Isolated primary neurolymphomatosis with cranial multineuritis: a case presentation
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Isolated primary neurolymphomatosis with cranial multineuritis: a case presentation Sen Sheng 1
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Rohan Sharma 1 & Rohan Samant 2 & Junliang Yuan 3 & Krishna Nalleballe 1 & Nidhi Kapoor 1
Received: 21 June 2020 / Accepted: 6 October 2020 # Fondazione Società Italiana di Neurologia 2020
Abstract Background Isolated primary neurolymphomatosis (NL) of cranial multineuritis is a very rare condition that refers to the lymphomatous invasion of cranial nerves only. There are sparse cases of isolated cranial nerves NL reported worldwide. Case presentation We present magnetic resonance imaging (MRI) and cerebrospinal fluid (CSF) findings of a 63-year-old female patient suffering from isolated neurolymphomatosis of cranial multineuritis with a wide constellation of syndromes including binocular diplopia, left facial paralysis and pain, syncope episodes, and progressive dysphagia. A contrasted MRI brain showed multiple cranial nerves enhancement. Extensive workup for infectious, autoimmune, neoplastic, paraneoplastic, or inflammatory etiologies had been unrevealing except CSF cytology revealed large atypical monotypic B cells that were suspicious for non-Hodgkin lymphoma on the third large volume tap. The decision of biopsy was deferred after the risks and benefits discussion. Following the four cycles of empiric methotrexate-based induction chemotherapy, the patient’s symptoms resolved, and a complete radiographic response was achieved without whole-brain radiation or autologous hematopoietic cell transplantation. In the latest follow-up, she is independent with her daily activities and remains in clinical and radiographic remission more than 3 years since initial chemotherapy. Conclusion Isolated NL of cranial nerves can present diagnostic and management pitfalls for the neurologist, neurosurgeons, and oncologists. Since current diagnostic modalities have modest sensitivity and a pathological diagnosis is often difficult, empiric treatment once other possibilities are ruled out can carry a good prognosis. Keywords Neurolymphomatosis . Cranial neuritis . CNS lymphoma
Case presentation A 63-year-old woman presented with new-onset binocular diplopia, left facial paralysis and pain, syncope episodes, and progressive dysphagia for 3 months. She was diagnosed with Bell’s palsy 3 months ago before the current presentation manifesting as left facial weakness and severe facial pain. She * Sen Sheng [email protected] 1
Department of Neurology, The University of Arkansas for Medical Sciences, 4301 West Markham Street, Slot 500, Little Rock, AR 72205, USA
2
Department of Radiology, The University of Arkansas for Medical Sciences, Little Rock, USA
3
Department of Neurology, Peking University Sixth Hospital, National Clinical Research Center for Mental Disorders, Beijing, China
was treated with 60 mg prednisone once daily and 1000 mg valacyclovir three times a day for one week without significant improvement. She had no history of limb weakness, shoulder or hip pain, fever, weight loss, night sweat, recent vaccinatio
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