CIDP and Spinal Cord Compression: A Case Report

Case Diagnosis: Incomplete paraplegia 2/2 thoracic myelopathy

Case Description or Program Description: Patient presented to the emergency department with progressive bilateral lower extremity numbness, tingling, weakness and gait difficulty over a 4 month period.
MRI of the lumbar spine significant for Moderate to severe spondylosis spinal canal stenosis at L5-S1, moderate spinal canal stenosis at L1-2 and L2-3. Spinal tap was significant for albuminocytologic dissociation managed with IVIG for 4 days and had functional improvement in rehab. Spinal tap subsequently significant for elevated Mag-Ab titer at 23,892. Patient was noted to have functional decline. Neurology consulted with plan to infuse Rituximab. Physical exam showed bilateral Babinski sign and impaired sensation to light touch to T5 dermatome and below. MRI T-spine revealed severe spinal stenosis with cord compression and signal change in cord at T3-4.

Setting: Tertiary Care Academic Hospital

Assessment/Results: Patient underwent T2-4 bilateral laminectomy. Her symptoms improved within 2 weeks post-surgery with strength improving to 3/5 in hip flexors bilaterally upon discharge from rehab.

Discussion (relevance): Severe spinal cord compression will typically present with upper motor neuron signs and is important to differentiate from demyelinating pathologies such as chronic inflammatory demyelinating polyneuropathy (CIDP) which could present with weakness and paresthesia. The complexity of this case is compounded by the patient initially presenting with the Anti-Mag variant of CIDP with worsening symptoms post treatment. Thorough physical examination and MRI report confirmed demyelination of the lumbar spine. The presence of a confirmed diagnosis, such as Anti-MAG CIDP, can create a cognitive bias, potentially delaying the identification of coexisting pathologies.

Conclusions: When a patient exhibits acute neurological deterioration, a comprehensive re-evaluation is imperative to prevent misdiagnosis and ensure timely intervention.