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Case Reports
. 1999 May;6(3):357-61.
doi: 10.1046/j.1468-1331.1999.630357.x.

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

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Case Reports

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

K Hojo et al. Eur J Neurol. 1999 May.

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.

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