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Anti-Ma and anti-Ma2-associated paraneoplastic neurological syndromes.
Neurologia (Engl Ed). 2018 Jan - Feb; 33(1):18-27.N

Abstract

OBJECTIVE

Analyse the clinical profile, associated tumour types, and response to treatment of paraneoplastic neurological syndromes associated with antibodies against Ma proteins.

METHODS

A retrospective study of patients with antibodies against Ma proteins identified in a neuroimmunology laboratory of reference.

RESULTS

Of the 32 patients identified, 20 showed reactivity against Ma2 only (anti-Ma2 antibodies), 11 against Ma1 and Ma2 (anti-Ma antibodies), and 1 with reactivity against Ma1 only (anti-Ma1 antibodies). The most common clinical presentations were limbic encephalopathy, diencephalic dysfunction, or brainstem encephalopathy, frequently appearing as a combination of these features. Three patients had isolated cerebellar dysfunction with anti-Ma antibodies, and 2 exhibited peripheral nervous system syndrome with anti-Ma2 antibodies. Testicular tumours were the most common neoplasms (40%) in the anti-Ma2 cases. In the group associated with anti-Ma1 antibodies, the most common were lung tumours (36%), followed by testicular tumours. All idiopathic cases were reactive to Ma2. The clinical outcome was significantly better in the anti-Ma2 group. The patient with anti-Ma1 presented with limbic encephalitis and brainstem dysfunction associated with lymphoepithelioma of the bladder.

CONCLUSIONS

Specifically determining the different reactivities of anti-Ma protein antibodies in order to differentiate between Ma1 and Ma2 antibodies is important because anti-Ma2-associated paraneoplastic syndromes have a better outcome. Lastly, this study is the first to confirm that there may be cases that react exclusively to antibodies against Ma1.

Authors+Show Affiliations

Servicio de Neurología, Hospital Clínico San Carlos, Madrid, España. Electronic address: gloria271187@hotmail.com.Servicio de Neurología, Hospital Clínic, Barcelona, España.Servicio de Neurología, Hospital Clínic, Barcelona, España.Servicio de Neurología, Hospital Clínic, Barcelona, España.Servicio de Neurología, Hospital Clínic, Barcelona, España.

Pub Type(s)

Journal Article

Language

eng spa

PubMed ID

27460184

Citation

Ortega Suero, G, et al. "Anti-Ma and anti-Ma2-associated Paraneoplastic Neurological Syndromes." Neurologia (Barcelona, Spain), vol. 33, no. 1, 2018, pp. 18-27.
Ortega Suero G, Sola-Valls N, Escudero D, et al. Anti-Ma and anti-Ma2-associated paraneoplastic neurological syndromes. Neurologia (Engl Ed). 2018;33(1):18-27.
Ortega Suero, G., Sola-Valls, N., Escudero, D., Saiz, A., & Graus, F. (2018). Anti-Ma and anti-Ma2-associated paraneoplastic neurological syndromes. Neurologia (Barcelona, Spain), 33(1), 18-27. https://doi.org/10.1016/j.nrl.2016.05.010
Ortega Suero G, et al. Anti-Ma and anti-Ma2-associated Paraneoplastic Neurological Syndromes. Neurologia (Engl Ed). 2018 Jan - Feb;33(1):18-27. PubMed PMID: 27460184.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Anti-Ma and anti-Ma2-associated paraneoplastic neurological syndromes. AU - Ortega Suero,G, AU - Sola-Valls,N, AU - Escudero,D, AU - Saiz,A, AU - Graus,F, Y1 - 2016/07/25/ PY - 2016/04/20/received PY - 2016/05/05/accepted PY - 2016/7/28/pubmed PY - 2018/8/28/medline PY - 2016/7/28/entrez KW - Anti-Ma KW - Anti-Ma2 KW - Anticuerpos onconeuronales KW - Encefalitis límbica KW - Limbic encephalitis KW - Onconeural antibodies KW - Paraneoplastic neurological syndromes KW - Síndromes neurológicos paraneoplásicos SP - 18 EP - 27 JF - Neurologia (Barcelona, Spain) JO - Neurologia (Engl Ed) VL - 33 IS - 1 N2 - OBJECTIVE: Analyse the clinical profile, associated tumour types, and response to treatment of paraneoplastic neurological syndromes associated with antibodies against Ma proteins. METHODS: A retrospective study of patients with antibodies against Ma proteins identified in a neuroimmunology laboratory of reference. RESULTS: Of the 32 patients identified, 20 showed reactivity against Ma2 only (anti-Ma2 antibodies), 11 against Ma1 and Ma2 (anti-Ma antibodies), and 1 with reactivity against Ma1 only (anti-Ma1 antibodies). The most common clinical presentations were limbic encephalopathy, diencephalic dysfunction, or brainstem encephalopathy, frequently appearing as a combination of these features. Three patients had isolated cerebellar dysfunction with anti-Ma antibodies, and 2 exhibited peripheral nervous system syndrome with anti-Ma2 antibodies. Testicular tumours were the most common neoplasms (40%) in the anti-Ma2 cases. In the group associated with anti-Ma1 antibodies, the most common were lung tumours (36%), followed by testicular tumours. All idiopathic cases were reactive to Ma2. The clinical outcome was significantly better in the anti-Ma2 group. The patient with anti-Ma1 presented with limbic encephalitis and brainstem dysfunction associated with lymphoepithelioma of the bladder. CONCLUSIONS: Specifically determining the different reactivities of anti-Ma protein antibodies in order to differentiate between Ma1 and Ma2 antibodies is important because anti-Ma2-associated paraneoplastic syndromes have a better outcome. Lastly, this study is the first to confirm that there may be cases that react exclusively to antibodies against Ma1. SN - 2173-5808 UR - https://www.unboundmedicine.com/medline/citation/27460184/Anti_Ma_and_anti_Ma2_associated_paraneoplastic_neurological_syndromes_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S0213-4853(16)30086-X DB - PRIME DP - Unbound Medicine ER -