Sign in to NCBI

Display Settings:

Format

Send to:

Choose Destination
We are sorry, but NCBI web applications do not support your browser and may not function properly. More information
    Eur J Neurol. 1999 May;6(3):357-61.

    Hereditary sensory neuropathy with deafness and dementia: a clinical and neuroimaging study.

    Hojo K, Imamura T, Takanashi M, Ishii K, Sasaki M, Imura S, Ozono R, Takatsuki Y, Takauchi S, Mori E.

    Source

    Division of Clinical Neurosciences, Hyogo Institute for Aging Brain and Cognitive Disorders, Himeji, Japan.

    Abstract

    We describe three sibling patients with autosomal dominantly inherited sensory neuropathy, sensorineural hearing loss and dementia. The features of cognitive-behavioral deficits in the patients, including executive dysfunction, apathy, indifference and inattention, were consistent with a frontal lobe dysfunction. Magnetic resonance imaging showed a diffuse brain atrophy. A fluorodeoxyglucose positron emission tomography in one patient and a single photon emission computed tomography in another demonstrated a glucose hypometabolism or a hypoperfusion in the medial frontal and thalamic regions. Primary frontal involvement or frontal dysfunction secondary to thalamic lesions may contribute to the nature of dementia in these patients.

    Copyright 1999 Lippincott Williams & Wilkins

    PMID:
    10210919
    [PubMed - indexed for MEDLINE]

    Publication Types, MeSH Terms

    Publication Types

    MeSH Terms

    LinkOut - more resources

    Full Text Sources

    Medical

      Supplemental Content

      Icon for Blackwell Publishing

      Save items

      loading

      Related citations in PubMed

      See reviews... See all...

      Cited by 2 PubMed Central articles

      Related information

      • Related Citations
        Calculated set of PubMed citations closely related to the selected article(s) retrieved using a word weight algorithm. Related articles are displayed in ranked order from most to least relevant, with the “linked from” citation displayed first.
      • ClinVar
        Clinical variations associated with publication
      • Gene (OMIM)
        Gene records associated with Online Mendelian Inheritance in Man (OMIM) records that cite the current articles in their reference lists.
      • MedGen
        Related information in MedGen
      • MedGen (Bookshelf cited)
        Related records in MedGen based on citations in GeneReviews and Medical Genetics Summaries
      • Nucleotide (Weighted)
        Nucleotide records associated with the current articles through the Gene database. These are the related sequences on the Gene record that are added manually by NCBI.
      • OMIM (calculated)
        Online Mendelian Inheritance in Man (OMIM) records that include the current articles as references in the light bulb links within or in the citations at the end of the OMIM record. The references available through the light bulb link are collected using the PubMed related articles algorithm to identify records with similar terminology to the OMIM record.
      • OMIM (cited)
        Online Mendelian Inheritance in Man (OMIM) records that include the current articles as reference cited at the end of the OMIM record.
      • Protein (Weighted)
        Protein records associated with the current articles through related Gene database records. These are the related sequences on the Gene record that are added manually by NCBI.
      • Cited in PMC
        Full-text articles in the PubMed Central Database that cite the current articles.
      • Cited in Books
        NCBI Bookshelf books that cite the current articles.

      Recent activity

      Clear Turn Off Turn On

      Your browsing activity is empty.

      Activity recording is turned off.

      Turn recording back on

      See more...
      You are here: NCBI > Literature > PubMed
      Write to the Help Desk