It is the most frequent acquired demyelinating disease of the central nervous system.

Estimated incidence: 0,13-0,6/100.000/year. Approximately 10 % of the  adults suffering from multiple sclerosis have their first attack before 18 years of age. An onset before 10 years of age occurs in 20 to 30 % of the pediatric cases.

Before puberty (10 years of age) there is a slight male preponderance. After puberty, women are more frequently affected.

Possible contributing factors have been identified: HLA-DRB1*15:01 group, history of EBV infections, low vitamin D blood levels.

The first attack may present as:

-        uni- or bilateral optic neuritis: sudden decrease in visual acuity, painful eye movements, loss of color vision, central scotoma

-        complete or partial transverse myelitis

-        disseminated encephalomyelitis, often preceded by a viral disease or vaccination, especially before the age of 10 years

-         mono- or polyfocal nervous deficits: urinary disorders, loss of sensitivity

These clinical manifestations can be combined.

MRI criteria: at least 2 of the 3 following criteria: 5 lesions or +; more than 2 periventricular lesions, 1 lesion in the brainstem; simultaneous presence of lesions fixing or not, gadolinium. In children, lesions are most often infratentorial and larger with perilesional edema.

Children have a greater relapse rate than adults, especially at the beginning of the disease: 75 % have a relapse in the year following the first attack. In 50 % of cases, entry in the secondary progressive form of the disease occurs approximately 20 years after the start of the disease.

In children, in contrast to the adult, multiple sclerosis causes cognitive disorders including at speech disorders and the visiospatial memory problems. Depression and fatigue are common in adolescence.

Treatment: when the diagnosis of multiple sclerosis is clearly established, the same treatments are used as in adults, in high doses:

-        fingolimod: a sphingosine-1-phosphate receptor modulator

-        dimethyl fumarate: protects neurons from oxidative stress

-        teriflunomide: an immunomodulator with anti-inflammatory properties that selectively and reversibly inhibits dihydroorotate dehydrogenase, a mitochondrial enzyme required for de novo pyrimidine synthesis

MRI and the clinical evolution are important to make the differential  diagnosis  with other neurological conditions (CADASIL, leukodystrophy, deficiency in vitamin B12, etc) or systemic (systemic lupus erythematosus, lyme disease, sarcoidosis, lymphoma, etc).

Other acquired pediatric demyelinating diseases of the central nervous  system can also present with relapses: they are,

-        the disease linked to the presence of anti-AQP4 (aquaporin A) antibodies

-        the disease linked to the presence of anti-MOG (myelin oligodendrocyte glycoprotein) antibodies

-        the form without any known antibodies

Anesthetic implications:

any perioperative stress should be avoided because stress favors relapses; the anesthetic management should be adapted according to the lesions (dysautonomia ? obstructive sleep apneas ?) and to the current treatment (corticotherapy ? monoclonal antibodies ?); there is no problem performing peripheral nerve blocks, but given the high risk of relapse, caution should be exercised with perimedullar blocks (an epidural with low doses of local anesthetics should be preferred to spinal anesthesia) even if no risk of relapse has been described in adults when the disease is stabilized; continous IV lidocaine infusions should probably be avoided as blockade of the Na channels could have a facilitating effect for relapse. N2O should be used with caution. Regarding baclofen (oral or intrathecal), dose adaptation  should be discussed with the patient’s neurologist.

References : 

-         Duignan S, Brownlee W, Wassmer E, Hemingway C, Lim M, Cicarelli O, Hacohen Y.
Paediatric multiple sclerosis : a new era in diagnosis and treatment.
Develop  Med Child Neurol 2019 ; 61 : 1039-49

-        de Maere d’Aetrycke O, Dubuisson N, Marta M, Gnanapavan S et al.
Anesthetic management of people with multiple sclerosis.
Acta Anaesth Belg 2019 ; 70, S1, 1-11.

Updated: March 2024