Andoni Echaniz-Laguna, MD, PhD and
Nathalie Philippi, MD
From the Département de Neurologie, Hôpital Civil, Strasbourg, France.
Address correspondence and reprint requests to Dr. A. Echaniz-Laguna, Département de Neurologie, Hôpital Civil, BP 426, 67091 Strasbourg, France Echaniz-Laguna{at}medecine.u-strasbg.fr.
A 54-year-old man presented with 6 months of progressive gaitunsteadiness and weakness of four limbs. Examination showedsevere symmetric proximal and distal weakness of all limbs,hypesthesia below the knees, vibratory sensation loss in lowerlimbs and hands, absent tendon reflexes, and bilateral Babinskisigns. CSF contained 19 g/L protein (normal <0.45) and 2lymphocytes/mm3. Electrodiagnostic studies revealed absent motorand sensory responses in all limbs, and evidence of denervationin hand and foot muscles. He was diagnosed with chronic inflammatorydemyelinating polyradiculoneuropathy (CIDP) and treated withIV immunoglobulin (figure). Babinski signs disappeared and therewas 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.1Repetitive demyelination and remyelination with onion bulb formationcan result in gross enlargement of spinal nerves and roots.1Although rare, cases of CIDP with spinal cord compression dueto hypertrophic spinal roots have been reported.1,2
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