Complete unilateral ophthalmoplegia as the presenting manifestation of Waldenstrom's macroglobulinemia

In Waldenstrom's macroglobulinemia, an excess number of plasma cells produces a large amount of a single class of immunoglobulin M globulins (which are associated with immune defenses). A monoclonal IgM present in large amounts is an example of a paraprotein, an antibody or antibody fragment present in excessive quantities. Waldenstrom's macroglobulinemia is most common among men over 50. Macroglobulinemia is associated with hepatomegaly, adenopathy, splenomegaly (enlarged liver, lymph nodes and spleen), hemorrhage, and anemia. Examination of the bone marrow reveals numerous lymphocytes and plasmacytes. Recently, however, a case was seen in which the presenting symptom of macroglobulinemia was ophthalmoplegia (paralysis) in the right eye. The patient, a 75-year-old man, had diplopia (double vision), pain, and ptosis (drooping of the eyelid). Neurological evaluation revealed a total ophthalmoplegia of the right eye, involving three separate cranial nerves (the abducens, oculomotor, and trochlear nerves). IgM kappa chain paraprotein was found at a level of 4 grams per deciliter. Treatment with chlorambucil resulted in a complete resolution of the condition within a month. Since no tissue specimens are available, it is impossible to determine what histopathological features may have contributed to the clinical symptoms. Neurological symptoms, including various palsies of cranial nerves, are not rare among patients with macroglobulinemia. However, this case is remarkable in that common symptoms, such as hepatomegaly and splenomegaly, were not present. (Consumer Summary produced by Reliance Medical Information, Inc.)