Accessory Nerve Palsy as the Initial Manifestation of Chronic Lymphocytic Leukemia: A Case Report

BACKGROUND Chronic lymphocytic leukemia is the most common leukemia affecting adults, classically presenting with painless lymphadenopathy or diagnosed incidentally on routine full blood count. Neurological complications rarely arise in chronic lymphocytic leukemia and the underlying causes for such complications remain poorly understood. Cranial nerve involvement is uncommon and there are currently no reported cases of accessory nerve palsy as the first presentation of chronic lymphocytic leukemia. CASE REPORT A woman in her 40s presented with a 4-week history of left-sided neck swelling and pain associated with left shoulder restriction. Left trapezius weakness and scapular winging was found on examination, consistent with a left accessory nerve palsy. Examination also revealed multiple tender lymph nodes in the left posterior triangle of the neck. Investigations included a staging CT scan, flexible nasendoscopy, nerve conduction studies, and a full blood count showing lymphocytosis. A work-up involving Ear, Nose, and Throat Surgery, Hematology, and Orthopedic Surgery led to the diagnosis of chronic lymphocytic leukemia. There was no evidence of Richter's transformation. A multidisciplinary team approach was used to treat the patient, and there was complete resolution of her neurological symptoms on follow-up. CONCLUSIONS This report describes an unusual first presentation of chronic lymphocytic leukemia. It serves as a reminder to consider hematological malignancies in cases of accessory nerve palsy associated with a neck swelling. Early input from Hematology and multidisciplinary involvement can aid early diagnosis and prevent unnecessary investigations. Further research is warranted to explore the neurological spectrum of CLL.