Systematic Review of a Rare Immune Disorder Involving the Proximal Nerve Roots
Saurabh Singhal1, Rahul Khanna2, Anudeep Surendranath3, Jayksh Chhabra4, Vismay Thakkar5, Rajesh Gupta6
1Well Smart Neurology Clinic, Opelousas General Health System, 2Neurology, Neurology and Sleep clinic, PLC, 3CHI St. Vincent, 4Neurology, University of Texas Health Science Center, 5Neurology, Wesley Medical Center, Wichita, KS, 6UTHealth
Objective:

Chronic immune sensory polyradiculopathy (CISP) is a rare and often overlooked inflammatory/immune nervous disorder, first identified by Sinreich et al. in 2004. To our best knowledge, there has not been any recent significant study to inform us about the incidence of the disease and prevalence of characteristic presenting symptoms, clinical and diagnostic findings with treatment outcome. Here we conducted a systematic literature review of the CISP cases that presented with characteristic neurological symptoms, clinical, radiological, neurophysiological, CSF and other findings leading to the accurate diagnosis followed by treatment with improvement in the clinical condition.

Background:

CISP is characterised by the isolated involvement of the dorsal roots proximal to the dorsal root ganglion, typically sparing the distal nerve roots, distinguishing it from more commonly known conditions such as acute inflammatory demyelinating polyneuropathy ( AIDP) and chronic inflammatory demyelinating polyneuropathy ( CIDP). The diagnosis is often missed due to normal EDX and CNS imaging findings leading to patients' clinical presentation being incorrectly labelled as hysterical or non-organic. 

Design/Methods:

We conducted a systematic review of CISP cases published on PubMed. Our review included a total of 22 patients from 8 articles. Specific Inclusion and Exclusion criteria diagnostic for CISP were used to select the articles and reduce any potential bias. 

Results:

Many patients presented with gait difficulties and sensory ataxia, were found to have normal nerve conduction studies (NCS) and electromyography (EMG), but exhibited characteristic abnormalities in somatosensory evoked potentials (SSEP), elevated CSF protein levels, thickened nerve roots on contrast-enhanced lumbar spine MRIs, and histological changes on nerve root biopsies. Clinical improvement was observed following treatment with steroids and/or intravenous immunoglobulin (IVIG).

Conclusions:

The study concluded that while CISP is rare, it is an important clinical entity to consider, as accurate diagnosis and appropriate treatment can lead to significant improvements in neurological symptoms and disabilities

10.1212/WNL.0000000000210831
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