Paraneoplastic Brachial Amyotrophic Diplegia and Encephalitis With Ma2-IgG During Immune Checkpoint Inhibitor Use for...

Immune checkpoint inhibitors (ICIs) improve cancer survival but can cause neurologic immune-related adverse events. Ma2-IgG-associated paraneoplastic neurologic syndrome typically presents with central nervous system involvement in patients with testicular or lung cancers. We present a case of paraneoplastic brachial amyotrophic diplegia (BAD) associated with Ma2-IgG which developed after ICI.

This is a case report of an 86-year-old man with pleural mesothelioma treated with nivolumab and ipilimumab, who presented with progressive, asymmetric weakness of his upper extremities after 5 cycles of ICI. Examination did not reveal associated sensory deficits. Blood creatine kinase level was normal and acetylcholine receptor antibody testing result was negative. Magnetic resonance imaging revealed findings consistent with bilateral frontal temporal lobe encephalitis, but without concurrent cervical cord lesions. Cerebrospinal fluid analysis revealed mild pleocytosis and oligoclonal bands. Ma2-IgG was present in the cerebrospinal fluid (>1:32) and serum (>1:3200). Electrodiagnostic testing revealed diffuse chronic innervation and active denervation changes involving the upper extremities raising concern for a motor neuron disorder. He was diagnosed with having paraneoplastic BAD and encephalitis. A progressive course ensued, despite initial transient improvement with escalation of immunosuppression.

Paraneoplastic BAD is an uncommon paraneoplastic phenomenon that can occur with Ma2-IgG in the setting of ICI use and atypical tumors.