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):
3. 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)
4. Progressive course, or initial progression followed by stationary course; and
5. No evidence of a compressive lesion of the spinal cord.
6. Disease duration of ≤4 years
7. 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.