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LETTERS TO THE EDITOR
Year : 2022 | Volume
: 25
| Issue : 6 | Page : 1206-1208
Primary CNS lymphoma masquerading as ventriculitis
Shalesh Rohatgi1, Prajwal Rao1, Satish Nirhale1, Pravin Naphade1, Yogeshwari Deshmukh2
1 Department of Neurology, Dr. D Y Patil Medical College, Hospital and Research Center, Dr. DY Patil Vidyapeeth Pune, Maharashtra, India
2 Department of Radiology, Star Imaging and Research Center, Pune, Maharashtra, India
Correspondence Address:
Shalesh Rohatgi
Dr. D Y Patil Medical College, Hospital and Research Center, Pimpri, Pune - 411 060, Maharashtra
India
Source of Support: None, Conflict of Interest: None
CheckDOI: 10.4103/aian.aian_625_22
How to cite this article:Sir,
We report a case of a 74-year-old female patient who presented with complaints of irrelevant talk, excessive sleepiness, and delayed verbal response for 25 days. No h/o fever, seizures, headache, or vomiting. No history of any rash. The patient was admitted to a hospital and was found to have hyponatremia, which was corrected. However, there was no improvement in her condition and subsequently, the patient was transferred to our hospital. Clinical examination revealed that she was afebrile and had no lymphadenopathy or organomegaly. She was drowsy and disoriented. No signs of meningeal irritation were present and there was no other neurological deficit. Routine lab investigations including hemogram, liver function tests, renal function tests, electrolytes, and thyroid function tests were normal. The erythrocyte sedimentation rate was 65 mm fall.
MRI brain [Figure 1] revealed T2 and Fluid-attenuated inversion recovery (FLAIR) hyperintensity involving the wall of the ventricular system that showed diffusion restriction and post-contrast enhancement, suggestive of ventriculitis. MRI of the spine was unremarkable. Her CSF examination revealed a pressure of 18 Cms, proteins 235 mg%, glucose 7 mg%, (blood sugar 126 mg%), and200 cells; predominantly lymphocytes. There were no malignant cells seen. CSF Gram's stain and Acid Fast Bacilli (AFB) stain were negative. Cryptococcal antigen was negative. The bacterial culture showed no growth.
Figure 1: MRI Brain Images. (a and b) T2 FLAIR images showing hyperintensities along the ventricles and septum (black arrows); (a and d) Contrast enhanced T1 images showing enhancement along the ventricular walls (black arrows); (e and f) DWI images showing diffusion restriction along the ventricular walls (black arrows)TB PCR was negative. HIV was negative. EBV, VZV, HSV1, HSV2, CMV PCR was negative. Initial magnetic resonance imaging (MRI) spine was normal. In view of the CSF picture, the possibility of malignancy was considered, and repeat CSF was sent for flow cytometry that showed 50% of large atypical lymphoid cells which were myeloperoxidase positive. Cells were characterized by the expression of CD19, eCD79a, CD10, CD20, CD22, and HLA-DR and negative for cCD3, sCD3, CD8, CD34, CD13, CD117, CD33, Kappa, and Lambda. The findings were suggestive of B cell non-Hodgkin's lymphoma. PET-CT was done that showed hypermetabolic nodular thickening in the interventricular septum and along the ventricles. There was no increased uptake in any other organ, suggesting it to be primary CNS lymphoma. The patient was treated with high-dose methotrexate with leucovorin and rituximab. She was discharged after the first pulse. She reported 2 weeks later with complains of burning pain in both lower limbs along with weakness. Contrast-enhanced, fat-saturated T1W image of the lumbar spine showed diffuse leptomeningeal enhancement surrounding lower dorsal cord and conus, suggestive of leptomeningeal dissemination of lymphoma [Figure 2]. The patient refused any further treatment and was discharged.
Figure 2: Contrast enhanced fat saturated T!W midsagittal image of lumbar spine shows diffuse leptomeningeal enhancement surrounding lower dorsal cordPrimary CNS lymphoma (PCNL) is a rare form of extra-nodal highly invasive non-Hodgkin's lymphoma. Ninety percent arise from B cells and 10% arise from T cells. It can occur in both immune-competent and immune-compromised patients. In immune-competent patients, it accounts for almost 4% of all intracranial neoplasms and 4-6% of extra-nodal lymphomas.[1] Radiological imaging usually reveals a solitary homogenously enhancing mass in the supratentorial region. PCNL involving the ventricular system is very rare and accounts only for 1% of all primary CNS tumors. Only a few cases of involvement of the ventricles have been reported in the literature and that too all presented as mass in the ventricles.[2],[3],[4],[5]
Isolated case reports of involvement of only lateral ventricles,[6] only fourth ventricle[3],[7],[8] and only third ventricle[3],[9],[10] are available but to the best of our knowledge; this is the first case of primary CNS lymphoma presenting as ventriculitis on imaging involving the entire ventricular system.
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