Frontotemporal dementia

fronto-temporal dementiaFrontotemporal dementia (FTD)Dementia, frontotemporalfamilial frontotemporal dementiafrontal lobe dementiaFrontotemporal dementiasFTD
The frontotemporal dementias (FTD) encompass six types of dementia involving the frontal or temporal lobes.wikipedia
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Dementia

senilesenilitysenile dementia
The frontotemporal dementias (FTD) encompass six types of dementia involving the frontal or temporal lobes.
Other common types include vascular dementia (25%), Lewy body dementia (15%), and frontotemporal dementia.

Frontotemporal lobar degeneration

frontotemporal lobar degeneration with ubiquitin-positive inclusionsFTLD
One variant is the clinical presentation of frontotemporal lobar degeneration, which is characterized by progressive neuronal loss predominantly involving the frontal or temporal lobes, and typical loss of over 70% of spindle neurons, while other neuron types remain intact.
Frontotemporal lobar degeneration (FTLD) is a pathological process that occurs in frontotemporal dementia.

Corticobasal syndrome

They are: behavioral variant of FTD, semantic variant primary progressive aphasia, nonfluent agrammatic variant primary progressive aphasia, corticobasal syndrome, progressive supranuclear palsy, and FTD associated with motor neuron disease.
Corticobasal syndrome (CBS) is a progressive neurodegenerative disease and a type of frontotemporal dementia.

Amyotrophic lateral sclerosis

ALSLou GehrigLou Gehrig's disease
In rare cases, FTD can occur in patients with motor neuron disease (MND) (typically amyotrophic lateral sclerosis). FTD caused by FTLD-TDP43 has numerous genetic causes. Some cases are due to mutations in the GRN gene, also located on chromosome 17. Others are caused by VCP mutations, although these patients present with a complex picture of multisystem proteinopathy that can include amyotrophic lateral sclerosis, inclusion body myopathy, Paget's disease of bone, and FTD. The most recent addition to the list is a hexanucleotide repeat expansion in intron 1 of C9ORF72. Only one or two cases have been reported describing TARDBP (the TDP-43 gene) mutations in a clinically pure FTD (FTD without MND).
Around half of people with ALS will experience mild changes in cognition and behavior, and 10–15% will show signs of frontotemporal dementia.

Progressive supranuclear palsy

supranuclear palsysupranuclear palsy, progressiveParkinson's disease with hyperextension
They are: behavioral variant of FTD, semantic variant primary progressive aphasia, nonfluent agrammatic variant primary progressive aphasia, corticobasal syndrome, progressive supranuclear palsy, and FTD associated with motor neuron disease.
Some consider PSP, corticobasal degeneration, and frontotemporal dementia to be variations of the same disease.

Granulin

GRNprogranulinhuman granulin gene
FTD caused by FTLD-TDP43 has numerous genetic causes. Some cases are due to mutations in the GRN gene, also located on chromosome 17. Others are caused by VCP mutations, although these patients present with a complex picture of multisystem proteinopathy that can include amyotrophic lateral sclerosis, inclusion body myopathy, Paget's disease of bone, and FTD. The most recent addition to the list is a hexanucleotide repeat expansion in intron 1 of C9ORF72. Only one or two cases have been reported describing TARDBP (the TDP-43 gene) mutations in a clinically pure FTD (FTD without MND).
The 2006 discovery of the GRN mutation in a population of patients with frontotemporal dementia has spurred much research in uncovering the function and involvement in disease of progranulin in the body.

Progressive nonfluent aphasia

progressive nonfluent aphasia (PNFA)
Progressive nonfluent aphasia (PNFA) is characterized by progressive difficulties in speech production.
Problems with writing, reading and speech comprehension can occur as can behavioural features similar to frontotemporal dementia.

C9orf72

FTD caused by FTLD-TDP43 has numerous genetic causes. Some cases are due to mutations in the GRN gene, also located on chromosome 17. Others are caused by VCP mutations, although these patients present with a complex picture of multisystem proteinopathy that can include amyotrophic lateral sclerosis, inclusion body myopathy, Paget's disease of bone, and FTD. The most recent addition to the list is a hexanucleotide repeat expansion in intron 1 of C9ORF72. Only one or two cases have been reported describing TARDBP (the TDP-43 gene) mutations in a clinically pure FTD (FTD without MND). Another gene that has been associated with this condition is C9orf72.
The mutations in C9orf72 are significant because it is the first pathogenic mechanism identified to be a genetic link between familial frontotemporal dementia (FTD) and of amyotrophic lateral sclerosis (ALS).

Pick's disease

pick disease of the brainMorbus PickPick 's disease
It was first described by Arnold Pick in 1892 and was originally called "Pick's disease", a term now reserved for Pick disease, one specific type of frontotemporal dementia. The most severe brain atrophy appears to be associated with Pick's disease, corticobasal degeneration, and TDP pathology associated with behavioral-variant FTD.
This condition is now more commonly called frontotemporal dementia by professionals, and the use of Pick's disease as a clinical diagnosis has fallen out of fashion.

Spindle neuron

spindle neuronsSpindle cellsvon Economo neurons
One variant is the clinical presentation of frontotemporal lobar degeneration, which is characterized by progressive neuronal loss predominantly involving the frontal or temporal lobes, and typical loss of over 70% of spindle neurons, while other neuron types remain intact.
Frontotemporal dementia involves loss of mostly spindle neurons.

Semantic dementia

semantic dementia (SD)
Semantic dementia (SD) is characterized by the loss of semantic understanding, resulting in impaired word comprehension, although speech remains fluent and grammatically faultless.
As the disease progresses, behavioral and personality changes are often seen similar to those seen in frontotemporal dementia.

Corticobasal degeneration

The most severe brain atrophy appears to be associated with Pick's disease, corticobasal degeneration, and TDP pathology associated with behavioral-variant FTD.
Frontotemporal dementia can be an early feature.

Tau protein

tautau proteinsMAPT
Tau-positive frontotemporal dementia with parkinsonism (FTDP-17) is caused by mutations in the MAPT gene on chromosome 17 that encodes the Tau protein It has been determined that there is a direct relationship between the type of tau mutation and the neuropathology of gene mutations. The mutations at the splice junction of exon 10 of tau lead to the selective deposition of the repetitive tau in neurons and glia. The pathological phenotype associated with mutations elsewhere in tau is less predictable with both typical neurofibrillary tangles (consisting of both 3 repeat and 4 repeat tau) and Pick bodies (consisting of 3 repeat tau) having been described. The presence of tau deposits within glia is also variable in families with mutations outside of exon 10. This disease is now informally designated FTDP-17T. FTD shows a linkage to the region of the tau locus on chromosome 17, but it is believed that there are two loci leading to FTD within megabases of each other on chromosome 17.
Hyperphosphorylation of the tau protein (tau inclusions, pTau) can result in the self-assembly of tangles of paired helical filaments and straight filaments, which are involved in the pathogenesis of Alzheimer's disease, frontotemporal dementia, and other tauopathies.

TARDBP

TDP-43TAR DNA Binding Protein (TDP-43)
FTD caused by FTLD-TDP43 has numerous genetic causes. Some cases are due to mutations in the GRN gene, also located on chromosome 17. Others are caused by VCP mutations, although these patients present with a complex picture of multisystem proteinopathy that can include amyotrophic lateral sclerosis, inclusion body myopathy, Paget's disease of bone, and FTD. The most recent addition to the list is a hexanucleotide repeat expansion in intron 1 of C9ORF72. Only one or two cases have been reported describing TARDBP (the TDP-43 gene) mutations in a clinically pure FTD (FTD without MND).
A hyper-phosphorylated, ubiquitinated and cleaved form of TDP-43—known as pathologic TDP43—is the major disease protein in ubiquitin-positive, tau-, and alpha-synuclein-negative frontotemporal dementia (FTLD-TDP, previously referred to as FTLD-U ) and in amyotrophic lateral sclerosis (ALS).

Valosin-containing protein

VCPcdc48
FTD caused by FTLD-TDP43 has numerous genetic causes. Some cases are due to mutations in the GRN gene, also located on chromosome 17. Others are caused by VCP mutations, although these patients present with a complex picture of multisystem proteinopathy that can include amyotrophic lateral sclerosis, inclusion body myopathy, Paget's disease of bone, and FTD. The most recent addition to the list is a hexanucleotide repeat expansion in intron 1 of C9ORF72. Only one or two cases have been reported describing TARDBP (the TDP-43 gene) mutations in a clinically pure FTD (FTD without MND).
MSP can manifest clinically as classical amyotrophic lateral sclerosis (ALS), frontotemporal dementia (FTD), inclusion body myopathy (IBM), Paget's disease of bone (PDB), or as a combination of these disorders.

Disinhibition

disinhibiteddisinhibitoryloss of inhibition
1) Disinhibition
Frontotemporal dementia

Aphasia

aphasicdysphasiaaphasics
Aphasia
Primary progressive aphasia (PPA) is a neurodegenerative focal dementia that can be associated with progressive illnesses or dementia, such as frontotemporal dementia / Pick Complex Motor neuron disease, Progressive supranuclear palsy, and Alzheimer's disease, which is the gradual process of progressively losing the ability to think.

Spontaneous cerebrospinal fluid leak

intracranial hypotensionCSF leaksleaking of CSF
CSF leaks are a known cause of reversible frontotemporal dementia.
There are documented cases of reversible frontotemporal dementia and coma.

Frontal lobe

frontal cortexfrontalfrontal lobes
The frontotemporal dementias (FTD) encompass six types of dementia involving the frontal or temporal lobes. One variant is the clinical presentation of frontotemporal lobar degeneration, which is characterized by progressive neuronal loss predominantly involving the frontal or temporal lobes, and typical loss of over 70% of spindle neurons, while other neuron types remain intact.

Temporal lobe

medial temporal lobetemporaltemporal cortex
The frontotemporal dementias (FTD) encompass six types of dementia involving the frontal or temporal lobes. One variant is the clinical presentation of frontotemporal lobar degeneration, which is characterized by progressive neuronal loss predominantly involving the frontal or temporal lobes, and typical loss of over 70% of spindle neurons, while other neuron types remain intact.

Primary progressive aphasia

progressive aphasiaaphasiaprimary progressive aphasia (PPA)
They are: behavioral variant of FTD, semantic variant primary progressive aphasia, nonfluent agrammatic variant primary progressive aphasia, corticobasal syndrome, progressive supranuclear palsy, and FTD associated with motor neuron disease.

Neuron

neuronsnerve cellsnerve cell
One variant is the clinical presentation of frontotemporal lobar degeneration, which is characterized by progressive neuronal loss predominantly involving the frontal or temporal lobes, and typical loss of over 70% of spindle neurons, while other neuron types remain intact.

Arnold Pick

It was first described by Arnold Pick in 1892 and was originally called "Pick's disease", a term now reserved for Pick disease, one specific type of frontotemporal dementia.

Prevalence

lifetime prevalenceprevalence ratepoint prevalence
Second only to Alzheimer's disease (AD) in prevalence, FTD accounts for 20% of young-onset dementia cases.

Binge eating

bingebinge eatbinging
FTD patients tend to struggle with binge eating and compulsive behaviors.