[Peripheral neuropathy with mixed cryoglobulinemia].
Among 54 patients with mixed cryoglobulinemia a peripheral neuropathy was demonstrated in 23 patients (42.6%). Five patients had a secondary form while the majority (18) had essential cryoglobulinemia. The immunoglobulin components were polyclonal in 5 cases, only present in trace amounts in 5 and of type II in 13 patients. Fifteen patients had symptoms of polyneuropathy, but in 18 cases the symptomatology had symmetrical features and in two cases asymmetrical characteristics. In 8 patients a mono-polyneuropathy was observed. The most significant electrophysiological finding was the decreased amplitude of the sensory action potential of the sural nerve, which we observed in 95% of the instances, whereas a decreased sensory conduction velocity was seen in 8/23 cases only. A biopsy of the sural nerve, performed in eleven cases, always showed abnormal epi- and endoneurial vessels. Epineurial vessel vasculitis was found in 4 cases and fibrous thickening of the vessel wall in two cases. Endothelial swelling of the endoneurial vessels was observed in 7 patients and luminal obliteration in 3 samples. Axonal degeneration demonstrated in 7 of the 11 processed sample tissues was the main pathological finding. Signs of de/remyelination were associated in 3 cases, and were the dominant lesions in a single specimen. The simultaneous biopsy of the peroneus brevis muscle showed signs of denervation in all samples, vasculitis in 7 cases and deposits of filaments plus annular bodies of the vessel wall in 2 samples. All these data support the hypothesis that ischaemic damage may be the final common pathway of the majority of peripheral neuropathies in mixed cryoglobulinemia patients.
Manganelli
P
P
II Divisione medica e Reumatologia, USL 4, Parma.
Pavesi
G
G
Fiocchi
A
A
Gemignani
F
F
Ferraccioli
G
G
Nervetti
A
A
Marbini
A
A
D000328:Adult; D000368:Aged; D001706:Biopsy; D003449:Cryoglobulinemia / Q000150:complications / Q000175:diagnosis* / Q000473:pathology; D004576:Electromyography; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009132:Muscles / Q000473:pathology; D010523:Peripheral Nervous System Diseases / Q000175:diagnosis* / Q000209:etiology / Q000473:pathology; D013497:Sural Nerve / Q000473:pathology
La neuropatia periferica della criglobulinemia mista.