Early Aggressive Immunotherapy Improves Functional Outcome in Chronic Immune Sensory Polyradiculopathy

Area of Science:

• Neurology
• Immunology

Background:

• Chronic immune sensory polyradiculopathy (CISP) is an uncommon inflammatory disorder affecting proximal sensory nerve roots.
• Patients typically exhibit sensory ataxia, gait instability, and normal motor findings, often with normal nerve conduction studies.

Observation:

• An elderly male presented with acute progressive leg numbness and falls, demonstrating hyporeflexia and severe proprioceptive/vibratory sensory loss.
• Neurological examination revealed severe sensory ataxia, rendering him unable to stand or walk. Standard nerve conduction studies and spinal MRI were unremarkable.

Findings:

• Tibial somatosensory evoked potentials identified a proximal conduction defect in lumbar sensory nerve roots.
• Cerebrospinal fluid analysis showed cytoalbuminergic dissociation, indicating inflammation. Diagnosis of CISP was confirmed.
• The patient received aggressive immunotherapy, including corticosteroids, mycophenolate mofetil, and intravenous immunoglobulin, leading to significant recovery.

Implications:

• This case highlights the importance of recognizing CISP as a treatable cause of sensory ataxia.
• Early and aggressive combination immunotherapy can lead to dramatic functional recovery, contrasting with previous reports of slower improvement with single-line treatments.
• Physicians should consider CISP in patients with unexplained sensory ataxia, as timely intervention is key for optimal outcomes.