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Abstracts of the 8th Meeting of the Italian Peripheral Nerve Study Group: 35
Author(s) -
Mazzeo A,
Nastasi V,
Autunno M,
Musolino C,
Majorana G,
Girlanda P,
Vita G,
Messina C
Publication year - 2003
Publication title -
journal of the peripheral nervous system
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1
H-Index - 67
eISSN - 1529-8027
pISSN - 1085-9489
DOI - 10.1046/j.1529-8027.2003.00035.x
Subject(s) - medicine , multiple myeloma , pathology , polyneuropathy , immunofixation , bone marrow , biopsy , melphalan , peripheral neuropathy , monoclonal gammopathy of undetermined significance , plasmacytoma , monoclonal , antibody , immunology , monoclonal antibody , diabetes mellitus , endocrinology
A 41‐year‐old man suffered for 1 year of severe progressive sensorimotor polyneuropathy that rendered him bedridden in few months. Routine laboratory investigations, including serum protein immunoelectrophoresis and immunofixation, were normal. Electrophysiological studies and sural nerve biopsy showed a severe mixed axonal and demyelinating polyneuropathy. X‐ray showed multiple osteosclerotic lesions in the spine. MRI disclosed pachymeningeal thickening of the brain and meningeal infiltration at cauda equina level with gadolinium enhancement. Bone marrow biopsy revealed 12% of plasma cells with a monotypic immunocytochemical profile for lambda light chain. A diagnosis of osteosclerotic myeloma (OM) was done. He received two courses of IVIg followed by melphalan and prednisone without any benefit. Very recently he underwent a bone marrow autotransplantation. OM is a rare variant of multiple myeloma, representing less than 3% of all myeloma cases. In contrast to multiple myeloma, in OM half of patients have a peripheral neuropathy similar to CIDP, with or without features of POEMS syndrome. MRI meningeal involvement, concomitant with a severe neuropathy, was not yet described in OM. A paraneoplastic process in addition to a direct neoplastic infiltration may account for the severity of the disease in our patient, despite different therapeutic approaches.