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Predictors of amyotrophic lateral sclerosis mimic syndrome

  • Cecilia Quarracino
  • María Constanza Segamarchi
  • Gabriel E. RodríguezEmail author
Original article
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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.

Keywords

ALS differential diagnosis ALS mimic syndrome ALS mimic disorder Spondylotic myelopathy 

Notes

Compliance with ethical standards

Conflict of interest

The authors declare that they have no conflicts of interest.

Ethical approval

This article does not contain any studies with human participants or animals performed by any of the authors.

Informed consent

For this type of study, formal consent is not required.

References

  1. 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 CrossRefGoogle Scholar
  2. 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 CrossRefGoogle Scholar
  3. 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 CrossRefGoogle Scholar
  4. 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 CrossRefGoogle Scholar
  5. 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 CrossRefGoogle Scholar
  6. 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 CrossRefGoogle Scholar
  7. 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 CrossRefGoogle Scholar
  8. 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 CrossRefGoogle Scholar
  9. 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 CrossRefGoogle Scholar
  10. 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 CrossRefGoogle Scholar
  11. 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 CrossRefGoogle Scholar
  12. 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 CrossRefGoogle Scholar
  13. 13.
    Ghasemi M (2016) Amyotrophic lateral sclerosis mimic syndromes. Iran J Neurol 15(2):85–91 (PMID: 27326363) Google Scholar

Copyright information

© Belgian Neurological Society 2019

Authors and Affiliations

  • Cecilia Quarracino
    • 1
  • María Constanza Segamarchi
    • 2
  • Gabriel E. Rodríguez
    • 3
    Email author
  1. 1.Neurology DepartmentInstituto de Investigaciones Médicas Alfredo LanariBuenos AiresArgentina
  2. 2.Neurology Residency, Neurology DepartmentHospital Ramos MejíaBuenos AiresArgentina
  3. 3.ALS Clinic, Neurology DepartmentHospital Ramos MejíaBuenos AiresArgentina

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