Anti-neuronal Autoantibody Type 1 (Anti-Hu) Paraneoplastic Neurologic Syndrome Causing Jaw Dystonia
DOI:
https://doi.org/10.17161/rrnmf.v5i2.21976Keywords:
paraneoplastic syndrome, dystoniaAbstract
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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Dalmau J, Graus F, Villarejo A, et al. Clinical analysis of anti-Ma2-associated encephalitis. Brain. 2004;127(Pt 8):1831-1844. doi:10.1093/brain/awh203
Graus F, Cordon-Cardo C, Posner JB. Neuronal antinuclear antibody in sensory neuronopathy from lung cancer. Neurology. 1985 Apr;35(4):538-43.doi: 10.1212/wnl.35.4.538. PMID:2984600.
Graus F, Keime-Guibert F, Rene R, Benyahia B, Ribalta T, Ascaso C, Escaramis G, Delattre JY. Anti-Hu-associated paraneoplastic encephalomyelitis: analysis of 200 patients. Brain. 2001 Jun;124(Pt 6):1138-48. Doi: 10.1093/brain/125.6.1138. PMID: 11353730.
Malek N, Damian M. Trismus caused by paraneoplastic brainstem encephalitis. Pract Neurol. 2018 Apr;18(2):146-150. doi: 10.1136/practneurol-2017-001819. Epub 2018 Feb 13. PMID: 29440480.
Sillevis Smitt P, Grefkens J, de Leeuw B, van den Bent M, van Putten W, Hooijkaas H, Vecht C. Survival and outcome in 73 anti-Hu positive patients with paraneoplastic encephalomyelitis/sensory neuronopathy. J Neurol. 2002 Jun;249(6):745-53. doi: 10.1007/s00415-002-0706-4. PMID: 12111309.
Tisavipat N, Chang BK, Ali F, Pittock SJ, Kammeyer R, Declusin A, Cohn SJ, Flanagan EP. Subacute Horizontal Diplopia, Jaw Dystonia, and Laryngospasm. Neurol Neuroimmunol Neuroinflamm. 2023 Jun 13;10(4):e200128. Doi:10.1212/NXI.0000000000200128. PMID: 37311643; PMCID: PMC10265402.
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Copyright (c) 2024 Joseph Conway, Katherine Havard, Justin Abbatemarco, Albert Aboseif, Emily Maly, Amy Kunchok, Payal Soni, James Liao
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