Descriptions of the neurodegenerative diseases seen and studied at the MAC

Progressive Supranuclear Palsy

Progressive supranuclear palsy (PSP) is a degenerative disease of the brain leading to difficulties with walking and balance, problems with eye movements, changes in behavior, difficulty with speech and swallowing, and dementia.

What is progressive supranuclear palsy (PSP)?

Progressive supranuclear palsy (PSP) is a degenerative brain disease leading to difficulties with walking and balance, problems with eye movements, changes in behavior, difficulty with speech and swallowing, and dementia.

Primary Progressive Aphasia

Primary progressive aphasia is caused by degeneration in the areas of the brain that control speech and language. This type of aphasia begins gradually, with speech or language symptoms that will vary depending on the brain areas affected by the disease.

What is primary progressive aphasia (PPA)?

Posterior Cortical Atrophy

Posterior cortical atrophy (PCA), also called Benson's syndrome, is the visual variant of Alzheimer’s disease. It is a clinical syndrome in which complex visual processing is progressively disrupted owing to a neurodegenerative disorder.

What is posterior cortical atrophy (PCA)?

Mild Cognitive Impairment

In contrast to Alzheimer's where other cognitive skills are affected, mild cognitive impairment (MCI) is defined by deficits in memory that do not significantly impact daily functioning. Memory problems may be minimal to mild and hardly noticeable to the individual.

What is mild cognitive impairment (MCI)?

HIV-Related Cognitive Impairment

The human immunodeficiency virus (HIV) causes chronic inflammation throughout the body and brain. Without treatment, over 50% of subjects will develop clinically relevant central nervous system (CNS) symptoms. While antiretroviral therapies that reduce inflammation have greatly decreased the frequency of dementia in HIV, milder forms of impairment remain frequent.

What is HIV-related cognitive impairment?

The Human Immunodeficiency Virus (HIV) can infect the brain and impair central nervous system (CNS) function. With the advent of antiretroviral therapies, the most severe forms of HIV-associated dementia significantly decreased in prevalence. The subtler forms of HIV-associated neurocognitive disorders, however, remain frequent.

The major focus of our research is the impact of HIV on the central nervous system and cognitive functioning. Cognitive impairment in HIV is categorized into three groups—

Huntington's Disease

Huntington disease (HD) is a progressive, neurodegenerative disorder typically characterized by involuntary movements (chorea), behavioral and personality changes and cognitive decline (dementia). It is caused by a dominantly inherited gene mutation that can be passed down from generation to generation. HD is an illness with profound neurological and psychiatric features.

What is Huntington disease (HD)?

Huntington disease (HD) results from degeneration of neurons of structures deep within the brain, the basal ganglia, which are responsible for movement and coordination. Structures and circuitry responsible for thought, perception, emotions and memory are also affected, likely due to connections from the basal ganglia to the frontal lobes. Subsequently, there is great variability in the expression of HD, even within the same family.

Frontotemporal Dementia

Frontotemporal dementia is a group of related conditions resulting from the progressive degeneration of the temporal and frontal lobes of the brain. These areas of the brain play a significant role in decision-making, behavioral control, emotion and language.

Lewy Body Dementias

Lewy body dementias include dementia with Lewy bodies (DLB) and Parkinson’s disease with dementia (PDD) and are the second most frequent cause of dementia in elderly adults. Common symptoms include problems with movement, visual hallucinations, and fluctuations in thinking skills or attention.

What are Lewy body dementias?

Creutzfeldt-Jakob Disease

Creutzfeldt-Jakob disease is a human prion disease caused by a normal protein that becomes misshapen into a “prion,” builds up in the brain and disrupts normal brain function. It is a rare disorder that is fatal, usually within 6 months of diagnosis and a year of the first symptom.

Corticobasal Degeneration

Corticobasal degeneration is a progressive neurological disorder that affects nerve cells that control walking, balance, mobility, vision, speech and swallowing.

What is corticobasal degeneration (CBD)?

Corticobasal degeneration, sometimes referred to as corticobasal ganglionic degeneration (CBGD), is considered to be part of the "Pick complex" of neurodegenerative diseases because it is clinically, genetically and pathologically similar to frontotemporal dementia (FTD). It is characterized by nerve cell loss and atrophy (shrinkage) of multiple areas of the brain including the cerebral cortex and the basal ganglia.

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