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Predictors of amyotrophic lateral sclerosis mimic syndrome

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Abstract

The term amyotrophic lateral sclerosis mimic syndrome (ALSms) includes pathologies that present signs or symptoms similar to those caused by amyotrophic lateral sclerosis (ALS), which can lead to misdiagnosis. In general, any kind of misdiagnosis can result in negative clinical, psychological and economic consequences as well diagnostic and treatment delay. The objectives were to determine the frequency and to compare the demographic and clinical characteristics of patients with ALS and ALSms in our ALS clinic. We retrospectively studied all patients evaluated from 2007 to 2017 including only patients with a definite final diagnosis. Out of 368 patients with motor neuron disease symptomatology, 43 (11.7%) had an ALSms. The most frequent etiology was compressive myelopathy (32.6%). Multivariate analysis considering positive associations was statistically significant for patients having only upper or lower motor neuron signs in the physical examination, a non-compatible electromyogram (EMG), as well as atypical first symptoms. ALS misdiagnosis is an ongoing and not infrequent problem. From our series of patients, atypical symptoms, absence of EMG pathological findings or isolated upper or lower motor neuron disease should prompt suspicion of a differential diagnosis.

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References

  1. Brooks BR (1994) El Escorial World Federation of Neurology criteria for the diagnosis of amyotrophic lateral sclerosis. Subcommittee on Motor Neuron Diseases/Amyotrophic Lateral Sclerosis of the World Federation of Neurology Research Group on Neuromuscular Diseases and the El Escorial "Clinical limits of amyotrophic lateral sclerosis" workshop contributors. J Neurol Sci 124(Suppl):96–107. https://doi.org/10.1016/0022-510X(94)90191-0

    Article  PubMed  Google Scholar 

  2. Oskarsson B, Gendron TF, Staff NP (2018) Amyotrophic lateral sclerosis: an update for 2018. Mayo Clin Proc 93(11):1617–1628. https://doi.org/10.1016/j.mayocp.2018.04.007

    Article  PubMed  Google Scholar 

  3. Cellura E, Spataro R, Taiello AC, La Bella V (2012) Factors affecting the diagnostic delay in amyotrophic lateral sclerosis. Clin Neurol Neurosurg 114(6):550–554. https://doi.org/10.1016/j.clineuro.2011.11.026

    Article  PubMed  Google Scholar 

  4. Solomon AJ, Weinshenker BG (2013) Misdiagnosis of multiple sclerosis: frequency, causes, effects, and prevention. Curr Neurol Neurosci Rep 13(12):403. https://doi.org/10.1007/s11910-013-0403-y

    Article  CAS  PubMed  Google Scholar 

  5. Brooks BR, Miller RG, Swash M, Munsat TL (2000) El Escorial revisited: revised criteria for the diagnosis of amyotrophic lateral sclerosis. Amyotroph Lateral Scler Other Motor Neuron Disord 1(5):293–299. https://doi.org/10.1080/146608200300079536

    Article  CAS  PubMed  Google Scholar 

  6. Carvalho MD, Swash M (2009) Awaji diagnostic algorithm increases sensitivity of El Escorial criteria for ALS diagnosis. Amyotroph Lateral Scler 10(1):53–57. https://doi.org/10.1080/17482960802521126

    Article  PubMed  Google Scholar 

  7. Traynor BJ, Codd MB, Corr B, Forde C, Frost E, Hardiman O (2000) Amyotrophic lateral sclerosis mimic syndromes: a population-based study. Arch Neurol 57(1):109–113. https://doi.org/10.1001/archneur.57.1.109

    Article  CAS  PubMed  Google Scholar 

  8. Davenport RJ, Swingler RJ, Chancellor AM, Warlow CP (1996) Avoiding false positive diagnoses of motor neuron disease: lessons from the Scottish Motor Neuron Disease Register. J Neurol Neurosurg Psychiatry 60(2):147–151. https://doi.org/10.1136/jnnp.60.2.147

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  9. Pupillo E, Messina P, Logroscino G, Beghi E, SLALOM Group (2014) Long-term survival in amyotrophic lateral sclerosis: a population-based study. Ann Neurol. 75(2):287–297. https://doi.org/10.1002/ana.24096

    Article  PubMed  Google Scholar 

  10. Jacobson RD, Goutman SA, Callaghan BC (2016) Pearls & Oy-sters: the importance of atypical features and tracking progression in patients misdiagnosed with ALS. Neurology. 86(13):e136–e139. https://doi.org/10.1212/WNL.0000000000002522

    Article  PubMed  PubMed Central  Google Scholar 

  11. Cortés-Vicente E, Pradas J, Marín-Lahoz J, De Luna N, Clarimón J, Rojas-Garcia R et al (2017) Early diagnosis of amyotrophic lateral sclerosis mimic syndromes: pros and cons of current clinical diagnostic criteria. Amyotroph Lateral Scler Frontotemporal Degener 18(5–6):333–340. https://doi.org/10.1080/21678421.2017.1316408

    Article  PubMed  Google Scholar 

  12. Verma A, Bradley WG (2001) Atypical motor neuron disease and related motor syndromes. Semin Neurol 21(2):177–187. https://doi.org/10.1055/s-2001-15263

    Article  CAS  PubMed  Google Scholar 

  13. Ghasemi M (2016) Amyotrophic lateral sclerosis mimic syndromes. Iran J Neurol 15(2):85–91 (PMID: 27326363)

    PubMed  PubMed Central  Google Scholar 

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Correspondence to Gabriel E. Rodríguez.

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Quarracino, C., Segamarchi, M.C. & Rodríguez, G.E. Predictors of amyotrophic lateral sclerosis mimic syndrome. Acta Neurol Belg 119, 253–256 (2019). https://doi.org/10.1007/s13760-019-01135-1

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  • DOI: https://doi.org/10.1007/s13760-019-01135-1

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