Cervical Fixation Surgery Cervical Collar for Management of Hirayama Disease: A Randomized Study

Posterior Cervical Fixation Versus Long-term Cervical Collar for Management of Hirayama Disease: Prospective Randomized Open Blinded Endpoint (PROBE), Phase III Study

The goal of this clinical trial] is to compare cervical collar versus neck stabilization surgery in diagnosed patients of Hirayama disease who have been reporting worsening of problems in the past six months. The main question[s] it aims to answer are:

• Is cervical stabilization surgery (Posterior cervical fixation) superior to conservative management in the form of cervical collar placement in patients with progressive Hirayama disease, observed at six months after intervention

Participants will have equal chance to:

• Undergo cervical fixation surgery
• Cervical collar management The investigators will study and compare the efficacy of both treatments upto six months after intervention

Study Overview

• Status: Recruiting
• Conditions: Hirayama Disease
• Intervention / Treatment: Procedure: Posterior cervical fixation surgery

Detailed Description

1. Study design:

   Prospective randomized open blinded endpoint (PROBE), phase III study

2. Inclusion criteria:

All the following:

1. Age ≥ 18 years

2. Patients with Hirayama disease as per the following criteria (All of the following) (16):

   1. Clinical evidence of wasting and weakness confined to one limb (EMG evidence of denervation in the opposite limb will not be a reason for exclusion)
   2. Progressive course, or initial progression followed by stationary course; and
   3. No evidence of a compressive lesion of the spinal cord.
3. Disease duration of ≤4 years
4. Progression of clinical symptoms in the past six-months c. Exclusion criteria:

Any of the following:

1) Refusal to consent for randomization 2) Not willing to come for three- and six-months follow-up e. Procedures at baseline:

1. Clinical, electrophysiology and autonomic function assessments:

   After the clinical diagnosis by inclusion criteria, the patients will undergo a detailed clinical examination (as per the case record form (CRF), which includes power, autonomic features, grip strength), and the objective assessment of the electrophysiological parameters

2. Radiological assessment:

   All patients will undergo MR imaging on a 1.5 T MR scanner (Optima 450w, General Electric, Milwaukee, USA), using an 8-channel cervical-thoracic-lumbar array spine coil. The imaging will include axial and sagittal T2-WIs and post-gadolinium T1+C in flexion, neutral and extension positions. In addition, DTI protocol will include sagittal and axial acquisition of cervical cord in neutral position. MR-DTI data will process on a dedicated workstation (Advantage Windows workstation, GE Healthcare, WI, USA) using commercially available software (Functool 14.3.01, GE HealthCare, WI, USA). After co-registration of raw images to correct the motion and distortion artefacts, the ADC and FA images will be generated. The mean, minimum and maximum values of FA and ADC will be recorded from region of maximum cord compression/ T2-hyperintensity.

   The investogators will get a cervical spine radiograph at the time of discharge and at 6 months.

3. Surgical procedure:

The patients who are randomized to undergo posterior cervical fixation will be admitted in Neurosciences Centre, All India Institute of Medical Sciences, New Delhi. These patients will undergo routine preoperative work up, including routine blood investigations and a detailed preanesthetic checkup (PAC). These patients will undergo posterior cervical fixation in the form of cervical lateral mass screw fixation in extension without decompression under general anesthesia.

• Study Type: Interventional
• Enrollment (Estimated): 80
• Phase: Not Applicable

Contacts and Locations

• Study Contact: Name: Deepti Vibha; Phone Number: +91-011-26594485; Email: deeptivibha@aiims.edu
• Study Contact Backup: Name: Kanwaljeet Garg; Phone Number: +91-968569905; Email: kanwaljeet@aiims.edu

Study Locations

• India
  • Delhi
    • New Delhi, Delhi, India, 110029
      • Recruiting
      • Deepti Vibha
      • Contact: Deepti Vibha; Phone Number: +91-011-26594485; Email: deeptivibha@aiims.edu
      • Principal Investigator: Kanwaljeet Garg
      • Sub-Investigator: Ajay Garg

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: Adult
• Accepts Healthy Volunteers: No

Description

Inclusion Criteria:

• All the following:

  1. Age ≥ 18 years

  2. Patients with Hirayama disease as per the following criteria (All of the following) (16):

     1. Clinical evidence of wasting and weakness confined to one limb (EMG evidence of denervation in the opposite limb will not be a reason for exclusion)
     2. Progressive course, or initial progression followed by stationary course; and
     3. No evidence of a compressive lesion of the spinal cord.
  3. Disease duration of ≤4 years
  4. Progression of clinical symptoms in the past six-months

Exclusion Criteria:

• Any of the following:

  1. Refusal to consent for randomization
  2. Not willing to come for three- and six-months follow-up

Study Plan

How is the study designed?

Design Details

• Primary Purpose: Treatment
• Allocation: Randomized
• Interventional Model: Parallel Assignment
• Masking: Single

Number of Arms

2

Arms and Interventions

Participant Group / Arm	Intervention / Treatment
Experimental: Surgical arm; Will undergo posterior cervical fixation surgery within 4 weeks of randomization	Procedure: Posterior cervical fixation surgery; These patients will undergo posterior cervical fixation in the form of cervical lateral mass screw fixation in extension without decompression under general anesthesia.
No Intervention: Cervical collar arm; Will be given cervical collar for regular use

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Time Frame
Odom's criteria score at six months	Six months after intervention

Secondary Outcome Measures

Outcome Measure	Time Frame
Odom's criteria score at three months	Three months after intervention
interval change in DTI metrics of fractional anisotropy (FA) and apparent diffusion coefficient (ADC) of cervical cord	at six-months post-intervention
Electrophysiological and grip strength	6 month

Collaborators and Investigators

• Sponsor: All India Institute of Medical Sciences, New Delhi
• Investigators: Principal Investigator: Deepti Vibha, AIIMS, New Delhi

Study record dates

Study Major Dates

• Study Start (Actual): October 1, 2023
• Primary Completion (Estimated): September 1, 2026
• Study Completion (Estimated): December 1, 2026

Study Registration Dates

• First Submitted: July 18, 2023
• First Submitted That Met QC Criteria: July 18, 2023
• First Posted (Actual): July 25, 2023

Study Record Updates

• Last Update Posted (Actual): January 30, 2024
• Last Update Submitted That Met QC Criteria: January 27, 2024
• Last Verified: January 1, 2024

More Information

Terms related to this study

• Keywords: cervical collar; Hirayama disease; posterior cervical fixation surgery
• Additional Relevant MeSH Terms: Central Nervous System Diseases; Nervous System Diseases; Genetic Diseases, Inborn; Neuromuscular Diseases; Neurodegenerative Diseases; Spinal Cord Diseases; Heredodegenerative Disorders, Nervous System; Motor Neuron Disease; Muscular Atrophy, Spinal; Spinal Muscular Atrophies of Childhood
• Other Study ID Numbers: AC-60

Plan for Individual participant data (IPD)

• Plan to Share Individual Participant Data (IPD)?: UNDECIDED
• IPD Plan Description: Anonymized patient data may be shared on pertinent request

Drug and device information, study documents

• Studies a U.S. FDA-regulated drug product: No
• Studies a U.S. FDA-regulated device product: No