Nineteen‐year follow‐up of Waldenström's‐associated neuropathy and Bing–Neel syndrome

Abstract

We describe the follow-up of a patient with Waldenström's macroglobulinemia who developed mild predominantly sensory peripheral neuropathy, Bing-Neel syndrome, and, after 17 years, acute mononeuropathy multiplex associated with increasing paraprotein levels. Nerve biopsy demonstrated deposition of IgM in the endoneurium and perineurium. Magnetic resonance imaging showed extension of the cerebral white-matter abnormality. We suggest that the pathogenetic mechanism of the mononeuropathy multiplex may include direct IgM deposition. Late peripheral nerve complications appeared to be related to the paraprotein level.

Amyloid‐like IgM deposition neuropathy: a distinct clinico‐pathologic and proteomic profiled disorder

“…Endoneurial aggregates were confined by the perineurium without evidence of epineurial involvement. Prior reports have emphasized the same nerve distribution ( Darn-ley, 1962 ; Iwashita et al, 1974 ; Lamarca et al, 1987 ; Yee et al, 1989 ; Moorhouse et al, 1992 ; Vallat et al, 2008b ; Leschziner et al, 2009 ; Luigetti et al, 2010 ) . The mechanisms of deposition and endoneurial selectivity remain unknown.…”

Repeated treatments with rituximab in a patient with amyloid-like I g M deposition neuropathy

“… 1 – 6 Sensory disturbances preceded development of weakness by 6 months to 20 years in 5 cases. 1 – 4 Immune-mediated treatments, such as prednisolone, 1 , 3 chlorambucil, 2 , 6 and cyclophosphamide, 2 , 4 , 5 plasma exchange, 1 , 6 and RTX infusion, 1 , 4 , 5 were introduced in an early stage of development of weakness but resulted in poor improvement of limb weakness in 6 cases. 1 – 3 , 5 , 6 One patient with mononeuropathy multiplex after 17 years of sensory neuropathy showed almost complete recovery of power after oral administration of cyclophosphamide.…”

“… 1 – 3 , 5 , 6 One patient with mononeuropathy multiplex after 17 years of sensory neuropathy showed almost complete recovery of power after oral administration of cyclophosphamide. 4 …”

“…The pathophysiology of amyloid-like IgM deposition neuropathy is unclear and may be heterogeneous. In addition to a direct effect of deposited materials on nerve fibers, a vasculitic process is suggested by clinicopathologic features such as acute-onset multiple mononeuropathy 4 – 6 and epineurial inflammatory changes. 1 In our patient, sural nerve biopsy findings indicated that sensory polyneuropathy is likely to result from progressive deposition of amyloid-like IgM and subsequent compression of nerve fibers.…”

“…Amyloid-like immunoglobulin M (IgM) deposition neuropathy associated with Waldenström macroglobulinemia is a rare phenotype of IgM-related neuropathy. The prominent clinical features are painful sensory-dominant neuropathy followed by distal motor weakness and atrophy over a long clinical course 1 – 4 that are refractory to immunosuppressive treatment in many patients. 1 – 6 Herein, we report a case of amyloid-like IgM deposition neuropathy that was successfully treated with rituximab (RTX).…”