarcts andleukoencephalopathy. J Neuropathol Exp Neurol 1997; 56: 947–64.30. Schultz A, Santoianni R, Hewan-Lowe K. Vasculopathic changes ofCADASIL can be focal in skin biopsies. Ultrastruct Pathol 1999; 23:241–7.31. Caselli RJ. Giant cell (temporal) arteritis. A treatable cause of multiinfarctdementia. Neurology 1990; 40: 753–5.32. Larner AJ, Kidd D, Elkington P et al. Spatz–Lindenberg disease: arare cause of vascular dementia. Stroke 1999; 30: 687–9.33. Hachinski V. Preventable senility: a call for action against thevascular dementias. Lancet 1992; 340: 645–8.