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Primary progressive non fluent aphasia

MedGen UID:
148373
•Concept ID:
C0751706
•
Mental or Behavioral Dysfunction
Synonym: Progressive non-fluent aphasia
SNOMED CT: Primary progressive non fluent aphasia (716281000); Progressive non-fluent aphasia (716281000)
Modes of inheritance:
Non-Mendelian inheritance
MedGen UID:
109109
•Concept ID:
C0600599
•
Genetic Function
Source: Orphanet
A mode of inheritance that depends on genetic determinants in more than one gene.
Not genetically inherited
MedGen UID:
988794
•Concept ID:
CN307044
•
Finding
Source: Orphanet
clinical entity without genetic inheritance.
See: This record
Non-Mendelian inheritance (Orphanet)
Not genetically inherited (Orphanet)
 
Monarch Initiative: MONDO:0015059
Orphanet: ORPHA100070

Definition

A form of frontotemporal dementia with characteristics of agrammatism, laborious speech, alexia, and agraphia, frequently accompanied by apraxia of speech. Language comprehension is relatively preserved. [from SNOMEDCT_US]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • Primary progressive non fluent aphasia
Follow this link to review classifications for Primary progressive non fluent aphasia in Orphanet.

Professional guidelines

PubMed

Speech and language therapy approaches to managing primary progressive aphasia.
Volkmer A, Rogalski E, Henry M, Taylor-Rubin C, Ruggero L, Khayum R, Kindell J, Gorno-Tempini ML, Warren JD, Rohrer JD
Pract Neurol 2020 Apr;20(2):154-161. Epub 2019 Jul 29 doi: 10.1136/practneurol-2018-001921. PMID: 31358572Free PMC Article
Frontotemporal dementia: diagnosis, deficits and management.
Bott NT, Radke A, Stephens ML, Kramer JH
Neurodegener Dis Manag 2014;4(6):439-54. doi: 10.2217/nmt.14.34. PMID: 25531687Free PMC Article
Criteria for the diagnosis of corticobasal degeneration.
Armstrong MJ, Litvan I, Lang AE, Bak TH, Bhatia KP, Borroni B, Boxer AL, Dickson DW, Grossman M, Hallett M, Josephs KA, Kertesz A, Lee SE, Miller BL, Reich SG, Riley DE, Tolosa E, Tröster AI, Vidailhet M, Weiner WJ
Neurology 2013 Jan 29;80(5):496-503. doi: 10.1212/WNL.0b013e31827f0fd1. PMID: 23359374Free PMC Article

Recent clinical studies

Etiology

Decreased striatal dopamine transporter uptake in the non-fluent/agrammatic variant of primary progressive aphasia.
Gil-Navarro S, Lomeña F, Cot A, Lladó A, Montagut N, Castellví M, Bosch B, Rami L, Antonell A, Balasa M, Pavia J, Iranzo A, Molinuevo JL, Sánchez-Valle R
Eur J Neurol 2013 Nov;20(11):1459-e126. Epub 2013 May 17 doi: 10.1111/ene.12196. PMID: 23679075
Primary progressive non-fluent aphasia: a case study.
Kartsounis LD, Crellin RF, Crewes H, Toone BK
Cortex 1991 Mar;27(1):121-9. doi: 10.1016/s0010-9452(13)80275-4. PMID: 2055038

Diagnosis

Foreign accent syndrome as the initial sign of primary progressive aphasia.
Luzzi S, Viticchi G, Piccirilli M, Fabi K, Pesallaccia M, Bartolini M, Provinciali L, Snowden JS
J Neurol Neurosurg Psychiatry 2008 Jan;79(1):79-81. Epub 2007 Jul 17 doi: 10.1136/jnnp.2006.113365. PMID: 17635973
Primary progressive non-fluent aphasia: a case study.
Kartsounis LD, Crellin RF, Crewes H, Toone BK
Cortex 1991 Mar;27(1):121-9. doi: 10.1016/s0010-9452(13)80275-4. PMID: 2055038

Prognosis

Primary progressive non-fluent aphasia: a case study.
Kartsounis LD, Crellin RF, Crewes H, Toone BK
Cortex 1991 Mar;27(1):121-9. doi: 10.1016/s0010-9452(13)80275-4. PMID: 2055038

Supplemental Content

Table of contents

    Clinical resources

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    Reviews

    Related information

    • ClinVar
      Related medical variations
    • MeSH
      Related Medical Subject Headings
    • PMC Articles
      Related information in PubMed Central Links
    • PubMed
      Related literature resources in PubMed

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