Polyneuropathy with osmiophilic membrane-bound, cytoplasmic inclusions in Schwann cells (POMCIS).
In the cytoplasm of Schwann cells of a sural nerve biopsy from a 21-year-old female patient with chronic neuropathy we noted numerous unique, usually double membrane-bound, osmiophilic, granular or globular inclusions, approximately 30-600 microm in diameter. Some of these membrane-bound vesicular or tubular structures contained less dense or no osmiophilic inclusions. Morphometry revealed a reduction of the myelin area per endoneural area to approximately 13% (normal value: 20- 30%) and of the density of myelinated nerve fibers to 5,412/mm(2) (normal value at this age: 6,000-9,000/mm(2)). Large myelinated nerve fibers were predominantly reduced in number, and no myelinated nerve fibers with diameters larger than 4.5 microm were seen. Numerous, usually small onion bulb formations indicated a predominantly demyelinating type of neuropathy. This is to the best of our knowledge the first case of a chronic demyelinating neuropathy in which this kind of presumably pathognostic deposits in the cytoplasm of Schwann cells was detected.
Schröder
J M
JM
Institut für Neuropathologie, Universitätsklinikum, Rheinisch-Westfälische Technische Hochschule Aachen, Pauwelsstrasse 30, D-52074 Aachen, Germany. jmschroeder@post.klinikum.rwth-aachen.de
Wang
J F
JF
Sindern
E
E
Malin
J P
JP
D000328:Adult; D001369:Axons / Q000473:pathology / Q000648:ultrastructure; D002462:Cell Membrane / Q000473:pathology / Q000648:ultrastructure; D004396:Coloring Agents; D003593:Cytoplasm / Q000473:pathology / Q000648:ultrastructure; D005260:Female; D006801:Humans; D002479:Inclusion Bodies / Q000473:pathology* / Q000648:ultrastructure; D008854:Microscopy, Electron; D008928:Mitochondria / Q000473:pathology / Q000648:ultrastructure; D009413:Nerve Fibers, Myelinated / Q000473:pathology / Q000648:ultrastructure; D009431:Neural Conduction / Q000502:physiology; D009992:Osmium; D011115:Polyneuropathies / Q000473:pathology*; D012583:Schwann Cells / Q000473:pathology* / Q000648:ultrastructure; D013497:Sural Nerve / Q000473:pathology / Q000648:ultrastructure
D004396:Coloring Agents; D009992:Osmium