Objective:

A descriptive series of seven cases diagnosed with cranial neurolymphomatosis are presented. Analysis includes clinical manifestations, radiographic findings, management, and outcomes.

Background:

Neurolymphomatosis (NL) occurs due to the infiltration of a nerve by malignant cells. Nerves of the peripheral nervous system are commonly involved including the cranial nerves. NL is a rare disease process and distinct from paraneoplastic syndromes, and leptomeningeal carcinomatosis. Symptoms commonly present as motor weakness, hyporeflexia, foot drop, mono-neuritis multiplex. Naturally, cranial neruolymphomatosis (CNL) presents with a singular or multiple cranial nerve neuropathies.  Primary CNL is defined as the initial presenting symptom leading to a new diagnosis of cancer. Secondary CNL is defined as cancer progression with objectively measured spread to a cranial nerve.

Design/Methods:

A descriptive case series is presented on seven patients who presented to a tertiary care center from the years 2018-2022.

Results:

A total of seven cases are presented. 5/7 (71.4%) patients presented as primary CNL. Diffuse large B-cell lymphoma was the most prevalent malignancy presenting in 3/7 (42.8%) of cases. Other malignancies observed include non-Hodgkin lymphoma, monoclonal B-cell lymphocytosis, peripheral T-cell lymphoma, and olfactory neuroblastoma. The most commonly affected cranial nerve was the trigeminal nerve in 4/7 (57.1%) of patients. The most common neuro-radiographic finding being lesion to Meckel's cave. Other cranial nerve neuropathies include the optic, facial, and vestibulocochlear nerve. Treatment was guided based on the individual malignancy and 3/7 (42.8%) of patients achieved complete remission, 2/7 (28.5%) were deceased within one year, and 1/7 (14.2%) was referred to hospice care.

Conclusions:

Cranial neurolymphomatosis (CNL) is a rare disease process associated with non-solid tumors (i.e. lymphoma, leukemia,etc.) This case series provides significant insight on the clinical manifestation, etiologies, neuro-radiographic findings, management and outcomes.