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We describe a patient with intravenous immunoglobulin (IVIg) dependent relapsing chronic inflammatory demyelinating polyneuropathy (CIDP), unresponsive to steroids or conventional immunosuppressive agents, who achieved remission following treatment with alemtuzumab.
A 19 year old women presented with a two week history of distal lower limb paraesthesiae and distal upper limb weakness. Her symptoms worsened over four weeks but she remained mobile. There was no history of preceding infections, pain, or autonomic dysfunction. Examination revealed reduced limb muscle tone and distal weakness, more marked in the upper limbs. She was areflexic with flexor plantar responses and had reduced pin prick sensation in the hands and toes.
A lumbar puncture showed a raised protein of 1.8 g/l, with normal glucose and cell counts and negative oligoclonal bands. A preliminary diagnosis of Guillain-Barré syndrome was made and she was treated conservatively, with good recovery.
Three months later she developed further weakness, predominantly affecting her legs. Examination revealed normal muscle tone, distal but asymmetrical limb weakness, absent reflexes, and distal sensory loss. She remained mobile with the aid of unilateral support.
Further investigations were normal or unremarkable; these included full blood count, blood film, urea and electrolytes, liver function tests, glucose, erythrocyte sedimentation rate, creatinine kinase, vitamin B-12, folate, serum electrophoresis, antinuclear antibodies, antineutrophilic cytoplasmic antibody, antiganglioside antibodies including anti-GM1, anti-double-stranded DNA antibodies, HIV, hepatitis A, B, and C, and …
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