Summary
Electron microscopic, histochemical and biochemical studies of a case of juvenile lipidosis have been presented. The neuronal inclusions were mostly unit membranebound with fingerprint-like patterns. Membrano-vesicular bodies were also found in the neuronal perikarya, but “typical” granular lipofuscin bodies were not seen. Cortical astrocytes contained a similar type of inclusion but the inclusions in the astrocytes in the white matter were quite different. The localization of acid phosphatase activity in all types of inclusions suggests their possible lysosomal nature. Biochemical analysis failed to pin-point any specific abnormalities.
Zusammenfassung
Elektronenmikroskopische, histochemische und biochemische Untersuchungen eines Falles von juveniler Lipidose werden mitgeteilt. Die neuronalen Einschlüsse sind zumeist von einer unit membrane umgeben und zeigen fingerabdruckähnliche Muster. Membranovesiculäre Körperchen finden sich ebenfalls in den Nervenzellperikaryen; typische granuläre Lipofuscinkörperchen werden aber nicht gesehen. Die Astrocyten der Rinde enthalten eine ähnliche Art von Einschlüssen, von denen aber die Einschlüsse in den Astrocyten der Marksubstanz völlig verschieden sind. Die Lokalisation der Aktivität von saurer Phosphatase in allen Einschlußtypen läßt an ihre mögliche lysosomale Natur denken. Die biochemische Analyse deckt keine spezifischen Abweichungen auf.
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Supported by grants NB-02255, NB-03356, NB-07298 and 5TI-MH-6418 from the National Institutes of Health, United State Public Health Service and R-160-65C from the United Cerebral Palsy Foundation.
The authors gratefully acknowledge the cooperation of the staff of the departments of neurology and neurosurgery, Albert Einstein College of Medicine, Bronx Municipal Hospital Center. They also thank Dr.Robert D. Terry for his review and criticism of the manuscript and the staff of the laboratory of the neuropathology for the excellent technical assistance.
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Suzuki, K., Johnson, A.B. & Marquet, E. A case of juvenile lipidosis: Electron microscopic, histochemical and biochemical studies. Acta Neuropathol 11, 122–139 (1968). https://doi.org/10.1007/BF00690216
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DOI: https://doi.org/10.1007/BF00690216