Teaching NeuroImages: Chronic inflammatory demyelinating polyradiculoneuropathy causing spinal cord compression

A 54-year-old man presented with 6 months of progressive gait unsteadiness and weakness of four limbs. Examination showed severe symmetric proximal and distal weakness of all limbs, hypesthesia below the knees, vibratory sensation loss in lower limbs and hands, absent tendon reflexes, and bilateral Babinski signs. CSF contained 19 g/L protein (normal <0.45) and 2 lymphocytes/mm³. Electrodiagnostic studies revealed absent motor and sensory responses in all limbs, and evidence of denervation in hand and foot muscles. He was diagnosed with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and treated with IV immunoglobulin (figure). Babinski signs disappeared and there was gradual improvement in ataxia and strength.

Figure Contrast-enhanced T1-weighted MRI studies demonstrating massive hypertrophy of cervical nerve roots causing cervical spinal cord compression (A, B; dotted arrow: spinal cord; white arrows: nerve roots) and major hypertrophy of brachial plexi (C, white arrows)

CIDP is one of the main causes of hypertrophic neuropathy.¹ Repetitive demyelination and remyelination with onion bulb formation can result in gross enlargement of spinal nerves and roots.¹ Although rare, cases of CIDP with spinal cord compression due to hypertrophic spinal roots have been reported.^1,2