B Lymphoblastic Leukemia/Lymphoma Presenting As Fifth And Seventh Cranial Nerves Palsy. Case Report

OBJECTIVE: To report a V and VII cranial nerves palsy as initial presentation of B lymphoblastic leukemia/lymphoma (B-LBL).BACKGROUND: B Lymphoblastic Leukemia/Lymphoma (B-LBL) typically presents with symptoms related to anemia, thrombocytopenia, and neutropenia. Central nervous system (CNS) involvement is not uncommon during the course of this disease, it can be accompanied by cranial neuropathies or other neurological symptoms, predominantly meningeal, related to increased intracranial pressure, but it is considered rare as initial presentation.DESIGN/METHODS: A 27-year-old, previously healthy woman had a 7-week history of right-sided facial weakness and paresthesia. Initially, she was evaluated at an outside facility for right facial tingling and numbness involving the lower lip, difficulty in closing her right eye and right facial droop. She was diagnosed with Bell's palsy, and treated with acyclovir and oral prednisone without clinical improvement. Five weeks later, she was referred to MD Anderson Cancer Center (MDACC) for further evaluation of her symptoms and leukocytosis.RESULTS: A bone marrow biopsy showed B-LBL. Several cerebrospinal fluid samples were negative for neoplastic cells. Brain MRI demonstrated enhancement of the right cranial nerve VII and VIII complex, extra-axial nodular enhancement in the right middle cranial fossa, and lymphomatous infiltration in the right angle of mandible. The patient was treated with high dose intravenous steroids, systemic chemotherapy with PEG-asparaginase, rituximab, cyclophosphamide, doxorubicin, and vincristine; intrathecal methotrexate twice weekly and a single course of intrathecal cytarabine. Ten days later, she was discharged with slight improvement of her right facial palsy, remaining on systemic chemotherapy.CONCLUSIONS: This is the first reported case that we know of B-LBL presenting with multiple cranial neuropathies. This presentation illustrates the importance in differentiating between Bell's palsy and multiple cranial neuropathies, which may indicate an alternative diagnosis.Disclosure: Dr. Garciarena has nothing to disclose. Dr. Kamiya Matsuoka has nothing to disclose. Dr. Amin has nothing to disclose. Dr. Tremont has nothing to disclose. Dr. De Groot has received personal compensation for activities with Genentech, Inc., VBL Therapeutics, and Merck & Co., Inc. Dr. De Groot has received research support from Sanofi-Aventis Pharmaceuticals, Inc., AstraZeneca Pharmaceuticals, and EMD Serono.