Summary
The case is described of a 35-year-old housewife diagnosed as having membranous lipodystrophy (as described by Nasu et al. in 1970 and called lipomembranous polycystic osteodysplasia by Hakola in 1972). The main symptom of this patient was a slowly progressive dementia. Skeletal symptoms were not seen. The computerized tomogram of the brain showed calcification of bilateral basal ganglia and the plain roentgenograms of the bones revealed cystic radiolucent areas at the distal end of the bones of the patient's extremities. Histological examination of the curetted material from the right talus revealed a ‘membranocystic’ pattern. The fatty tissue curetted from the cyst of the talus and the lysosomal enzymes of the white blood cells were biochemically normal. A possible relationship between this disease entity and connective disorders is considered.
Zusammenfassung
Klinische und histologische Befunde des Knochens bei einer 35jährigen Hausfrau mit membranöser Lipodystrophie (Nasu) oder lipomembranöser polycystischer Osteodysplasie (Hakola et al.) wurden beschrieben. Hauptsymptome der Patientin war progressive Demenz. Knochensymptome waren bis jetzt nicht bemerkt. Das Computertomogram des Gehirns zeigte Verkalkung der Basalganglien beiderseits. Multiple cystische Herde wurden auf dem Röntgenogramm der Beine dargestellt, und membrano-cystische Struktur wurde im Knochenmark des Talus mikroskopisch gefunden. Die chemische Analyse der Fettsäure des Talusknochenmarkes und Lysosomenzyme der weißen Blutkörperchen enthalten keine spezifischen Befunde. Mögliche Beziehung zwischen membranöser Lipodystrophie und Bindegewebserkrankungen wurde diskutiert.
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Matsuo, T., Suetsugu, M., Eguchi, M. et al. Membranous lipodystrophy. Arch Psychiatr Nervenkr 231, 123–130 (1982). https://doi.org/10.1007/BF00343833
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DOI: https://doi.org/10.1007/BF00343833
Key words
- Membranous lipodystrophy
- Lipomembranous polycystic osteodysplasia
- Calcification of basal ganglia
- Dementia
- Enzyme deficiency