Interdisciplinary, Intensive, Activity-Based Treatment for Intrauterine Spinal Cord Infarct: A Case Report

The child was delivered by caesarean section at 35 weeks due to breech presentation and loss of fetal movement. She was born apneic without response to tactile stimulation. Her brain magnetic resonance imaging (MRI) was normal, and spine MRIs were consistent with SCI from the cervicomedullary junction to T2 with sparing at C3-C5. She was initially diagnosed with in utero transverse myelitis and received IV steroids and IVIg. At 1 month old, she was deemed medically stable and discharged to home; however, she was unable to perform the expected gross motor functions of a 1 month old. Following discharge, she presented for two brief hospitalizations for steroid-induced gastritis, failure to thrive, and UTIs. At 9 months, a follow-up MRI showed spinal cord volume loss from C4-T1 and she was evaluated in a neuroimmunology clinic. Based on prenatal history, MRIs, electromyelogram, nerve conduction study, and laboratory testing, her diagnosis was changed at that time to IUSCI.

At 11 months old, she could not perform any expected developmental milestones, so she began a 4-week course of inpatient ABT 6 days per week, 2 to 3 hours per day. Upon inpatient discharge, her caregivers had been trained in a home program to encourage developmental milestone achievement; however, she still required assistance with performing all developmental skills. At 12 months, she enrolled in outpatient therapy twice weekly for 4 months to promote motor skill acquisition and refine the home program. At 15 months, she transitioned to weekly clinic-based visits with an extensive home program for the rest of the week. Outpatient weekly, intensive interventions (5 hours per visit, including 2 hours of OT, 2 hours of PT, and 1 hour of AT) were complemented by in-home interventions carried out by the family, home-based therapists, and other caregivers. Activities practiced at nearly all visits and the relationship of those activities to the ABT principles are outlined in Table 1. A total of 146 OT visits, 120 PT visits, and 82 AT visits were completed between June 2015 and January 2018. Frequency and duration of the visits were based on available literature for similar conditions29,33,35–44 as literature specific to IUCSI is very limited. The child's tolerance for therapy and the family's schedule also influenced the plan of care.

The child was routinely assessed using the Gross Motor Function Measure-88 (GMFM-88),45 which is comprised of 88 gross motor skills that should be attained by 5 years old in typically developing children. The child's first assessment was performed upon inpatient admission in April 2015, and the most recent was conducted January 2018. She has shown steady progress on this measure, except for two instances of decline (see Figure 1). In one of those, the assessment was performed by a different evaluator; the other was after a 2-month break from therapy due to hospitalization for respiratory failure. Most notably, she has made progress with neck strength and control, rolling, sitting balance, and prone scooting. She has also demonstrated progress with transitions from sitting to supine but is limited due to upper extremity weakness.

The child has also been evaluated using the Physical Abilities and Mobility Scale (PAMS).46 This scale is a 20-item measure created to complement the WeeFIM and is geared for children aged 2 years and up. Children are rated on a scale of 1 to 5 to determine recovery after injury, level of caregiver assistance, and tolerance to activities (ie, positioning, wheelchair use). It is primarily used in inpatient settings but is being developed for outpatient settings. The child is scored out of 100, with 0 being the most impaired and 100 being no impairments. Scoring allows for the use of compensatory movements to perform the tasks. Over 2 years, the child demonstrated improvements in the following domains: tolerance to positioning, head control, trunk control, rolling, transitioning supine/sit, and supported standing (see Figure 2). The PAMS was performed twice on inpatient and twice on outpatient. The large gap in time is due to the measure not initially being utilized in outpatient settings.

Clinically, she developed lower extremity (LE) sensory responses to tactile stimulation, volitional stepping, trunk and neck strength sufficient to support herself against gravity for up to 40 seconds, and upper extremity (UE) bilateral coordination for developmental play and fine motor skills. She achieved developmental milestones in functional mobility (ie, rolling supine to side-lying, sitting 5 minutes wearing a dynamic trunk orthosis [SPIO vest] with minimal assistance), social interactions (attending preschool with typically developing peers using her power wheelchair with supervision, playing hide-and-seek with friends), and UE function (self-feeding of finger foods and using a spoon with her dominant hand). She demonstrates spontaneous stepping with body weight supported treadmill training (BWSTT) combined with transcutaneous spinal cord stimulation (TSCS).47 She began utilizing FES cycling and demonstrates reciprocal LE cycling activity. She has improved bladder sensation, which will be useful for continence as she can now recite the steps for intermittent catheterization (IC) and is beginning to work on opening packages for ICs. An additional goal of OT has been to assess, fabricate, and modify orthotics for her UEs to prevent contractures and increase function. While ABT promotes optimal neuromotor function through neuroplasticity, maintaining range of motion complements this goal and will allow for options like tendon transfer surgery should that be necessary to promote additional UE function in the future.

The child's parents and caregivers were trained in the implementation of activities (ie, prone mobile stander, FES, functional mobility, use of splints/orthoses) to carryover concepts from the weekly sessions into daily life at home, in her community, and at her preschool. The family demonstrated exemplary dedication to the program, averaging 10 hours per week of home program activities in addition to therapy visits.

Evidence regarding IUSCI is very limited. Based on our review of the literature, we believe this may be the youngest reported case in which an ABT approach has been initiated. Children have developing nervous systems and are particularly suited to benefit from the technologies and advances utilized in an ABT paradigm.48 Young children, especially infants, have shown better recovery than expected relative to the severity of their SCI, and this recovery can continue over many years due to increased neuroplasticity in the age range and, in part, due to their developmental processes.49 

Infants have a very immature central nervous system that is undergoing reorganization and modification. This may mask the level of their injury due to the decreased myelination that slows signals from the brain to the spinal cord.50 As their nervous system develops, we may find they have undamaged areas of the spinal cord that were masked by their immaturity. It has been determined that young children have excessive neuronal growth and connections. These connections will be naturally pruned from the central nervous system over time, in a nonjudgmental fashion.51 This means we can take advantage of the excess neurons for the child's benefit.

We are also limited in our ability to fully assess children's level of function due to their limited ability to follow directions. Evaluations in this population are further impeded by a lack of appropriate outcome measures. For example, based on age, standardized OT assessments were not completed for this child due to the lack of available normative data. As for measuring gross motor skills, the GMFM-88 has only been validated for children with cerebral palsy and Down syndrome. The mechanism of this child's injury was not cerebral; however, it was acquired just before birth and impacts performance of the items tested with the GMFM-88. Therefore, we have found it useful to track her individual progress over time in the absence of comparable data. Specific developmental measures do not exist for children with IUSCI. Despite these challenges, clinical observations described above are sufficient to detail functional progress.

The child in this case has actively participated in ongoing therapy consistently for 3 years, demonstrating steady improvements in functional mobility and social interaction, as well as avoiding multiple hospitalizations which are usually seen in this population.52,53 Over 3 years of ABT, she experienced only one hospitalization. This was due to respiratory syncytial virus and pneumonia and resulted in a 2-month interruption in ABT to allow her to recover. Upon return to ABT, it was noted that a significant decrease in trunk strength and endurance had occurred.

In this specific case, ABT did not restore function as IUSCI had impacted the child's baseline. Rather, the neurological and musculoskeletal system were trained to function as intended. While some compensatory aids were used, they were thoughtfully chosen to complement the ABT principles. For example, to participate in ABT the body must be free to move. However, this child could not achieve optimal trunk posture without support. A dynamic trunk orthosis was chosen over a rigid trunk orthosis to offer support while not completely restricting freedom of movement. Further, we acknowledged the need for short-term mobility and independence to promote social-cognitive development, so short-term goals were addressed (ie, use of wheeled mobility) while still incorporating ABT concepts such as TSP and MP.

This case supports the use of ABT to achieve constantly evolving therapeutic and functional goals. We believe that the gains made by the child are attributable to a combination of factors, not least of which is the consistent commitment of her family who regularly travelled to therapy sessions and performed the home program. This case provides a model to address therapy goals by coupling weekly intensive treatment with a home program to demonstrate meaningful progress for this child and her family. While IUSCIs are rare, the model implemented suggests possibilities for other children with early-onset neurological insult. Establishing a balanced, sustainable therapy program that focuses on improving function not only through compensation is vitally important to facilitate adaptive motor patterns in young children.