Brain Disorders

Corticobasal Degeneration (CBD)

(A,B) T2-weighted image showing diffuse asymmetric (L > R) bilateral frontoparietal atrophy (arrows) in a 54-year-old woman with progressive nonfluent aphasia and mild parkinsonism due to pathology-proven corticobasal degeneration.

Huntington's Disease

Huntington's disease is a hereditary disorder caused by a faulty gene for a protein called huntingtin. The children of people with the disorder have a 50% chance of inheriting it. The disease causes degeneration in many regions of the brain and spinal cord. Symptoms of Huntington's disease usually begin when patients are in their thirties or forties, and the average life expectancy after diagnosis is about 15 years. Cognitive symptoms of Huntington's disease typically begin with mild personality changes, such as irritability, anxiety, and depression, and progress to severe dementia. Many patients also show psychotic behavior. Huntington's disease causes chorea - involuntary jerky, arrhythmic movements of the body - as well as muscle weakness, clumsiness, and gait disturbances.

HIV-associated Dementia (HAD)

A 40-year-old woman diagnosed with HIV presented with confusion and decline in memory. The MRI shows atrophy and white matter hyperintensity on T2 not involving U-fibers.

Frontotemporal Dementia (FTD)

Frontotemporal dementia, sometimes called frontal lobe dementia, describes a group of diseases characterized by degeneration of nerve cells - especially those in the frontal and temporal lobes of the brain. Unlike Alzheimer's disease, frontotemporal dementia usually does not include formation of amyloid plaques. In many people with frontotemporal dementia, there is an abnormal form of tau protein in the brain, which accumulates into neurofibrillary tangles. This disrupts normal cell activities and may cause the cells to die.

Lewy Body Dementia

Lewy body dementia (LBD) is one of the most common types of progressive dementia. Lewy body dementia usually occurs sporadically, in people with no known family history of the disease. However, rare familial cases have occasionally been reported. In Lewy body dementia, cells die in the brain's cortex (outer layer), and in a part of the mid-brain called the substantia nigra. Many of the remaining nerve cells in the substantia nigra contain abnormal structures called Lewy bodies that are the hallmark of the disease. The symptoms of Lewy body dementia overlap with Alzheimer's disease in many ways, and may include memory impairment, poor judgment, and confusion. However, Lewy body dementia typically also includes visual hallucinations, parkinsonian symptoms such as a shuffling gait (walk) and flexed posture, and day-to-day fluctuations in the severity of symptoms. Patients with Lewy body dementia live an average of 7 years after symptoms begin. There is no cure for Lewy body dementia, and treatments are aimed at controlling the parkinsonian and psychiatric symptoms of the disorder.

Vascular (Multi-Infarct) Dementia 2

There are several types of vascular dementia, which vary slightly in their causes and symptoms. One type, called multi-infarct dementia (MID), is caused by numerous small strokes in the brain. Multi-infarct dementia typically includes multiple damaged areas, called infarcts, along with extensive lesions in the white matter, or nerve fibers, of the brain. Although not all strokes cause dementia, in some cases a single stroke can damage the brain enough to cause dementia. This condition is called single-infarct dementia. Dementia is more common when the stroke takes place on the left side (hemisphere) of the brain and/or when it involves the hippocampus, a brain structure important for memory. Other types of vascular dementia include Binswanger's disease and CADASIL (cerebral autosomal dominant arteriopathy with subcortical infarct and leukoencephalopathy).

Vascular (Multi-Infarct) Dementia 1

Vascular dementia is the second most common cause of dementia, after Alzheimer's disease. It accounts for up to 20 % of all dementias and is caused by brain damage from cerebrovascular or cardiovascular problems - usually strokes. It also may result from genetic diseases, endocarditis (infection of a heart valve), or amyloid angiopathy (a process in which amyloid protein builds up in the brain's blood vessels, sometimes causing hemorrhagic or "bleeding" strokes). In many cases, it may coexist with Alzheimer's disease. Unlike people with Alzheimer's disease, people with vascular dementia often maintain their personality and normal levels of emotional responsiveness until the later stages of the disease. People with vascular dementia frequently wander at night and often have other problems commonly found in people who have had a stroke, including depression and incontinence.

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