What are these conditions?
Frontotemporal degeneration (FTD) refers to a family of disorders characterized by the progressive loss of neurons (brain cells) in the frontal and temporal regions of the brain. Although the precise cause is unknown, FTD is thought to result from abnormal accumulation of misfolded proteins, which disrupt, and eventually kill, brain cells. Patients with Frontotemporal degeneration can present with different clinical symptoms, ranging from behavioral impairments to language or motor dysfunction. Clinical diagnoses that fall within the FTD spectrum include: behavioral variant FTD (bvFTD), primary progressive aphasia (PPA), Corticobasal Syndrome (CBS), Progressive Supranuclear Palsy (PSP), and FTD with Amyotrophic Lateral Sclerosis (ALS).
The Penn FTD Center's research interests also include other neurological disorders such as Alzheimer's Syndromes, Parkinson's Syndromes and Rapidly Progressing Dementias.
|Rapidly Progressing Dementias||
Most neurodegenerative diseases such as Frontotemporal degeneration or Alzheimer’s disease have a slow, gradual progression over several years. In some patients, however, cognitive, motor or behavioral symptoms may emerge and progress over the course of weeks to months. These conditions are collectively referred to as “Rapidly-Progressive Dementias.”
|Behavioral variant FTD (bvFTD)||
The behavioral variant of Frontotemporal dementia (bvFTD) is the most common presentation of Frontotemporal degeneration. This variant is characterized by progressive atrophy (cell loss) in frontal and anterior temporal regions of the brain leading to alterations in complex thinking, personality and behavior.
|Primary progressive aphasia (PPA)||As the name implies, primary progressive aphasia is a disorder of language (aphasia) that is progressive in nature and not due to neurological diseases that are easily seen on an MRI like stroke, head trauma, and cancer.|
|Corticobasal Syndrome (CBS)||
Corticobasal Syndrome (CBS) is a progressive condition affecting the cortex or grey matter (outer surface) of the brain. This part of the brain is most concerned with higher level cognition. CBS also affects the basal ganglia and brainstem, which are deeper structures in the brain responsible for the smooth execution of movements. Because CBS affects the parts of the brain responsible for both cognitive function and movement, both of these abilities may be affected in this condition. Coticobasal Syndrome can often be confused with Parkinson’s disease, as individuals with CBS may move slowly, be rigid in their movements, and have a tremor.
|Progressive Supranuclear Palsy (PSP)||
Overview and Age of Onset
Progressive Supranuclear Palsy (PSP) is a progressive condition that affects mobility, vision, speech, and cognition. Symptoms can begin as young as 40, but the average age of diagnosis is often in the 60s.
|FTD with Amyotrophic Lateral Sclerosis (ALS)||
Overview and Age of Onset
Amyotrophic Lateral Sclerosis (ALS), also known as Motor Neuron Disease (MND) or Lou Gehrig’s Disease, is a disorder that is characterized by the progressive degeneration of neurons in the brain and spinal cord responsible for the voluntary control of muscle. Loss of these neurons and the resulting weakness that it produces causes ever increasing disability. Because the muscles of swallowing and or breathing are eventually affected, ALS is inevitably a life threatening disorder.
|Alzheimer's Syndromes||Typical Alzheimer's disease is diagnosed when there are problems with memory, language, visuospatial functioning and executive resources. Microscopic inspection of the brain often reveals the accumulation of misfolded proteins known as tau and amyloid. There are also atypical presentations of Alzheimer's disease.|
Overview and Pathology
Some patients with neurodegenerative disease may develop significant difficulty with balance, rigidity, tremor or gait. The major diseases in this category are Parkinson’s disease (PD), Parkinson’s disease with Dementia (PDD) and Dementia with Lewy bodies (DLB). These related conditions are collectively known as “synucleinopathies” or “Lewy body spectrum disorders.” This is because the characteristic neuronal inclusions (“Lewy bodies”) seen at autopsy in these disorders are primarily composed of the protein, alpha-synuclein, and these inclusions were first observed by Frederic Lewy.