TY - JOUR
T1 - Hereditary sensory neuropathy with deafness and dementia
T2 - A clinical and neuroimaging study
AU - Hojo, Kaori
AU - Imamura, Toru
AU - Takanashi, Mayako
AU - Ishii, Kazunari
AU - Sasaki, Masahiro
AU - Imura, Shigeaki
AU - Ozono, Reiko
AU - Takatsuki, Yoko
AU - Takauchi, Shigeru
AU - Mori, Etsuro
PY - 1999/1/1
Y1 - 1999/1/1
N2 - 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.
AB - 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.
KW - Dementia
KW - Hereditary sensory neuropathy
KW - Positron emission tomography
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U2 - 10.1046/j.1468-1331.1999.630357.x
DO - 10.1046/j.1468-1331.1999.630357.x
M3 - Article
C2 - 10210919
AN - SCOPUS:0032970457
VL - 6
SP - 357
EP - 361
JO - European Journal of Neurology
JF - European Journal of Neurology
SN - 1351-5101
IS - 3
ER -