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Disease Profile

Logopenic progressive aphasia

Prevalence
Prevalence estimates on Rare Medical Network websites are calculated based on data available from numerous sources, including US and European government statistics, the NIH, Orphanet, and published epidemiologic studies. Rare disease population data is recognized to be highly variable, and based on a wide variety of source data and methodologies, so the prevalence data on this site should be assumed to be estimated and cannot be considered to be absolutely correct.

Unknown

Age of onset

Adult

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ICD-10

G31.0

Inheritance

Autosomal dominant A pathogenic variant in only one gene copy in each cell is sufficient to cause an autosomal dominant disease

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Autosomal recessive Pathogenic variants in both copies of each gene of the chromosome are needed to cause an autosomal recessive disease and observe the mutant phenotype

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X-linked
dominant X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.

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X-linked
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder

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Mitochondrial or multigenic Mitochondrial genetic disorders can be caused by changes (mutations) in either the mitochondrial DNA or nuclear DNA that lead to dysfunction of the mitochondria and inadequate production of energy.

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Multigenic or multifactor Inheritance involving many factors, of which at least one is genetic but none is of overwhelming importance, as in the causation of a disease by multiple genetic and environmental factors.

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Not applicable

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Other names (AKA)

LPA; Logopenic primary progressive aphasia; Logopenic variant PPA

Categories

Nervous System Diseases

Summary

Logopenic progressive aphasia (LPA) is a type of dementia characterized by language disturbance, including difficulty making or understanding speech (aphasia). It is a type of primary progressive aphasia (PPA). Affected individuals have slow, hesitant speech due to difficulty retrieving the correct words, names, or numbers. Difficulty with phase and sentence repetition are additionally present. Speech is typically well articulated and grammatically correct with good single-word comprehension. But over time, affected individuals may have trouble understanding long or complex verbal information, due to problems holding onto lengthy information that they hear. Language difficulties associated with LPA are due to shrinking, or atrophy, in the left posterior temporal cortex and inferior parietal lobule. Click here to view an image of the lobes of the brain.[1][2][3]

Treatment

Although no medications or interventions have demonstrated long-term stabilization of logopenic progressive aphasia (LPA), different treatment methods have shown promising short-term benefits.[2][4] Studies utilizing language therapy and behavioral interventions have shown encouraging results. Neuromodulation through methodologies such as Transcranial Direct Current Stimulation (tDCS) and transcranial magnetic stimulation (rTMS) have additionally been identified as a promising therapies to potentially use in combination with behavioral treatment and language therapy.[2]

As the most common underlying pathology of LPA is Alzheimer's disease (AD) pathology, limited research has been completed on interventions shown to reduce the rate of decline in cognitive symptoms in AD. So far cholinesterase inhibitors and memantine, medications used in Alzheimer’s disease, have not been proven effective in treating logopenic progressive aphasia. Case studies involving steriod use and Omentum Transposition Therapy have reported improvement; however, the results have not been replicated in other cases and as with other treatment options, long-term studies are lacking.[2]

The National Aphasia Association provides further information on the medical management of primary progressive aphasias at the following link:
https://www.aphasia.org/aphasia-resources/aphasia-therapy-guide/

Organizations

Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Organizations Supporting this Disease

    Organizations Providing General Support

      Learn more

      These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

      Where to Start

        In-Depth Information

        • The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
        • Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
        • PubMed is a searchable database of medical literature and lists journal articles that discuss Logopenic progressive aphasia. Click on the link to view a sample search on this topic.

          References

          1. Primary Progressive Aphasia. UCSF Memory and Aging Center. February 2011; https://memory.ucsf.edu/education/diseases/ppa. Accessed 9/14/2011.
          2. Donna C. Tippett, Argye E. Hillis,Kyrana Tsapkini. Treatment of Primary Progressive Aphasia. Curr Treat Options Neurol.. August 2015; 17(8):362. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4600091/.
          3. M.L. Gorno-Tempini,A.E. Hillis, S. Weintraub, A. Kertesz, M. Mendez, S.F. Cappa, J.M. Ogar, J.D. Rohrer, S. Black, B.F. Boeve, F. Manes, N.F. Dronkers, R. Vandenberghe, K. Rascovsky, K. Patterson, B.L. Miller, D.S. Knopman, J.R. Hodges, M.M. Mesulam, M. Grossman. Classification of primary progressive aphasia and its variants. Neurology. March 2011; 76(11):1006-1014. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3059138/.
          4. Beeson PM, King RM, Bonakdarpour B, Henry ML, Cho H, Rapcsak SZ. Positive effects of language treatment for the logopenic variant of primary progressive aphasia. J Mol Neurosci. 2011 Nov;45(3):724-36; https://www.ncbi.nlm.nih.gov/pubmed/21710364.
          5. Le Rhun E, Richard F, Pasquier F. Natural history of primary progressive aphasia. Neurology. 2005 Sep 27;65(6):887-91; https://www.ncbi.nlm.nih.gov/pubmed/16186529. Accessed 1/18/2013.
          6. Hsieh S, Hodges JR, Leyton CE, Mioshi E. Longitudinal changes in primary progressive aphasias: differences in cognitive and dementia staging measures. Dement Geriatr Cogn Disord. 2012;34(2):135-41; https://www.ncbi.nlm.nih.gov/pubmed/23006977. Accessed 1/18/2013.
          7. Zanetti O, Solerte SB, Cantoni F. Life expectancy in Alzheimer's disease (AD). Arch Gerontol Geriatr. 2009;49 Suppl 1:237-43; https://www.ncbi.nlm.nih.gov/pubmed/19836639. Accessed 1/18/2013.
          8. Miller BL Lee SE,. Frontotemporal dementia: Treatment. In: DeKosky ST, Eichler AF. UpToDate. Waltham, MA: UpToDate; 2013;
          9. Amici S, Gorno-Tempini ML, Ogar JM, Dronkers NF, Miller BL. An overview on Primary Progressive Aphasia and its variants. Behav Neurol. 2006;17(2):77-87; https://www.ncbi.nlm.nih.gov/pubmed/16873918. Accessed 1/18/2013.
          10. Randolph C . Frontotermporal dementia: Clinical features and diganosis. In: DeKasky ST, Eichler AF. UpToDate. Waltham, MA: UpToDate; 2012;

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