Anti-neuronal nuclear autoantibody type1 (ANNA-1), or anti-Hu, paraneoplastic neurologic syndrome (PNS) classically manifests with sensory neuronopathy and encephalomyelitis. We describe a rare case of anti-ANNA-1 PNS presenting with marked jaw dystonia and cognitive impairment. Her symptoms complicated the evaluation for an underlying malignancy and severely impacted her functional status due to malnutrition and increased disability. Symptomatic management focused on reducing the severity of jaw dystonia, which improved her overall function and allowed for treatment of her underlying malignancy.
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Conway et al. (2024) studied this question.
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