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Neurolymphomatosis of the Brachial Plexus and its Branches: Case Series and Literature Review

  • Pierre R. Bourque (a1) (a2), Jodi Warman Chardon (a1) (a2) (a3), Mark Bryanton (a4), Melissa Toupin (a5), Bruce F. Burns (a6) and Carlos Torres (a2) (a7)...

Abstract

Background: Neurolymphomatosis is a process of neoplastic endoneurial invasion, most strongly associated with non-Hodgkin’s lymphoma. It must be distinguished from paraneoplastic, metabolic, nutritional and treatment-related causes of neuropathy that are common in this patient population. Methods: This brief case series illustrates the protean manifestations of neurolymphomatosis of the brachial plexus, ranging from focal distal mononeuropathy to multifocal brachial plexopathy, either as the index manifestation of lymphoma or as a complication of relapsing disease. Results: Prominent asymmetry, pain and nodular involvement on neuroimaging may help distinguish neurolymphomatosis from paraneoplastic immune demyelinating radiculoneuropathy. MR neurography criteria for the diagnosis of neurolymphomatosis include hyperintensity on T2 and STIR sequences, focal and diffuse nerve enlargement with fascicular disorganization and gadolinium enhancement. No specific anatomical distribution within the brachial plexus has, however, been found to be characteristic. Fluorodeoxyglucose-positron emission tomography (FDG-PET) imaging is the imaging modality with the highest sensitivity for detection of nodal or extranodal spread in lymphoma. Conclusions: Brachial plexus neuropathy in neurolymphomatosis is highly protean in its distribution, semiology and relation to lymphoma staging. Dedicated MRI and PET-CT imaging are leading diagnostic modalities.

Neurolymphomatose du plexus brachial et de ses branches : étude de série de cas et recension des écrits. Contexte: La neurolymphomatose constitue un processus d’invasion endoneurale néoplasique le plus souvent associé à des lymphomes non hodgkiniens. Il doit ainsi être distingué de l’ensemble des causes de la neuropathie : paranéoplasiques, métaboliques, nutritionnelles ainsi que celles liées à l’administration d’un traitement. À noter que ces causes sont répandues au sein du groupe de patients à l’étude. Méthodes: Cette étude succincte d’une série de cas entend se pencher sur les manifestations protéiformes de la neurolymphomatose du plexus brachial, lesquelles, que ce soit comme indices de la présence de lymphomes ou résultant des complications d’une maladie récurrente, vont de la mono-neuropathie focale distale à la plexopathie brachiale multifocale. Résultats: Il est possible qu’une asymétrie importante, de la douleur et la présence de nodules détectés par neuro-imagerie contribuent à établir une distinction entre la neurolymphomatose et la radiculoneuropathie démyélinisante paranéoplasique d’origine immunitaire. Les critères diagnostiques de la neurolymphomatose au moyen de la neurographie par résonance magnétique (NRM) incluent notamment l'hyper-intensité en séquences STIR (pondération en T2) ainsi que l’augmentation focale et diffuse du volume des nerfs associée à une désorganisation fasciculaire et à l’utilisation du gadolinium afin d’améliorer le contraste des images. Cela dit, précisons qu’aucune distribution anatomique spécifique à l’intérieur du plexus brachial n’est apparue caractéristique. La tomographie par émission de positrons (18FDG) est la modalité d’imagerie dont la sensibilité de contraste, la plus élevée, permet de détecter une propagation nodulaire ou extra-nodulaire dans le cas de lymphomes. Conclusions: Dans le cas de la neurolymphomatose, on a observé que la neuropathie du plexus brachial était grandement protéiforme tant en ce qui concerne sa distribution, sa sémiologie qu’en relation avec la stadification des lymphomes. Tant des examens spécifiques d’IRM que la tomographie par émission de positrons demeurent des techniques diagnostiques de premier plan.

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Copyright

Corresponding author

Correspondence to: Dr. J. Warman Chardon, The Ottawa Hospital, 1053 Carling Avenue, Ottawa, ON K1H8L6, Canada. Email: jwarman@toh.ca

References

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Neurolymphomatosis of the Brachial Plexus and its Branches: Case Series and Literature Review

  • Pierre R. Bourque (a1) (a2), Jodi Warman Chardon (a1) (a2) (a3), Mark Bryanton (a4), Melissa Toupin (a5), Bruce F. Burns (a6) and Carlos Torres (a2) (a7)...

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