U.S. flag

An official website of the United States government


Send to:

Choose Destination

Progressive language deterioration

MedGen UID:
Concept ID:
HPO: HP:0007064


Progressive loss of previously present language abilities. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVProgressive language deterioration

Conditions with this feature

GRN-related frontotemporal lobar degeneration with Tdp43 inclusions
MedGen UID:
Concept ID:
Disease or Syndrome
The spectrum of GRN frontotemporal dementia (GRN-FTD) includes the behavioral variant (bvFTD), primary progressive aphasia (PPA; further subcategorized as progressive nonfluent aphasia [PNFA] and semantic dementia [SD]), and movement disorders with extrapyramidal features such as parkinsonism and corticobasal syndrome (CBS). A broad range of clinical features both within and between families is observed. The age of onset ranges from 35 to 87 years. Behavioral disturbances are the most common early feature, followed by progressive aphasia. Impairment in executive function manifests as loss of judgment and insight. In early stages, PPA often manifests as deficits in naming, word finding, or word comprehension. In late stages, affected individuals often become mute and lose their ability to communicate. Early findings of parkinsonism include rigidity, bradykinesia or akinesia (slowing or absence of movements), limb dystonia, apraxia (loss of ability to carry out learned purposeful movements), and disequilibrium. Late motor findings may include myoclonus, dysarthria, and dysphagia. Most affected individuals eventually lose the ability to walk. Disease duration is three to 12 years.
Cortical dysplasia-focal epilepsy syndrome
MedGen UID:
Concept ID:
Disease or Syndrome
Pitt-Hopkins-like syndrome-1 (PTHSL1) is an autosomal recessive neurodevelopmental disorder characterized by delayed psychomotor development, intellectual disability, severe speech impairment or regression, and behavioral abnormalities. Most patients have onset of seizures within the first years of life. Some patients may have cortical dysplasia on brain imaging (summary by Smogavec et al., 2016).

Professional guidelines


Taylor-Rubin C, Croot K, Nickels L
Expert Rev Neurother 2021 Apr;21(4):419-430. Epub 2021 Apr 8 doi: 10.1080/14737175.2021.1897253. PMID: 33641570
Kok DE, Winkels RM, van Herpen CM, Kampman E
Eur J Cancer 2018 Nov;104:145-150. Epub 2018 Oct 20 doi: 10.1016/j.ejca.2018.09.018. PMID: 30352382
Varadkar S, Bien CG, Kruse CA, Jensen FE, Bauer J, Pardo CA, Vincent A, Mathern GW, Cross JH
Lancet Neurol 2014 Feb;13(2):195-205. doi: 10.1016/S1474-4422(13)70260-6. PMID: 24457189Free PMC Article

Recent clinical studies


Andermann F
Prog Brain Res 2002;135:479-85. doi: 10.1016/S0079-6123(02)35045-3. PMID: 12143366


Dougherty M, Lazar J, Klein JC, Diaz K, Gobillot T, Grunblatt E, Hasle N, Lawrence D, Maurano M, Nelson M, Olson G, Srivatsan S, Shendure J, Keene CD, Bird T, Horwitz MS, Marshall DA
Cold Spring Harb Mol Case Stud 2016 Nov;2(6):a001222. doi: 10.1101/mcs.a001222. PMID: 27900365Free PMC Article
Knels C, Danek A
Brain Lang 2010 May;113(2):96-100. Epub 2010 Jan 19 doi: 10.1016/j.bandl.2009.09.007. PMID: 20034661

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.
    • Bookshelf
      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Consumer resources

    Recent activity

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...