Items tagged “neurodegenerative”
12 results found
Article
Frontotemporal lobar degeneration
Frontotemporal lobar degeneration (FTLD) is the pathological description of a group of neurodegenerative disorders characterized by focal atrophy of the frontal and/or temporal cortices. These results in variable clinical manifestations collectively known as frontotemporal dementia (FTD) syndrom...
Article
Multiple system atrophy
Multiple system atrophy (MSA) is a sporadic neurodegenerative disease (one of the synucleinopathies) characterized by varying degrees of cerebellar ataxia, autonomic dysfunction, parkinsonism, and corticospinal dysfunction.
Epidemiology
Multiple systemic atrophy is a sporadic disease, with a ...
Article
Neurodegenerative disease
Neurodegenerative disease is a blanket term encompassing a wide variety of disorders, typically slowly progressive, with variable gradual neurologic dysfunction.
Over the years numerous classification schemas have been described, each adding a layer of confusion for students. The main distinct...
Article
Synucleinopathy
Synucleinopathies are a subgroup of neurodegenerative diseases, characterized by impairment of alpha-synuclein metabolism, resulting in abnormal intracellular deposits. They can further be divided into those with and those without the formation of Lewy bodies, although it should be noted that so...
Article
Shy-Drager syndrome
The Shy-Drager syndrome is a neurodegenerative disease, and one of the manifestations of multiple systemic atrophy (MSA).
Terminology
It is important to note that the current belief that olivopontocerebellar degeneration, Shy-Drager syndrome and striatonigral degeneration are different manifes...
Article
Parkinson disease
Parkinson disease (PD), also known as idiopathic Parkinson disease (iPD), is a neurodegenerative disease and movement disorder characterized by resting tremor, rigidity and hypokinesia due to progressive degeneration of dopaminergic neurons in the substantia nigra.
Epidemiology
Parkinson dise...
Article
Kufor-Rakeb syndrome
Kufor-Rakeb syndrome, also known as Parkinson disease-9 (PARK9), is a rare neurodegenerative disease, considered a form of Mendelian parkinsonism.
Epidemiology
This syndrome is very rare and was first described in an Arab consanguineous kindred 4. It has since been described in select familie...
Article
Cerebral transthyretin-associated amyloidoses
Cerebral involvement can be seen transthyretin-associated amyloidoses and presents as a neurodegenerative disease.
Epidemiology
Age of presentation is very wide, ranging from adolescence to old age 1.
Clinical presentation
Clinical presentation is variable, but includes 1:
dementia
spast...
Article
Posterior cortical atrophy
Posterior cortical atrophy, also known as Benson syndrome, is an uncommon neurodegenerative disease typically affecting individuals in the sixth and seventh decades of life. It is characterized by dysfunction of the parietal, posterior temporal, and occipital lobes which results in progressive a...
Article
Neuronal intranuclear hyaline inclusion disease
Neuronal intranuclear hyaline inclusion disease (NIID) is a slowly progressive neurodegenerative disease characterized by eosinophilic hyaline intranuclear inclusions in the central and peripheral nervous system, and also in the visceral organs.
Epidemiology
Originally considered as primarily ...
Article
Alzheimer disease
Alzheimer disease is a common neurodegenerative disease, responsible for 60-80% of all dementias, and imposing a significant burden on developed nations. It is associated with an accumulation and deposition of cerebral amyloid-β (Aβ) and is the most common cerebral amyloid deposition disease.
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Article
Woodhouse-Sakati syndrome
Woodhouse-Sakati syndrome is a rare autosomal recessive genetic disorder characterized by neurodegeneration resulting from mutations in the DCAF17 gene.
Clinical presentation
Its primary clinical features involve endocrine abnormalities, such as hypogonadism, the absence of secondary sexual ch...
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