Advertisement

Spectrum of pediatric acquired demyelinating syndromes (PADS) of the central nervous system in a tropical developing country: A 10-year retrospective study

  • Author Footnotes
    1 Joint first authors.
    ,
    Author Footnotes
    2 ORCID: 0000-0002-7220-1405.
    Maria Ruby Rose Q. Castañeda
    Correspondence
    Corresponding author at: Department of Neurosciences Office, Philippine General Hospital, Taft Avenue, Ermita, Manila 1000, Philippines.
    Footnotes
    1 Joint first authors.
    2 ORCID: 0000-0002-7220-1405.
    Affiliations
    Division of Pediatric Neurology, Department of Pediatrics and Neurosciences, College of Medicine and Philippine General Hospital, University of the Philippines Manila, Manila, Philippines
    Search for articles by this author
  • Author Footnotes
    1 Joint first authors.
    ,
    Author Footnotes
    3 ORCID: 0000-0001-5621-1833.
    Adrian I. Espiritu
    Footnotes
    1 Joint first authors.
    3 ORCID: 0000-0001-5621-1833.
    Affiliations
    Division of Adult Neurology, Department of Neurosciences, College of Medicine and Philippine General Hospital, University of the Philippines Manila, Manila, Philippines

    Department of Clinical Epidemiology, College of Medicine, University of the Philippines Manila, Manila, Philippines

    Division of Neurology, Department of Medicine, St. Michael’s Hospital, University of Toronto, Toronto, Ontario, Canada
    Search for articles by this author
  • Author Footnotes
    4 ORCID: 0000-0001-9622-6810.
    Marilyn A. Tan
    Footnotes
    4 ORCID: 0000-0001-9622-6810.
    Affiliations
    Division of Pediatric Neurology, Department of Pediatrics and Neurosciences, College of Medicine and Philippine General Hospital, University of the Philippines Manila, Manila, Philippines
    Search for articles by this author
  • Author Footnotes
    1 Joint first authors.
    2 ORCID: 0000-0002-7220-1405.
    3 ORCID: 0000-0001-5621-1833.
    4 ORCID: 0000-0001-9622-6810.

      Highlights

      • Data on pediatric acquired demyelinating syndromes (PADS) in the Philippines is lacking.
      • A total of 77 patients with PADS were identified at our institution.
      • The most common PADS subtypes were TM, ADEM and MS.
      • IVMP is the most utilized treatment during an acute demyelinating attack.
      • The majority of patients with PADS experienced partial recovery.

      Abstract

      Background

      Epidemiological data on the pediatric acquired demyelinating syndromes (PADS) in the Philippines has not been described previously in the literature. There may be geographic differences in frequencies of PADS where true disease burden is not known or underestimated in resource-limited settings due to lack of case recognition and/or diagnostic facilities. The purpose of this study was to determine the frequencies and clinical characteristics of the different subtypes of PADS at our institution and compare these with those in published literature.

      Methods

      We conducted a retrospective cross-sectional study of children diagnosed with PADS who were admitted at the Philippine General Hospital from January 2009 to December 2018. Charts of these patients were reviewed to determine frequencies, clinical profile, diagnostic findings and outcomes.

      Results

      A total of 77 patients were identified with PADS using the appropriate diagnostic criteria. The frequencies of the PADS subtypes were the following: transverse myelitis (n = 21; 27.3 %); acute disseminated encephalomyelitis (n = 20, 26.0 %); multiple sclerosis (n = 17, 22.1 %); optic neuritis (n = 13, 16.9 %); clinically isolated syndrome (n = 4, 5.2 %); and neuromyelitis optica spectrum disorder (n = 2, 2.6 %). Overall, the mean age at initial event and at diagnosis were 10.6 ± 4.6 years. Female:male ratio was 1.02:1. On admission, the majority of patients had motor paralysis (n = 49, 63.6 %) while several patients manifested with sensory deficits (n = 31, 40.3 %), visual changes (n = 26, 33.8 %) and brainstem involvement (n = 20, 26.0 %). Nearly all patients had evidence of lesions in magnetic resonance imaging (n = 72, 93.5 %) located in spinal cord (n = 25; 32.5 %), cerebral white matter (n = 24; 31.2 %), and optic nerve (n = 12, 15.6 %). Among patients who underwent cerebrospinal fluid analysis (n = 34), 7 patients had abnormal findings (20.6 %). The most utilized treatment regimens during admission were intravenous methylprednisolone (n = 53, 68.8 %) and oral prednisone (n = 43, 55.8 %). The majority had partial recovery (n = 56, 72.7 %) and 16 experienced full recovery (20.8 %) at discharge. Five patients died (6.5 %).

      Conclusions

      Our study provided the first comprehensive summary on the clinical features of children with PADS admitted in a Philippine tertiary hospital with limited resources. Our study highlights the value of using clinical diagnostic criteria in improving case recognition especially in low-and middle-income countries. Regional disparities in disease burden warrant international registries with wider geographic representation in order to come up with diagnostic and management guidelines suitable for various levels of care.

      Abbreviations:

      ADEM (Acute disseminated encephalomyelitis), CIS (Clinically isolated syndrome), DMD (Disease-modifying drug), EEG (Electroencephalography/ electroencephalogram), IVMP (Intravenous methylprednisolone), MRI (Magnetic resonance imaging), MS (Multiple sclerosis), NMOSD (Neuromyelitis optica spectrum disorders), ON (Optic neuritis), PADS (Pediatric acquired demyelinating syndrome), TM (Transverse myelitis), VEP (Visual evoked potential)

      Keywords

      To read this article in full you will need to make a payment

      Purchase one-time access:

      Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online access
      One-time access price info
      • For academic or personal research use, select 'Academic and Personal'
      • For corporate R&D use, select 'Corporate R&D Professionals'

      Subscribe:

      Subscribe to Journal of Clinical Neuroscience
      Already a print subscriber? Claim online access
      Already an online subscriber? Sign in
      Institutional Access: Sign in to ScienceDirect

      References

        • Langer-Gould A.
        • Zhang J.L.
        • Chung J.
        • Yeung Y.
        • Waubant E.
        • Yao J.
        Incidence of acquired CNS demyelinating syndromes in a multiethnic cohort of children.
        Neurology. 2011; 77: 1143-1148https://doi.org/10.1212/WNL.0b013e31822facdd
        • Absoud M.
        • Lim M.J.
        • Chong W.K.
        • De Goede C.G.
        • Foster K.
        • Gunny R.
        • et al.
        Paediatric acquired demyelinating syndromes: Incidence, clinical and magnetic resonance imaging features.
        Mult Scler J. 2013; 19: 76-86
        • Hintzen R.Q.
        • Dale R.C.
        • Neuteboom R.F.
        • Mar S.
        • Banwell B.
        Pediatric acquired CNS demyelinating syndromes.
        Neurology. 2016; 87: S67-S73
        • Boesen M.S.
        • Magyari M.
        • Born A.
        • Thygesen L.C.
        Pediatric acquired demyelinating syndromes: A nationwide validation study of the Danish National Patient Register.
        Clin Epidemiol. 2018; 10: 391-399https://doi.org/10.2147/CLEP.S156997
        • Neuteboom R.
        • Wilbur C.
        • Van Pelt D.
        • Rodriguez M.
        • Yeh A.
        The spectrum of inflammatory acquired demyelinating syndromes in children.
        Semin Pediatr Neurol. 2017; 24: 189-200https://doi.org/10.1016/j.spen.2017.08.007
        • Espiritu A.I.
        • Mesina B.V.Q.
        • Puerto A.A.D.
        • Reyes N.G.D.
        • Damian L.F.
        • Pascual V.J.L.R.
        Neuromyelitis optica spectrum disorder in a tertiary hospital in the Philippines: A case series.
        Mult Scler Relat Disord. 2019; 31: 124-130https://doi.org/10.1016/j.msard.2019.04.006
        • Mesina B.
        • San L.A.
        Spectrum of demyelinating diseases in a tertiary hospital from 2000–2010.
        Heal Sci J. 2012; 1: 63-69
        • Espiritu A.I.
        • Leochico C.F.D.
        • Separa K.J.N.J.
        • Jamora R.D.G.
        Scientific impact of multiple sclerosis and neuromyelitis optica spectrum disorder research from Southeast Asia: A bibliometric analysis.
        Mult Scler Relat Disord. 2020; 38: 101862
        • Krupp L.B.
        • Tardieu M.
        • Amato M.P.
        • Banwell B.
        • Chitnis T.
        • Dale R.C.
        • et al.
        International Pediatric Multiple Sclerosis Study Group criteria for pediatric multiple sclerosis and immune-mediated central nervous system demyelinating disorders: Revisions to the 2007 definitions.
        Mult Scler J. 2013; 19: 1261-1267
        • Thompson A.J.
        • Banwell B.L.
        • Barkhof F.
        • Carroll W.M.
        • Coetzee T.
        • Comi G.
        • et al.
        Diagnosis of multiple sclerosis: 2017 revisions of the McDonald criteria.
        Lancet Neurol. 2018; 17: 162-173
        • Chhabda S.
        • Malik P.
        • Reddy N.
        • Muthusamy K.
        • Mirsky D.
        • Sudhakar S.
        • et al.
        Relapsing demyelinating syndromes in children: A practical review of neuroradiological mimics.
        Front Neurol. 2020; 11https://doi.org/10.3389/fneur.2020.00627
        • Ignacio K.H.D.
        • Espiritu A.I.
        • Jamora R.D.G.
        The current status and challenges in multiple sclerosis management in the Philippines.
        Mult Scler Relat Disord. 2020; 46102510https://doi.org/10.1016/j.msard.2020.102510
        • Banwell B.
        • Kennedy J.
        • Sadovnick D.
        • Arnold D.L.
        • Magalhaes S.
        • Wambera K.
        • et al.
        Incidence of acquired demyelination of the CNS in Canadian children.
        Neurology. 2009; 72: 232-239
        • Ketelslegers I.A.
        • Catsman-Berrevoets C.E.
        • Neuteboom R.F.
        • Boon M.
        • Dijk K.G.J.
        • Eikelenboom M.J.
        • et al.
        Incidence of acquired demyelinating syndromes of the CNS in Dutch children: a nationwide study.
        J Neurol. 2012; 259: 1929-1935
        • Yamaguchi Y.
        • Torisu H.
        • Kira R.
        • Ishizaki Y.
        • Sakai Y.
        • Sanefuji M.
        • et al.
        A nationwide survey of pediatric acquired demyelinating syndromes in Japan.
        Neurology. 2016; 87: 2006-2015
        • Banwell B.
        • Tenembaum S.
        • Lennon V.A.
        • Ursell E.
        • Kennedy J.
        • Bar-Or A.
        • et al.
        Neuromyelitis optica-IgG in childhood inflammatory demyelinating CNS disorders.
        Neurology. 2008; 70: 344-352
        • Mikaeloff Y.
        • Suissa S.
        • Vallée L.
        • Lubetzki C.
        • Ponsot G.
        • Confavreux C.
        • et al.
        First episode of acute CNS inflammatory demyelination in childhood: Prognostic factors for multiple sclerosis and disability.
        J Pediatr. 2004; 144: 246-252
        • Neuteboom R.F.
        • Boon M.
        • Catsman Berrevoets C.E.
        • Vles J.S.
        • Gooskens R.H.
        • Stroink H.
        • et al.
        Prognostic factors after a first attack of inflammatory CNS demyelination in children.
        Neurology. 2008; 71: 967-973
        • Cole J.
        • Evans E.
        • Mwangi M.
        • Mar S.
        Acute disseminated encephalomyelitis in children: An updated review based on current diagnostic criteria.
        Pediatr Neurol. 2019; 100: 26-34https://doi.org/10.1016/j.pediatrneurol.2019.06.017
        • Waldman A.T.
        • Stull L.B.
        • Galetta S.L.
        • Balcer L.J.
        • Liu G.T.
        Pediatric optic neuritis and risk of multiple sclerosis: Meta-analysis of observational studies.
        J Am Assoc Pediatr Ophthalmol Strabismus. 2011; 15: 441-446https://doi.org/10.1016/j.jaapos.2011.05.020
        • Pidcock F.S.
        • Krishnan C.
        • Crawford T.O.
        • Salorio C.F.
        • Trovato M.
        • Kerr D.A.
        Acute transverse myelitis in childhood: Center-based analysis of 47 cases.
        Neurology. 2007; 68: 1474-1480https://doi.org/10.1212/01.wnl.0000260609.11357.6f
        • Alper G.
        • Petropoulou K.A.
        • Fitz C.R.
        • Kim Y.
        Idiopathic acute transverse myelitis in children: An analysis and discussion of MRI findings.
        Mult Scler J. 2011; 17: 74-80https://doi.org/10.1177/1352458510381393
        • Swaiman K.F.
        • Ashwal S.
        • Ferriero D.M.
        • Schor N.F.
        • Finkel R.S.
        • Gropman A.L.
        • et al.
        Pediatric Neurology Principles and Practice.
        6th edition. Elsevier Inc., 2017: 1725-1728
        • Scotti G.
        • Gerevini S.
        Diagnsis and differential diagnosis of acute transverse myelopathy. The role of neuroradiological investigations and review of the literature.
        Neurological Sciences. 2001; 22: S69-S73https://doi.org/10.1007/s100720100038
        • Waldman A.T.
        • Gorman M.P.
        • Rensel M.R.
        • Austin T.E.
        • Hertz D.P.
        • Kuntz N.L.
        Management of pediatric central nervous system demyelinating disorders: Consensus of United States neurologists.
        J Child Neurol. 2011; 26: 675-682https://doi.org/10.1177/0883073810395141
        • Brenton J.N.
        • Banwell B.L.
        Therapeutic approach to the management of pediatric demyelinating disease: Multiple sclerosis and acute disseminated encephalomyelitis.
        Neurotherapeutics. 2016; 13: 84-95https://doi.org/10.1007/s13311-015-0396-0
        • Ghezzi A.
        • Amato M.P.
        • Makhani N.
        • Shreiner T.
        • Gärtner J.
        • Tenembaum S.
        Pediatric multiple sclerosis.
        Neurology. 2016; 87: S97-S102