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Case 365

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Male patient, 42 years old, infected with HIV for 15 years, on regular antirretroviral therapy (ART), was referred to Tertiary Care to investigate bradypsychia, mental confusion, walking difficulty, overall weakness (especially on the right), nausea, vomiting, diarrhea and low right visual acuity, with one month of evolution. Presented an episode of seizure without fever. Lumbar puncture was performed (which revealed atypical B-cell infiltration) and brain magnetic resonance imaging (MRI) was requested.

Considering the common disorders of the central nervous system (CNS) in patients with AIDS (Acquired Human Immunodeficiency Syndrome), what is the most likely diagnosis?

a) Neurotoxoplasmosis

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b) Primary CNS lymphoma

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c) Progressive multifocal leukoencephalopathy

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d) Neurocryptococcosis

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Image Analysis

Image 1: Brain MRI, axial view, FLAIR-weighted. Heterogeneous intracranial expansive lesion located in the left temporo-occipital region (outlined in red), infiltrating the trunk/splenium of the corpus callosum, associated with intense perilesional edema (outlined in blue) and mass effect, causing shift of the midline structures.

 

Image 2: Brain MRI, axial view, diffusion (DWI) - (A) and ADC Map - (B). Diffusion restriction is observed. This restriction generates the observed hypersignal to diffusion (outlines in purple) and the hyposignal in the ADC Map (highlighted in green).

 

Image 3: Brain MRI, sagittal view, T1-weighted, after the intravenous gadolinium-based contrast injection, revealing intense uptake of contrast in the lesion (outlined in orange).

Highlights

- CNS is the most common site of primary lymphoma in HIV-infected patients;

- They present high aggressiveness, high histological grade and strong association with Epstein-Barr virus;

- Neurological symptoms are progressive and ophthalmic symptoms should be investigated;

- The most appropriate imaging exam for investigation is brain MRI; if contraindicated and/or inaccessible, CT may contribute to the diagnosis;

- High cellularity results in the findings of hyperdensity (CT), hyposignal/isosignal on T2, intense post-contrast enhancement and diffusion restriction (MRI).

References

- Moreira, Raoni. Infecções Oportunistas do Sistema Nervoso Central em HIV/AIDS na Emergência. Revista QualidadeHC. 2017 Jul. [Available from: http://www.hcrp.usp.br/revistaqualidade/uploads/Artigos/214/214.pdf];

- Korfel A, Schlegel U. Diagnosis and treatment of primary CNS lymphoma. Nat Rev Neurol. 2013 Jun;

- Ferreri AJ, Marturano E. Primary CNS lymphoma.Best Pract Res Clin Haematol. 2012 Mar;

- Scott BJ, Douglas VC, Tihan T, Rubenstein JL, Josephson SA. A systematic approach to the diagnosis of suspected central nervous system lymphoma. JAMA Neurol. 2013 Mar;

- Reis F, Schwingel R, Nascimento FBP. Central nervous system lymphoma: iconographic essay. Radiol Bras. 2013.

Author

Lara Hemerly De Mori, 4th year medical student at Universidade Federal de Minas Gerais.

E-mail: larahemerly[at]gmail.com

Supervisor

Cristiana Buzelin Nunes, Adjunct Professor at the Department of Anatomical Pathology at Medical School of Universidade Federal de Minas Gerais.

E-mail: cristianabnunes[at]gmail.com

Carlos Magno da Silva, neurosurgeon, neuroradiologist of Hospital das Clínicas - UFMG.

E-mail: carlosbresil[at]hotmail.com

Reviewers

Jhonatas Pereira Santos, Leandra Prates Diniz, Felipe Eduardo Fagundes Lopes, Mateus Nardelli; Professor José Nelson Mendes Vieira and Professor Viviane Santuari Parisotto Marino.

Translated by

Lara Hemerly De Mori, 4th year medical student at Universidade Federal de Minas Gerais.

E-mail: larahemerly[at]gmail.com

Test question

(State Health Department - Espírito Santo - Brazil) Regarding primary central nervous system lymphoma, related to HIV infection, check the correct option:

a) It is diagnosed mainly by neuroimaging exams, with findings of diffuse brain lesions with little mass effect. The main differential diagnosis is progressive multifocal leukoencephalopathy.

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b) If possible, complete surgical resection of all identified lesions is desirable as standard treatment to avoid chemotherapy and radiotherapy.

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c) It is commonly one of the first AIDS-related complications to present in the patient because it requires a lower degree of immunosuppression than other HIV-associated diseases.

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d) The onset of this complication is strongly associated with Epstein-Barr virus infection. Epstein-Barr DNA sequences can be found in the CSF of most affected individuals.

25%

e) The most common clinical condition is progressive loss of muscle strength, which has been going on for more than twelve months, with signs of intracranial hypertension. Cognition is usually preserved.

25%
   

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