VASCULAR DEMENTIA

CHAPTER 47 VASCULAR DEMENTIA



Gustavo C. Román


Cerebrovascular disease constitutes a well-defined cause of dementia first recognized by Sir Thomas Willis in 1672 under the name postapoplectic dementia. Vascular dementia is considered the second major cause of dementia in elderly persons, after Alzheimer’s disease, representing 15% to 20% of all cases of dementia worldwide.1,2 Furthermore, it has been recognized that cerebrovascular disease may serve as a catalyst for converting low-grade Alzheimer’s disease to clinical dementia. The diagnosis of vascular dementia is relatively straightforward, particularly in cases with abrupt loss of cognitive functions after stroke or in individuals with a genetic history of cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL).3 The diagnosis may be less obvious in very old patients, in whom cerebrovascular disease and degenerative pathology (neurofibrillary tangles, amyloid deposits, Lewy bodies) may coexist. However, even in these cases of so-called mixed dementia, the contribution of the vascular pathology seems to outweigh the age-associated degenerative pathology. Moreover, vascular dementia is usually unrecognized in patients in whom cerebral hypoperfusion complicates cardiac failure and circulatory diseases. Judicious treatment of vascular risk factors at population level may lessen the predicted epidemic increase of dementia among elderly persons.



DEFINITIONS



Vascular Dementia


Vascular dementia is an etiological category of dementia characterized by severe cognitive impairment resulting from ischemic or hemorrhagic stroke or from hypoperfusion affecting brain regions important for memory, cognition, and behavior.


Vascular dementia is most frequently caused by the following lesions:


1. Hypoperfusive lesions such as global or selective ischemia, border-zone infarcts, and incomplete white matter ischemia.

2. Ischemic lesions involving large or small vessels: strategic single strokes, multi-infarct dementia (MID), small-vessel disease, lacunes, and venous occlusions.


Diagnostic Criteria


In most controlled trials in vascular dementia, investigators have used the clinical criteria proposed by Román and colleagues4 on behalf of the National Institute of Neurological Disorders and Stroke–Association Internationale pour la Recherche et l’Enseignement en Neurosciences (NINDS-AIREN criteria). Most cases of acute poststroke vascular dementia fulfill criteria for probable vascular dementia, modified as follows:5


1. There is acute onset of dementia, demonstrated by executive dysfunction causing loss of functional independence.

2. Relevant cerebrovascular lesions are demonstrated by neuroimaging.

3. A temporal relation between stroke and cognitive loss is evident. This last criterion usually cannot be fulfilled in subacute forms of vascular dementia.


Vascular Cognitive Disorder


Numerous diseases and syndromes can produce a vascular cognitive disorder.5 Table 47-1 provides a comprehensive list of potential causes of vascular cognitive disorder.


TABLE 47-1 Potential Etiologies of the Vascular Cognitive Disorder








































































































































































































































































































































































Primary vascular causes or hypoperfusion Amyloidosis angiopathy
Arterial dissection
Atherosclerosis of large or mid-sized arteries
Cardiopulmonary arrest
Diabetes mellitus
Dural arteriovenous malformation
Fibromuscular dysplasia
Giant cerebral aneurysm with compression
Hyperlipidemia
Hypertensive arteriolosclerosis
Moyamoya disease
Recurrent hypotension with syncope
Vasospasm/migraine
Hemorrhage Amyloid angiopathy
Aneurysms
Arteriovenous malformations
Cavernous angioma
Charcot-Bouchard aneurysm
CNS hemosiderosis with recurrent bleeding
Hematologic bleeding diathesis
Hemorrhagic transformation of infarction
Hypertension
Neoplasm (glioblastoma or metastatic)
Pharmacologic (e.g., anticoagulants)
Posthemorrhagic normal-pressure hydrocephalus
Telangiectasis
Vasculitis
Venous angioma
Venous thrombosis
Embolic causes Atherosclerotic stenosis with embolization
Atrial fibrillation
Atrial myxoma
Cardiac surgery, including CAGB
Cardiomyopathy
Congenital heart disease
Libman-Sacks endocarditis
Marantic endocarditis
Mitral valve prolapse syndrome
Myocardial infarction with mural thrombus
Nitrogen bubble emboli
Prosthetic valves
Rheumatic endocarditis and valvulopathies
Septal defect with paradoxical emboli
Septic, air, or fat emboli
Subacute bacterial endocarditis
Hematologic disorders Antiphospholipid/anticardiolipin antibodies
Antithrombin III deficiency
Arylsulfatase A pseudodeficiency
Cryoglobulinemia
Disseminated intravascular coagulation
Dysfibrinogenemias
Dysproteinemias
Factor V Leyden mutation
Factors V, VII, XII, and XIII deficiencies
Hemoglobinopathies (e.g., sickle cell disease)
Heparin cofactor II deficiency
Hyperviscosity syndromes
Idiopathic thrombocytosis
Leukemias
Nephrotic syndrome
Polycythemia vera
Pregnancy and oral contraceptives
Protein C or S deficiency
Thrombotic thrombocytopenic purpura
Waldenström’s macroglobulinemia
Toxic causes Amphetamines
Arsenic
Carbon monoxide
Cocaine or crack
Ergot alkaloids
Oral contraceptives
Radiation-induced vasculopathy
Noninfectious inflammatory or autoimmune causes Allergic or hypersensitivity angiitis
Anticardiolipin or antiphospholipid antibodies
Behçet’s syndrome
Burgher’s disease
Cogan’s syndrome
Dermatomyositis-polymyositis
Disseminated neocortical and subcortical encephalopathies
Drug-induced vasculitis
Endocardial fibroelastosis
Henoch-Schönlein purpura (radiation, tumor)
Kawasaki syndrome
Köhlmeier-Degos disease
Lupus anticoagulant
Polyarteritis nodosa
Primary CNS vasculitis
Relapsing polychondritis
Rheumatoid arthritis with arteritis
Sarcoidosis
Scleroderma
Sjögren’s syndrome
Systemic lupus erythematosus
Takayasu’s arteritis
Temporal (giant cell) arteritis
Thromboangiitis obliterans
Thrombotic microangiopathy
Ulcerative colitis
Vogt-Koyanagi-Harada syndrome
Wegener’s granulomatosis
Infectious causes of stroke AIDS
Bacterial meningitis with arteritis
Cat-scratch disease
Cysticercosis
Herpes zoster ophthalmicus
Lyme disease
Meningovascular infectious arteritis
Parasites and ova
Rickettsial arteritis
Syphilis
Tuberculosis
Viral arteritis
Yeast and fungal arteritis
Genetic causes 11β-hydroxylase deficiency
11β-ketoreductase deficiency
17α-hydroxylase deficiency
Antithrombin III deficiency
β-Thalassemia major
Bannayan-Zonana syndrome
Bloom’s syndrome
CADASIL
Chronic familial cerebral vasculopathy
Cockayne’s syndrome
Dysfibrinogenemia
Ehlers-Danlos syndrome, especially type IV
Fabry’s disease
Factors VII to XIII deficiencies
Familial atrial myxoma
Familial cavernous angioma
Familial hemiplegic migraine
Familial hypercholesterolemia
Familial hypoalphalipoproteinemia
Familial intracranial aneurysm
Familial oculoleptomeningeal amyloidosis
Familial porencephaly
Familial triglyceridemia
Fibromuscular dysplasia
Hemoglobin SC
Heparin cofactor II deficiency
Hereditary arteriovenous malformations
Hereditary cardiac conduction disorder
Hereditary cardiomyopathies
Hereditary cerebral amyloidosis
Hereditary platelet defect (Wiskott-Aldrich syndrome)
Hereditary polycythemia
Homocystinuria
Hyperlipoproteinemia (types III and IV)
Klippel-Trénaunay-Weber syndrome
Leigh’s disease
Marfan syndrome
MELAS syndrome
Menkes’ syndrome
Methylmalonic, propionic, and isovaleric acidemia; glutamic aciduria type I
Mitral valve prolapse
Moyamoya disease
Neurofibromatosis
Plasminogen deficiency
Progeria
Prekallikrein deficiency
Protein C or protein S deficiency
Pseudoxanthoma elasticum
Rendu-Osler-Weber syndrome
Rhabdomyomas
Sickle-cell disease
Sturge-Weber syndrome
Sulfite oxidase deficiency
Tangier disease
Tuberous sclerosis
Von Hippel–Lindau syndrome
Miscellaneous causes Metastatic deposits
Neoplastic anagioendotheliosis
Sneddon’s syndrome
Spatz-Lindenberg disease
Susac’s disease

AIDS, acquired immunodeficiency syndrome; CABG, coronary artery bypass graft; CADASIL, cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy; CNS, central nervous system; MELAS, mitochondrial encephalomyopathy, lactic acidosis, and strokelike symptoms (syndrome).


Modified From Mendez MF, Cummings JL: Dementia: A Clinical Approach, 3rd ed. Philadelphia: Butterworth-Heinemann (Elsevier Science), 2003.



Vascular Cognitive Impairment


This term designates a wide range of severity of cognitive alterations of vascular nature. However, the term is preferably restricted to cases of vascular cognitive impairment without dementia (vascular mild cognitive impairment).5



EPIDEMIOLOGY


In some of the most heavily populated regions of the world—including China, Japan, India, and Mexico—a vascular etiology is the most frequent cause of dementia. As the population in most of the world ages, likely increases in stroke and heart disease associated with old age will render vascular dementia the most frequent cause of dementia worldwide.



Prevalence


Jorm and Jolley6 performed a meta-analysis of 47 international studies on the prevalence of vascular dementia and found the following consistent trends:


1. Age-specific rates: Vascular dementia increases exponentially with age. Prevalence rates double after age 70 with every 5.3-year increase in age (in comparison with 4.5-year increases for Alzheimer’s disease), ranging from 1.5% at ages 70 to 75 years to 14% to 16.3% at ages 80 years and older.

2. Gender distribution: Vascular dementia is more common in men, especially before age 75.

3. Geographic (racial) variation: Vascular dementia is more prevalent in Asian, black, and Hispanic populations than in white populations. Hypertensive small-vessel disease is particularly common in the former groups.


Incidence


Incidence data for vascular dementia are quite limited. Dubois and Herbert2 used age-standardized incidence ratios to analyze data from 10 incidence studies of vascular dementia. These ratios ranged from 0.42 to 2.68, confirming the geographic and racial variations of vascular dementia.



CLINICAL FEATURES


Despite the apparent complexity of vascular dementia, the clinical diagnosis may be simplified into two major groups, acute and subacute, according to the temporal profile of presentation (Table 47-2).


TABLE 47-2 Clinical Forms of Vascular Dementia According to Temporal Pattern of Presentation




















Acute Forms

1. Posterior forms caused by lesions in the territory of the PCA, inferomedial temporal lobes, hippocampus, thalamus
Clinical features Amnesia, visual disturbances (homonymous hemianopsia, color agnosia, visual agnosia)
Left-sided lesions: transcortical sensory aphasia, alexia without agraphia
Right-sided lesions: spatial disorientation
Bilateral occipital lesions: Anton’s syndrome
Bilateral parieto-occipital lesions: Balint’s syndrome

2. Lesions in the ACA territory and medial frontal lobe
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Jun 19, 2016 | Posted by in NEUROLOGY | Comments Off on VASCULAR DEMENTIA

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