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Isolated primary neurolymphomatosis with cranial multineuritis: a case presentation
Sen Sheng1, Rohan Sharma2, Rohan Samant3
1Department of Neurology, The University of Arkansas for Medical Sciences, 4301 West Markham Street, Slot 500, Little Rock, AR, 72205, USA. SSheng@uams.edu.
Summary
Isolated neurolymphomatosis (NL) of cranial nerves is rare. Empiric chemotherapy can lead to good prognosis and remission in patients with cranial nerve lymphoma when other causes are excluded.
Area of Science:
- Neurology
- Oncology
- Radiology
Background:
- Isolated primary neurolymphomatosis (NL) of cranial multineuritis is a rare condition involving lymphomatous invasion of cranial nerves.
- Sparse cases of isolated cranial nerves NL are reported globally, presenting diagnostic challenges.
Observation:
- A 63-year-old female presented with binocular diplopia, facial paralysis, syncope, and dysphagia.
- Cranial magnetic resonance imaging (MRI) revealed multiple cranial nerve enhancements.
- Cerebrospinal fluid (CSF) cytology identified atypical monotypic B cells, suspicious for non-Hodgkin lymphoma.
Findings:
- Empiric methotrexate-based chemotherapy led to symptom resolution and complete radiographic response.
- The patient achieved clinical and radiographic remission for over 3 years without radiation or stem cell transplant.
- Diagnostic modalities for NL have modest sensitivity, often necessitating empiric treatment.
Implications:
- Isolated NL of cranial nerves poses diagnostic and management difficulties for clinicians.
- Empiric treatment, after ruling out other etiologies, can yield a favorable prognosis for cranial nerve lymphoma.
- This case highlights the efficacy of chemotherapy in managing rare neurolymphomatosis presentations.
