Chronic immune sensory polyradiculopathy (CISP)

Area of Science:

• Neurology
• Immunology
• Clinical Neuroscience

Background:

• Chronic immune sensory polyradiculopathy (CISP) is a rare variant of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP).
• CISP typically presents with sensory deficits without significant motor weakness.
• Early diagnosis and treatment are crucial for managing CISP.

Purpose of the Study:

• To describe a case of CISP presenting with isolated sensory ataxia.
• To propose diagnostic criteria for CISP.
• To evaluate the efficacy of intravenous immunoglobulin (IVIg) in treating CISP.

Main Methods:

• Case report of a patient with isolated sensory ataxia.
• Utilized nerve conduction studies, MRI, somatosensory evoked potentials (SSEPs), and cerebrospinal fluid (CSF) analysis.
• Proposed diagnostic criteria based on clinical and electrodiagnostic findings.

Main Results:

• The patient presented with normal initial nerve conduction studies and MRI but showed delayed SSEPs, elevated CSF protein, and nerve root enhancement on subsequent imaging.
• Diagnostic criteria for CISP were proposed, including sensory symptoms, normal motor/sensory nerve conduction studies, and specific electrodiagnostic/imaging findings.
• The patient demonstrated significant improvement following treatment with IVIg.

Conclusions:

• CISP is a distinct CIDP variant characterized by sensory ataxia and specific diagnostic findings.
• Proposed criteria aid in the diagnosis of CISP, differentiating it from other neuropathies.
• Intravenous immunoglobulin is an effective treatment for CISP, leading to clinical improvement.