Hereditary sensory neuropathy with deafness and dementia: A clinical and neuroimaging study

Kaori Hojo, Toru Imamura, Mayako Takanashi, Kazunari Ishii, Masahiro Sasaki, Shigeaki Imura, Reiko Ozono, Yoko Takatsuki, Shigeru Takauchi, Etsuro Mori

Research output: Contribution to journalArticlepeer-review

16 Citations (Scopus)


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.

Original languageEnglish
Pages (from-to)357-361
Number of pages5
JournalEuropean Journal of Neurology
Issue number3
Publication statusPublished - 1999 Jan 1


  • Dementia
  • Hereditary sensory neuropathy
  • Positron emission tomography

ASJC Scopus subject areas

  • Neurology
  • Clinical Neurology


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