Spinal Cord Stimulation Surgery for Improving Lower Limb Motor Dysfunction in Adrenomyeloneuropathy (AMN): A Prospective, Multicenter, Single-Arm, Self-Controlled Study.
Trial ID: NCT06796920
Status: RECRUITING
Study type: INTERVENTIONAL
Phase: NA
Start date: 2025-02-10
Completion date: 2027-10
Sponsor: Third Military Medical University
Collaborators: Beijing Tiantan Hospital; Jingmen No.1 People's Hospital; The 958th Hospital of the Chinese People's Liberation Army
Conditions: Adrenomyeloneuropathy Without Cerebral Involvement
Interventions: Spinal cord stimulation
Locations: Beijing TianTan Hospital; The 958 Hospital of Chinese People's Liberation Army, The Jiangbei Campus of Southwest Hospital, The First Affiliated Hospital of Army Medical University; the Southwest hospital; Jingmen No.1 People's Hospital
Countries: China; China; China; China
Enrollment: 10
Age range: 22 Years - 50 Years
Sex: MALE

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.

Eligibility criteria:
Inclusion Criteria:

* Conforming to the diagnostic criteria of AMN, with a definite genetic testing report, and complicated by lower limb motor function disorders;
* Capable of normal communication and able to complete scale tests independently (as determined by on-site scale tests);
* Willing to participate in this study after giving informed consent;
* The muscle tone of the patient's bilateral lower extremities was elevated.

Exclusion Criteria:

* Other inherited diseases;
* Other severe central nervous system diseases;
* History of brain surgery;
* Psychiatric and psychological diseases such as depression and anxiety;
* The presence of metallic foreign bodies or prostheses (such as cardiac pacemakers, insulin pumps) in the body, claustrophobia, and other contraindications for MRI;
* Informed consent was not obtained;
* Unable to tolerate MRI-related examinations;
* Received anticoagulant, antispasmodic or antiepileptic drug therapies throughout the entire study period;
* Postoperative wound infection;
* Other motor disorders, spinal cord pathologies, fractures, osteoarthritis, amputations, scoliosis and other movement-affecting diseases.

Outcomes:
6-minute walk test (6MWT)