Recovery of functional ambulation through stepwise rehabilitation treatment in a patient with encephalomyeloradiculoneuropathy: A case report

Abstract

Encephalomyeloradiculoneuropathy is a rare inflammatory disorder for which rehabilitation strategies and functional prognosis are not well established. This report describes the clinical course of a patient with severe lower-limb paralysis caused by encephalomyeloradiculoneuropathy. A man in his 50s developed a fever, impaired consciousness, and respiratory failure requiring mechanical ventilation. Based on the combined involvement of the central and peripheral nervous systems, a clinical diagnosis of encephalomyeloradiculoneuropathy was made. Rehabilitation treatment was initiated early during mechanical ventilation in the acute-care hospital, at a time when he required total assistance for ambulation. Despite immunotherapy, near-complete paralysis of the trunk and bilateral lower limbs persisted with orthostatic hypotension. Rehabilitation treatment was continued in acute-care and rehabilitation hospitals with monthly goal setting. At 8 months, he achieved independent outdoor ambulation.

Introduction

Encephalomyeloradiculoneuropathy (EMRN) is a rare inflammatory disorder of the central and peripheral nervous systems that often results in severe neurological deficits. ¹ However, previous reports have primarily focused on its immunological features and clinical spectrum,^1,2 with little attention to the impact of rehabilitation interventions on functional recovery. This lack of evidence limits clinicians’ ability to plan rehabilitation and predict functional prognosis.

This case report describes a patient with EMRN who achieved functional ambulation through continuous and stepwise treatment from an acute-care hospitalizationto rehabilitation hospital, accompanied by marked improvements in muscle strength and activities of daily living. It provides clinically relevant insights into rehabilitation planning for this condition.

Case description

The patient was a male mechanical engineer in his 50s with no major neurological history. He developed flu-like symptoms, a high fever, fatigue, and anorexia, and was admitted to a referring hospital with suspected meningitis/meningoencephalitis. His neurological status subsequently deteriorated, and he developed respiratory failure requiring mechanical ventilation.

The clinical course and neurological treatment timeline are summarized in Figure 1. During the acute phase, a neurological examination revealed a mild disturbance of consciousness, gaze-evoked nystagmus, mild upper-limb weakness, absent deep tendon reflexes in the lower limbs, and a distal-dominant sensory disturbance. Although he was successfully extubated, severe motor paralysis of the trunk and bilateral lower limbs subsequently became evident, accompanied by autonomic dysfunction, orthostatic hypotension, and a neurogenic bladder. Brain magnetic resonance imaging later demonstrated T2-weighted hyperintense lesions involving the bilateral basal ganglia, thalamus, midbrain, cerebral peduncles, pons, and medulla, with contrast enhancement of cranial nerves. Nerve conduction studies revealed A-waves and reduced F-wave variability in the tibial nerves, without significant conduction velocity slowing or amplitude reduction. Differential diagnoses, including infectious meningoencephalitis, Guillain–Barré syndrome, and other inflammatory neurological disorders, were considered. Based on the combined involvement of the central and peripheral nervous systems, the patient was clinically diagnosed with EMRN at the time of transfer to our hospital. He received immunotherapy, including intravenous corticosteroid pulse therapy, intravenous immunoglobulin, plasma exchange, cyclophosphamide, and azathioprine.

Figure 1.

Clinical course and neurological treatment timeline.

Figure 2 summarizes the longitudinal course of rehabilitation treatment and functional recovery. Rehabilitation treatment was initiated early during mechanical ventilation in the acute-care hospital. In the initial phase, the primary goals were prevention of disuse syndrome. Range-of-motion (ROM) exercises and bedside strengthening exercises were performed, followed by reclining wheelchair sitting and tilt-table standing as tolerated. Because orthostatic hypotension markedly limited mobilization, both lower limbs were wrapped with elastic bandages to prevent venous pooling, and pharmacological treatment, including an α1-adrenergic agonist, was initiated. At approximately 1 month after onset, the patient still required total assistance for most activities of daily living and ambulation. The total Functional Independence Measure motor score was 13 points, and the functional ambulation categories score was 0. At approximately 2 months after onset, consciousness and cognitive function improved, and deep tendon reflexes began to reappear in the lower limbs. Rehabilitation treatment progressed to edge-of-bed sitting, transfer practice, and more active strengthening exercises as orthostatic hypotension gradually improved. At approximately 3 months after onset, early motor recovery was observed in the trunk and lower limbs, enabling progression to more intensive standing training. A hip–knee–ankle–foot orthosis with a medial single hip joint (MSH-KAFO) was prescribed.^3,4 This orthosis stabilized the hip, knee, and ankle joints and restricted excessive degrees of freedom, enabling safe standing and gait training with parallel bars and therapist assistance despite profound weakness.

Figure 2.

Longitudinal course of rehabilitation treatment and functional recovery.

After transfer to a rehabilitation hospital, the amount of rehabilitation substantially increased. The primary goals were updated monthly according to neurological recovery. At approximately 4 months after onset, the focus was on improving trunk strength and trunk control during standing and gait. Gait training progressed from the parallel bars to walking with bilateral Lofstrand crutches while wearing the MSH-KAFO. However, moderate assistance was still required because of insufficient proximal lower-limb strength and a risk of forward falls. At approximately 5 months after onset, further improvement in trunk and proximal lower-limb strength allowed removal of the medial hip joint component, and training was continued using bilateral KAFOs. Repetitive peripheral magnetic stimulation was added to facilitate strengthening of antigravity muscles, especially the gluteus maximus and quadriceps muscles.^5,6 During this phase, lower-limb muscle strength improved markedly, and a supervised gait with bilateral KAFOs and bilateral Lofstrand crutches became possible. At approximately 6 months after onset, the rehabilitation focus shifted to knee control during gait. Although overall lower-limb strength improved only gradually, gait pattern learning progressed. The patient then advanced to short-distance walking using bilateral ankle–foot orthoses (AFOs) and bilateral single-point canes. To increase gait volume, treadmill training was incorporated. At approximately 7 months after onset, the rehabilitation focus shifted further distally to ankle control during gait. Functional electrical stimulation was applied to the common peroneal nerves to facilitate appropriate activation of the tibialis anterior muscles during swing. ⁷ As dorsiflexor strength improved, gait became more stable and efficient. Outdoor gait training was initiated, and the patient progressed from supervised ambulation with bilateral AFOs and bilateral single-point canes to walking with minimal assistance. Because ankle control improved, the AFOs were replaced with lighter AFOs. At 8 months after onset, the patient achieved independent outdoor ambulation using bilateral single-point canes and bilateral lightweight AFOs, with a maximum continuous walking distance of approximately 1 km. He did not require a cane or orthoses for indoor furniture-assisted ambulation and was discharged home. By discharge, the total functional independence measure score had improved to 82 points, with a functional ambulation category of 5. He subsequently continued outpatient follow-up and planned return to work. No major adverse events related to rehabilitation treatment were observed during the recovery phase.

Discussion

This case demonstrates that even a patient with severe EMRN-related paralysis can regain functional ambulation through goal-oriented and stepwise rehabilitation treatment. EMRN is a rare inflammatory disorder affecting both the central and peripheral nervous systems, and its functional prognosis remains poorly understood. Consequently, rehabilitation planning should be individualized according to the distribution and severity of neurological deficits. Because no standardized rehabilitation protocol for EMRN has been established, direct comparison across cases remains difficult. Nevertheless, the present case illustrates a phase-specific rehabilitation approach aligned with neurological recovery and may provide clinically relevant insights for rehabilitation planning in patients with EMRN.

Several factors may have contributed to the favorable outcome. First, patient-related factors may have played an important role, including relatively young age, absence of clear poor prognostic indicators, preserved cognition after the acute phase, and improvement of orthostatic hypotension, which allowed progressive mobilization.^8–10 Second, the design of the rehabilitation program was likely important. In severe neurological disorders, early rehabilitation is essential to prevent disuse syndrome and contractures.^11–13 In the present case, ROM training and early mobilization were initiated in the acute phase. Once neurological recovery began, the rehabilitation program shifted appropriately from the prevention of secondary complications to the active facilitation of gait recovery, which appears to have been a critical turning point. Third, staged orthotic management may have facilitated motor learning by progressively increasing task complexity. Lower-limb orthoses can facilitate gait recovery by constraining excessive degrees of freedom, increasing stability, and simplifying motor tasks during early motor relearning.^3,4 In the present case, the MSH-KAFO first enabled safe standing and gait training despite profound weakness. As recovery progressed, orthotic constraints were gradually reduced in a proximal-to-distal sequence, from the hip to the knee and then to the ankle. This strategy was consistent with motor learning principles because task difficulty increased in parallel with the patient’s recovery. ¹⁴ The sequential changes in orthotic configuration also aligned well with the changing rehabilitation targets: trunk control, hip control, knee control, and finally ankle control. Adjunctive therapies may also have contributed to recovery, including repetitive peripheral magnetic stimulation, treadmill training, and functional electrical stimulation. Finally, clinicians managing peripheral neuropathies must remain aware of possible overwork weakness. ¹⁵ In the present case, intensive rehabilitation treatment was provided, but no clear clinical or laboratory evidence of overwork weakness was observed. This suggests that carefully monitored intensive rehabilitation treatment may be feasible even in severe EMRN. In interpreting the recovery process, although it is not possible to distinguish the relative contributions of immunotherapy, natural recovery, and the rehabilitation, the temporal relationship between neurological recovery and functional improvement provides clinically relevant insights. In this case, the reappearance of deep tendon reflexes and early motor recovery preceded the progression of standing and gait training, suggesting that the rehabilitation strategy was adapted according to the evolving neurological status.

This report has limitations. Because this is a single case report, generalizability is limited, and the specific effects of rehabilitation treatment cannot be clearly distinguished from those of immunotherapy and spontaneous neurological recovery. Nonetheless, this case provides practical insights into rehabilitation planning for a rare disorder with limited available evidence.

Conclusion

This case suggests that even patients with severe paralysis caused by EMRN may achieve functional ambulation through individualized, goal-oriented rehabilitation treatment that integrates accurate diagnosis, staged orthotic management, motor learning principles, and interdisciplinary collaboration. These findings provide practical insights into rehabilitation planning for patients with rare inflammatory disorders involving both the central and peripheral nervous systems.