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Pick’s Disease

Updated : March 11, 2024





Background

With temporal and frontal cortical deterioration, frontotemporal dementia is a progressive neurodegenerative disease characterized by changes in behavior and linguistic deficits. It is a heterogeneous disorder characterized by a complex constellation of interlocking pathological clinical, and genetic factors that culminate in a unique disease phenotype.

Pick’s disease is a dementia illness that affects pre-senile people and is split into two subtypes: primary progressive aphasia (PPA) and behavioral variety (bvFTD).

Nonfluent/agrammatic variant (nfvPPA), logopenic aphasia, and semantic variation (svPPA) are the three types of PPA. The frontotemporal diagnosis includes overlapping syndromes such as motor neuron disease (FTD-MND), corticobasal syndrome (CBS), and progressive supranuclear palsy (PSP-S).

Frontotemporal dementia has an insidious onset, followed by a gradual deterioration from identification to death that places a considerable physical, social, mental, and financial burden on caregivers, patients, and families.

Epidemiology

Frontotemporal dementia is the 2nd most prevalent cause of early-onset dementia in people under sixty-five, accounting for ten percent of all pathologically diagnosed cases. Frontotemporal dementia accounts for three percent of all dementias in patients of all ages.

While the illness is most common in people in their fifth and sixth decades of life, it has also been seen in people as young as their 30s and as old as seventy. 60% of participants of frontotemporal dementia are behavioral, with rapidly progressive aphasia subtypes appearing less commonly.

Due to the diagnostic challenges of frontotemporal dementia, estimates for prevalence and incidence range from 15 to 22 per 100,000 and 2.7 to 4.1 per 100,000, respectively. These values are likely under-representing figures.

The numbers in the United States and in other nations are comparable; however, developing nations are underrepresented mostly in epidemiologic research, and statistics on non-white communities are scarce. Gender disparities in distribution have not been discovered in studies.

Anatomy

Pathophysiology

While the pathophysiology of Alzheimer’s disorder is well-understood and similar across instances, the mechanism underlying frontotemporal dementia is varied and complex, necessitating further research.

The predominant pathogenic emphasis is atrophy of the anterior temporal and frontal lobes caused by the intraneuronal accumulation of aberrant protein inclusion bodies.

The main proteins responsible for frontotemporal dementia have been identified as tau, RNA-binding protein fused in sarcoma (FUS), and transactive response DNA binding protein 43 (TDP-43).

They’ve been linked to diverse clinical expressions of different frontotemporal dementia subtypes and have been linked to individual genetic variants.

Etiology

Even though most frontotemporal dementia cases are sporadic, the disease is highly heritable, with up to forty percent of individuals reporting a family history of dementia. 10 to 20 percent of participants have genetic alterations that are inherited in an autosomal dominant way.

Microtubule-associated protein tau (MAPT), C9orf72, and progranulin (GRN) are the 3 primary genes involved in illness etiology, with a variety of other genes involved less frequently.

Few researchers have looked at modifiable complications for frontotemporal dementia; nevertheless, diabetes, autoimmune illnesses, and a history of head injury have all been connected to the concept of frontotemporal dementia.

Hypertension, obesity, a history of cerebrovascular accidents, and smoking were all investigated. More research is needed to establish these links and to explicitly advocate lifestyle changes as a way to reduce the incidence of frontotemporal dementia.

Genetics

Prognostic Factors

Frontotemporal dementia is variable, and its prevalence is minimal compared to Alzheimer’s disease, life-expectancy data and making reliable predictions are difficult to come by.

Depending on the subset of phenotypes being studied, survival rates have a wide range. From diagnosis until death, patients with the motor neuron disease subtype have the shortest illness course, lasting two to three years.

Patients with behavioral variant frontotemporal dementia have a nine-year median life expectancy, whereas those with semantic dementia have a twelve-year median life expectancy, which is comparable to the generally delayed progressive Alzheimer’s dementia.

Clinical History

Physical Examination

Age group

Associated comorbidity

Associated activity

Acuity of presentation

Differential Diagnoses

Laboratory Studies

Imaging Studies

Procedures

Histologic Findings

Staging

Treatment Paradigm

by Stage

by Modality

Chemotherapy

Radiation Therapy

Surgical Interventions

Hormone Therapy

Immunotherapy

Hyperthermia

Photodynamic Therapy

Stem Cell Transplant

Targeted Therapy

Palliative Care

Medication

 

arimoclomol (FDA Approval Pending) 

FDA Approval Pending for treatment of Niemann-Pick disease Type C



 
 

Media Gallary

References

https://www.ncbi.nlm.nih.gov/books/NBK562226/

Pick’s Disease

Updated : March 11, 2024




With temporal and frontal cortical deterioration, frontotemporal dementia is a progressive neurodegenerative disease characterized by changes in behavior and linguistic deficits. It is a heterogeneous disorder characterized by a complex constellation of interlocking pathological clinical, and genetic factors that culminate in a unique disease phenotype.

Pick’s disease is a dementia illness that affects pre-senile people and is split into two subtypes: primary progressive aphasia (PPA) and behavioral variety (bvFTD).

Nonfluent/agrammatic variant (nfvPPA), logopenic aphasia, and semantic variation (svPPA) are the three types of PPA. The frontotemporal diagnosis includes overlapping syndromes such as motor neuron disease (FTD-MND), corticobasal syndrome (CBS), and progressive supranuclear palsy (PSP-S).

Frontotemporal dementia has an insidious onset, followed by a gradual deterioration from identification to death that places a considerable physical, social, mental, and financial burden on caregivers, patients, and families.

Frontotemporal dementia is the 2nd most prevalent cause of early-onset dementia in people under sixty-five, accounting for ten percent of all pathologically diagnosed cases. Frontotemporal dementia accounts for three percent of all dementias in patients of all ages.

While the illness is most common in people in their fifth and sixth decades of life, it has also been seen in people as young as their 30s and as old as seventy. 60% of participants of frontotemporal dementia are behavioral, with rapidly progressive aphasia subtypes appearing less commonly.

Due to the diagnostic challenges of frontotemporal dementia, estimates for prevalence and incidence range from 15 to 22 per 100,000 and 2.7 to 4.1 per 100,000, respectively. These values are likely under-representing figures.

The numbers in the United States and in other nations are comparable; however, developing nations are underrepresented mostly in epidemiologic research, and statistics on non-white communities are scarce. Gender disparities in distribution have not been discovered in studies.

While the pathophysiology of Alzheimer’s disorder is well-understood and similar across instances, the mechanism underlying frontotemporal dementia is varied and complex, necessitating further research.

The predominant pathogenic emphasis is atrophy of the anterior temporal and frontal lobes caused by the intraneuronal accumulation of aberrant protein inclusion bodies.

The main proteins responsible for frontotemporal dementia have been identified as tau, RNA-binding protein fused in sarcoma (FUS), and transactive response DNA binding protein 43 (TDP-43).

They’ve been linked to diverse clinical expressions of different frontotemporal dementia subtypes and have been linked to individual genetic variants.

Even though most frontotemporal dementia cases are sporadic, the disease is highly heritable, with up to forty percent of individuals reporting a family history of dementia. 10 to 20 percent of participants have genetic alterations that are inherited in an autosomal dominant way.

Microtubule-associated protein tau (MAPT), C9orf72, and progranulin (GRN) are the 3 primary genes involved in illness etiology, with a variety of other genes involved less frequently.

Few researchers have looked at modifiable complications for frontotemporal dementia; nevertheless, diabetes, autoimmune illnesses, and a history of head injury have all been connected to the concept of frontotemporal dementia.

Hypertension, obesity, a history of cerebrovascular accidents, and smoking were all investigated. More research is needed to establish these links and to explicitly advocate lifestyle changes as a way to reduce the incidence of frontotemporal dementia.

Frontotemporal dementia is variable, and its prevalence is minimal compared to Alzheimer’s disease, life-expectancy data and making reliable predictions are difficult to come by.

Depending on the subset of phenotypes being studied, survival rates have a wide range. From diagnosis until death, patients with the motor neuron disease subtype have the shortest illness course, lasting two to three years.

Patients with behavioral variant frontotemporal dementia have a nine-year median life expectancy, whereas those with semantic dementia have a twelve-year median life expectancy, which is comparable to the generally delayed progressive Alzheimer’s dementia.

arimoclomol (FDA Approval Pending) 

FDA Approval Pending for treatment of Niemann-Pick disease Type C



https://www.ncbi.nlm.nih.gov/books/NBK562226/