A 22-year-old man presented with new-onset, gradually progressive diffuse headache for 2 months. He exhibited no apparent physical or neurologic abnormalities except for limited bilateral lateral rectus movement and bilateral papilledema on fundoscopic examination. An MRI examination of the brain showed multiple nodular-enhancing lesions along both sides of the falx cerebri with perilesional edema. In addition, these nodular structures formed a branching network of thickened dura mater to adjacent nodular structures (Figure 1). Due to these atypical features, the differential diagnosis included tuberculosis, sarcoidosis, immunoglobulin G4–related disorders, lymphoproliferative disorders, meningioma, hemangiopericytoma, and Erdheuim-Chester disease.1 A stereotactic biopsy was performed. The pathologic and immunohistochemistry results and systemic evaluation were compatible with primary CNS anaplastic large cell lymphoma with ALK+.2 After a total of 6 courses of high-dose methotrexate and ifosfamide followed by whole-brain radiation, the patient achieved complete remission and resolution of headache (Figure 2).
T1-weighted imaging with gadolinium revealed multiple bilateral nodular-enhancing lesions along the falx cerebri, primarily located in the frontal lobe, and thickening of the dura mater (A: sagittal view, B: coronal view, and C: axial view). The red arrows demonstrated a branching network of thickened dura mater to adjacent enhancing nodular structures (A). Axial T2–fluid-attenuated inversion recovery image showed hypersignal intensity in the bilateral frontal lobes, suggesting the presence of perilesional edema (D).
T1-weighted imaging with gadolinium revealed the nearly complete resolution of thickening dura mater, nodular-enhancing lesions, and a branching network (A: sagittal view, B: coronal view, and C: axial view). The white arrow demonstrated the presence of poststereotactic brain biopsy and mild thickening of adjacent dura mater, primarily resulted from the postoperative change (B). Axial T2–fluid-attenuated inversion recovery image showed the resolution of hypersignal intensity in the bilateral frontal lobes (D).
Author Contributions
W. Rattanawong: drafting/revision of the article for content, including medical writing for content; major role in the acquisition of data; study concept or design; and analysis or interpretation of data. A. Tekarnjnavanit: drafting/revision of the article for content, including medical writing for content; major role in the acquisition of data; study concept or design; and analysis or interpretation of data. C. Marukatat: drafting/revision of the article for content, including medical writing for content; major role in the acquisition of data; study concept or design; and analysis or interpretation of data. P. Hemachudha: drafting/revision of the article for content, including medical writing for content; major role in the acquisition of data; study concept or design; and analysis or interpretation of data. T. Pongpitakmetha: drafting/revision of the article for content, including medical writing for content; major role in the acquisition of data; study concept or design; and analysis or interpretation of data. P. Anukoolwittaya: drafting/revision of the article for content, including medical writing for content; major role in the acquisition of data; study concept or design; and analysis or interpretation of data.
Study Funding
No targeted funding reported.
Disclosure
The authors report no relevant disclosures. Go to Neurology.org/N for full disclosures.
Acknowledgment
The authors thank the Hematology and Radiation Oncology team, King Chulalongkorn Memorial Hospital, for the support and care of the patient.
Footnotes
Go to Neurology.org/N for full disclosures. Funding information and disclosures deemed relevant by the authors, if any, are provided at the end of the article.
Submitted and externally peer reviewed. The handling editor was Resident & Fellow Section Editor Whitley Aamodt, MD, MPH, MSCE.
All authors contributed equally to this work.
- Received April 19, 2023.
- Accepted in final form July 7, 2023.
- © 2023 American Academy of Neurology
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