Membranous lipodystrophy presenting with palilalia: a PET study of cerebral glucose metabolism.
A case of membranous lipodystrophy (Nasu-Hakola disease; NHD) associated with palilalia was reported. A 38-year-old Japanese woman developed walking difficulty in her twenties. At age 35 she manifested neuropsychiatric symptoms characterized by euphoria, palilalia and dementia. A bone marrow biopsy showed periodic acid Schiff-positive membranous cystic lesions in the adipose tissue. Positron emission tomography with (18F)-2-fluoro-2-deoxy-D-glucose disclosed that regional cerebral glucose metabolism was decreased in the bilateral frontal white matter with mild hypometabolism in the thalamus and basal ganglia; all predominantly on the right. Taken together with the previous postmortem findings, it is postulated that frontal lobe hypofunction, predominantly in the right hemisphere, produced the unique neuropsychiatric symptoms in this patient.
Ueki
Y
Y
Department of Neurology, Faculty of Medicine, Kyoto University, Japan.
Kohara
N
N
Oga
T
T
Fukuyama
H
H
Akiguchi
I
I
Kimura
J
J
Shibasaki
H
H
D000328:Adult; D001479:Basal Ganglia / Q000378:metabolism / Q000503:physiopathology; D001921:Brain / Q000378:metabolism* / Q000503:physiopathology; D003704:Dementia / Q000000981:diagnostic imaging* / Q000378:metabolism; D003937:Diagnosis, Differential; D004734:Energy Metabolism; D005260:Female; D005625:Frontal Lobe / Q000378:metabolism / Q000503:physiopathology; D005947:Glucose / Q000378:metabolism*; D006801:Humans; D008060:Lipodystrophy / Q000000981:diagnostic imaging* / Q000378:metabolism; D008279:Magnetic Resonance Imaging; D013064:Speech Disorders / Q000000981:diagnostic imaging* / Q000378:metabolism; D013788:Thalamus / Q000378:metabolism / Q000503:physiopathology; D014055:Tomography, Emission-Computed
D005947:Glucose