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Functional electrical stimulation to aid walking in patients with adrenomyeloneuropathy: A case study and observational series.

ABSTRACT: Adrenomyeloneuropathy (AMN) is a rare inherited condition where affected individuals develop slowly progressive spastic paraparesis with a gradual decline in walking ability. There is no cure for AMN and treatment focuses on supportive measures and aids. One treatment option is functional electrical stimulation (FES), a treatment, approved by The National Institute for Health and Care Excellence (NICE), for managing foot drop in upper motor neuron disorders. Limited evidence exists for its use in AMN patients. We describe the effects of FES in an individual case and more broadly within a cohort of 21 patients successfully treated with FES. Patients with AMN referred for FES typically report frequent falls (71%) and foot drop (57%) as the most common barriers to walking. When using FES, walking speed at baseline (0.70 m/s [SD = 0.2]) was maintained at the 2-year review (0.68 m/s [SD = 0.2]) with a persistent orthotic effect (improvement in walking speed when device on vs. off) seen from wearing FES over the same 2-year period (11%-19%). Patient walking satisfaction (visual analogue scale: 0 - very dissatisfied; 10 - very satisfied) was also greater when comparing no-FES versus FES over the same period (Year 1: 2.5 vs. 7.7; Year 2: 2.1 vs. 6.1). FES is not effective in all patients. Twelve patients referred found no benefit from the device; although there was no clear evidence, this was related to the degree of AMN associated peripheral neuropathy. However, FES is a safe, cost-effective treatment option and should be considered, along with assessment in a multidisciplinary clinic, for all AMN patients with walking difficulties.

SUBMITTER: Goodison W

PROVIDER: S-EPMC8743338 | biostudies-literature |

REPOSITORIES: biostudies-literature

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Project description:Background and purposeCerebral palsy (CP) is characterized by decreased passive joint range-of-motion and impaired walking, resulting in progressive loss of function. Typical gait training interventions for children with CP appear insufficient to mitigate these effects. The purpose of this case report is to describe the use of a new treadmill-based gait training intervention using active correction with functional electrical stimulation (FES) in 2 adolescents with CP.Case descriptionTwo participants with CP (13-year-old girls, Gross Motor Function Classification System [GMFCS] level II and III) trained by walking on a treadmill, with FES assistance, for 30 minutes, 3 times per week, for 12 weeks. The intervention used a feedback control system to detect all 7 phases of gait in real time and triggered FES to the appropriate muscle groups (up to 5 bilaterally) based on the detected gait phase. Joint kinematics, step width, stride length, walking endurance, peak oxygen uptake ($\dot{v}^{o}_{2}$), and oxygen (O2) cost of walking were evaluated preintervention and postintervention.OutcomesBoth participants showed improved knee and ankle angles and step width relative to children who are typically developing, and both exhibited increased stride length. One participant (GMFCS III) improved peak $\dot{v}^{o}_{2}$and walking endurance but not O2 cost of walking at her original self-selected walking speed. The other participant (GMFCS II) improved O2 cost of walking but not peak $\dot{v}^{o}_{2}$ or walking endurance. These differences are partly explained by differences in gait type, functional abilities, and initial fitness levels. Most improvements persisted at follow-up, indicating short-term neurotherapeutic effects.DiscussionMost improvements persisted at follow-up, suggesting short-term neurotherapeutic effects. This case series demonstrates the promising utility of FES-assisted gait-training interventions, tailored to target individual gait deviations, in improving walking performance.

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Functional electrical stimulation to aid walking in patients with adrenomyeloneuropathy: A case study and observational series.

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