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. 2011 Apr;14(2):81-92.
doi: 10.4103/0972-2327.82789.

Chronic dysimmune neuropathies: Beyond chronic demyelinating polyradiculoneuropathy

Affiliations

Chronic dysimmune neuropathies: Beyond chronic demyelinating polyradiculoneuropathy

Satish V Khadilkar et al. Ann Indian Acad Neurol. 2011 Apr.

Abstract

The spectrum of chronic dysimmune neuropathies has widened well beyond chronic demyelinating polyradiculoneuropathy (CIDP). Pure motor (multifocal motor neuropathy), sensorimotor with asymmetrical involvement (multifocal acquired demylinating sensory and motor neuropathy), exclusively distal sensory (distal acquired demyelinating sensory neuropathy) and very proximal sensory (chronic immune sensory polyradiculopathy) constitute the variants of CIDP. Correct diagnosis of these entities is of importance in terms of initiation of appropriate therapy as well as prognostication of these patients. The rates of detection of immune-mediated neuropathies with monoclonal cell proliferation (monoclonal gammopathy of unknown significance, multiple myeloma, etc.) have been facilitated as better diagnostic tools such as serum immunofixation electrophoresis are being used more often. Immune neuropathies associated with malignancies and systemic vasculitic disorders are being defined further and treated early with better understanding of the disease processes. As this field of dysimmune neuropathies will evolve in the future, some of the curious aspects of the clinical presentations and response patterns to different immunosuppressants or immunomodulators will be further elucidated. This review also discusses representative case studies.

Keywords: Multifocal motor neuropathy; chronic inflammatory demyelinating neuropathy; distal acquired demyelinating predominately sensory; multifocal acquired demyelinating sensory and motor.

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Conflict of interest statement

Conflict of Interest: Nil

Figures

Figure 1
Figure 1
(a) Magnetic resonance imaging axial scan at L5-S1 (arrows show the extreme thickening of the sacral nerve roots) (b) Whole body substraction magnetic resonance image showing tubershaped lumbosacral root enlargement from the L1 to the S1 levels (most prominent in S1 root as shown by the arrow)
Figure 2
Figure 2
Loss of architecture, vascular proliferation, infiltrate of small and large lymphocytes with prominent nucleoli, mitotic figures and Reed Sternberg cells Hodgkin's disease – nodular sclerosis ×40 Haematoxylin and eosin

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