Epidural Spinal Cord Stimulation for Lower-limb Impairment in Adrenomyeloneuropathy

Summary

Adrenal spinal neuropathy (AMN) is a rare X-linked genetic disease caused by mutations in the ABCD1 gene, and belongs to a special type of adrenal leukodystrophy. The patient's lower limb strength is weakened, the range of motion of the ankle joint is reduced, the hip flexors are weak and affect walking, and the peripheral nerves and vibration sensation are damaged. As the condition worsens, the lower limb muscle tone changes from hyperactivity to decrease, ultimately transitioning from spastic paralysis to flaccid paralysis. At present, the treatment plan for AMN is not yet perfect, and effective therapies are urgently needed to alleviate symptoms.

Spinal cord electrical stimulation (SCS) is the implantation of a thin electrode into the epidural space of the corresponding spinal segment within the spinal canal. Then connect the electrodes to a nerve stimulator implanted subcutaneously in the iliac region, and use electrical pulses to stimulate the conduction of sensory neurons in the posterior column and posterior horn of the spinal cord for treatment, which can achieve the goal of controlling pain. In addition, SCS has also conducted research on the recovery of lower limb function in paraplegic patients and upper limb function in post-stroke hemiplegic patients, and has improved corresponding motor dysfunction to a certain extent. Spinal cord stimulation may be a potential treatment for motor dysfunction in AMN. Based on the above, this study attempts to evaluate the efficacy of SCS in treating lower limb muscle tone and movement disorders in AMN patients, and explore the potential therapeutic effects and related mechanisms of SCS on AMN.

In this study, 10 AMN patients will be recruited. After enrollment, preoperative evaluation will be conducted. After preliminary assessment of motor function, neurological evaluation, and other related examinations, lumbar spinal nerve stimulators and pulse generators were implanted in our hospital. After the implantation surgery is completed, depending on the patient's recovery status, they will be transferred to various centers for subsequent rehabilitation treatment within one to two weeks, and then turned on for treatment. Before starting up, a second corresponding inspection and evaluation will be conducted. The third and fourth corresponding inspections and evaluations will be conducted one week and four weeks after startup, respectively. The patient will be discharged 4 weeks after starting up, and then return to the hospital for the fifth and sixth corresponding examinations and evaluations at 4 weeks and 6 months after discharge. Evaluate the effectiveness and safety of SCS in improving lower limb motor dysfunction in AMN patients through statistical analysis.

Conditions

• Adrenomyeloneuropathy Without Cerebral Involvement

Interventions

PROCEDURE

Spinal cord stimulation

Upon completion of the initial assessments, a lumbar spinal cord nerve stimulator and pulse generator will be implanted. Following surgery, based on the patient's recovery status, the patient will be transferred to the respective center for rehabilitation within one to two weeks. Afterward, electrical stimulation therapy will be initiated. Stimulation should be applied at least three days per week. Each day, the total stimulation time should be between 4 and 8 hours. Stimulation Modes: Continuous Stimulation: For example, if 6 hours of stimulation are prescribed, the stimulator will run uninterrupted for 6 hours. Intermittent Stimulation: For instance, a cycle of 40 seconds on and 20 seconds off. If a total of 6 hours of active stimulation is needed, the stimulator must remain on for 9 hours in total to accommodate rest intervals.The ranges for each parameter are as follows:Stimulation Frequency: 2 Hz - 2000 Hz, Pulse Width: 20 μs - 1000 μs, Stimulation Amplitude:Voltage: 0 V - 10 v

Sponsors & Collaborators

• Beijing Tiantan Hospital

  collaborator OTHER

• Jingmen No.1 People's Hospital

  collaborator OTHER

• The 958th Hospital of the Chinese People's Liberation Army

  collaborator UNKNOWN

• Third Military Medical University

  lead OTHER

Study Design

Allocation

NA

Purpose

TREATMENT

Masking

NONE

Model

SINGLE_GROUP

Eligibility

Min Age

22 Years

Max Age

50 Years

Sex

MALE

Healthy Volunteers

No

Timeline & Regulatory

Start

2025-02-10

Primary Completion

2027-06-30

Completion

2027-10-31

Countries

• China

Study Locations