A Comprehensive Management of Devic's Disease: A Pediatric Case Study

Devic's disease, also known as neuromyelitis optica (NMO), is an uncommon autoimmune condition that affects the optic nerves and spinal cord. It is characterized by recurrent optic neuritis and myelitis, which can cause paralysis and visual impairment. Because NMO mimics multiple sclerosis, diagnosing it is difficult and necessitates particular testing, such as magnetic resonance imaging (MRI) and aquaporin-4 antibody detection. Patients with NMOs are susceptible to severe, erratic episodes that can result in rapid impairment. As such, timely and efficient therapy with immunosuppressive medicines and continued supportive care are crucial. Improving mobility, strength, coordination, and quality of life while treating the functional deficiencies associated with NMOs requires the use of physiotherapy. This case study emphasizes how crucial it is to manage a young NMO patient using a multidisciplinary strategy in order to maximise results. This case report discusses a 16-year-old male presenting with a sudden onset of balance impairment, slurred speech, difficulty walking and breathing, and weakness in limbs, with the right side more affected. Over three months, he experienced increasing eyesight issues, fatigue, tremors during activities of daily living, difficulty swallowing, and night cramps. Diagnostic investigations including MRI, angiography, visual evoked potentials (VEP) study, and cerebrospinal fluid (CSF) analysis confirmed demyelinating changes consistent with NMO, also known as Devic's disease. The patient received management with steroidal medications, immunosuppressants, and plasma therapy, along with physiotherapy rehabilitation. The physiotherapy protocol aimed to address muscle weakness, coordination impairment, balance issues, fine motor deficits, fatigue, sensory impairment, and dependence on activities of daily living. Motor, sensory, and cranial nerve assessments were conducted, revealing impairments consistent with NMO. Outcome measures pre- and post-intervention showed improvements in functional independence, balance, and fatigue severity. The medical management included a combination of medications and investigations to manage NMO symptoms and monitor disease progression. The physiotherapeutic approach employed a multidisciplinary strategy focusing on education, exercise, and functional tasks to improve the patient's quality of life and independence.


Introduction
Devic's disease, also known as neuromyelitis optica (NMO), is a rare autoimmune disorder primarily affecting the optic nerves and spinal cord [1,2].It is characterized by inflammation and demyelination of these central nervous system structures, leading to a range of neurological symptoms [3,4].It frequently manifests as transverse myelitis, which causes limb weakness, sensory loss, and bladder/bowel problems, as well as optic neuritis, which causes vision loss.The prevalence of NMO is estimated to be around 1-2 individuals per 100,000 in the general population, making it less common than conditions like multiple sclerosis (MS) [5].The exact cause of NMO is not fully understood, but it is thought to involve an autoimmune response targeting a protein called aquaporin-4, which is found in the optic nerves and spinal cord [6,7].
Diagnostic investigations for NMO include magnetic resonance imaging (MRI) scans, which may reveal characteristic features such as myelitis, and blood tests to detect specific antibodies like aquaporin-4 [8].Lumbar puncture (cerebrospinal fluid (CSF) analysis) may also be conducted to assess for abnormalities indicative of NMO [9,10].Signs and symptoms of NMO can vary but often include optic neuritis, leading to visual disturbances, and transverse myelitis, resulting in limb weakness, numbness, and loss of bladder and bowel control [11,12].Other symptoms may include slurred speech, balance difficulties, and pain.NMO typically follows a relapsing-remitting course, with periods of exacerbation and partial or complete recovery [13,14].Corticosteroids, plasma exchange for acute episodes, and immunosuppressive medication for longterm care are all included in the treatment plan.Enhancing mobility, strength, coordination, and general quality of life with physiotherapy necessitates a multidisciplinary approach for the best possible patient treatment.
Physiotherapy plays a crucial role in managing NMO by addressing functional deficits and enhancing the patient's overall quality of life [15,16].Physiotherapeutic interventions focus on improving mobility, balance, and coordination [17].Specific exercises are designed to address muscle weakness, spasticity, and impaired gait.Moreover, physiotherapists employ neurorehabilitation techniques to enhance motor control and re-educate neural pathways [18,19].This comprehensive approach helps individuals with NMO regain independence in daily activities, minimize the impact of symptoms, and prevent complications associated with immobility.Regular physiotherapy sessions contribute to a holistic management strategy for individuals living with NMO.

Case Presentation
We are discussing a case report representing a 16-year-old male who presented in October 2023 with complaints of sudden balance impairment, slurred speech, and difficulty in walking and breathing.He reported weakness in his limbs, with the right side being more affected.Additional history included a recent increase in myopia, fatigue, the occurrence of tremors during activities of daily living (ADLs), difficulty swallowing, and night cramps.All these symptoms manifested over the span of three months.
Upon admission to the neurology department, the following investigations were conducted: MRI, angiography, a visual evoked potentials (VEP) study, and CSF analysis.These investigations revealed demyelinating changes.The diagnosis was confirmed through NMO and myelin oligodendrocyte glycoprotein (MOG) antibody tests.The patient was managed with steroidal medications, immunosuppressants, and plasma therapy.
Simultaneously, a physiotherapy rehabilitation program was initiated.A thorough assessment was conducted, and a physiotherapy protocol was planned accordingly.The assessments included evaluations of reflexes, muscle strength (manual muscle testing (MMT)), cranial nerve function, and sensory function.The detailed results are presented in the tables below.

Motor assessment
Prior to conducting reflex examinations and MMT, the patient's consent was obtained (Table 1 and Table 2

Cranial nerve assessment
A thorough cranial nerve examination was done which revealed impairment of the optic nerve.All other cranial nerves are intact.

Sensory assessment
Patient consent was obtained prior to assessing sensations, as indicated in Table 3.

Timeline of events
The patient underwent different investigations, and the timeline is mentioned in Table 4, to simplify the occurrence of events.

Investigation and medical management
The investigations the patient went through are mentioned with the drugs given on the day of screening in Table 5.

Physiotherapeutic management
A comprehensive physiotherapy protocol was developed based on the initial assessment, focusing on muscle strengthening, coordination, balance, fine motor skills, and endurance.Detailed intervention is mentioned in Table 6.

Outcome measures
The outcome measures were evaluated at the beginning and at the end of the physiotherapy treatment period as mentioned in Table 7.

Discussion
A 16-year-old male patient with NMO is described in this case study.He presented with sudden balance impairment, slurred speech, breathing and walking difficulties, limb weakness (particularly on the right side), vision issues, exhaustion, tremors, difficulty swallowing, and night cramps.His symptoms worsened over the course of three months, leading to a hospital stay.There, diagnostic tests such as an MRI, a VEP study, and a CSF analysis showed NMO.In addition to receiving immunosuppressants, steroids, and plasma therapy for medical care, the patient also participated in an organised physical therapy rehabilitation program.
Nechemia et al. conducted a study focusing on the efficacy of multidisciplinary inpatient rehabilitation for individuals diagnosed with NMO, comparing their outcomes with patients diagnosed with MS.The objective was to evaluate the extent to which existing rehabilitation protocols designed for MS could be adapted and effective for NMO patients [20].The study analyzed data from 15 NMO and 32 MS inpatients, assessing various parameters to gauge the effectiveness of rehabilitation interventions.One notable finding was the longer length of stay required for NMO patients compared to those with MS.Despite this difference, both groups experienced significant benefits from the rehabilitation program.Specifically, NMO patients demonstrated greater improvements in Functional Independence Measure (FIM) scores compared to MS patients.The FIM scores serve as a comprehensive measure of a patient's ability to perform ADLs and their level of independence.The higher improvement in FIM scores among NMO patients suggests that the rehabilitation interventions were particularly effective in addressing the functional deficits associated with NMO [20]. Furthermore

Conclusions
This case report highlights the importance of a comprehensive multidisciplinary approach in managing NMO.Early diagnosis and prompt initiation of medical management, including immunosuppressive therapy, are crucial in controlling disease activity and preventing relapses.Physiotherapy plays a vital role in addressing functional deficits and enhancing the patient's overall quality of life.Tailored physiotherapeutic interventions focusing on muscle strengthening, coordination, balance, fine motor skills, endurance, and sensory awareness contribute to improving mobility and independence in ADLs and reducing the impact of symptoms such as fatigue.Regular monitoring of outcomes and adjustment of interventions based on the patient's progress are essential for optimizing rehabilitation outcomes in individuals with NMO.
The 16-year-old male patient with NMO showed significant improvements following a structured four-week physiotherapy rehabilitation program.Initially presenting with severe balance impairment, slurred speech, limb weakness, vision problems, fatigue, tremors, and difficulties in walking, breathing, and performing ADLs, the patient underwent a comprehensive intervention plan targeting these impairments.Postintervention assessments revealed notable enhancements in muscle strength, coordination, balance, and overall mobility.The FIM score increased, indicating greater functional independence, while the Pediatric Balance Scale (PBS) score improved, reflecting better balance and stability.The patient's endurance and fine motor skills also saw significant progress, leading to increased independence in ADLs and an overall improvement in quality of life.This case underscores the effectiveness of tailored physiotherapy interventions in managing NMO, demonstrating that a multidisciplinary and individualized approach can yield substantial functional gains and enhance patient outcomes.

TABLE 6 : Physiotherapeutic management
ROM: range of motion; ADLs: activities of daily living

TABLE 7 : Outcome measures taken pre-and post-intervention
FSS: Fatigue Severity Scale; FIM: Functional Independence Measure; PEDI: Pediatric Evaluation of Disability Inventory; PBS: Pediatric Balance Scale , the study observed lower scores on the Expanded Disability Status Scale (EDSS) at discharge for NMO patients compared to MS patients.The EDSS is a widely used scale to quantify disability in MS, assessing various functional systems affected by the disease.The lower EDSS scores in NMO patients indicate less disability and better functional outcomes following the rehabilitation program.These findings underscore the potential for significant functional gains in NMO patients through tailored rehabilitation interventions.Despite the inherent challenges and complexities associated with NMO, such as optic nerve involvement and spinal cord inflammation, the study suggests that rehabilitation strategies developed for MS can be adapted and effectively applied to NMO with appropriate modifications.The study by Nechemia et al. emphasizes the importance of individualized rehabilitation approaches that address the specific needs and challenges of NMO patients.By tailoring interventions to target the unique symptoms and impairments associated with NMO, healthcare providers can optimize outcomes and enhance the quality of life for individuals living with this rare autoimmune disorder.