N.F. AKHMETGALEEVA¹˒ ², O.V. LYUTOV ², K.Z. BAKHTIYAROVA²

 ¹Republican Children’s Clinical Hospital, Ufa

²Bashkir State Medical University, Ufa

  Contact details:

Akhmetgaleeva N.F. — neurologist, postgraduate student of the Department of Neurology

Address: 98 Stepana Kuvykina St., 450106 Ufa, Russian Federation, tel.: +7-347-282-25-23, e-mail: nailya_9292@list.ru

The article presents an analysis of data on children with a first-time demyelinating episode who were initially diagnosed with ADEM. In most cases, the diagnostic search was complicated by an atypical clinical picture and imperfections in diagnostic methods. A thorough examination of patients is necessary, as well as differential diagnosis with other CNS diseases, timely pathogenetic therapy to prevent residual neurological deficit, and long-term dynamic observation of patients.

The purpose — to analyze the first demyelination episode in children, to assess the severity of the patient’s condition and remote outcomes.

Material and methods. Data of 19 patients observed at the Children’s Center for Neuropsychiatry and Epileptology of the Republican Children’s Clinical Hospital (Ufa) from 1994 to 2024. Statistical analysis was performed in Statistica 22.0.

Results. 19 children with a first demyelination episode were observed. Girls predominated among the patients. The first demyelination episode occurred more often in children of middle school age. In 10 patients, the first demyelination episode was preceded by a viral infection, in 3 children the fact of injury was recorded, in one child the disease was preceded by a DPT revaccination. In other patients, no factors triggering the disease onset were identified. Common infectious symptoms were often observed — 10 children; motor dysfunction was observed in 15 children, oculomotor disorders — 6, ataxia — 6, pelvic organ dysfunction — 4, epileptic seizures — 2, hearing and vision loss — 1, pseudobulbar disorders — 1. 11 children (57.8%) had only brain damage at their debut, 7 (36.8%) had brain and spinal cord damage, and 1 child (5.2%) had only spinal cord damage. In eight children, the initial diagnosis of ADEM was revised.

Conclusions. It is important to apply diagnostic criteria at the first demyelination episode to establish a correct diagnosis. In order to prevent the development of persistent neurological disorders, timely therapy is necessary.

Key words: acute disseminated encephalomyelitis, children, demyelination episode.

REFERENCES

1. Avdeeva V.E., Kotov A.S. Clinical polymorphism of acute disseminated encephalomyelitis: diagnosis, treatment, long-term prognosis. Lecture with description of clinical cases. Russkiy zhurnal detskoy nevrologii, 2023, no. 1, pp. 10–19 (in Russ.).
2. Averchenkov D.M., Volik A.V., Fominykh V.V. et al. Acute disseminated encephalomyelitis. Zhurnal nevrologii i psikhiatrii im. S.S. Korsakova, 2021, vol. 121, no. 11, pp. 119–128 (in Russ.).
3. Noorbakhsh F., Johnson R.T., Emery D. et al. Acute disseminated encephalomyelitis: clinical and pathogenesis features. Neurol. Clin, 2008, vol. 6 (3), pp. 759–780.
4. Tenembaum S.N. Acute disseminated encephalomyelitis. Handb. Clin. Neurol,2013, vol. 112, pp. 1253–1262.
5. Pohl D., Alper G., van Haren K. et al. Acute disseminated encephalomyelitis: Updates on an inflammatory CNS syndrome. Neurology, 2016, vol. 87 (9 Suppl 2), pp. 38–45.
6. Boesen M.S., Blinkenberg M., Koch-Henriksen N. et al. Implications of the International Paediatric Multiple Sclerosis Study Group consensus criteria for paediatric acute disseminated encephalomyelitis: a nationwide validation study. Dev. Med. Child Neurol, 2018, vol. 60 (11), rr. 1123–1131.
7. Banwell B., Kennedy J., Sadovnick D. et al. Incidence of acquired demyelination of the CNS in Canadian children. Neurology, 2009, vol. 72 (3), pp. 232–239.
8. de Mol C.L., Wong Y.Y.M., van Pelt E.D. et al. Incidence and outcome of acquired demyelinating syndromes in Dutch children: update of a nationwide and prospective study. J. Neurol, 2018, vol. 265 (6), rr. 1310–1319.
9. Anilkumar A.C., Foris L.A., Tadi P. Acute disseminated encephalomyelitis. In: StatPearls. Treasure Island: StatPearls Publishing, 2022, available at: https://www.ncbi.nlm.nih.gov/books/NBK430934/
10. Paolilo R.B., Deiva K., Neuteboom R. et al. Acute disseminated encephalomyelitis: current perspectives. Children (Basel), 2020, vol. 7 (11), r. 210.
11. Massa S., Fracchiolla A., Neglia C. et al. Update on acute disseminated encephalomyelitis in children and adolescents. Children (Basel), 2021, vol. 8 (4), r. 280.
12. Krupp L.B., Tardieu M., Amato M.P. et al. International Pediatric Multiple Sclerosis Study Group. International Pediatric Multiple Sclerosis Study Group criteria for pediatric multiple sclerosis and immune-mediated central nervous system demyelinating disorders. Mult. Scler,2013, vol. 19 (10), rr. 1261–1267.
13. Thompson A.J., Banwell B.L., Barkhof F. et al. Diagnosis of multiple sclerosis: 2017 revisions of the McDonald criteria. Lancet Neurol, 2018, vol. 17 (2), pp. 162–173.
14. Wang C.X. Assessment and management of acute disseminated encephalomyelitis (ADEM) in the pediatric patient. Paediatr. Drugs, 2021, vol. 23 (3), pp. 213–221.
15. Pohl D., Alper G., van Haren K., Kornberg A.J., Lucchinetti C.F., Tenembaum S., Belman A.L. Acute disseminated encephalomyelitis: Updates on an inflammatory CNS syndrome. Neurology, 2016, vol. 87 (9 Suppl 2), pp. S38–S45.
16. Spalice A., Parisi P., Papetti L., Nicita F., Ursitti F., Del Balzo F. et al. Clinical and pharmacological aspects of inflammatory demyelinating diseases in childhood: an update. Current Neuropharmacol, 2010, vol. 8 (2), pp. 135–148.