Q J Med 2003; 96: 453-454
© 2003 Association of Physicians
Correspondence |
Intracranial plasmacytoma causing acute unilateral loss of vision
Department of Internal Medicine
American University of Beirut-Medical Center
Beirut
Lebanon
e-mail (Dr Khalifeh): gm04{at}aub.edu.lb
Sir,
Some patients with multiple myeloma may present with neurological complaints,1 but intracranial plasmacytomas are rare. We describe a patient with a plasmacytoma compressing the optic nerve, causing unilateral loss of vision as the first presentation of multiple myeloma.
A previously healthy 62-year-old man consulted his ophthalmologist because of sudden onset of decreased vision in the right eye. He denied any other symptom. A provisional diagnosis of optic neuritis was made, and oral steroids (prednisone 1 mg/kg daily) were started, but without improvement. Three weeks later, his internist requested a magnetic resonance imaging (MRI) of the brain that revealed a mass at the apex of the right orbit compressing the optic nerve (Figure 1), and referred him to our centre.
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Physical examination showed multiple areas of bone tenderness, a pale right optic disc, minimal light perception in the nasal field, and counting fingers at 30 cm in the temporal field of the right eye. Investigations showed mild anaemia, with a haemoglobin of 111 g/l. Serum chemistry revealed a calcium level of 2.925 mmol/l, total protein of 111 g/l with 37 g/l albumin and 74 g/l globulin. Serum protein electrophoresis and immunofixation showed a monoclonal gammopathy in the gamma region (47.38 g/l) of the IgA-Kappa type. A bone scan and a CT scan of orbits and base of skull showed multiple bony lesions in the skull and vertebral column. The diagnosis of multiple myeloma was confirmed by bone-marrow aspirate showing 20% infiltration of plasma cells. The mass compressing the optic nerve was considered to be a plasmacytoma, in view of the above. The patient was started on vincristine, adriamycin, dexamethasone, and radiation therapy to the orbit. He then underwent successful peripheral stem-cell transplantation. He is currently doing well with improvement of vision in the right eye (counting fingers at 1 m in the nasal field, and at 2 m in the temporal field).
Involvement of cranial nerve foramina by myelomatous processes affecting the base of skull, or direct nerve compression by tumour masses are the two possible mechanisms explaining cranial nerve signs in multiple myeloma patients.1 An intracranial plasmacytoma may underlie the presentation in systemic myeloma,2–4 as in our case. However, optic nerve compression from a plasmacytoma has been reported in only four cases in the literature, to our knowledge.5 Two of these cases were initially diagnosed as retrobulbar neuritis and treated for few months with steroids until the correct diagnosis was made by craniotomy, lacking advanced imaging. A third case presenting with headache and visual disturbances was also treated with steroids for four and a half years as a case of cranial arteritis. Maini and Macewen in 1997 reported a fourth case, with its MRI appearance. Similarly, our patient sought ophthalmological advice initially and was treated as a case of retrobulbar neuritis. The characteristic presentation of our patient is that his only complaint was sudden unilateral deterioration in vision despite diffuse myelomatous disease.
References
1. Pollard JD, Young GAR. Neurology and the bone marrow. J Neurol Neurosurg Psychiatry 1997; 63:706–18.
2. Stark RJ, Henson RA. Cerebral compression by myeloma. J Neurol Neurosurg Psychiatry 1981; 44:833–6.
3. Mäntylä R, Kinnunen J, Bohling T. Intracranial plasmacytoma: a case report. Neuroradiol 1996; 38:646–9.[Medline]
4. Jordan DR, Drouin J, Berry G, Watson G. Intracranial plasmacytoma associated with multiple myeloma. Can J Ophthalmol 1984; 19:275–8.[Medline]
5. Maini R, Macewen CJ. Intracranial plasmacytoma presenting with optic nerve compression. Br J Ophthalmol 1997; 81: 417–18.[Medline]
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