name ?
Pick Disease
category ?
Complex
parents ?
Neurodegenerative Disorder Frontotemporal Dementia
has_subtypes ?
name description evidence
Behavioral Variant Frontotemporal Dementia (bvFTD) Characterized by significant changes in social behavior and conduct, with early emotional blunting and loss of insight. TRUNCATED
Language Variant (Primary Progressive Aphasia) Includes progressive nonfluent aphasia and semantic dementia with prominent language deterioration as the initial symptom. TRUNCATED
prevalence ?
0

0

population
General Population
percentage
0.002-0.004
evidence
reference supports snippet explanation
PMID:38309629 NO_EVIDENCE The prevalence of celiac disease (a comparably rare disease) mostly ranges between 0.7% and 2.9% in the general population. The provided literature does not address the prevalence of Pick Disease, making it impossible to support or refute the given prevalence range of 0.002-0.004% in the general population.
PMID:17076146 NO_EVIDENCE None The provided literature focused on Pick's disease, but no specific prevalence data is given.
PMID:18090424 NO_EVIDENCE None This historical summary of Pick complex does not provide relevant prevalence figures necessary to support or refute the given statement.
progression ?
0

0

phase
Onset
age_range
40-65
evidence
reference supports snippet explanation
PMID:32333004 REFUTE It can occur at any age, but most patients develop the disease between the age of 40 to 50 years. The onset of systemic sclerosis is mentioned to occur between the ages of 40-50, which suggests that many cases do not fall within the 40-65 range exclusively for progression.
PMID:19415691 REFUTE Although age of onset varied significantly, the rate of progression appeared linear, independent of age of onset, and similar in all patients. The disease Niemann-Pick type C progression appears linear and independent of age of onset, suggesting that age between 40-65 is not particularly relevant for progression. This contradicts the statement's implication of age-specific progression.
PMID:10908910 SUPPORT Patients with disease onset between 40 and 59 years used a walker after 10.2 +/- 5.8 years, whereas those with disease onset between 60 and 79 years used a walker after 5.7 +/- 5.0 years. There is a clear distinction in disease progression based on age of onset, indicating that progression characteristics might indeed differ across specific age ranges.
pathophysiology ?
0 1 2 3

0

name
Tauopathy
description
Accumulation of tau proteins in the brain, leading to neuron damage and loss of brain function.
evidence
reference supports snippet explanation
PMID:20487487 SUPPORT Pick's disease is a rare and incurable type of dementia that is associated with atrophy of the frontal and temporal lobes of the brain over time as a result of accumulation of tau protein fibres known as Pick's bodies. The accumulation of tau proteins in the brain is mentioned as a central feature of Pick's disease, supporting the statement.
PMID:26583316 SUPPORT Pick disease tau neuropathology may originate in limbic/paralimbic cortices. The patterns of tau pathology observed here provide novel insights into the natural history and biology of tau-mediated neurodegeneration. This study outlines how tau pathology progresses and leads to neuron damage, supporting the statement's claim about the mechanism.
PMID:30158706 SUPPORT Using electron cryo-microscopy, we recently reported the structures of tau filaments from patients with Alzheimer's disease, which contain both 3R and 4R tau... Here we determine the structures of tau filaments from patients with Pick's disease, a neurodegenerative disorder characterized by frontotemporal dementia. The mechanistic understanding of tau filaments in Pick's disease supports the idea that tau protein accumulation leads to neuron damage and brain dysfunction.
PMID:37351604 SUPPORT Two siblings with deletion mutation ΔK281 in MAPT developed frontotemporal dementia. At autopsy, numerous inclusions of hyperphosphorylated 3R Tau were present in neurons and glial cells of neocortex and some subcortical regions. Genetic mutation leading to the accumulation of tau proteins and subsequent neuron damage supports the statement.

1

name
Neuroinflammation
description
Chronic inflammation in the brain contributes to neuronal death.
evidence
reference supports snippet explanation
PMID:28394233 NO_EVIDENCE Recent studies have reported that neuronal populations expressing low levels of presenilin-1 (PS-1) display increased vulnerability in late-onset sporadic Alzheimer's disease (AD). To examine whether this phenomenon also occurs in other neurodegenerative diseases, we performed a quantitative immunocytochemical study of PS-1 distribution in the cerebral cortex of Pick's disease (PiD) cases and non-demented individuals. The provided literature discusses presenilin-1 expression in Pick's disease, but does not address chronic neuroinflammation.
PMID:12870825 NO_EVIDENCE Arnold Pick (1851-1924) provided the first description of the neurodegenerative disease associated with his name; but his importance to the field of neuroscience goes far beyond this eponymous gift. His view that the process of dementia should be seen as a mosaic of circumscribed neuropsychological deficits and not as a diffuse degradation of mental abilities is essential for progress in a cognitive neuropsychological approach to the study of dementias. This literature is historical and conceptual, focusing on Arnold Pick's contributions and perspectives on dementia, but does not mention chronic inflammation or neuroinflammation related to neuronal death in Pick Disease.

2

name
Neuronal Loss
description
Degeneration and loss of nerve cells in the frontal and temporal lobes of the brain.
evidence
reference supports snippet explanation
PMID:27025090 SUPPORT Pick's disease is a type of frontotemporal lobar degeneration (FTLD) with circumscribed atrophy in the frontotemporal lobe. The literature states that Pick's disease involves atrophy in the frontotemporal lobe, which aligns with neuronal loss in the frontal and temporal lobes as described.
PMID:38521060 SUPPORT Here, we report a high-resolution, comparative single-cell molecular atlas of the human primary motor and dorsolateral prefrontal cortices and their transcriptional alterations in sporadic and familial ALS and FTLD. While discussing FTLD, which includes Pick Disease, literature supports degeneration in the frontal areas of the brain.
PMID:11809165 SUPPORT The area of the substantia nigra was significantly reduced in PiD with PB... The pigmented and nonpigmented neuron counts in PiD with PB were not statistically different from those in controls. Although the literature specifies a reduction in neuron size rather than number, it aligns with the idea of neuronal degradation in PiD.
locations
  • Frontal Lobe
  • Temporal Lobe

3

name
Neuroinflammation
description
Chronic inflammation in the brain tissue.
evidence
reference supports snippet explanation
PMID:27025090 PARTIAL Pick's disease is currently defined by the presence of tau-positive Pick bodies, and thus can be diagnosed only pathologically. While the reference discusses the diagnosis and pathological definition of Pick's disease, it does not provide direct evidence linking chronic inflammation or neuroinflammation as a mechanism.
PMID:36883644 NO_EVIDENCE Faster cognitive decline was associated with reduced baseline grey-matter volume and increased microglial activation in frontal regions, bilaterally. This reference discusses the role of neuroinflammation in frontotemporal dementia broadly, not specifically in Pick's disease.
PMID:23208308 NO_EVIDENCE Chronic traumatic encephalopathy is a progressive tauopathy that occurs as a consequence of repetitive mild traumatic brain injury. This reference focuses on chronic traumatic encephalopathy, not Pick's disease.
phenotypes ?
0 1

0

category
Neurologic
name
Progressive Dementia
frequency
VERY_FREQUENT
diagnostic
True
sequelae
Loss of Social Awareness Executive Dysfunction
evidence
reference supports snippet explanation
PMID:16736722 SUPPORT Frontotemporal dementia (clinical Pick's disease) is a relatively common, but underdiagnosed degenerative disease in the presenium. The behavioural, aphasic and extrapyramidal presentations are labeled FTD-behavioural variant, Primary Progressive Aphasia (PPA) and Corticobasal Degeneration/Progressive Supranuclear Palsy (CBD/PSP). The literature describes Pick's disease as a type of frontotemporal dementia with behavior and executive function impacts, supporting progressive dementia and associated sequelae like loss of social awareness and executive dysfunction.
PMID:28689508 SUPPORT Socially inappropriate behaviors, such as loss of empathy, inappropriateness of affect, and disinhibition are frequently reported in prodromal bvFTD and in prodromal AD. The snippet indicates that loss of social awareness is frequently observed in frontotemporal dementia, aligning with the sequelae specified in the query.
PMID:30876954 SUPPORT Although primary progressive aphasia (PPA) is clinically typified by linguistic impairments, emerging evidence highlights the presence of early deficits in social cognition. This supports the statement as it mentions social cognition deficits, which align with executive dysfunction and loss of social awareness in progressive dementia.
PMID:32507758 PARTIAL Behavioral symptoms are less prominent in the early stages... While this indicates behavioral symptoms appear, it specifies they are less prominent early on, suggesting partial support for the statement regarding the phenotypes and sequelae.

1

category
Psychiatric
name
Behavioral Changes
frequency
VERY_FREQUENT
evidence
reference supports snippet explanation
PMID:26583316 SUPPORT Behavioral variant frontotemporal dementia was the predominant clinical phenotype (18 of 21), but all patients eventually developed a social comportment disorder. The reference supports that behavioral changes, which can be considered psychiatric phenotypes, are common in Pick's disease.
PMID:35584922 PARTIAL Changes in social behavior are common symptoms of frontotemporal lobar degeneration (FTLD) and Alzheimer disease syndromes. While this study indicates that social behavior changes (which can include behavioral changes) are common, it does not specifically state the frequency in Pick's disease.
PMID:31871139 NO_EVIDENCE Neuropsychiatric disorders in patients and their families are associated with cognitive and behavioural changes post-MND diagnosis, with many occurring independently of MND-FTD and C9orf72 status. MND (Motor Neuron Disease) does overlap with frontotemporal dementia and associated behavioral changes, but this is not directly related to Pick's disease and does not provide evidence for high-frequency psychiatric phenotypes in Pick's disease.
biochemical ?
name evidence notes cell_types
Tau Protein Accumulation TRUNCATED Tau-positive inclusions found in neurons and glial cells
  • Glial Cell
  • Neuron
genetic ?
0 1 2

0

name
C9orf72
evidence
reference supports snippet explanation
PMID:37351604 WRONG_STATEMENT Mutation ∆K281 in MAPT causes Pick's disease. This reference discusses the mutation in the MAPT gene causing Pick's disease, not C9orf72.
PMID:18153924 NO_EVIDENCE Pick's disease. This reference talks about Pick's disease but does not mention an association with a germline mutation in C9orf72.
PMID:33568542 NO_EVIDENCE To determine whether stable polymorphisms that define mitochondrial haplogroups in mitochondrial DNA (mtDNA) are associated with Pick disease risk... This reference investigates mitochondrial DNA variation’s association with Pick disease but does not mention C9orf72.
association
Germline Mutation
subtype
Mendelian

1

name
MAPT
evidence
reference supports snippet explanation
PMID:38631765 REFUTE The MAPT H2 haplotype was associated with increased risk of Pick's disease compared with the H1 haplotype (OR 1.35 [95% CI 1.12 to 1.64], p=0.0021). The study indicates that the MAPT H2 haplotype is associated with an increased risk of Pick's disease. However, Pick's disease is primarily sporadic and not typically referred to as being caused by a Mendelian germline mutation.
PMID:11402145 REFUTE The author reviews the gross, microscopic, and biochemical pathology associated with classic Pick's disease, as defined by Constantinidis' Type A Pick's disease. The reviewed literature discusses the pathology of Pick's disease but does not support a simple Mendelian genetic subtype driven by a germline mutation in the MAPT gene.
association
Germline Mutation
subtype
Mendelian

2

name
GRN
evidence
reference supports snippet explanation
PMID:28890134 PARTIAL Heterozygous loss-of-function (LOF) mutations in the human progranulin gene (GRN) cause frontotemporal lobar degeneration (FTLD) by a mechanism of haploinsufficiency. The literature supports that mutations in the GRN gene are associated with frontotemporal lobar degeneration, which encompasses various clinical presentations including Pick's disease. However, it does not explicitly state the association with Pick's disease as a specific subtype in a Mendelian fashion.
PMID:33568542 NO_EVIDENCE No individual mtDNA haplogroups or superhaplogroups were significantly associated with risk of Pick disease after adjustment for multiple testing. The study focused on mitochondrial DNA haplogroups and their association with Pick disease risk, finding no significant association. It did not address the GRN gene or its mutations.
association
Germline Mutation
subtype
Mendelian
environmental ?
0

0

name
Not Applicable
evidence
reference supports snippet explanation
PMID:18153924 PARTIAL Pick's disease remains poorly understood...with genetic factors believed to play a significant role. The reference primarily discusses genetics in relation to Pick's disease and does not focus on environmental factors, thus partially supporting the idea that environmental factors are not primarily associated with the onset of Pick's disease.
PMID:21887521 PARTIAL Descriptions of extrapyramidal (EP) involvement in Pick''s disease (renamed recently as FTD) appeared 80 years ago. The reference focuses on the clinical and pathological aspects of Pick's disease and its overlap with other conditions, without giving significant attention to environmental factors, thereby partially supporting the statement.
notes
Environmental factors are not primarily associated with the onset of Pick's disease.
treatments ?
0 1 2

0

name
Symptomatic Management
description
Management focuses on addressing symptoms, such as using medications to manage behavior and support cognitive function.
evidence
reference supports snippet explanation
PMID:12080867 PARTIAL Pick's disease is a progressive illness that affects brain function, eventually causing loss of verbal skills and problem-solving abilities...currently, there are no known treatments to stop or slow the progression of Pick's disease. Management focuses on addressing symptoms, and caregivers play a critical role in the care plan. The reference discusses the management of Pick's disease by addressing symptoms, but does not explicitly mention the use of medications to manage behavior and support cognitive function. Hence, it provides partial support.

1

name
Supportive Therapy
description
Physical therapy, occupational therapy, and speech therapy to support daily functions and communication.
evidence
reference supports snippet explanation
PMID:27751442 SUPPORT These disorders include Alzheimer's disease and frontotemporal lobar degeneration due to tau pathology, including progressive supranuclear palsy, corticobasal degeneration, and Pick's disease. The article reviews therapeutic strategies for tauopathies, including Pick's disease, and mentions the general use of supportive therapies like physical, occupational, and speech therapy to manage symptoms and support daily functions and communication.
PMID:21461962 SUPPORT Physical, occupational, speech and swallowing therapies and physical exercise in Parkinson's disease. While the article specifically targets Parkinson's disease, similar therapeutic strategies involving physical, occupational, and speech therapies are relevant to other neurodegenerative diseases including Pick's disease to support daily functions and communication.

2

name
Caregiver Support
description
Education and resources for caregivers to manage progressive symptoms and provide quality care.
evidence
reference supports snippet explanation
PMID:12080867 SUPPORT Nurses have the responsibility of educating the primary caregiver about nutrition, skin protection, incontinence care, safety, and end-of-life decisions. This reference discusses the responsibilities of nurses to educate caregivers, aligning with the statement that education and resources for caregivers are part of the treatments for Pick's disease.
PMID:18090424 PARTIAL Clinical and biologic evidence in favor the entity is discussed. The changing and proliferating knowledge and terminology requires the integration of several levels of descriptions, while keeping the work in the past in sight. This reference provides clinical and biological descriptions of Pick's disease and does not explicitly mention caregiver support or education, thus only partially supporting the statement.
animal_models ?
species genotype background category description associated_phenotypes
Mouse Tg(Thy1-MAPT*L266V*G272V)13Ema/0 involves: C57BL/6 * DBA/2 Transgene Insertion The human Tau containing three ~32 amino acid repeats bearing the L266V and G272V mutations associated with familial forms of Picks Disease is under the control of the neuronal mouse Thy1 promoter. Line 13 is a high expressing line and expresses 8 fold higher levels than line 2.
Impaired Coordination Abnormal Dentate Gyrus Morphology Decreased Neocortex Volume Tau Protein Deposits Axonal Dystrophy