1Georges Saab, 2Nabil El Ayoubi, 2Samia Khoury
1University of Toronto-BARLO Multiple Sclerosis Center, Toronto, Canada; 2Nehme and Therese Tohme Multiple Sclerosis Center at the American University of Beirut, Beirut, Lebanon
Multiple Sclerosis (MS) is an autoimmune demyelinating disease that affects the central nervous system of individuals with peak incidence between the ages of 20-40. A distinct form of MS initially presents in the pediatric population (less than 18 years of age) with the same clinical presentation but a different disease course.
Pediatric Onset Multiple Sclerosis (POMS) occurs in 3-10% of MS cases worldwide with most patients (80-85%) occurring at a peripubertal age. Clinically these patients were found to have a more destructive disease with more aggressive and more frequent attacks throughout their disease course. Risk factors in multiple studies found them to be equivalent to the risk factors in their adult counterparts including low vitamin D levels, smoking, and EBV infections.
The clinical presentations or first clinical relapses in POMS are essentially the same as in adults including optic neuritis, sensory, motor, brainstem, and cerebellar manifestations with an emphasis on brainstem attacks being more highly associated with the development of MS in this population. Optic neuritis as a first clinical attack was found to be less prevalent in the POMS patients than as a first clinical attack in adults with MS. The individual attacks in these patients exhibit more extensive demyelination depicted in their more severe clinical presentation, but also have a better recovery with most patients having a complete recovery with treatment.
POMS patients have been classified in other regions with studies on their epidemiology, phenotype, and disease classification. They have yet to be classified in Lebanon where no study exists to date where the POMS patients are classified. Such a study can help clinicians further confirm the diagnosis in their patients, predict the disease course, and benefit from treatment data to better treat their patients.
Material(s) and Method(s):
All patients presenting to the multiple sclerosis center for the first time younger than 18 years of age who have consented to the AMIR study
Cross sectional (at onset) and longitudinal (retrospective/at last follow up)
Sample Size will depend on the number of patients presenting under the age of 18 from the start of the AMIR study till 15 February 2020
Data was collected from the redcap database of the AMIR study
Data was explored cross sectionally for eligible patients and means, standard deviations, and proportions were explored as appropriate. At last follow up, proportions of patients with certain characteristics was explored (stability of disease including imaging and clinical). Correlations between different clinical predictors and physical disabilities were explored.
62 patients identified, 54 with the relapsing remitting phenotype, 4 with a radiologically isolated syndrome, 2 with neuromyelitis optica spectrum disorder, and 2 with MOG antibody associated disease.
- Mean age at symptom onset (SD): 14.37 (2.64) years
- Mean age at diagnosis (SD): 15.4 (1.9) years
- Female sex (Male): 66.1 (33.9) %
- Median number of attacks before first visit (range): 1 (0-4)
- Mean follow up time (SD): 4.08 (2.26) years (Range=0-9)
- Presenting Symptoms:
- 27.8% Sensory
- 22.2% Motor
- 29.5% Optic Neuritis
- 46.3% Brainstem/Cerebellar
- 1.9% Bowel/Bladder
- 22.2% with a positive family history of MS
- Median EDSS at onset (range): 1.5 (0-6)
- Mean 25-Foot Walk Time at onset (SD): 4.3 (1.6) seconds
- Mean Single Digit Memory Test (SD): 52.89 (13.9)
- Imaging Characteristics:
- Brain lesion load:
- Mild in 17.6%
- Moderate in 45.1%
- Severe in 17.6%
- Spinal Cord lesion load:
- Mild in 47.2%
- Moderate in 22.2%
- Severe in 13.9%
Subgroup of patients with a positive family history of MS did not have significant differences in presenting symptoms, number of relapses, lesion load, severity of disease, or age at onset of diesase.
Treatment with Disease Modifying Therapies:
- 35.3% were on Interferons
- 23.7% on Fingolimod
- 13.5% on Natalizumab
- 10.9% on Dimethyl Fumarate
- 9.4% on Rituximab
- 6.8% on Teriflunomide
- 0.4% on Azathioprine
Patients seemed similar to their adult counterparts in most disease characteristics except for presenting symptom
Good response to treatment achieved and improvement of EDSS with a long follow up time
Limited by the small number of patients and MRI parameters