Behavioral variant of frontotemporal dementia

Behavioral variant of frontotemporal dementia (bv-FTD) is a form of frontotemporal dementia (FTD; see this term), characterized by progressive behavioral impairment and a decline in executive function with frontal lobe-predominant atrophy.



Input patient's signs and symptoms


Narrow down the case reports



Total: 21 (papers)

   


(per page)
Matched Phenotype  Gene  Mutation  MeSH
Rank
(Similarity)		 PMID
(PMCID)
1
(9.2%)		 29662677
(5896852)
 Auto-antibodies against P/Q- and N-type voltage-dependent calcium channels mimicking frontotemporal dementia.
Younes K, Lepow LA, Estrada C, Schulz PE.
SAGE Open Med Case Rep. 2018;6:2050313X17750928.
Inappropriate behavior Episcleritis
2
(5.0%)		 31114177
 Early pathological gambling in co-occurrence with semantic variant primary progressive aphasia: a case report.
Battista P, Capozzo R, Rizzo G, Zecca C, Anastasia A, De Blasi R, Logroscino G.
Clin Interv Aging. 2019;14:727-733.
Aphasia
Gambling Homo sapiens Male Middle Aged
2
(5.0%)		 30415651
 Argyrophilic grain disease presenting as behavioral frontotemporal dementia.
Gil MJ, Serrano S, Manzano MS, Cuadrado ML, Gomez E, Rabano A.
Clin Neuropathol. 2019;38(1):8-13.
Cognitive impairment
Homo sapiens Male Tauopathies
2
(5.0%)		 27737532
 An autopsy case of dementia with Lewy bodies clinically diagnosed to have a behavioral variant of frontotemporal dementia.
Sekiguchi H, Moriwaki M, Iritani S, Habuchi C, Torii Y, Umeda K, Fujishiro H, Yoshida M, Fujita K.
Clin Neuropathol. 2017;36 (2017)(1):23-30.
Dementia
Autopsy Brain Differential Diagnosis Homo sapiens Lewy Body Disease Male
2
(5.0%)		 27327087
 C9ORF72 G4C2-repeat expansion and frontotemporal dementia first reported case in Argentina.
Fernandez Suarez M, Surace E, Harris P, Tapajoz F, Sevlever G, Allegri R, Russo GN.
Neurocase. 2016;22(3):281-4.
Parkinsonism
C9orf72
C9orf72 Protein DNA Repeat Expansion Females Homo sapiens Middle Aged
2
(5.0%)		 27223596
(5790990)
 Schizophrenia as a mimic of behavioral variant frontotemporal dementia.
Kerssens CJ, Krudop WA, Prins ND, van Berckel BN, Rozemuller A, Seeley WW, Scheltens P, Stek ML, Pijnenburg YA.
Neurocase. 2016;22(3):285-8.
Psychosis
Diagnosis Differential Diagnosis Females Homo sapiens Middle Aged Schizophrenia
2
(5.0%)		 27143117
 The behavioral variant of frontotemporal dementia: An analysis of the literature and a case report.
Birkhoff JM, Garberi C, Re L.
Int J Law Psychiatry. 2016;47:157-63.
Apathy
Antisocial Personality Disorder Disruptive, Impulse Control, and Conduct Disorders Expert Testimony Homo sapiens Insanity Defense Male Middle Aged Neuropsychological Tests Personality Disorders Theft
2
(5.0%)		 26329153
 [Developmental Disorders and Dementia].
Midorikawa A.
Brain Nerve. 2015;67(9):1125-32.
Dementia
Age Factors Autism Spectrum Disorders Dementia Developmental Disabilities Differential Diagnosis Homo sapiens Male Parkinson Disease
2
(5.0%)		 25658417
 Neuropsychological findings in a case of punding before and after cessation of pramipexole.
Larson ER.
Clin Neuropsychol. 2015;29(1):166-78.
Punding
Benzothiazoles Cognitive Therapy Dopamine Agonists Homo sapiens Male Middle Aged Parkinson Disease
2
(5.0%)		 23962693
 Neurodegenerative and psychiatric overlap in frontotemporal lobar degeneration: a case of familial frontotemporal dementia presenting with catatonia.
Holm AC.
Int Psychogeriatr. 2014;26(2):345-7.
Cognitive impairment
C9orf72
C9orf72 Protein Catatonia Electroconvulsive Therapy Homo sapiens Male Neuropsychological Tests Proteins Psychiatric Status Rating Scales Psychomotor Agitation Psychotropic Drugs X-Ray Computed Tomography
        

Phenotype(s) retrieved from Orphanet

    Total: 40

HPO ID Term Frequency
HP:0000474 Thickened nuchal skin fold Very frequent (99-80%)
HP:0000708 Behavioral abnormality Very frequent (99-80%)
HP:0000710 Hyperorality Very frequent (99-80%)
HP:0000711 Restlessness Very frequent (99-80%)
HP:0000718 Aggressive behavior Very frequent (99-80%)
HP:0000719 Inappropriate behavior Very frequent (99-80%)
HP:0000723 Restrictive behavior Very frequent (99-80%)
HP:0000733 Stereotypy Very frequent (99-80%)
HP:0000734 Disinhibition Very frequent (99-80%)
HP:0000737 Irritability Very frequent (99-80%)
HP:0000751 Personality changes Very frequent (99-80%)
HP:0000757 Lack of insight Very frequent (99-80%)
HP:0001268 Mental deterioration Very frequent (99-80%)
HP:0002145 Frontotemporal dementia Very frequent (99-80%)
HP:0002354 Memory impairment Very frequent (99-80%)
HP:0002357 Dysphasia Very frequent (99-80%)
HP:0002371 Loss of speech Very frequent (99-80%)
HP:0002442 Dyscalculia Very frequent (99-80%)
HP:0002465 Poor speech Very frequent (99-80%)
HP:0006892 Frontotemporal cerebral atrophy Very frequent (99-80%)
HP:0010522 Dyslexia Very frequent (99-80%)
HP:0010526 Dysgraphia Very frequent (99-80%)
HP:0010529 Echolalia Very frequent (99-80%)
HP:0030213 Emotional blunting Very frequent (99-80%)
HP:0030223 Perseveration Very frequent (99-80%)
HP:0002500 Abnormality of the cerebral white matter Frequent (79-30%)
HP:0011204 EEG with continuous slow activity Frequent (79-30%)
HP:0012658 Abnormal brain FDG positron emission tomography Frequent (79-30%)
HP:0030212 Collectionism Frequent (79-30%)
HP:0000709 Psychosis Occasional (29-5%)
HP:0000741 Apathy Occasional (29-5%)
HP:0001288 Gait disturbance Occasional (29-5%)
HP:0001347 Hyperreflexia Occasional (29-5%)
HP:0002069 Generalized tonic-clonic seizures Occasional (29-5%)
HP:0002071 Abnormality of extrapyramidal motor function Occasional (29-5%)
HP:0002300 Mutism Occasional (29-5%)
HP:0002380 Fasciculations Occasional (29-5%)
HP:0002446 Astrocytosis Occasional (29-5%)
HP:0002493 Upper motor neuron dysfunction Occasional (29-5%)
HP:0012671 Abulia Occasional (29-5%)


Phenotype(s) retrieved from case reports

    Total: 7

HPO ID Term # of case reports
HP:0000726 Dementia 2
HP:0001300 Parkinsonism 2
HP:0002381 Aphasia 2
HP:0000605 Supranuclear gaze palsy 1
HP:0004305 Involuntary movements 1
HP:0100315 Lewy bodies 1
HP:0100754 Mania 1


Causative gene(s) retrieved from Orphanet

    Total: 9

Gene Symbol Gene Name Entrez Gene ID
TMEM106B transmembrane protein 106B 54664
PSEN1 presenilin 1 5663
CHMP2B charged multivesicular body protein 2B 25978
TREM2 triggering receptor expressed on myeloid cells 2 54209
VCP valosin containing protein 7415
MAPT microtubule associated protein tau 4137
GRN granulin precursor 2896
SQSTM1 sequestosome 1 8878
C9ORF72 C9orf72-SMCR8 complex subunit 203228