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Home arrow Environment arrow Inflammatory Disorders of the Nervous System: Pathogenesis, Immunology, and Clinical Management
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CIDP Variants

Only 50% of patients with CIDP present with classic features described above [77]. Other variants of CIDP include sensory-predominant, motor-predominant, ataxic, chronic inflammatory sensory polyradiculopathy (CISP), and multifocal acquired demyelinating sensory and motor (MADSAM) neuropathy.

Five to thirty-five percent of CIDP patients present with sensory symptoms in their lower extremities [78]. Despite this purely sensory presentation from the clinical standpoint, motor nerve conduction abnormalities consistent with demyelination can be found in many of these patients, and a pure sensory variant of CIDP has only been reported rarely [79, 80]. On the other hand, many of the patients with purely sensory variant will develop motor involvement years later [81]. Sensory CIDP may mimic sensory ganglionopathy if the sensory action potentials are absent and motor conduction studies are entirely normal. In these instances, nerve biopsy may be required for the diagnosis [79, 82]. A rare (~5%) predominantly sensory ataxic form of CIDP (chronic immune sensory polyradiculopathy (CISP)) is a distinct clinical entity that involves large fibers of the dorsal roots rather than distal sensory nerves [83, 84]. In these cases peripheral nerve conduction studies may be unrevealing, and somatosensory conduction potentials may need to confirm demyelination of the sensory nerve roots [85]. The motor-predominant variant of CIDP presents with relatively symmetric proximal and distal muscle weakness, demyelination on the nerve conduction study, and minimal or absent sensory involvement, which occurs in about 7-10% of patients with CIDP, more commonly in young adults <20 years of age [78, 86, 87]. The main differential diagnosis for motor variant of CIDP is multifocal motor neuropathy. Multifocal acquired demyelinating sensory and motor (MADSAM, aka Lewis-Sumner syndrome) neuropathy is a focal variant which occurs in about 6-15% of CIDP patients [78]. MADSAM presents with an asymmetrical muscle weakness and sensory changes, usually starting in one or both upper extremities. Later in its clinical course, MADSAM may become more diffuse and involve both lower extremities as well.

It is differentiated from axonal mononeuritis multiplex by the presence of segmental demyelination in the nerve conduction study, involving both motor and sensory nerves.

 
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