Neuropathy associated with cryoglobulinemia

Abstract

A patient with severe subacute sensory ataxia was found to have an IgM (kappa) cryoglobulin. Clinical, electrophysiologic, and sural nerve biopsy studies indicated that axonal degeneration and segmental demyelination both played a role in the pathogenesis of this neuropathy. Corticosteroid therapy was associated with notable clinical improvement and a 50% decrease in cryoglobulin concentration.

Peripheral neuropathy in essential mixed cryoglobulinaemia.

“…Unmyelinated fibres were involved only in more severely damaged nerves (patients 5, 6, 18). Changes of the endoneurial microvessels, consisting of thickening of the vessel walls and obliteration of the lumen, were seen in all specimens but were especially severe in four (patients 1,5,9,12). Scattered erythrocytes around endoneurial capillaries ("endoneurial purpura"'9 were seen in patient 12).…”

“…Scattered erythrocytes around endoneurial capillaries ("endoneurial purpura"'9 were seen in patient 12). Necrotising vasculitis of the epineurial arterioles was present in two specimens (patients 5, 15), whereas in four others (patients 7,9,12,18) there were signs of probable or healed vasculitis. Severe changes of the endoneurial vessels were more common in type II EMC (3/5 v 1/3), whereas vasculitis tended to be more common in type III EMC (3/3 v 3/5).…”

“…A sufficient number of teased fibres were available for examination in five biopsy specimens, whereas in three others no fibre could be isolated due to severe myelinated fibre loss (table 3). Axonal degeneration coexisted with variable degrees of segmental demyelination and remyelination, possibly secondary to axonal degeneration or atrophy.33"35 Direct immunofluorescence examination showed IgM deposits in the epineurial vessel walls in patient 12 and in the endoneurial vessels in patient 5, whereas it was negative in patients 9 and 15. In all examined patients peroneus brevis muscle biopsy specimens showed denervation of variable entity.…”

“…Compression at the usual entrapment sites was excluded. In five patients with isolated polyneuropathy (numbers 6,7,12,19,21), the nerves of the upper limbs were examined in addition to peroneal and sural nerves, showing abnormal motor and sensory examination of the median and ulnar nerve in two of them (patients 12 and 21) as features of subclinical polyneuropathy of the upper limbs. In all 18 patients abnormal EMG findings, of variable severity, were constantly observed in the distal muscles of the lower limbs, even in patients with normal electroneurographic examination of the peroneal nerves.…”

HCV-Related Nervous System Disorders

“…A patient with subacute sensory ataxia and IgMk cryoglobulin with demyelinating and axonal features was reported by Lippa et al [69]. In addition, sensory demyelinating polyneuropathy, responsive to immunomodulatory treatment, has been also detected in subjects with polyclonal hypergammaglobulinemia or IgM monoclonal gammopathy, in the absence of CG [70], suggesting the occurrence of humoral immune-mediated demyelination.…”