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Progressive non-fluent aphasia
1 OMIM reference -
6 associated genes
355 connected diseases
No signs/symptoms info
Disease Type of connection
Behavioral variant of frontotemporal dementia
Semantic dementia
Amyotrophic lateral sclerosis
Early-onset autosomal dominant Alzheimer disease
Familial isolated dilated cardiomyopathy
Frontotemporal dementia with motor neuron disease
Inclusion body myopathy with Paget disease of bone and frontotemporal dementia
Classical progressive supranuclear palsy
Progressive supranuclear palsy - corticobasal syndrome
Progressive supranuclear palsy - parkinsonism
Progressive supranuclear palsy - progressive non fluent aphasia
Progressive supranuclear palsy - pure akinesia with gait freezing
Adult-onset distal myopathy due to VCP mutation
CLN11 disease
Spastic paraplegia - Paget disease of bone
Young adult-onset Parkinsonism
Familial isolated arrhythmogenic ventricular dysplasia, biventricular form
Familial isolated arrhythmogenic ventricular dysplasia, left dominant form
Familial isolated arrhythmogenic ventricular dysplasia, right dominant form
Common variable immunodeficiency
Giant cell glioblastoma
Gliosarcoma
Autosomal dominant hypohidrotic ectodermal dysplasia
Hereditary cerebral hemorrhage with amyloidosis, Arctic type
Hereditary cerebral hemorrhage with amyloidosis, Dutch type
Hereditary cerebral hemorrhage with amyloidosis, Flemish type
Hereditary cerebral hemorrhage with amyloidosis, Iowa type
Hereditary cerebral hemorrhage with amyloidosis, Italian type
Hereditary cerebral hemorrhage with amyloidosis, Piedmont type
Lethal acantholytic epidermolysis bullosa
Split hand-split foot malformation
Acute promyelocytic leukemia
Familial idiopathic steroid-resistant nephrotic syndrome with focal segmental hyalinosis
Retinitis pigmentosa
Idiopathic pulmonary fibrosis
Familial prostate cancer
Hereditary breast and ovarian cancer syndrome
Cerebellar ataxia - hypogonadism
Familial pancreatic carcinoma
22q11.2 deletion syndrome
Cowden syndrome
Proteus syndrome
Congenital bilateral absence of vas deferens
Cystic fibrosis
Hereditary chronic pancreatitis
Idiopathic bronchiectasis
Male infertility with normal virilization due to meiosis defect
Heritable pulmonary arterial hypertension
Periventricular nodular heterotopia
Li-Fraumeni syndrome
Alveolar soft-part sarcoma
Hyperlipidemia type 3
Lipoprotein glomerulopathy
Sea-blue histiocytosis
Translocation renal cell carcinoma
Diffuse cutaneous systemic sclerosis
Limited cutaneous systemic sclerosis
Follicular lymphoma
Chuvash erythrocytosis
Von Hippel-Lindau disease
Precursor B-cell acute lymphoblastic leukemia
Dedifferentiated liposarcoma
Well-differentiated liposarcoma
B-cell chronic lymphocytic leukemia
X-linked non-syndromic intellectual deficit
17q11 microdeletion syndrome
Familial or sporadic hemiplegic migraine
Noonan syndrome
Spinocerebellar ataxia type 7
X-linked dominant chondrodysplasia, Chassaing-Lacombe type
Atelosteogenesis type I
Atelosteogenesis type III
Autosomal dominant Larsen syndrome
Boomerang dysplasia
Essential thrombocythemia
Precursor T-cell acute lymphoblastic leukemia
Spondylocarpotarsal synostosis
Acute myeloblastic leukemia with maturation
Acute myeloblastic leukemia without maturation
Acute myelomonocytic leukemia
Autosomal dominant macrothrombocytopenia
Cataract - intellectual deficit - hypogonadism
Distal 22q11.2 microdeletion syndrome
Fanconi anemia
Juvenile primary lateral sclerosis
Megalencephaly - polymicrogyria - postaxial polydactyly - hydrocephalus
Micro syndrome
Multiple endocrine neoplasia type 1
Baraitser-Winter syndrome
Developmental malformations - deafness - dystonia
Keratosis palmoplantaris striata
Skin fragility-woolly hair-palmoplantar keratoderma syndrome
Woolly hair-palmoplantar keratoderma-dilated cardiomyopathy syndrome
Autoimmune lymphoproliferative syndrome
Autosomal recessive systemic lupus erythematosus
Left ventricular noncompaction
Autoimmune lymphoproliferative syndrome with recurrent infections
Isolated NADH-CoQ reductase deficiency
Machado-Joseph disease type 1
Machado-Joseph disease type 2
Machado-Joseph disease type 3
Autosomal dominant nonsyndromic intellectual deficit
Craniopharyngioma
Desmoid tumor
Hepatocellular carcinoma, childhood-onset
Pilomatrixoma
Hypohidrotic ectodermal dysplasia with immunodeficiency
Chronic intestinal pseudoobstruction
Congenital short bowel syndrome
Congenital valvular dysplasia
Ehlers-Danlos syndrome with periventricular heterotopia
Frontometaphyseal dysplasia
Hereditary breast cancer
Hereditary site-specific ovarian cancer syndrome
Osteodysplasty, Melnick-Needles type
Otopalatodigital syndrome type 1
Otopalatodigital syndrome type 2
Parkinsonian-pyramidal syndrome
Primary peritoneal carcinoma
Terminal osseous dysplasia - pigmentary defects
Familial gastric cancer
Gastric linitis plastica
Alexander disease type I
Alexander disease type II
2p21 microdeletion syndrome
Naxos disease
Monosomy 5p
Congenital fibrosis of extraocular muscles
Cortical dysgenesis with pontocerebellar hypoplasia due to TUBB3 mutation
Dyssegmental dysplasia, Silverman-Handmaker type
Schwartz-Jampel syndrome
Coffin-Lowry syndrome
T-B+ severe combined immunodeficiency due to CD3delta / CD3epsilon / CD3zeta
Werner syndrome
Berardinelli-Seip congenital lipodystrophy
Huntington disease
Juvenile Huntington disease
Intermittent hydrarthrosis
Osteosarcoma
TRAPS syndrome
Tyrosinemia type 2
Intravascular large B-cell lymphoma
Catecholaminergic polymorphic ventricular tachycardia
Distal hereditary motor neuropathy type 7
Perry syndrome
Maternal uniparental disomy of chromosome 14
Paternal uniparental disomy of chromosome 14
Adrenocortical carcinoma
Athabaskan brainstem dysgenesis syndrome
Bosley-Salih-Alorainy syndrome
Charcot-Marie-Tooth disease type 4D
Early infantile epileptic encephalopathy
Hereditary sensory and autonomic neuropathy with spastic paraplegia
Homozygous familial hypercholesterolemia
Papilloma of choroid plexus
ADULT syndrome
Ankyloblepharon - ectodermal defects - cleft lip / palate
Bladder exstrophy
EEC syndrome
Glutaryl-CoA dehydrogenase deficiency
Limb-mammary syndrome
Autosomal dominant nonsyndromic sensorineural deafness type DFNA
Autosomal recessive centronuclear myopathy
17q11.2 microduplication syndrome
Alternating hemiplegia of childhood
Benign paroxysmal torticollis of infancy
Complete androgen insensitivity syndrome
Familial hypospadias
Familial paroxysmal ataxia
Giant cell arteritis
Granulomatosis with polyangiitis
Juvenile myelomonocytic leukemia
Juvenile rheumatoid factor-negative polyarthritis
Kennedy disease
Neurofibromatosis type 1 due to NF1mutation or intragenic deletion
Oligoarticular juvenile arthritis
Partial androgen insensitivity syndrome
Pediatric systemic lupus erythematosus
Spinocerebellar ataxia type 1
Spinocerebellar ataxia type 6
Watson syndrome
Chronic myeloid leukemia
17p13.3 microduplication syndrome
3-phosphoglycerate dehydrogenase deficiency
3M syndrome
ALDH18A1-related De Barsy syndrome
Adult-onset proximal spinal muscular atrophy, autosomal dominant
Alpha-1-antitrypsin deficiency
Apolipoprotein A-I deficiency
Atypical Werner syndrome
Autosomal codominant severe lipodystrophic laminopathy
Autosomal dominant Charcot-Marie-Tooth disease type 2A2
Autosomal dominant Charcot-Marie-Tooth disease type 2B
Autosomal dominant Charcot-Marie-Tooth disease type 2F
Autosomal dominant Emery-Dreifuss muscular dystrophy
Autosomal dominant hypocalcemia
Autosomal dominant intermediate Charcot-Marie-Tooth disease type E
Autosomal dominant limb-girdle muscular dystrophy type 1B
Autosomal dominant limb-girdle muscular dystrophy type 1F
Autosomal dominant optic atrophy plus syndrome
Autosomal dominant spastic paraplegia type 13
Autosomal dominant spastic paraplegia type 4
Autosomal recessive Emery-Dreifuss muscular dystrophy
Autosomal recessive ataxia due to ubiquinone deficiency
Autosomal recessive distal renal tubular acidosis with deafness
Autosomal recessive limb-girdle muscular dystrophy type 2P
Autosomal recessive limb-girdle muscular dystrophy type 2Q
Autosomal recessive spastic paraplegia type 18
Bartter syndrome with hypocalcemia
Brugada syndrome
Bullous pemphigoid
CLN4A disease
CLN6 disease
COG4-CDG
COG5-CDG
Cardiofaciocutaneous syndrome
Charcot-Marie-Tooth disease type 2B1
Chorioretinopathy, Birdshot type
Chronic mucocutaneous candidiasis
Chronic respiratory distress with surfactant metabolism deficiency
Combined deficiency of factor V and factor VIII
Congenital factor VII deficiency
Congenital muscular dystrophy due to LMNA mutation
Congenital pulmonary alveolar proteinosis
Congenital stationary night blindness
Cree leukoencephalopathy
Dentatorubral pallidoluysian atrophy
Desquamative interstitial pneumonia
Dilated cardiomyopathy - hypergonadotropic hypogonadism
Distal 17p13.3 microdeletion syndrome
Distal hereditary motor neuropathy type 2
Dravet syndrome
Enchondromatosis
Endocrine-cerebro-osteodysplasia syndrome
Epidermolysis bullosa simplex with muscular dystrophy
Epidermolysis bullosa simplex with pyloric atresia
Epidermolysis bullosa simplex, Ogna type
Epilepsy with myoclonic-astatic seizures
Estrogen resistance syndrome
Extraskeletal Ewing sarcoma
Familial dilated cardiomyopathy with conduction defect due to LMNA mutation
Familial hypocalciuric hypercalcemia type 1
Familial idiopathic steroid-resistant nephrotic syndrome with diffuse mesangial proliferation
Familial idiopathic steroid-resistant nephrotic syndrome with diffuse mesangial sclerosis
Familial idiopathic steroid-resistant nephrotic syndrome with minimal changes
Familial infantile bilateral striatal necrosis
Familial isolated hypoparathyroidism due to impaired PTH secretion
Familial melanoma
Familial partial lipodystrophy, Dunnigan type
Familial partial lipodystrophy, Köbberling type
Familial renal amyloidosis due to Apolipoprotein AI variant
Fibronectin glomerulopathy
GM1 gangliosidosis type 1
GM1 gangliosidosis type 2
GM1 gangliosidosis type 3
Generalized epilepsy with febrile seizures-plus context
Glycogen storage disease due to liver phosphorylase kinase deficiency
Glycogen storage disease due to muscle phosphorylase kinase deficiency
Goldberg-Shprintzen megacolon syndrome
Gray platelet syndrome
Heart-hand syndrome, Slovenian type
Hemorrhagic disease due to alpha-1-antitrypsin Pittsburgh mutation
Hereditary cerebral cavernous malformation
Hereditary motor and sensory neuropathy type 5
Hereditary motor and sensory neuropathy type 6
Hereditary orotic aciduria
Hereditary sensory and autonomic neuropathy type 2
Hutchinson-Gilford progeria syndrome
Hyperinsulinism due to HNF1A deficiency
Infant acute respiratory distress syndrome
Infantile Refsum disease
Infantile-onset ascending hereditary spastic paralysis
Intellectual deficit, X-linked, Turner type
Joubert syndrome with renal defect
Juvenile amyotrophic lateral sclerosis
LEOPARD syndrome
LMNA-related cardiocutaneous progeria syndrome
Laminopathy type Decaudain-Vigouroux
Leber congenital amaurosis
Lennox-Gastaut syndrome
Lethal restrictive dermopathy
Limited systemic sclerosis
Lissencephaly due to TUBA1A mutation
MMEP syndrome
MODY syndrome
Maffucci syndrome
Malignant migrating partial seizures of infancy
Mandibuloacral dysplasia with type A lipodystrophy
Mendelian susceptibility to mycobacterial diseases due to complete ISG15 deficiency
Mendelian susceptibility to mycobacterial diseases due to partial STAT1 deficiency
Metaphyseal chondromatosis with D-2-hydroxyglutaric aciduria
Miller-Dieker syndrome
Mucopolysaccharidosis type 4B
Multiple acyl-CoA dehydrogenation deficiency, mild type
Multiple acyl-CoA dehydrogenation deficiency, severe neonatal type
Multiple endocrine neoplasia type 4
Muscle-eye-brain disease with bilateral multicystic leucodystrophy
Narcolepsy without cataplexy
Narcolepsy-cataplexy
Neonatal adrenoleukodystrophy
Neonatal severe primary hyperparathyroidism
Nephrogenic diabetes insipidus
Nephrogenic syndrome of inappropriate antidiuresis
Ovarioleukodystrophy
Partial acquired lipodystrophy
Pelizaeus-Merzbacher-like due to HSPD1 mutation
Pilocytic astrocytoma
Primary biliary cirrhosis
Primary systemic amyloidosis
Progeria-associated arthropathy
Pseudohypoaldosteronism type 2C
Pseudohypoaldosteronism type 2E
Pulverulent cataract
Recessive intellectual disability - motor dysfunction - multiple joint contractures
Retinitis punctata albescens
Sarcoidosis
Senior-Loken syndrome
Severe early-onset axonal neuropathy due to MFN2 deficiency
Spinocerebellar ataxia type 10
Spinocerebellar ataxia type 15 / 16
Spinocerebellar ataxia type 29
Succinyl-CoA:3-ketoacid CoA transferase deficiency
Susceptibility to viral and mycobacterial infections
Timothy syndrome
Weaver syndrome
Williams syndrome
Zellweger syndrome
Alobar holoprosencephaly
Familial advanced sleep-phase syndrome
Lobar holoprosencephaly
Microform holoprosencephaly
Midline interhemispheric variant of holoprosencephaly
Semilobar holoprosencephaly
Septopreoptic holoprosencephaly
Autosomal dominant severe congenital neutropenia
Budd-Chiari syndrome
Cyclic neutropenia
Familial thrombocytosis
Myelofibrosis with myeloid metaplasia
Polycythemia vera
Acroosteolysis dominant type
Alagille syndrome due to a NOTCH2 point mutation
Autosomal agammaglobulinemia
CADASIL
Hereditary spherocytosis
Infantile myofibromatosis
Partial chromosome Y deletion
Rubinstein-Taybi syndrome due to EP300 haploinsufficiency
SHORT syndrome
Familial Alzheimer-like prion disease
Fatal familial insomnia
Gerstmann-Straussler-Scheinker syndrome
Huntington disease-like 1
Inherited Creutzfeldt-Jakob disease
Leigh syndrome with leukodystrophy
Synonym(s):
- Agramatic variant of PPA
- Agramatic variant of primary progressive aphasia
- Non-fluent variant PPA
Classification (Orphanet):
- Rare genetic disease
- Rare neurologic disease
Classification (ICD10):
- Diseases of the nervous system -
Epidemiological data:
Class of prevalence: 1-9 / 100 000
Average age onset: adulthood
Average age of death: -
Type of inheritance: multigenic/multifactorial
External references:
1 OMIM reference -
1 MeSH reference: D057178
No signs/symptoms info available.