Efficacy of intravenous immunoglobulin in patients with IgG monoclonal gammopathy and polyneuropathy
ABSTRACT The optimal treatment of patients with neuropathy associated with IgG monoclonal gammopathy of undetermined significance is unknown. Plasma exchange has been shown to be effective but alternative therapies have not been systematically evaluated.
To report our experience with intravenous immunoglobulin (IVIG) in patients with IgG monoclonal gammopathy of undetermined significance polyneuropathy.
Retrospective review of clinical and electrodiagnostic features of 20 consecutive patients treated with IVIG over an 8-year period.
Academic medical center.
Medical Research Council strength (maximum, 40 points) and sensory (maximum, 26 points) scores, modified Rankin Disability Scale score.
There were 14 men and 6 women (mean age, 65 years; age range, 36-82 years). The mean strength score was 35.6 points and the mean sensory score was 15.8 points prior to therapy. After IVIG therapy, the mean strength score increased by 1.1 points (P =.22) and the sensory score increased by 1.7 points (P =.11). Eight patients (40%) improved by 2 points or more in their motor or sensory score and 1 point or more in the modified Rankin Disability Scale score and were considered IVIG therapy responders. They had a shorter duration of symptoms (P =.03), numb hands (P =.02), and falling episodes (P =.02), and had greater proximal leg weakness (P =.02) compared with nonresponders. In IVIG therapy responders, the ulnar motor conduction velocity was slower, ulnar and peroneal distal motor latencies were prolonged, and the frequency of conduction block was higher (13 of 36 motor nerves in responders vs 6 of 53 in nonresponders, P =.008).
Intravenous immunoglobulin therapy was beneficial in 8 (40%) of our 20 patients with polyneuropathy and IgG monoclonal gammopathy of undetermined significance. Proximal leg weakness, short duration of symptoms, and demyelinating features on electrodiagnostic studies were associated with a response to IVIG therapy.
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ABSTRACT: Chronic inflammatory demyelinating polyneuropathy (CIDP) is a chronic, acquired immune and inflammatory disorder of the peripheral nervous system. The classic form of the disorder is manifested by progressive or relapsing proximal or generalized limb weakness and areflexia, and usually easily recognized; it is the large number of regional and functional variants and variety of associated illnesses that pose a challenge to the clinician in practice. Similarly, laboratory and electromyography criteria have been developed to confirm the diagnosis; however, these various schemes are contrived because only 50% to 60% of patients with typical clinical features of CIDP fulfill these strict electrodiagnostic research criteria. Several studies have established the efficacy of immune therapies such as corticosteroids, plasma exchange, and intravenous immune globulin as the mainstay of treatment of CIDP, but these treatments might provide only short-term benefit. This review offers an approach to the evaluation and management of patients with CIDP and highlights the difficult clinical problems in those who do not respond or frequently relapse after treatment with standard therapies such as patients with CIDP and concomitant axonal loss, and the assessment of those with CIDP and concurrent diseases such as diabetes mellitus.Journal of clinical neuromuscular disease 07/2003; 4(4):174-89. DOI:10.1097/00131402-200306000-00004
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ABSTRACT: The review concentrates on the use of clinical neurophysiology in peripheral nerve disorders covered in the present issue. It is pertinent to distinguish different types of involvement of fibers in diabetic neuropathy, including the involvement of small and large fibers, to outline the diagnostic criteria of inflammatory neuropathies, and to describe the spectrum of peripheral nerve pathophysiology in inherited neuropathies. Painful neuropathies represent a particular challenge to clinical neurophysiology since it is mainly small fibers, which are difficult to study, that are affected. Electrodiagnostic studies have relevance in distinguishing neuropathies with different etiologies in diabetes mellitus, and different strategies and methods are necessary to study patients with autonomic and small-fiber involvement. The involvement of motor or sensory fibers, or both, and primary axonal or demyelinative pathology are important questions relating to immune-mediated neuropathies studied in the context of the specificity of antibodies against various neuronal and Schwann-cell structures. In hereditary neuropathy, electrophysiological studies are also used to distinguish axonal neuropathies from demyelinating neuropathies, though overlap and 'intermediate' patterns have become well recognized. In pain syndromes, conventional electrophysiological studies may give normal results if large fibers are not involved, and the use of autonomic measures in these situations has particular relevance. The usefulness of electrodiagnostic measures depends on the clinical, diagnostic, or pathophysiological question involved, and the strategy employed should reflect the advantages and limitations of these methods. If adequate consideration is paid to these properties, then such studies have a central role in the diagnosis and adequate treatment of patients with neuromuscular disorders.Current Opinion in Neurology 11/2003; 16(5):603-12. DOI:10.1097/01.wco.0000093104.34793.94 · 5.31 Impact Factor
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ABSTRACT: 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.European Journal of Neurology 12/2003; 10(6):677-85. DOI:10.1046/j.1468-1331.2003.00687.x · 4.06 Impact Factor