Paraneoplastic Antibody Syndromes
Common Paraneoplastic Antibody Syndromes
Limbic encephalitis
Rapid onset neuro-behavioral/cognitive decline
- anti-Hu (ANNA-1) antibody
- small cell lung cancer
Opsoclonus Myoclonus
Opsoclonus, myoclonus, and ataxia
- anti-Ri (ANNA-2) antibody
- commonly: breast cancer
Stiff-person syndrome
Periodic muscle rigidity/stiffness of the limbs and trunk (begins axially, progresses to the limbs). Manifests with exaggerated startle, painful spasms,
- anti-amphiphysin antibody is the most common paraneoplastic cause.
- anti-GAD65 antibody is the most common non-paraneoplastic cause.
Lambert-Eaton myasthenic syndrome
Subactue, progressive weakness of the shoulders, feet/hands, and oropharynx, followed by the eyes, secondary to a neuromuscle junction attack (like myasthenia gravis).
- anti-voltage-gated P/Q type calcium channel antibodies
- when paraneoplastic, often associated w/AGNA (SOX1), in association w/small cell lung cancer
Paraneoplastic cerebellar degeneration
Rapidly progressive (days to weeks) cerebellar (gait, balance, coordination) disorder
- anti-Yo antibody
- classically: ovarian cancer,
- commonly: breast cancer
Paraneoplastic retinal degeneration
Painless, progressive degeneration of peripheral vision (later central vision), secondary to antibody attack of retinal bipolar cell antigens
- anti-recoverin antibody (aka, anti-CAR (cancer-associated retinopathy))
- also associated with anti-MAR (melanoma associated retinopathy)
Paraneoplastic sensory neuropathy/neuronopathy
Asymmetric, rapidly progressive loss of sensation of the limbs with profound loss of proprioception, secondary to an attack at the dorsal root ganglion.
- anti-Hu (ANNA-1) antibody
- most commonly: small cell lung cancer
Paraneoplastic choreoathetosis (aka, basal ganglionitis)
Choreoathetosis is the prominent symptom, parkinsonism and hemiballismus have also been reported.
- anti-CRMP-5/CV2 antibody (other antibodies also reported)
- commonly: small cell lung cancer
- also: thymoma, thyroid, renal cancer
References
- Aydin, Dogu, Finn Somnier, and Lisbeth H. Lassen. “Paraneoplastic Choreoathetosis in a Patient with Small Cell Lung Carcinoma and Anti-CRMP5/CV2: A Case Report.” Case Reports in Neurology 8, no. 1 (January 8, 2016): 16–19. https://doi.org/10.1159/000443522.
- Daroff, Robert B., Joseph Jankovic, John C. Mazziotta, and Scott L. Pomeroy. Bradley’s Neurology in Clinical Practice E-Book. Elsevier Health Sciences, 2015.
- Fanous, Ibrahim, and Patrick Dillon. “Paraneoplastic Neurological Complications of Breast Cancer.” Experimental Hematology & Oncology 5 (October 24, 2016). https://doi.org/10.1186/s40164-016-0058-x.
- Graus, Francesc, and Josep Dalmau. “Paraneoplastic Neuropathies:” Current Opinion in Neurology 26, no. 5 (October 2013): 489–95. https://doi.org/10.1097/WCO.0b013e328364c020.
- Gray, Frangoise, Charles Duyckaerts, and Umberto De Girolami. Escourolle and Poirier’s Manual of Basic Neuropathology. OUP USA, 2013.
- Klaas, James P., J. Eric Ahlskog, Sean J. Pittock, Joseph Y. Matsumoto, Allen J. Aksamit, J. D. Bartleson, Rajeev Kumar, Kathleen F. McEvoy, and Andrew McKeon. “Adult-Onset Opsoclonus-Myoclonus Syndrome.” Archives of Neurology 69, no. 12 (December 2012): 1598–1607. https://doi.org/10.1001/archneurol.2012.1173.
- “Lambert-Eaton Myasthenic Syndrome.” NORD (National Organization for Rare Disorders) (blog). Accessed August 4, 2018. https://rarediseases.org/rare-diseases/lambert-eaton-myasthenic-syndrome/.
- “Paraneoplastic Retinopathy.” Accessed August 4, 2018. https://www.aao.org/bcscsnippetdetail.aspx?id=1c93a96a-be47-4a6e-af91-03970c86d3f2.
- Samuels, Martin A., Allan H. Ropper, and Joshua Klein. Adams and Victor’s Principles of Neurology 10th Edition. McGraw-Hill Education, 2014.
- Schiff, David, Santosh Kesari, and Patrick C. Y. Wen. Cancer Neurology in Clinical Practice: Neurologic Complications of Cancer and Its Treatment. Springer Science & Business Media, 2008.
- Shams’ili, Setareh, Joost Grefkens, Bertie de Leeuw, Martin van den Bent, Herbert Hooijkaas, Bronno van der Holt, Charles Vecht, and Peter Sillevis Smitt. “Paraneoplastic Cerebellar Degeneration Associated with Antineuronal Antibodies: Analysis of 50 Patients.”
- Brain: A Journal of Neurology 126, no. Pt 6 (June 2003): 1409–18.