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Beriberi is an uncommon disorder related to thiamine deficiency. It is mainly found in underdeveloped countries among populations with poorly diversified diet, consisting largely of milled white cereals, a poor source of thiamine. In industrialized countries, thiamine deficiency with cardiac failure is more frequently found than the dry beriberi in high risk groups like chronic alcoholics. Nevertheless our attention was drawn to an outbreak of 70 cases of dry beriberi which occurred from 1997 to 2005 in the French territories of Reunion and Mayotte islands. It was characterized by an acute or sub-acute sensorimotor polyneuropathy with axonal lesions, affecting the lower limbs and occasionally the upper limbs, sometimes associated with cardiac beriberi. It affected young, non alcoholic individuals from the Mahoran and Comorian community who were in apparent good health when the illness occurred. Our study highlighted the feeding habits which are partly responsible for the development of the disease due to a chronic lack of thiamine and which probably contributed together with multiple cofactors to trigger off the illness. But many elements and mainly biological ones, also lead us to think that there is a genetic predisposition to develop this neuropathy.

Peripheral neuropathy has been widely reported in patients with monoclonal gammopathy (MG), more frequently immunoglobulin M (IgM) or IgG than IgA. Nevertheless, it remains unclear whether this association has clinical or pathogenic relevance. In order to clarify the possible role of IgG/IgA MG in neuropathy, we studied the clinical and electrophysiological features of 15 consecutive patients with polyneuropathy and IgG/IgA-MG, and compared them to those of 40 patients with polyneuropathy associated with IgM-MG, previously reported. Nine middle-aged patients (60%) had a chronic progressive or relapsing demyelinating polyneuropathy (DP) that was clinically and electrophysiologically indistinguishable from classic chronic inflammatory demyelinating polyneuropathy (CIDP) and frequently responded to immunosuppressive treatments, both characteristics supporting a dysimmune process. Six older patients (40%) had a chronic axonal distal polyneuropathy similar to the so-called chronic cryptogenic sensory polyneuropathy: there was no clear relationship with the MG in these patients and the response to immunosuppressive treatments was poor. Several features allowed us to distinguish between polyneuropathies associated with IgG/IgA-MG (IgG/IgA-PN) considered together and polyneuropathies associated with IgM-MG (IgM-PN). In the first group, the proportion of patients with a predominantly sensory clinical picture (27%) was less than that in the second group (75%), and there were fewer changes in nerve conduction studies. In addition, we found that the nine patients with DP associated with IgG/IgA-MG (IgG/IgA-DP) differed from the 31 with DP associated with IgM-MG (IgM-DP): clinical and electrophysiological studies clearly showed that the demyelinating pattern was more heterogeneous in IgG/IgA-DP than in IgM-DP. The spectrum of polyneuropathies associated with IgG/IgA-MG is heterogeneous, including DP, which is similar to classic CIDP, and axonal polyneuropathy, in which the pathogenic role of the MG remains elusive. In addition, IgG/IgA-DP differ from IgM-DP on clinical and electrophysiological grounds, suggesting probable different physiopathological mechanisms.

The clinical and laboratory phenotype of a paraproteinaemic neuropathy syndrome termed chronic sensory ataxic neuropathy with anti-disialosyl IgM antibodies is described in a series of 18 cases. Previous single case reports have outlined some features of this syndrome. All 18 cases were defined by the presence of serum IgM antibodies which react principally with NeuAc (alpha2-8)NeuAc(alpha2-3)Gal-configured disialosyl epitopes common to many gangliosides including GDlb, GD3, GTlb and GQlb. In 17 out of 18 cases, the serum contained benign IgM paraproteins, and in four of these cases at least two IgM paraproteins were present. The IgM antibodies were also cold agglutinins in 50% of cases. The clinical picture comprised a chronic neuropathy with marked sensory ataxia and areflexia, and with relatively preserved motor function in the limbs. In addition, 16 out of 18 cases had motor weakness affecting oculomotor and bulbar muscles as fixed or as relapsing-remitting features. When present in their entirety, these clinical features have been described previously under the acronym CANOMAD: chronic ataxic neuropathy, ophthalmoplegia, IgM paraprotein, cold agglutinins and disialosyl antibodies. This distribution of clinical features is reminiscent of Miller Fisher syndrome, in which acute-phase anti-disialylated ganglioside IgG antibodies are found. Clinical electrophysiology and nerve biopsy show both demyelinating and axonal features. A partial response to intravenous immunoglobulin and other treatments is reported in some cases.

The authors report here the case of a female patient who developed symmetrical polyneuropathy of the lower limbs a few months after she was found to have a myeloma with cryoglobulinemia. In musculocutaneous nerve biopsy material from this patient, electron microscopy showed pathological changes in the nervous tissue together with axonal degeneration and demyelination. But the most striking finding was the presence in the endoneurial space, of numerous masses made up of closely packed tubular structures. These masses occurred also in the walls and even in the lumen of all the vasa nervorum. Their morphologic features and dimensions were identical to cryoprecipitate which the authors extracted from the serum and which they examined with electron microscope.

A 53-year-old woman developed symmetrical polyneuropathy of the lower limbs a few months after she was found to have myeloma with cryoglobulinemia. In musculocutaneous nerve biopsy material, electron microscopy showed both axonal degeneration and demyelination. The most striking finding was the presence, in the endoneurial space, of numerous masses of closely packed tubular structures. These masses also were found in the walls of all the vasa nervorum and within the lumen of some vessels. The morphological features and dimensions of the deposits within nerve were identical to those of cryoprecipitates extracted from serum and examined with the electron microscope. An example of myeloma neuropathy with cryoglobulin deposits within the endoneurial space has not been reported previously.

Peripheral neuropathy can arise from various mechanisms during hepatitis C virus (HCV) infection, mainly involving associated mixed cryoglobulinemia. The frequency of demyelinating polyneuropathy is probably underestimated in these patients. We report two cases of demyelinating polyneuropathy in HCV-infected patients. The first case concerned a 76-year-old woman followed for hepatitis C associated with a mixed cryoglobulinemia (type II), who developed a chronic progressive distal motor weakness and sensory disturbances concomitant with a raise in serum aspartate aminotransferase (GOT/AST) and alanine aminotransferase (GPT/ALT) levels. Other laboratory studies were normal except for a decrease in the hemolytic fraction of complement to 75 IU ( n = 400–520). The second case was a 68-year-old woman followed for hepatitis C associated with a mixed cryoglobulinemia (type II), who had sensory disturbances in the lower limbs. Laboratory studies were otherwise unremarkable. Cerebrospinal fluid studies showed a normal protein content without pleocytosis in both patients. In both cases nerve conduction studies were suggestive of a mixed axonal and demyelinating sensorimotor neuropathy. Sural nerve biopsy showed segmental demyelination and severe loss of large myelinated fibers as well as some onion bulb formation in both cases. The two patients subsequently improved, the first with an antiviral treatment and the second with oral steroids. [ABSTRACT FROM AUTHOR]

Abstract: Polyneuropathy is a common presenting component of POEMS syndrome whose symptoms are attributed to an overproduction of vascular endothelial growth factor (VEGF). We report two female patients with POEMS syndrome presenting as a severe predominantly axonal neuropathy. A nerve biopsy was performed for these patients; pathological data confirmed unusual numerous acute axonal lesions associated with other classical signs of POEMS syndrome. POEMS syndrome is usually associated with demyelinating neuropathy (and secondary axonal loss); however, prominent axonal neuropathy (with acute axonal lesions on nerve biopsy) can also be observed in this disease. These observations illustrate the heterogeneity of peripheral nervous system involvement in POEMS syndrome. [Copyright &y& Elsevier]