Primary Central Nervous System Lymphoma (PCNSL) is a rare type of non-Hodgkin’s lymphoma that tends to occur in the elderly and immunocompromised patients. The mainstay of treatment is that of high-dose methotrexate-based chemotherapy. Studies suggest that radiological response to high-dose methotrexate-based chemotherapy correlates with an improvement in neurocognitive ability that remains stable on follow-up. However, no studies involving patients with extremely poor neurological status prior to the commencement of chemotherapy have been reported, and the neurological prognosis of this group of patients remains unknown.
We describe 3 patients with biopsy-proven PCNSL that had comatose neurological states (Glasgow Coma Scale, GCS of 3-5) as a result of disease progression prior to treatment. The patients all experienced a drop in GCS in the short time frame between initial presentation and the start of treatment ranging from 12 days to 6 months.
Case 1 initially presented with lethargy, drowsiness and behavioural change, with disease foci in a periventricular distribution. Case 2 presented with diplopia and poor visual acuity, as a result of disease affecting the optic chiasma and brainstem. Finally, case 3 had unsteady gait, memory impairment and slow speech at diagnosis, with disease over periventricular regions involving the corpus callosum and corona radiata. All were treated with high-dose methotrexate-based chemotherapy. However, although excellent radiological responses to treatment were achieved, no meaningful neurological or cognitive recovery was documented.
PCNSL patients with a baseline comatose state have poor neurological prognosis even if there is excellent tumour response to chemotherapy. As it is an aggressive disease with an unpredictable clinical course, rapid detection and prompt treatment is crucial in this disease entity.
National Cancer Centre Singapore.
Has not received any funding.
All authors have declared no conflicts of interest.