[Improvement of severe and intravenous immunoglobulin-dependent multifocal motor neuropathy with conduction block after long-term rituximab]. / Amélioration d'un cas de neuropathie motrice multifocale avec blocs de conduction sévère, dépendant des immunoglobulines intraveineuses, après adjonction de rituximab au long cours.

ABSTRACT

INTRODUCTION: Some patients suffering from multifocal motor neuropathy with conduction blocks (MMNCB) are still disabled after treatment with intravenous immunoglobulin (IVIg). CASE REPORT We report the benefits of a combination of rituximab (RTX) and IVIg in the case of a 72-year-old man with MMNCB. DISCUSSION: Despite an IVIg treatment, the patient had severe motor weakness of the four limbs which limited daily living activity. Azathioprine, mycophenolate mofetyl and cyclophosphamid did not improve the patient's status. Adjunction of rituximab to IVIg therapy increased muscle strength measured on MRC sum score and reduced disability evaluated on ONLS (Overall Neuropathy Limitation Scale) score in the long term (37 months). In spite of the improvement of his neurological status, the patient remained dependent on IVIg. CONCLUSION: RTX could be proposed as a long-term complementary treatment for some severe cases of IVIg-dependent NMMBC. These results must be confirmed in a randomized controlled study.