Introduction
Lymphomatosis cerebri is a rare form of primary central nervous system lymphoma characterized by an atypical clinical presentation and neuroimaging, with a poor short-term prognosis.

Case Report
A 51-year-old woman began with clinical manifestations characterized by rapidly progressing cognitive impairment associated with a behavioral disorder, myoclonus, and gait disturbance. The brain magnetic resonance image showed extensive signaling in the bilateral periventricular white matter. The 18F-FDG PET-CT showed severe dorsolateral neocortical hypometabolism in the absence of focal hypermetabolic lesion, a metabolic pattern indicative of cerebri lymphomatosis. A brain biopsy confirmed the diagnosis. The patient started chemotherapy achieving complete remission. Eighteen months after diagnosis, the patient had improved clinically and neuroimaging.

Conclusion
This is the first report in Peru of an entity that should be considered in rapidly progressive dementia and leukoencephalopathy cases. Timely diagnosis and appropriate chemotherapy management can increase patient survival.

Authors: Miguel A. Vences[1], Adriana F. Saravia-Ayala[1], Elliot Barreto-Acevedo[1]

Affiliation:
[1] Hospital Nacional Edgardo Rebagliati Martins, Essalud, Lima, Perú

E-mail: vens1793@hotmail.com

Author address:
[1] Departamento de Neurología, Hospital Nacional Edgardo Rebagliati Martins
Av. Rebagliati 490
Jesús María
Lima, Perú

Citation: Vences MA, Saravia-Ayala AF, Barreto-Acevedo E. Rapidly progressive dementia associated with leukoencephalopathy: A case report of lymphomatosis cerebri. Medwave 2021;21(02):e8138 doi: 10.5867/medwave.2021.02.8138

Submission date: 7/10/2020

Acceptance date: 3/3/2021

Publication date: 26/3/2021

Origin: Not commissioned.

Type of review: Externally peer-reviewed by four reviewers, double-blind.

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1. Bakshi R, Mazziotta JC, Mischel PS, Jahan R, Seligson DB, Vinters HV. Lymphomatosis cerebri presenting as a rapidly progressive dementia: clinical, neuroimaging and pathologic findings. Dement Geriatr Cogn Disord. 1999 Mar- Apr;10(2):152-7. | CrossRef | PubMed |
2. Li L, Rong JH, Feng J. Neuroradiological features of lymphomatosis cerebri: A systematic review of the English literature with a new case report. Oncol Lett. 2018 Aug;16(2):1463-1474. | CrossRef | PubMed |
3. Lee PJ, Berrios I, Ionete C, Smith T. Lymphomatosis cerebri: diagnostic challenges and review of the literature. BMJ Case Rep. 2016 Nov 24;2016:bcr2016216591. | CrossRef | PubMed |
4. Hatanpaa KJ, Fuda F, Koduru P, Young K, Lega B, Chen W. Lymphomatosis Cerebri: A Diagnostic Challenge. JAMA Neurol. 2015 Sep;72(9):1066-7. | CrossRef | PubMed |
5. Gagnier JJ, Kienle G, Altman DG, Moher D, Sox H, Riley D; CARE Group*. The CARE Guidelines: Consensus-based Clinical Case Reporting Guideline Development. Glob Adv Health Med. 2013 Sep;2(5):38-43. | CrossRef | PubMed |
6. Bühring U, Herrlinger U, Krings T, Thiex R, Weller M, Küker W. MRI features of primary central nervous system lymphomas at presentation. Neurology. 2001 Aug 14;57(3):393-6. | CrossRef | PubMed |
7. Küker W, Nägele T, Korfel A, Heckl S, Thiel E, Bamberg M, et al. Primary central nervous system lymphomas (PCNSL): MRI features at presentation in 100 patients. J Neurooncol. 2005 Apr;72(2):169-77. | CrossRef | PubMed |
8. Fisher D, Mantell BS, Urich H. The clinical diagnosis and treatment of microgliomatosis: report of a case. J Neurol Neurosurg Psychiatry. 1969 Oct;32(5):474-8. | CrossRef | PubMed |
9. Izquierdo C, Velasco R, Vidal N, Sánchez JJ, Argyriou AA, Besora S, et al. Lymphomatosis cerebri: a rare form of primary central nervous system lymphoma. Analysis of 7 cases and systematic review of the literature. Neuro Oncol. 2016 May;18(5):707-15. | CrossRef | PubMed |
10. Kerbauy MN, Pasqualin DDC, Smid J, Iquizli R, Kerbauy LN, Nitrini R, et al. Diffuse large B-cell lymphoma of the central nervous system presenting as "lymphomatosis cerebri" and dementia in elderly man: Case report and review of the literature. Medicine (Baltimore). 2019 Feb;98(6):e14367. | CrossRef | PubMed |
11. Jiménez PP, Jiménez LO, García FP. Cerebral intravascular lymphomatosis as cause of subacute dementia: Report of a case. Rev. chil. neuro-psiquiatr. [Internet]. 2013;51(1):32-37. | CrossRef |
12. Santos PSF, Vinholi A, da Silva TR, Koike M, Sato HK. Linfomatose Cerebral: Um Raro Diagnóstico para Demência Rapidamente Progressiva. Relato de caso e Revisão de Literatura. jbnc. 31º de março de 2018;26(4):315 -319. [Internet] | Link |
13. Yu H, Gao B, Liu J, Yu YC, Shiroishi MS, Huang MM, et al. Lymphomatosis cerebri: a rare variant of primary central nervous system lymphoma and MR imaging features. Cancer Imaging. 2017 Oct 5;17(1):26. | CrossRef | PubMed |
14. Jiménez de la Peña MD, Vicente LG, Alonso RC, Cabero SF, Suárez AM, de Vega VM. The Multiple Faces of Nervous System Lymphoma. Atypical Magnetic Resonance Imaging Features and Contribution of the Advanced Imaging. Curr Probl Diagn Radiol. 2017 Mar-Apr;46(2):136-145. | CrossRef | PubMed |
15. Jain TK, Sharma P, Suman SK, Faizi NA, Bal C, Kumar R. Primary central nervous system lymphoma with lymphomatosis cerebri in an immunocompetent child: MRI and 18F-FDG PET-CT findings. Rev Esp Med Nucl Imagen Mol. 2013 Nov- Dec;32(6):394-6. | CrossRef | PubMed |
16. Kawai N, Okubo S, Miyake K, Maeda Y, Yamamoto Y, Nishiyama Y, et al. Use of PET in the diagnosis of primary CNS lymphoma in patients with atypical MR findings. Ann Nucl Med. 2010 Jun;24(5):335-43. | CrossRef | PubMed |
17. Gomez-Figueroa E, Peiro-Osuna RP, Reyes-Moreno I, Hernandez-Hernandez A, Gutierrez-Aceves A, Santos-Zambrano J, et al. Linfoma primario del sistema nervioso central: experiencia clinica en un centro neurologico [Primary central nervous system lymphoma: clinical experience in a neurological center]. Rev Neurol. 2019 Jan 16;68(2):59-65. | PubMed |

Bakshi R, Mazziotta JC, Mischel PS, Jahan R, Seligson DB, Vinters HV. Lymphomatosis cerebri presenting as a rapidly progressive dementia: clinical, neuroimaging and pathologic findings. Dement Geriatr Cogn Disord. 1999 Mar- Apr;10(2):152-7. | CrossRef | PubMed |

Li L, Rong JH, Feng J. Neuroradiological features of lymphomatosis cerebri: A systematic review of the English literature with a new case report. Oncol Lett. 2018 Aug;16(2):1463-1474. | CrossRef | PubMed |

Lee PJ, Berrios I, Ionete C, Smith T. Lymphomatosis cerebri: diagnostic challenges and review of the literature. BMJ Case Rep. 2016 Nov 24;2016:bcr2016216591. | CrossRef | PubMed |

Hatanpaa KJ, Fuda F, Koduru P, Young K, Lega B, Chen W. Lymphomatosis Cerebri: A Diagnostic Challenge. JAMA Neurol. 2015 Sep;72(9):1066-7. | CrossRef | PubMed |

Gagnier JJ, Kienle G, Altman DG, Moher D, Sox H, Riley D; CARE Group*. The CARE Guidelines: Consensus-based Clinical Case Reporting Guideline Development. Glob Adv Health Med. 2013 Sep;2(5):38-43. | CrossRef | PubMed |

Bühring U, Herrlinger U, Krings T, Thiex R, Weller M, Küker W. MRI features of primary central nervous system lymphomas at presentation. Neurology. 2001 Aug 14;57(3):393-6. | CrossRef | PubMed |

Küker W, Nägele T, Korfel A, Heckl S, Thiel E, Bamberg M, et al. Primary central nervous system lymphomas (PCNSL): MRI features at presentation in 100 patients. J Neurooncol. 2005 Apr;72(2):169-77. | CrossRef | PubMed |

Fisher D, Mantell BS, Urich H. The clinical diagnosis and treatment of microgliomatosis: report of a case. J Neurol Neurosurg Psychiatry. 1969 Oct;32(5):474-8. | CrossRef | PubMed |

Izquierdo C, Velasco R, Vidal N, Sánchez JJ, Argyriou AA, Besora S, et al. Lymphomatosis cerebri: a rare form of primary central nervous system lymphoma. Analysis of 7 cases and systematic review of the literature. Neuro Oncol. 2016 May;18(5):707-15. | CrossRef | PubMed |

Kerbauy MN, Pasqualin DDC, Smid J, Iquizli R, Kerbauy LN, Nitrini R, et al. Diffuse large B-cell lymphoma of the central nervous system presenting as "lymphomatosis cerebri" and dementia in elderly man: Case report and review of the literature. Medicine (Baltimore). 2019 Feb;98(6):e14367. | CrossRef | PubMed |

Jiménez PP, Jiménez LO, García FP. Cerebral intravascular lymphomatosis as cause of subacute dementia: Report of a case. Rev. chil. neuro-psiquiatr. [Internet]. 2013;51(1):32-37. | CrossRef |

Santos PSF, Vinholi A, da Silva TR, Koike M, Sato HK. Linfomatose Cerebral: Um Raro Diagnóstico para Demência Rapidamente Progressiva. Relato de caso e Revisão de Literatura. jbnc. 31º de março de 2018;26(4):315 -319. [Internet] | Link |

Yu H, Gao B, Liu J, Yu YC, Shiroishi MS, Huang MM, et al. Lymphomatosis cerebri: a rare variant of primary central nervous system lymphoma and MR imaging features. Cancer Imaging. 2017 Oct 5;17(1):26. | CrossRef | PubMed |

Jiménez de la Peña MD, Vicente LG, Alonso RC, Cabero SF, Suárez AM, de Vega VM. The Multiple Faces of Nervous System Lymphoma. Atypical Magnetic Resonance Imaging Features and Contribution of the Advanced Imaging. Curr Probl Diagn Radiol. 2017 Mar-Apr;46(2):136-145. | CrossRef | PubMed |

Jain TK, Sharma P, Suman SK, Faizi NA, Bal C, Kumar R. Primary central nervous system lymphoma with lymphomatosis cerebri in an immunocompetent child: MRI and 18F-FDG PET-CT findings. Rev Esp Med Nucl Imagen Mol. 2013 Nov- Dec;32(6):394-6. | CrossRef | PubMed |

Kawai N, Okubo S, Miyake K, Maeda Y, Yamamoto Y, Nishiyama Y, et al. Use of PET in the diagnosis of primary CNS lymphoma in patients with atypical MR findings. Ann Nucl Med. 2010 Jun;24(5):335-43. | CrossRef | PubMed |

Gomez-Figueroa E, Peiro-Osuna RP, Reyes-Moreno I, Hernandez-Hernandez A, Gutierrez-Aceves A, Santos-Zambrano J, et al. Linfoma primario del sistema nervioso central: experiencia clinica en un centro neurologico [Primary central nervous system lymphoma: clinical experience in a neurological center]. Rev Neurol. 2019 Jan 16;68(2):59-65. | PubMed |