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Article: Nonthymoma early-onset- and late-onset-generalized myasthenia gravis-A retrospective hospital-based study

TitleNonthymoma early-onset- and late-onset-generalized myasthenia gravis-A retrospective hospital-based study
Authors
Issue Date2007
PublisherElsevier BV. The Journal's web site is located at http://www.elsevier.com/locate/clineuro
Citation
Clinical Neurology And Neurosurgery, 2007, v. 109 n. 8, p. 686-691 How to Cite?
AbstractObjective: Acquired myasthenia gravis (MG) is predominantly due to nicotinic acetylcholine receptor (AChR) autoantibodies (Ab). Differences between nonthymoma early-onset and late-onset MG were reported. We studied the clinical and serological characteristics of nonthymoma AChR Ab-positive-generalized MG patients. Patients and methods: Chinese AChR Ab-positive-generalized MG patients who had generalized disease for 3 years or longer were studied. Results: Among 41 such patients, 25 (61%) were female. The mean onset age was 43.5 years (range 9-78 years) and the mean follow-up duration was 7.8 years (range 3-20 years). Sixteen (39%) patients had late-onset disease (onset age ≥50 years). Compared to early-onset patients (onset age <50 years), late-onset patients were characterized by male predominance (p = 0.002), absence of thymic lymphofollicular hyperplasia (p = 0.036), and a higher striated muscle Ab seropositivity rate (94% versus 4%, p < 0.001). Although there was no statistically significant difference in clinical severity and outcome or response to treatment between late-onset and early-onset patients, 50% and 75% of late-onset patients had moderate or severe disease at onset and worst status, respectively, compared to 28% and 52% for early-onset patients at onset and worst status, respectively. Also 63% of late-onset patients had disease progressed within first 3 years compared to only 40% of early-onset patients did. Conclusion: Nonthymoma late-onset-generalized MG patients were common among Hong Kong Chinese, with a statistically non-significant trend that it was clinically more severe than early-onset MG but with similar clinical outcome or response to treatment; >90% of these patients were seropositive for striated muscle Ab. © 2007 Elsevier B.V. All rights reserved.
Persistent Identifierhttp://hdl.handle.net/10722/163098
ISSN
2015 Impact Factor: 1.198
2015 SCImago Journal Rankings: 0.555
ISI Accession Number ID
References

 

DC FieldValueLanguage
dc.contributor.authorChan, KHen_US
dc.contributor.authorCheung, RTFen_US
dc.contributor.authorMak, Wen_US
dc.contributor.authorHo, SLen_US
dc.date.accessioned2012-09-05T05:27:34Z-
dc.date.available2012-09-05T05:27:34Z-
dc.date.issued2007en_US
dc.identifier.citationClinical Neurology And Neurosurgery, 2007, v. 109 n. 8, p. 686-691en_US
dc.identifier.issn0303-8467en_US
dc.identifier.urihttp://hdl.handle.net/10722/163098-
dc.description.abstractObjective: Acquired myasthenia gravis (MG) is predominantly due to nicotinic acetylcholine receptor (AChR) autoantibodies (Ab). Differences between nonthymoma early-onset and late-onset MG were reported. We studied the clinical and serological characteristics of nonthymoma AChR Ab-positive-generalized MG patients. Patients and methods: Chinese AChR Ab-positive-generalized MG patients who had generalized disease for 3 years or longer were studied. Results: Among 41 such patients, 25 (61%) were female. The mean onset age was 43.5 years (range 9-78 years) and the mean follow-up duration was 7.8 years (range 3-20 years). Sixteen (39%) patients had late-onset disease (onset age ≥50 years). Compared to early-onset patients (onset age <50 years), late-onset patients were characterized by male predominance (p = 0.002), absence of thymic lymphofollicular hyperplasia (p = 0.036), and a higher striated muscle Ab seropositivity rate (94% versus 4%, p < 0.001). Although there was no statistically significant difference in clinical severity and outcome or response to treatment between late-onset and early-onset patients, 50% and 75% of late-onset patients had moderate or severe disease at onset and worst status, respectively, compared to 28% and 52% for early-onset patients at onset and worst status, respectively. Also 63% of late-onset patients had disease progressed within first 3 years compared to only 40% of early-onset patients did. Conclusion: Nonthymoma late-onset-generalized MG patients were common among Hong Kong Chinese, with a statistically non-significant trend that it was clinically more severe than early-onset MG but with similar clinical outcome or response to treatment; >90% of these patients were seropositive for striated muscle Ab. © 2007 Elsevier B.V. All rights reserved.en_US
dc.languageengen_US
dc.publisherElsevier BV. The Journal's web site is located at http://www.elsevier.com/locate/clineuroen_US
dc.relation.ispartofClinical Neurology and Neurosurgeryen_US
dc.subject.meshAdolescenten_US
dc.subject.meshAdulten_US
dc.subject.meshAge Of Onseten_US
dc.subject.meshAsian Continental Ancestry Group - Statistics & Numerical Dataen_US
dc.subject.meshAutoantibodies - Blooden_US
dc.subject.meshChilden_US
dc.subject.meshChild, Preschoolen_US
dc.subject.meshFemaleen_US
dc.subject.meshHong Kong - Epidemiologyen_US
dc.subject.meshHumansen_US
dc.subject.meshMaleen_US
dc.subject.meshMotor Endplate - Metabolismen_US
dc.subject.meshMuscle, Skeletal - Metabolismen_US
dc.subject.meshMyasthenia Gravis - Diagnosis - Epidemiology - Therapyen_US
dc.subject.meshReceptors, Nicotinic - Immunologyen_US
dc.subject.meshRetrospective Studiesen_US
dc.subject.meshSeverity Of Illness Indexen_US
dc.titleNonthymoma early-onset- and late-onset-generalized myasthenia gravis-A retrospective hospital-based studyen_US
dc.typeArticleen_US
dc.identifier.emailCheung, RTF:rtcheung@hku.hken_US
dc.identifier.emailHo, SL:slho@hku.hken_US
dc.identifier.authorityCheung, RTF=rp00434en_US
dc.identifier.authorityHo, SL=rp00240en_US
dc.description.naturelink_to_subscribed_fulltexten_US
dc.identifier.doi10.1016/j.clineuro.2007.05.023en_US
dc.identifier.pmid17644246en_US
dc.identifier.scopuseid_2-s2.0-34547844246en_US
dc.identifier.hkuros143156-
dc.relation.referenceshttp://www.scopus.com/mlt/select.url?eid=2-s2.0-34547844246&selection=ref&src=s&origin=recordpageen_US
dc.identifier.volume109en_US
dc.identifier.issue8en_US
dc.identifier.spage686en_US
dc.identifier.epage691en_US
dc.identifier.isiWOS:000249498000010-
dc.publisher.placeNetherlandsen_US
dc.identifier.scopusauthoridChan, KH=7406034963en_US
dc.identifier.scopusauthoridCheung, RTF=7202397498en_US
dc.identifier.scopusauthoridMak, W=22948344000en_US
dc.identifier.scopusauthoridHo, SL=25959633500en_US

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