A case of progressive multifocal leukoencephalopathy in a patient with sarcoidosis

M. Pallin, C. O'sullivan, J. D. Dodd, K. McCreery, F. Brett, M. Farrell, D. O'brien, W. W. Hall, N. J. Tubridy, M. P. Keane
2011 QJM: Quarterly journal of medicine  
A 47-year-old caucasian man presented with a new visual field defect. He had been diagnosed with pulmonary sarcoidosis 15 years previously on the basis of mediastinal lymph node biopsy and chest imaging consistent with stage three disease. Other than an eruption of cutaneous sarcoidosis 5 years after initial diagnosis, his disease had been quiescent on maintenance medication of prednisolone 15 mg per day and hydroxychloroquine 200 mg per day. Several attempts to wean his steroids were
more » ... oids were unsuccessful. He had a documented longstanding T-cell lymphopenia, and at the time of presentation, his lymphocyte count was 488 Â 10 6 /l (T-cells 394 Â 10 6 /l, CD4 cells 131 Â 10 6 /l). The patient was a hotel manager, had no other significant medical conditions and was a life-long non-smoker with moderate alcohol consumption. On routine annual ophthalmologic follow-up, perimetry demonstrated a new partial left superior quadrantanopia. On further questioning, the patient admitted to recent visual field problems, particularly during playing golf when he had a tendency to 'lose' the ball out to the left. Pre-and post-contrast magnetic resonance imaging (MRI) of brain at this time demonstrated numerous high signal foci throughout both cerebral hemispheres including a lesion of the right geniculate-striate pathway as well as the right midbrain and upper pons. There was one isolated focus of enhancement in the left cerebellar peduncle. There was no meningeal thickening or enhancement (Figure 1 ). Analysis of cerebrospinal fluid including oligoclonal bands was within normal limits. Visual evoked responses were also normal. The consensus opinion from ophthalmology, pulmonology, neurology and radiology specialities was that of neurosarcoidosis, and immunosuppression was escalated with an increase in prednisolone therapy to 60 mg per day for 6 weeks followed by a taper to 40 mg per day and the addition of azathioprine. Upon review 10 weeks after increasing immunosuppression, at which stage the patient was receiving prednisolone 40 mg per day and azathioprine 150 mg per day, he reported no improvement in his visual symptoms and complained of increased blurring of vision. Perimetry demonstrated progression of the visual field defect and clinical examination confirmed the development of a right intranuclear ophthalmoplegia and right conjugate lateral gaze paresis as well as a left seventh nerve palsy. Repeat MRI of brain demonstrated stability of the number and size of lesions however several foci within frontal lobes and left cerebellum now enhanced post-contrast (Figure 2) . Given the progressive nature of his condition despite advanced immunosuppression, consideration was given to alternative diagnoses. Serum samples were analysed for cryptococcal antigen, toxoplasma
doi:10.1093/qjmed/hcr154 pmid:21893584 fatcat:rwtl7i6p5zgjlfx6yzfbllna34