Managing pediatric-onset multiple sclerosis in an austere setting: A case report

Background

Pediatric-onset multiple sclerosis (POMS) is the most common demyelinating disease in children with an incidence of 0.05 to 2.85 per 100,000 children and an overall prevalence of 0.69 to 26.92 per 100,000 children. ¹ Although the progression of POMS is slow compared to adult-onset multiple sclerosis, pediatric patients ultimately have earlier disability and worse cognitive impairment.^2,3 Therefore, it is critical to determine an effective treatment course for young patients presenting with POMS. ²

The McDonald criteria, which is a guideline to diagnose Multiple Sclerosis (MS) as discussed by the International Pediatric Multiple Sclerosis Study Group, was updated in 2017 empowering physicians to diagnose pediatric patients following just one demyelinating event by utilizing MRI with contrast and lumbar puncture to assess for oligoclonal bands.^4,5 An earlier diagnosis enables patients to initiate long-term disease-modifying therapy (DMTs) sooner, which is believed to decrease the risk of developing persistent disability. ⁶

Acute management of MS is directed toward first confirming that symptoms are attributable to a new lesion with an MRI and subsequently treating with high-dose steroids. Long-term management of MS aims to reduce relapses and disability progression while detecting and providing support for cognitive and/or psychological issues. ⁷ Surveillance treatment typically involves a brain (and sometimes spinal) MRI 3–4 months after initiation of immunomodulatory therapy and then every 6–12 months after to evaluate for new lesions indicating treatment failure. ⁸ Clinical tools can assess for deterioration in gross motor skills, fine motor skills, and attention/processing speed. Additionally, Optical Coherence Topography evaluates optic nerve health. A multidisciplinary team consisting of physical therapists, social workers, nutritionists, neuropsychology, and school liaisons work to optimize care for patients with POMS. ⁸ These resources are largely inaccessible in austere settings requiring patients with MS to be close to urban locations with specialists who can manage therapies.

Diagnosis, initiation of treatment, and surveillance for patients with POMS are resource-intensive. ⁹ For children who do not have adequate access to care, delays in diagnosis and therapy can result in worse long-term outcomes. Here, we present a case of a child with POMS in an austere area where adequate acute and chronic management was achieved through a combination of synchronous and asynchronous telemedicine and a collaborative effort with the local pediatrician. This study illustrates the challenges in managing MS in a pediatric patient in a remote setting and the evolving role of telemedicine in the treatment of POMS.

Case

An 11-year-old boy with POMS located in Guam presented to Navy Hospital (NH) Guam with acute left hemiparesis. The patient was previously managed at an academic hospital in California where he was diagnosed with Clinically Isolated Syndrome following progressive fatigue that culminatied in a flare with left hemiparesis and was treated with methylprednisolone. A subsequent MRI one year prior to assessment in Guam showed new interval lesions confirming the diagnosis of POMS. Although disease-modifying treatment had been discussed, the patient was unable to obtain this medication prior to moving to Guam due to family circumstances.

At the time of the presentation in Guam, local providers were without immediate access to an MRI, therefore, were unable to diagnose a flare or rule out an alternative diagnosis. The local Navy Pediatrician reached out to Tripler Army Medical Center (TAMC) child neurology located in Hawaii via pager and conversations initiated via telephone. Medical evacuation (MEDEVAC) was offered and relocation to an area with more comprehensive Child Neurology services was recommended; however, evaluation of the patient outside of Guam was not feasible. TAMC Child Neurology recommended treating the patient for presumptive POMS flare with corticosteroids and to repeat serologic mimicker screening for sarcoidosis, lupus, and Sjogren disease because prior laboratory studies were not readily available. Two days later, a cervical MRI showed a new enhancing lesion confirming the diagnosis of a POMS flare.

Following the acute flare and while awaiting MEDEVAC for in-person evaluation, NH Guam Pediatrics, TAMC Pediatric Neurology and TAMC Pediatric Cardiology collaborated via the Global Teleconsultation Portal (GTP), an asynchronous telecommunication system available to military physicians to discuss patient care in the most remote settings, to plan long-term management of POMS in the resource-limited location of Guam. NH Guam Pediatrics was able to successfully obtain fingolimod per neurology recommendations. Fingolimod was initiated during an inpatient admission while the patient was monitored on continuous telemetry for bradycardia.

NH Guam Pediatrics arranged screening laboratory studies including a complete blood count, varicella titers, surveillance imaging, and surveillance ophthalmology assessments per subspecialty recommendations. NH Guam Pediatrics also plans to provide regular depression screening, regular vitamin D levels, and will coordinate timed walk tests with local physical therapy. The local school has the capability of offering regular physical therapy and occupational therapy, both of which the patient would benefit from. The patient already had an Individualized Treatment Plan (ITP) due to slowness in processing information. Consideration for the use of Ampyra or Modafinil will be a collaborative discussion between Pediatrics, Behavioral Health, Child Neurology, and Physical Therapy. Written informed consent was obtained from the patient and parent regarding the case report.

Discussion

This case illustrates the nuances of both the acute and chronic management of POMS in an austere setting. Standard-of-care treatment of MS flares involves confirmation of a new demyelinating lesion with MRI and a comprehensive neurologic examination prior to initiation of high-dose steroids, which are ideally initiated within 24 hours of presentation. For our patient in Guam, a POMS flare could not be diagnosed via MRI for at least 24–72 hours after presentation due to limited resources. Based on the available information collected from patient history, the local pediatrician's neurologic exam, blood tests, and discussion with the patient and his family, it was decided that the most reasonable course of action was starting IV methylprednisolone without imaging confirmation. While there is the potential that IV methylprednisolone could be utilized in the setting of a pseudo-flare, there is the possibility that IV methylprednisolone could cause harm in a coincidental condition such as stroke or abscess. In this setting, authors argued that the low likelihood of the patient presenting with a comorbid condition coupled with the benefit from early flare treatment with methylprednisolone justified empiric steroid use.

Chronic management of POMS in Guam was challenging due to the logistics in obtaining DMTs, executing the screening and monitoring required during the initiation of a DMT, and coordination of ongoing surveillance for both sequelae of POMS and adverse effects of fingolimod. Fingolimod is one of only two DMTs that has undergone Phase III trials and is approved by regulatory agencies for patients with POMS. In 2018, Chitnis et al. showed that patients 10–17 years old with POMS who received oral fingolimod had significantly less enlarging or new lesions on T2-weighted MRI and less relapses compared to patients who received intramuscular interferon beta 1a. ¹⁰ Interestingly, fingolimod was one of three DMTs requested to be a part of the World Health Organization Essential Medicines List.^10,11 Despite the need for intensive initial cardiac monitoring and interval serologic and ophthalmologic screening, fingolimod was the most appropriate initial DMT in this case.

GTP is a military-specific asynchronous physician-to-physician consulting platform that enables virtual collaboration for complex cases in austere environments. In the field of neurology, telemedicine is becoming more accepted and is frequently utilized for follow-up encounters, for asynchronous interpretations of diagnostics, such as EEGs, and more recently, for telestroke in the acute setting. One telehealth platform targeting pediatric patients illustrated that their two most utilized specialties were behavioral health and neurology. ¹² During the COVID-19 pandemic, there was a large increase in telemedicine utilization among neurologists treating MS patients; overall, healthcare providers reported satisfaction with these encounters. ¹³ Telemedicine has also been shown to support patients with MS in physical therapy via telerehabilitation and managing the psychosocial implications of living with MS through a Digital Emotional Health Tool.^14–16

In this case, GTP enabled the child neurologist to take inventory of available services to support a POMS case in Guam and to collaborate with local pediatrics to determine the best course of treatment. While there is not a clear substitute for in-person child neurology evaluations as part of a multidisciplinary team, through a combination of synchronous telehealth appointments and asynchronous coordination via GTP, child neurology care, physical and occupational therapy, behavioral health screening, imaging screening, cognitive screening, and an emergency action plan were achieved.

Conclusion

This case illustrates the challenges of remote management of POMS and how a combination of synchronous and asynchronous telemedicine was able to achieve a satisfactory treatment plan in a resource-limited setting. The GTP system was especially effective at enabling multidisciplinary collaborative care and overcoming the severe lack of child neurology care in Guam. Though satisfactory medical care was achieved in this case, it highlights a glaring disparity in access to pediatric subspecialty care worldwide. While the benefits of teleneurology have been illustrated, the GTP system serves as a unique model for effective physician-to-physician asynchronous collaboration. A similar program expanded beyond military use may prove to be a viable way of overcoming the healthcare disparity inherent to austere medicine.