Characteristics
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Patient No 1
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Patient No 2
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Patient No 3
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Patient No 4
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Peripheral neuropathy | Sensory polyneuropathy of legs | Severe motor and sensory multiplex mononeuropathy of three limbs | Mild sensory multiplex mononeuropathy of four limbs | Sensory multiplex mononeuropathy of four limbs |
Initial electromyographic study | Axonal sensory polyneuropathy | Severe neuropathy with low motor and sensory velocities | Axonal sensory neuropathy | Axonal sensory neuropathy |
Neuromuscular biopsy before treatment | Muscular atrophy with mild denervation; axonal sensory nerve involvement | Normal muscle; axonal sensory nerve loss with perineural, venular, and arteriolar inflammatory infiltrates | Muscular atrophy; inflammatory axonal neuropathy with perivenular and arteriolar lymphoid infiltrates | Normal muscle; axonal sensory neuropathy without inflammatory infiltrate |
Treatment | Interferon α (3 mIU three times a week) | Plasmapheresis (10 sessions) | Not treated | Interferon α (3 mIU three times a week) for 10 months2-151 |
+ | + |
Ursodesoxycholic acid (400 mg/day) for 24 months | Prednisone 1 mg/kg/day during 2 months2-150 |
Outcome after treatment | Progressive improvement | Improvement | Stable | Improvement |