J Clin Neurol.  2024 May;20(3):276-284. 10.3988/jcn.2023.0469.

Sensory Chronic Inflammatory Demyelinating Polyradiculoneuropathy: Neglected ImmunotherapyResponsive Sensory Neuropathy

• Oh SJ
• King P

• KMID: 2555610
• DOI: http://doi.org/10.3988/jcn.2023.0469

Abstract

Background and Purpose
To report an improvement with immunotherapy in 34 (85%)/40 patients who required an immunotherapy among 56 patients with sensory chronic inflammatory demyelinating polyradiculoneuropathy (CIDP).
Methods
Sensory CIDP was diagnosed when two inclusion criteria are met: 1) acquired, chronic progressive or relapsing symmetrical or asymmetrical sensory polyneuropathy that had progressed for >2 months; and 2) definite electrophysiological and/or biopsy evidence of demyelinating neuropathy.
Results
Fifty-six patients with sensory CIDP were identified. Evidence of demyelination was obtained from by the routine motor nerve conduction study (NCS) in 39 (70%) patients, from a nerve biopsy in 10, and from a near-nerve needle sensory NCS in 7 patients. The most prominent laboratory abnormality was a high protein level in the cerebrospinal fluid in 21 (49%) of 43 tested patients. Immunotherapy was required in 41 (79%) of the 52 followed-up patients. An improvement with immunotherapy was observed in 36 (88%)/41 patients. In three patients, motor weakness developed in 5–8 years’ follow-up period and so, their diagnosis was changed to CIDP.
Conclusions
Sensory CIDP is responded to an immunotherapy in 88% of the treated patients. Sensory CIDP was diagnosed by the routine motor NCS in 70% of patients and by a sural nerve biopsy in 18% of patients. Thus, sensory CIDP should be recognized as a treatable CIDP variant among the different types of “idiopathic sensory neuropathy.”

Keyword

atypical CIDP; chronic sensory demyelinating neuropathy; idiopathic sensory neuropathy; sensory CIDP; sensory variant of CIDP