Page last updated: 2024-09-27

Aphasia, Primary Progressive

A progressive form of dementia characterized by the global loss of language abilities and initial preservation of other cognitive functions. Fluent and nonfluent subtypes have been described. Eventually a pattern of global cognitive dysfunction, similar to ALZHEIMER DISEASE, emerges. Pathologically, there are no Alzheimer or PICK DISEASE like changes, however, spongiform changes of cortical layers II and III are present in the TEMPORAL LOBE and FRONTAL LOBE. (From Brain 1998 Jan;121(Pt 1):115-26)

Synonyms(3)

Synonym
Mesulam Syndrome
Aphasia, Primary Progressive
Mesulam's Syndrome

Research Excerpts

Treatment

ExcerptReference
"Thirty-six behavioral variety FTD and primary progressive aphasia (PPA) patients were treated in an open-label period of 18 weeks and a randomized, placebo-controlled phase for 8 weeks with galantamine."( Blair, M; Brashear, R; Davidson, W; Jesso, S; Kertesz, A; Light, M; Morlog, D, 2008)

Research

Studies (97)

TimeframeStudies, This Condition (%)All Conditions %
pre-19900 (0.00)23.3326
1990's2 (2.06)12.5806
2000's16 (16.49)18.1394
2010's64 (65.98)28.8240
2020's15 (15.46)9.53
DrugIndicatedRelationship StrengthStudiesTrials
gamma-aminobutyric acid0low10
creatine0low30
donepezil0low20
fluoxetine0low10
memantine0low10
trazodone0low10
prednisone0low10
aspartic acid0low10
lysine0low10
cyclohexanol0low10
thiazoles0low210
galantamine0medium11
alpha-aminopyridine0low40
bromocriptine0medium11
transferrin0low10
venlafaxine hydrochloride0low10
n-acetylaspartic acid0low10
fluorodeoxyglucose f180medium421
n-methylpiperidin-4-yl propionate0low10
stilbenes0low10
2-(4'-(methylamino)phenyl)-6-hydroxybenzothiazole0low210
cystine0low10
cysteine0medium41
alpha-synuclein0low20
technetium tc 99m exametazime0medium51
florbetapir f 180low40
piperidines0low20
amyloid beta-peptides0low20
technetium tc 99m bicisate0medium41

Protein Targets (369)

ProteinPotency MeasurementsInhibition MeasurementsActivation MeasurementsDrugs
Amyloid-beta precursor protein0303
AR protein7007
retinoic acid nuclear receptor alpha variant 17007
estrogen nuclear receptor alpha5005
thyroid stimulating hormone receptor1001
thyroid hormone receptor beta isoform 26006
Voltage-dependent calcium channel gamma-2 subunit3003
Heat shock protein HSP 90-alpha0011
Botulinum neurotoxin type A 0101
Neutrophil cytosol factor 10101
Glutamate receptor 23003
Nitric oxide synthase, endothelial0101
Nitric oxide synthase, brain0101
Nitric oxide synthase, brain 0101
Nitric oxide synthase, inducible0101
Kappa-type opioid receptor0112
Phosphatidylinositol 4-kinase alpha0101
Mu-type opioid receptor0011
Tyrosine-protein kinase 0011
Phosphatidylinositol 4-kinase type 2-beta0101
Phosphatidylinositol 4-kinase type 2-alpha0101
Phosphatidylinositol 4-kinase beta0101
Chain A, MAJOR APURINIC/APYRIMIDINIC ENDONUCLEASE3003
Chain A, TYROSYL-DNA PHOSPHODIESTERASE2002
Chain A, Ferritin light chain2002
phosphopantetheinyl transferase1001
aldehyde dehydrogenase 1 family, member A14004
arylsulfatase A2002
euchromatic histone-lysine N-methyltransferase 26006
Bloom syndrome protein isoform 12002
cytochrome P450 2C19 precursor1001
lethal factor (plasmid)1001
lamin isoform A-delta106006
Metabotropic glutamate receptor 60011
Excitatory amino acid transporter 40101
Glutamate transporter homolog0011
Carbonic anhydrase 10001
Carbonic anhydrase 20001
N(4)-(beta-N-acetylglucosaminyl)-L-asparaginase0101
5-hydroxytryptamine receptor 1D0001
Glutamate receptor ionotropic, NMDA 1 0303
Excitatory amino acid transporter 10101
Excitatory amino acid transporter 20101
Excitatory amino acid transporter 30101
Glutamate receptor ionotropic, NMDA 2A 0303
Glutamate receptor ionotropic, NMDA 2B0303
Glutamate receptor ionotropic, NMDA 2C0303
Metabotropic glutamate receptor 10011
Metabotropic glutamate receptor 20011
Carbonic anhydrase-like protein, putative0001
Glutamate receptor ionotropic, NMDA 2D0303
Glutamate receptor ionotropic, NMDA 3B0303
Carbonic anhydrase 40001
Glutamate receptor ionotropic, NMDA 3A0303
Genome polyprotein 0112
dopamine D1 receptor1001
thioredoxin reductase5005
ATAD5 protein, partial2002
Fumarate hydratase1001
GLS protein1001
TDP1 protein3003
regulator of G-protein signaling 43003
cytochrome P450 family 3 subfamily A polypeptide 42002
glucocorticoid receptor [Homo sapiens]2002
pregnane X nuclear receptor6006
G2002
polyprotein1001
peroxisome proliferator activated receptor gamma3003
cytochrome P450, family 19, subfamily A, polypeptide 1, isoform CRA_a6006
D(1A) dopamine receptor4004
vitamin D3 receptor isoform VDRA3003
chromobox protein homolog 12002
serine/threonine-protein kinase mTOR isoform 13003
peptidyl-prolyl cis-trans isomerase NIMA-interacting 12002
5-hydroxytryptamine receptor 40606
Interferon beta2002
HLA class I histocompatibility antigen, B alpha chain 2002
Cellular tumor antigen p533003
ATP-dependent translocase ABCB10101
ATP-dependent translocase ABCB10303
Beta-1 adrenergic receptor0202
Cytochrome P450 3A40404
5-hydroxytryptamine receptor 1A0213
Alpha-2A adrenergic receptor0505
D(2) dopamine receptor0314
Androgen receptor0202
Alpha-2B adrenergic receptor0606
Alpha-2C adrenergic receptor0404
D0101
D(3) dopamine receptor0101
5-hydroxytryptamine receptor 1A0505
Alpha-2B adrenergic receptor0202
ATP-dependent translocase ABCB10101
D(1A) dopamine receptor0202
Alpha-2C adrenergic receptor0202
Alpha-2A adrenergic receptor0202
Alpha-1D adrenergic receptor0404
D(1B) dopamine receptor0101
5-hydroxytryptamine receptor 2A0617
5-hydroxytryptamine receptor 2C0617
5-hydroxytryptamine receptor 1B0404
D(4) dopamine receptor0101
D(3) dopamine receptor0404
Alpha-synuclein0101
5-hydroxytryptamine receptor 2B0404
5-hydroxytryptamine receptor 60404
D(2) dopamine receptor0213
Inositol hexakisphosphate kinase 12002
ATPase family AAA domain-containing protein 51001
Ataxin-22002
cytochrome P450 2C9, partial2002
ATP-dependent phosphofructokinase2002
Sodium- and chloride-dependent creatine transporter 10101
Proton-coupled amino acid transporter 10202
GLI family zinc finger 36006
retinoid X nuclear receptor alpha4004
aryl hydrocarbon receptor2002
geminin6006
peripheral myelin protein 224004
ATP-binding cassette sub-family C member 30404
Multidrug resistance-associated protein 40404
Acetylcholinesterase0505
Voltage-dependent L-type calcium channel subunit alpha-1F0101
Bile salt export pump0101
cGMP-specific 3',5'-cyclic phosphodiesterase0101
Bile salt export pump0505
Acetylcholinesterase0303
Cholinesterase0404
Amine oxidase [flavin-containing] B0101
Amine oxidase [flavin-containing] A 0101
Amine oxidase [flavin-containing] A0101
Amine oxidase [flavin-containing] A 0101
Acetylcholinesterase0303
Acetylcholinesterase06310
Liver carboxylesterase 10101
Acetylcholinesterase 0303
Amine oxidase [flavin-containing] B0101
Sodium-dependent serotonin transporter0628
Choline O-acetyltransferase 0101
Acetylcholinesterase0202
Glycogen synthase kinase-3 beta0101
Beta-secretase 10101
Cholinesterase0505
Voltage-dependent L-type calcium channel subunit alpha-1D 0101
Cholinesterase0101
Acetylcholine receptor subunit epsilon0101
cAMP-specific 3',5'-cyclic phosphodiesterase 4D0202
Potassium voltage-gated channel subfamily H member 20303
Voltage-dependent L-type calcium channel subunit alpha-1S0101
Voltage-dependent L-type calcium channel subunit alpha-1C0101
Cytochrome P450 1B10202
Sigma non-opioid intracellular receptor 10202
Canalicular multispecific organic anion transporter 10303
Acyl-CoA:cholesterol acyltransferase 0101
Sigma non-opioid intracellular receptor 10505
Carboxylic ester hydrolase 0101
Carboxylic ester hydrolase 0404
Histamine H3 receptor0202
RAR-related orphan receptor gamma3003
caspase 7, apoptosis-related cysteine protease1001
estrogen receptor 2 (ER beta)3003
nuclear receptor subfamily 1, group I, member 34004
progesterone receptor3003
estrogen-related nuclear receptor alpha4004
cytochrome P450 2D61001
peroxisome proliferator-activated receptor delta2002
caspase-31001
nuclear factor of kappa light polypeptide gene enhancer in B-cells 1 (p105), isoform CRA_a2002
v-jun sarcoma virus 17 oncogene homolog (avian)3003
Histone H2A.x2002
Caspase-72002
NPC intracellular cholesterol transporter 1 precursor1001
potassium voltage-gated channel subfamily H member 2 isoform d2002
caspase-32002
huntingtin isoform 22002
atrial natriuretic peptide receptor 2 precursor1001
ras-related protein Rab-9A1001
nuclear factor erythroid 2-related factor 2 isoform 14004
Gamma-aminobutyric acid receptor subunit pi0202
Gamma-aminobutyric acid receptor subunit delta0202
Sodium-dependent noradrenaline transporter0101
Potassium channel subfamily K member 20101
Albumin0022
Sodium channel protein type 1 subunit alpha0101
Aldo-keto reductase family 1 member B10202
5-hydroxytryptamine receptor 2C0202
Muscarinic acetylcholine receptor M50202
Cytochrome P450 2C80101
Cytochrome P450 2D60101
Muscarinic acetylcholine receptor M10202
Cytochrome P450 2C9 0101
Angiotensin-converting enzyme0202
Dipeptidyl peptidase 40101
5-hydroxytryptamine receptor 2A0303
Gamma-aminobutyric acid receptor subunit alpha-10213
Alpha-1B adrenergic receptor0303
Gamma-aminobutyric acid receptor subunit beta-10213
Gamma-aminobutyric acid receptor subunit gamma-20213
Muscarinic acetylcholine receptor M30202
Prostaglandin G/H synthase 10101
Alpha-1D adrenergic receptor0303
Sodium-dependent noradrenaline transporter 0314
Sodium-dependent dopamine transporter0202
Potassium voltage-gated channel subfamily C member 10101
cAMP-specific 3',5'-cyclic phosphodiesterase 4A0101
Gamma-aminobutyric acid receptor subunit beta-30213
5-hydroxytryptamine receptor 2B0202
Gamma-aminobutyric acid receptor subunit alpha-50213
Sodium-dependent serotonin transporter0202
Cytochrome P450 2C190101
Gamma-aminobutyric acid receptor subunit alpha-30213
Alpha-1A adrenergic receptor0303
Gamma-aminobutyric acid receptor subunit alpha-20213
Gamma-aminobutyric acid receptor subunit beta-20213
Gamma-aminobutyric acid receptor subunit alpha-40213
D(1A) dopamine receptor1001
Cytochrome P450 2J20101
Spike glycoprotein1001
Gamma-aminobutyric acid receptor subunit epsilon0202
Gastrin/cholecystokinin type B receptor 0101
Sodium-dependent dopamine transporter 0314
cAMP-specific 3',5'-cyclic phosphodiesterase 4B0101
Gamma-aminobutyric acid receptor subunit alpha-60213
Sigma intracellular receptor 20101
Sodium-dependent serotonin transporter0101
Sodium-dependent dopamine transporter0101
Transporter0101
Nuclear receptor subfamily 3 group C member 3 0202
Beta-2 adrenergic receptor0101
Gamma-aminobutyric acid receptor subunit gamma-10202
Gamma-aminobutyric acid receptor subunit gamma-30202
Sigma non-opioid intracellular receptor 10101
Gamma-aminobutyric acid receptor subunit theta0202
Transporter0101
Chain A, ACETYLCHOLINESTERASE0101
Chain A, Acetylcholinesterase0101
Vascular endothelial growth factor receptor 1 0101
Delta-type opioid receptor0101
Vascular endothelial growth factor receptor 30101
Vascular endothelial growth factor receptor 20101
Prolyl endopeptidase0101
Small conductance calcium-activated potassium channel protein 30101
Gamma-aminobutyric acid 0011
Gamma-aminobutyric acid receptor subunit rho-30011
Hsf1 protein0011
thyroid stimulating hormone receptor1001
bromodomain adjacent to zinc finger domain 2B1001
thyroid hormone receptor beta isoform a1001
transcriptional regulator ERG isoform 32002
histone-lysine N-methyltransferase 2A isoform 2 precursor1001
histone acetyltransferase KAT2A isoform 12002
neuropeptide S receptor isoform A1001
Gamma-aminobutyric acid receptor subunit pi0202
Histone deacetylase 30101
Gamma-aminobutyric acid type B receptor subunit 20011
Gamma-aminobutyric acid type B receptor subunit 20101
Gamma-aminobutyric acid receptor subunit beta-10202
Gamma-aminobutyric acid receptor subunit alpha-60011
Gamma-aminobutyric acid receptor subunit delta0202
Gamma-aminobutyric acid receptor subunit gamma-20213
Gamma-aminobutyric acid receptor subunit alpha-50202
Gamma-aminobutyric acid receptor subunit alpha-30202
Gamma-aminobutyric acid receptor subunit gamma-20011
Gamma-aminobutyric acid receptor subunit delta0011
Gamma-aminobutyric acid receptor subunit gamma-10202
Gamma-aminobutyric acid receptor subunit alpha-20202
Sodium- and chloride-dependent GABA transporter 10101
Gamma-aminobutyric acid receptor subunit rho-10022
Gamma-aminobutyric acid receptor subunit alpha-20011
Gamma-aminobutyric acid receptor subunit alpha-30011
Gamma-aminobutyric acid receptor subunit gamma-30011
Gamma-aminobutyric acid receptor subunit alpha-40202
Gamma-aminobutyric acid receptor subunit gamma-30202
Gamma-aminobutyric acid receptor subunit rho-20011
Gamma-aminobutyric acid receptor subunit alpha-60202
Sodium- and chloride-dependent GABA transporter 10101
Sodium- and chloride-dependent taurine transporter0101
Sodium- and chloride-dependent GABA transporter 20101
Sodium- and chloride-dependent GABA transporter 30101
Sodium- and chloride-dependent GABA transporter 10101
Sodium- and chloride-dependent GABA transporter 20101
Sodium- and chloride-dependent GABA transporter 30101
Sodium- and chloride-dependent betaine transporter0101
Sodium- and chloride-dependent betaine transporter0101
Sodium- and chloride-dependent GABA transporter 30101
Gamma-aminobutyric acid receptor subunit beta-10011
Histone deacetylase 40101
Gamma-aminobutyric acid receptor subunit alpha-10011
Gamma-aminobutyric acid receptor subunit alpha-10213
Gamma-aminobutyric acid receptor subunit beta-30202
Gamma-aminobutyric acid receptor subunit beta-30011
Gamma-aminobutyric acid receptor subunit beta-20213
4-aminobutyrate aminotransferase, mitochondrial0001
4-aminobutyrate aminotransferase, mitochondrial0001
TAR DNA-binding protein 431001
Histone deacetylase 10101
Sterol O-acyltransferase 10011
Gamma-aminobutyric acid receptor subunit alpha-50011
Gamma-aminobutyric acid receptor subunit pi0011
Histone deacetylase 70101
GABA theta subunit0202
Histone deacetylase 20101
Polyamine deacetylase HDAC100101
Histone deacetylase 11 0101
Histone deacetylase 80101
Gamma-aminobutyric acid receptor subunit alpha-40011
Gamma-aminobutyric acid receptor subunit epsilon0202
Platelet glycoprotein VI0011
Gamma-aminobutyric acid receptor subunit theta0011
Sodium- and chloride-dependent GABA transporter 20101
Gamma-aminobutyric acid receptor subunit gamma-10011
Histone deacetylase 60101
Gamma-aminobutyric acid type B receptor subunit 10011
Histone deacetylase 90101
Histone deacetylase 50101
Gamma-aminobutyric acid type B receptor subunit 10101
Chain A, Ribulose-1,5 bisphosphate carboxylase/oxygenase large subunit N-methyltransferase, chloroplast0101
Chain A, Ribulose-1,5 bisphosphate carboxylase/oxygenase large subunit N-methyltransferase, chloroplast0101
Glutathione reductase, mitochondrial0202
Cationic amino acid transporter 30101
Solute carrier family 22 member 20001
Solute carrier family 22 member 1 0101
Glutamate receptor ionotropic, NMDA 2D0101
Glutamate receptor ionotropic, NMDA 3B0101
Neuronal acetylcholine receptor subunit alpha-30101
Neuronal acetylcholine receptor subunit beta-40101
Neuronal acetylcholine receptor subunit alpha-60101
5-hydroxytryptamine receptor 70101
Glutamate receptor ionotropic, NMDA 10202
Glutamate receptor ionotropic, NMDA 2A0202
Glutamate receptor ionotropic, NMDA 2B0202
Glutamate receptor ionotropic, NMDA 2C0101
Glutamate receptor ionotropic, NMDA 3A0101
67.9K protein1001
nuclear factor erythroid 2-related factor 2 isoform 23003
DNA polymerase kappa isoform 11001
Solute carrier family 22 member 200101
Solute carrier family 22 member 60101
Rap guanine nucleotide exchange factor 41001
Sodium channel protein type 1 subunit alpha0101
Sodium channel protein type 2 subunit alpha0101
Sodium channel protein type 3 subunit alpha0101
Chain A, HADH2 protein2002
Chain B, HADH2 protein2002
pregnane X receptor1001
thioredoxin glutathione reductase1001
hypoxia-inducible factor 1, alpha subunit (basic helix-loop-helix transcription factor)2002
vitamin D (1,25- dihydroxyvitamin D3) receptor2002
nuclear receptor ROR-gamma isoform 11001
Glucocorticoid receptor0213
Glycine receptor subunit alpha-10202
Glycine receptor subunit beta0202
Glycine receptor subunit alpha-20202
Glycine receptor subunit alpha-30202
Nuclear receptor ROR-gamma1001
Luciferase2002
hypoxia-inducible factor 1 alpha subunit2002
farnesoid X nuclear receptor1001
heat shock protein beta-11001
histone deacetylase 9 isoform 31001
Tyrosinase0101
NAD(P)H dehydrogenase [quinone] 10001
Chain A, 2-oxoglutarate Oxygenase1001
USP1 protein, partial1001
cytochrome P450 2D6 isoform 11001
15-hydroxyprostaglandin dehydrogenase [NAD(+)] isoform 11001
mitogen-activated protein kinase 11001
muscarinic acetylcholine receptor M11001
Histamine H1 receptor0202
[prepared from compound, protein, and bioassay information from National Library of Medicine (NLM), extracted Dec-2023]