Successful Treatment of Refractory Guillain-Barré Syndrome with Alemtuzumab in a Patient with Chronic Lymphocytic Leukemia

Abstract

This is the case of a 79-year-old man with chronic lymphocytic leukemia who presented with Guillain-Barré syndrome with features overlapping with the Miller Fisher syndrome and Bickerstaff brainstem encephalitis and positive antiganglioside GQ1b antibody about 6 months after treatment with bendamustine and rituximab. His clinical and neurologic condition continued to deteriorate despite sequential treatment with corticosteroids, intravenous immunoglobulin and plasmapheresis, but in the end, he had a complete and durable response to treatment with alemtuzumab.

Lymphoma-associated dysimmune polyneuropathies

“…(2) In most patients, lymphoma was diagnosed before the development of GBS. Also this temporal correlation varied, so that the onset of GBS developed: (a) with lymphoma relapse [70,77,78,88,89,101]; (b) during lymphoma maintenance therapy [74]; (c) at varying intervals (2 weeks and 3 months) after completion of chemotherapy for lymphoma deemed in remission [72,73,75,76,82]; (d) while undergoing (interval 6 days to 3 weeks) chemotherapy for lymphoma (cycles #1 to #6) [91,93,95,97,98]; (e) after induction chemotherapy for lymphoma (interval 11 to 16 days) [87,96], or (f) during the course (as long as 10 years) of indolent disease not under active treatment e.g., CLL [73,86,101].…”

Novel pathomechanisms in inflammatory neuropathies

“…In IVIG-dependent CIDP, Alemtuzumab prolonged remission in young patients with short disease duration [ 192 , 193 ]. Another study reported successful treatment of a 79-year-old patient with overlapping features of Miller Fisher syndrome and Bickerstaff brainstem encephalitis with Alemtuzumab [ 194 ]. Despite these promising results, potential autoimmune adverse effects such as autoimmune thyroiditis and other immunologic effects may limit the applicability of the drug [ 192 , 195 ].…”

Biological Drugs in Guillain-Barré Syndrome: An Update

“…Thereafter, patient was started on escalating doses of subcutaneous Alemtuzumab to the goal dose of 30 mg three times a week, which was associated with significant improvement in symptoms. It was suggested by authors that observed recovery could be both due to eliminating effect of Alemtuzumab on anti-GQ1b autoantibodies, or as a result of controlling the underlying malignancy (CLL) [ 74 ]. In spite of this, no further study has investigated the effect of Alemtuzumab in GBS patients.…”