We report the case of a 55-year-old immunocompetent female with primary central nervous system
lymphoma (PCNSL). Seven years previously, the patient presented with left facial
dysesthesia, and a right thalamus lesion was revealed by brain MRI. Both her
dysesthesia and the lesion disappeared spontaneously in six months. One year previously, she noted motor disturbance of the right limbs, and brain MRI revealed lesions in the bilateral basal ganglia and the left internal capsule which gradually increased in size. She was admitted to our hospital owing to the brain MRI findings of a white matter lesion in the left occipital lobe and bilateral
optic neuritis. Previously, she had experienced two episodes of
steroid-responsive
uveitis in her left eye. An inflammatory disease such as
multiple sclerosis was initially suspected because of the relapsing/remitting
clinical course with a long time interval. Treatment with
corticosteroids improved her clinical symptoms and decreased the size of the lesions, but the brain lesions and bilateral
optic neuritis recurred within one month. At that time, the β2 microglobulin level in the cerebrospinal fluid was high and the left occipital lobe lesions showed increased 18F-fluoro-deoxyglucose uptake in positron emission tomography and decreased Cho/NAA ratio in 1H-MR spectroscopy. These findings suggested PCNSL. A brain biopsy confirmed the presence of
diffuse large B cell lymphoma. Both
uveitis and
optic neuritis were considered to be caused by
intraocular lymphoma associated with PCNSL. Although patients with PCNSL may experience temporary spontaneous remission, our present case suggests that the time interval from remission to relapse can be much longer than generally expected. We suggest that it is necessary to consider PCNSL and perform a brain biopsy on patients presenting with atypical clinical manifestations of an inflammatory disease, even in cases with a long
clinical course.