Acute presentation of Tangier polyneuropathy: a clinical and morphological study.
We describe a patient with Tangier disease and a peripheral neuropathy with an unusual acute onset. The morphological studies of sural nerve biopsy revealed both axonal degeneration and demyelination, and the fiber loss was preferentially restricted to two of ten nerve fascicles. The cytoplasm of Schwann cells, fibroblasts, macrophages and pericytes were vacuolated because of the presence of numerous lipid droplets. The clinical and morphological findings are consistent with the possibility that ischemia plays a major role in causing this neuropathy.
Department of Neurology, University of Milan, Istituto Scientifico S. Raffaele, Italy.
R
R
Fazio
R
R
Nemni
A
A
Quattrini
G
G
Ruotolo
S
S
Iannaccone
D
D
Mamoli
M
M
Lodi
N
N
Canal
D005260:Female; D006529:Hepatomegaly / Q000000981:diagnostic imaging / Q000473:pathology; D006651:Histocytochemistry; D006801:Humans; D007558:Italy; D008854:Microscopy, Electron; D008875:Middle Aged; D009046:Motor Neurons / Q000502:physiology; D009431:Neural Conduction / Q000502:physiology; D009475:Neurons, Afferent / Q000502:physiology; D016612:Paraffin Embedding; D013163:Splenomegaly / Q000000981:diagnostic imaging / Q000473:pathology; D013497:Sural Nerve / Q000473:pathology / Q000503:physiopathology; D013631:Tangier Disease / Q000000981:diagnostic imaging / Q000473:pathology* / Q000503:physiopathology; D014463:Ultrasonography