[Presenile dementia in polycystic lipomembranous osteodysplasia].
We present a 36-year-old woman with a 3-year history of cognitive decline followed by development of a small stepped gait and urinary and fecal incontinence. Workup revealed multiple bone cysts documented by X-ray and idiopathic hyperprolactinoma. An MRI confirmed the CT finding of massive bilateral basal ganglia calcification. This is the first case of polycystic lipomembranous osteodysplasia described in Germany. We conclude that patients with presenile dementia, psychosis, or early-onset Parkinsonism associated with basal ganglia calcification should undergo X-rays of hand and feet to rule out polycystic lipomembranous osteodysplasia.
Schäfer
C
C
Psychiatrische Klinik Sonnenhalde, CH-Riehen, Germany.
Klünemann
H H
HH
Ibach
B
B
Mueller
J
J
Putzhammer
A
A
Trender-Gerhard
I
I
Schuierer
G
G
Klein
H E
HE
D000328:Adult; D000544:Alzheimer Disease / Q000175:diagnosis / Q000235:genetics* / Q000523:psychology; D001479:Basal Ganglia / Q000473:pathology; D001480:Basal Ganglia Diseases / Q000175:diagnosis / Q000235:genetics* / Q000523:psychology; D001845:Bone Cysts / Q000175:diagnosis / Q000235:genetics* / Q000523:psychology; D002114:Calcinosis / Q000175:diagnosis / Q000235:genetics* / Q000523:psychology; D002869:Chromosome Aberrations; D003937:Diagnosis, Differential; D004569:Electroencephalography; D005260:Female; D005808:Genes, Recessive; D005858:Germany; D006801:Humans; D008060:Lipodystrophy / Q000175:diagnosis / Q000235:genetics* / Q000523:psychology; D008279:Magnetic Resonance Imaging; D009460:Neurologic Examination; D009483:Neuropsychological Tests; D014057:Tomography, X-Ray Computed