Cerebrovascular Disease Flashcards

Question

Clinical presentation of intracranial hemorrhage by location

Answer 1

Most often affects the parietal and occipital lobes. Parietal: contralateral sensory impairment. Associated with a higher incidence of seizures. Occipital: dense contralateral homonymous hemianopsia. Frontal: contralateral plegia or paresis of the leg with relative sparing of the arm. Putamenal Commonly occurs along white matter fiber tracts. Hemiplegia, hemisensory loss, homonymous hemianopsia, gaze palsy, stupor, and coma. Cerebellar Originates in the dentate nucleus, extends into the hemisphere and fourth ventricle, and possibly into the pontine tegmentum. Imbalance, vomiting, headache, neck stiffness, gaze palsy, and facial weakness. May become stuporous due to brain stem compression if the hemorrhage is unrecognized or untreated. Thalamic May extend in a transverse direction to the posterior limb of the internal capsule, downward to put pressure on the tectum of the midbrain, or may rupture into the third ventricle. Hemiparesis, hemisensory loss, occasionally transient homonymous hemianopsia or quadrantanopsia. May be an upgaze palsy with miotic pupils that are unreactive, peering at the tip of the nose, skewed, or “wrong way eyes” toward the weak side. Aphasia if dominant hemisphere; neglect or anosognosia in nondominant hemisphere. Aphasia if dominant hemisphere; neglect or anosognosia in nondominant hemisphere. Pontine Medial hematoma that extends into the base of the pons, disruption of the reticular activating system. Deep coma within first few minutes, total paralysis, pinpoint pupils, absent horizontal eye movements, facial palsy, deafness, and/ or dysarthria if awake.

Answer 2

Focal symptoms can occur if the AVM forms a pathway in which blood flows away from the site and causes hypoperfusion of brain tissue. AVMs are susceptible to spontaneous rupture because of their thin vessel walls. .

Answer 3

Patients with AVMs may be asymptomatic until later in life, though symptom onset is often between the ages of 10 and 40. ICH is the most common clinical presentation (between 40% to 80% of AVM cases). Prior to rupture, headaches and seizures are common.

Answer 4

The long-term benefit of treatment for unruptured AVMs is unclear. A systematic review and meta-analysis of observational studies concluded that all available treatments were associated with considerable risks, including a 5%– 7% median rate of permanent neurologic complications or death, and incomplete efficacy of 13%– 96%

Answer 5

AVMs less consistently result in well-lateralized or focal neuropsychological impairments compared to ischemic stroke. The variability in neuropsychological outcome may be due to AVM pathology resulting in differences in the development of cerebral (re-) organization, as well as variation in site and rate of expansion. When asymmetrical neuropsychological findings are present, the AVM laterality can be predicted as accurately as in embolic stroke. Interestingly, developmental learning disorders have been found in adults with AVMs at a rate four times that of the general population (Lazar et al., 1999). Neuropsycho-logical functioning has been shown to improve postsurgery and may be attributable to the reduced mass effect of the AVM or surrounding edema. Alternatively, improvement may be related to eliminating the “steal effect” in which shunting through the AVM results in decreased cerebral perfusion in the surrounding area

Answer 6

large vascular lumen with collagenous walls lined with a layer of endothelial cells and may vary in size from 2 mm to several centimeters.

Answer 7

Saccular Berry-shaped with a narrow stem. Most commonly located in the anterior circulation. Dissecting Caused by a tear along the innermost layer of the vessel wall, with blood subsequently leaking in between layers of the wall. Often result of traumatic brain injury. Fusiform Bulges out on all sides (circumferentially), forming a dilated artery. Often associated with atherosclerosis.

Answer 8

deposition of the protein beta amyloid in small and midsized blood vessels of the brain and leptomenginges, weakening the vessel walls and making them vulnerable to rupture. .

Answer 9

Pathology of CADASIL involves amyloid-negative angiopathy involving small arteries and capillaries primarily in the brain, which result in loss of the periventricular subcortical white matter, lacunar infarcts in the basal ganglia, thalamus, and brain stem, and chronic ischemia CADASIL typically manifests with ischemic episodes, migraine with aura, cognitive impairment, or psychiatric disturbance. Ischemic episodes are nearly always subcortical with a classic lacunar syndrome presentation (pure motor stroke, ataxic-hemiparesis, dysarthria– clumsy hand syndrome, sensorimotor deficit). Strokes are often recurrent, leading to gait disturbance, urinary incontinence, and pseudobulbar palsy. Migraine with aura occurs in about 30% of CADASIL cases and is usually an early symptom

Answer 10

Cognitive impairment is a common clinical manifestation of CADASIL and approximately 75% of carriers eventually develop dementia executive impairment was present in all individuals, and attention and memory were also affected (Buffon et al., 2006). Verbal fluency, ideational praxis, and error monitoring have also been described (Peters et al., 2005). Recognition memory, verbal episodic memory, and visuo-spatial skills may be relatively spared and severe aphasia and agnosia are rare, though the cognitive pattern becomes more homogeneous and diffuse late in the course of CADASIL. Major depression and apathy related to executive dysfunction occur in about 20%– 30% of patients with CADASIL. Other manifestations include severe mood swings, panic disorder, visual hallucinations, and transient delusions. These episodes can precede other signs of CADASIL pathology or MRI findings. Overall, CADASIL cases may initially present with psychiatric dysfunction that is followed by pervasive cognitive impairment later in disease course

Answer 11

It is a chronic progressive cerebrovascular disease characterized by bilateral stenosis or occlusion of the arteries around the circle of Willis with prominent arterial collateral circulation. seen in a number of other medical conditions; therefore, the term moyamoya phenomenon or moyamoya syndrome is used to differentiate from idiopathic moyamoya disease.

Answer 12

progressive, with stepwise cognitive changes due to repeated ischemic stroke or hemorrhage. In studies with long-term follow-up of untreated patients, progressive neurologic deficits and poor outcome were reported in 50%– 66%. Executive functioning was the most common area of difficulty.

Answer 13

20% of cases of dementia, second only to Alzheimer’s disease a diagnosis of VaD does not exclude the presence of Alzheimer pathology. In fact, the presence of vascular pathology increases the risk of Alzheimer’s disease up to one-third of all-cause late-life dementia cases show significant vascular pathology at postmortem

Answer 14

4 bodies have 4 criteria including DSM: criteria identify different patients and are not interchangeable, contributing to variations in epidemiologic estimates. four diagnostic approaches vary based on how dementia is defined: what types of cerebrovascular disease are included: what disorders must be specifically excluded: whether focal findings are required, whether the presence of vascular disease must be corroborated by neuroimaging, whether a temporal relationship between stroke event and cognitive decline is required. none of the available criteria distinguished mixed dementia from vascular dementia or recognized early vascular cognitive changes More recently, a fifth approach was proposed by a joint American Heart Association and American Stroke Association statement that provided diagnostic criteria for probable and possible VaD, and probable, possible, and unstable vascular cognitive impairment (VCI) (Gorelick et al., 2011). -> VCI is presently conceptualized as “a syndrome with evidence of clinical stroke or subclinical vascular brain injury and cognitive impairment affecting at least one cognitive domain” particular attention to tasks involving information processing speed, set-shifting, and working memory, based on the generally accepted notion that executive dysfunction is a key aspect of VCI.

Answer 15

At least three common pathologies contribute substantively to VaD. - large artery infarctions, - small artery subcortical infarctions or lacunes, - and chronic subcortical ischemia - Lacunar infarctions and chronic ischemic changes in the white matter share a common primary vascular pathology of small penetrating arteries— and therefore tend to occur together and are particularly common in individuals with hypertension or diabetes and in the elderly. - clinical manifestations attributed to small-artery disease and associated with Vascular Cog Impairment are retinopathy and the presence of cerebral microbleeds in the deep hemispheric and infratentorial regions. - evidence of diffuse blood-brain barrier dysfunction throughout the white matter with lacunar infarction. Chronic subcortical microischemia can result in cognitive impairment even in the absence of ischemic lesions (Balestrini et al., 2013) and is a more frequent clinical-pathologic correlate of VCI and VaD than multiple large infarcts (Hulette et al., 1997; Jellinger, 2013). Deep white matter tracts are particularly susceptible to vascular pathology, in part because white matter is marginally perfused and particularly vulnerable to alterations in CBF and disruption of the blood-brain barrier, both of which contribute to oxidative stress, inflammation, and subsequent demyelination. role of vascular insults as initiator, stimulator, or additive contributor to VaD is significantly related to lesion volume, number, and location. Areas of strategic importance for deficits may be cortical (i.e., hippocampus, angular gyrus, frontal lobe) or subcortical (i.e., thalamus, caudate, genu of the internal capsule). Thalamic damage may be a particularly important contributor to cognitive impairment Even a single stroke in so-called strategic areas can result in prominent cognitive impairment. This is sometimes referred to as “strategic infarct dementia,” though these cases generally have a static presentation rather than a degenerative course. Similarly, other vascular events may cause significant cognitive impairment (e.g., subarachnoid hemorrhage, impairment after cardiac bypass surgery, water-shed infarction) but are not generally considered with VaD because of the lack of expected progression.

Answer 16

vascular dementia classically is associated with an acute onset and fluctuating intensity of symptoms and a stepwise decline in cognitive functioning combined with evidence of cerebrovascular disease (e.g., focal neurological signs). stepwise trajectory is typically expected with frank strokes rather than chronic microischemic insults that may have a more slowly progressive course. Alzheimer’s disease is typically associated with greater memory dysfunction and fewer executive deficits than VaD in early stages; however, the reverse is not always true (Reed et al., 2007). Despite extensive study, there remains no clear consensus of which cognitive functions or neuropsychological tests best discriminate between VaD and Alzheimer’s disease White matter pathology and subsequent disruption of fronto-subcortical networks, particularly associated with chronic subcortical microischemia, are thought to lead to slowed information processing speed, the neurocognitive symptom most commonly associated with VCI/ VaD. In general, other symptoms associated with VaD include attention deficits, executive dys-function, reduced phonemic verbal fluency, and impaired motor programming. memory findings, relatively less prominent impairment is expected than in Alzheimer’s disease, and recognition memory performance tends to be better than free recall. However, the neuropsychological profile will depend on location and degree of the underlying cerebrovascular pathology. Gait disturbance, parkinsonism, urinary incontinence, and depression are more frequently implicated in VaD, relative to other dementias.

Answer 17

Alzheimer’s disease with cerebrovascular disease, is recognized as a separate entity due to the common co-occurrence of Alzheimer’s disease and VaD pathology. Approximately **30%** of patients with a clinical diagnosis of VaD demonstrate Alzheimer’s disease pathology at autopsy / / 50% of those with Alzheimer’s disease diagnoses will show vascular pathology at autopsy Elderly patients with dementia may in fact be more likely to have mixed pathology Alzheimer’s disease and VaD may have common etiologies and influence each other’s course.