Unilateral Painful Plexopathy: Early Diagnosis of Primary Neurolymphomatosis Leading to Remarkable Recovery
Shota Fukaura1, Hori Kentaro1, Junji Tokushige2, Shingo Kawakami1, Yoko Suzuki1
1Neurology, 2Hematology, Omori Red Cross Hospital
Objective:
To demonstrate that early diagnosis and prompt treatment of primary neurolymphomatosis can achieve rapid neurological recovery even in elderly patients, contrasting with the typically poor prognosis reported in the literature.
Background:
Primary neurolymphomatosis has poor prognosis with 55% mortality and median survival of 28 months. Diagnostic delays are common due to clinical mimicry of inflammatory conditions, particularly chronic inflammatory demyelinating polyneuropathy. The potential for neurological recovery with early intervention remains underexplored, especially in elderly patients.
Design/Methods:
We present a 71-year-old man with right upper limb weakness beginning one year prior and progressive pain emerging two months before presentation. Evaluation included neurological examination, laboratory studies, cerebrospinal fluid analysis, brachial plexus MRI, nerve conduction studies, gallium scintigraphy, and FDG-PET. PET-guided lymph node biopsy confirmed diagnosis.
Results:
Examination revealed mild atrophy and weakness in right upper extremity with globally decreased reflexes. Laboratory studies showed modest inflammatory markers with normal CSF. MRI demonstrated right brachial plexus enlargement with C5 nerve root enhancement. Nerve conduction studies revealed conduction block in the right axillary nerve. Chronic inflammatory demyelinating polyneuropathy was considered, but strictly unilateral distribution, prominent pain, and poor immunotherapy response were atypical. Steroid pulse therapy provided transient minimal relief with rapid rebound; intravenous immunoglobulin showed no response. Gallium scintigraphy was negative, but FDG-PET revealed hypermetabolic activity in the right brachial plexus and multiple lymph nodes. PET-guided biopsy confirmed diffuse large B-cell lymphoma. After one cycle of R-CHOP with methotrexate, the patient demonstrated significant improvement with pain resolution and conduction block improvement.
Conclusions:
Early diagnosis and prompt treatment of primary neurolymphomatosis can achieve rapid neurological improvement in elderly patients. Early intervention may lead to better outcomes than traditionally poor prognosis suggests. Unilateral pain-predominant plexopathy with poor immunosuppressive therapy response should prompt immediate oncological investigation. Timely diagnosis and treatment are critical for optimizing neurological recovery.
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