Outcomes after Cervical Duraplasty for Monomelic Amyotrophy (Hirayama Disease): Results of a Case-Control Study of 60 Patients.

Background The optimal management and the role of surgery in monomelic amyotrophy, also known as Hirayama disease (HD), remain controversial. In the largest series of patients with HD managed by cervical duraplasty (CD), this study compares the outcomes between conservative and surgical management. Methods A retrospective case-control study was performed on 60 patients with HD. The cases consisted of 30 patients who underwent CD, and 30 age- and sex-matched controls who managed with long-term collar wear. Clinical improvement was recorded using two patient-reported outcome measures-the Odom's scale and a self-rated hand grip strength score. Median and ulnar compound muscle action potential (CMAP) amplitudes and various magnetic resonance imaging parameters were recorded. Results None of the controls experienced clinical improvement at follow-up; 90% remained status quo at 24.33 ± 9.38 months. Seventy-six percent of the operated patients demonstrated clinical improvement at 29.61 ± 12.78 months, and a majority of them reported moderately better grip strength. The preoperative anteroposterior diameter (APD) of the surgical group improved from 0.74 ± 0.13 to 0.86 ± 0.21 ( p = 0.01), and the cord demonstrated a significant decrease in forward migration on flexion (0.20 ± 0.11 and 0.03 ± 0.07 preoperatively and at follow-up, respectively, p < 0.0001). Patients with clinical improvement demonstrated significantly better mean APD and median and ulnar CMAP values than those who did not improve ( p < 0.0001). The mean cervical alignment did not change significantly at follow-up ( p = 0.13). Conclusions This study shows that CD can significantly alter the clinical course of patients with progressive symptoms of HD. This approach successfully arrests the forward migration of the cervical cord on flexion and promotes morphological recovery of the cord. A majority of the patients undergoing CD demonstrate clinical and electrophysiological improvement at mid-term follow-up.

Deep flexor sarcopenia as a predictor of poor functional outcome after anterior cervical discectomy in patients with myelopathy.

BACKGROUND: Paraspinal muscle morphometry has been recognized to be a prognostic factor across various surgical conditions, but its utility in predicting disease-specific outcomes in spine surgery remains under-explored. METHODS: A prospective cohort study was performed on 45 consecutive patients undergoing anterior cervical discectomy (ACD) for single-level, symptomatic cervical degenerative disc disease causing radiculomyelopathy or myelopathy. Previously described predictors of outcome such as age, gender, smoking, comorbidities, duration of symptoms, preoperative Nurick grade, extent of cord compression, and signal intensity change in the cord were recorded. Additionally, MRI-based morphometrics of the superficial and deep paraspinal muscles were recorded. Logistic regression (LR) analysis was performed using a purposeful variable selection process to identify variables that independently predicted Nurick grade improvement (NGI). RESULTS: At a mean follow-up of 20.02 ± 8.63 months after ACD, 37 (82.22%) patients demonstrated NGI. LR analysis yielded three predictors of NGI of which two were related to the deep flexor muscles. While a worse preoperative Nurick grade negatively predicted NGI, a deep flexor area and deep flexor/deep extensor area ratio positively predicted NGI. The regression model demonstrated a good fit and was statistically significant (χ2(3) = 22.18, p < 0.0001). The model explained 64% of the variance in NGI and correctly classified 89% of cases. CONCLUSIONS: This study has for the first time identified the utility of paraspinal morphometrics in predicting disease-specific functional outcome after cervical spine surgery. Our results indicate that in addition to preoperative Nurick grade, an already accepted outcome predictor, the deep flexor cross-sectional area, and the deep flexor/deep extensor ratio are strong predictors of NGI following ACD for single-level, symptomatic cervical degenerative disc disease with myelopathy. Deep muscle morphometrics could be included in future risk stratification algorithms for patients with cervical disc disease.

A points-based algorithm for prognosticating clinical outcome of Chiari malformation Type I with syringomyelia: results from a predictive model analysis of 82 surgically managed adult patients.

OBJECTIVE Although various predictors of postoperative outcome have been previously identified in patients with Chiari malformation Type I (CMI) with syringomyelia, there is no known algorithm for predicting a multifactorial outcome measure in this widely studied disorder. Using one of the largest preoperative variable arrays used so far in CMI research, the authors attempted to generate a formula for predicting postoperative outcome. METHODS Data from the clinical records of 82 symptomatic adult patients with CMI and altered hindbrain CSF flow who were managed with foramen magnum decompression, C-1 laminectomy, and duraplasty over an 8-year period were collected and analyzed. Various preoperative clinical and radiological variables in the 57 patients who formed the study cohort were assessed in a bivariate analysis to determine their ability to predict clinical outcome (as measured on the Chicago Chiari Outcome Scale [CCOS]) and the resolution of syrinx at the last follow-up. The variables that were significant in the bivariate analysis were further analyzed in a multiple linear regression analysis. Different regression models were tested, and the model with the best prediction of CCOS was identified and internally validated in a subcohort of 25 patients. RESULTS There was no correlation between CCOS score and syrinx resolution (p = 0.24) at a mean ± SD follow-up of 40.29 ± 10.36 months. Multiple linear regression analysis revealed that the presence of gait instability, obex position, and the M-line-fourth ventricle vertex (FVV) distance correlated with CCOS score, while the presence of motor deficits was associated with poor syrinx resolution (p ≤ 0.05). The algorithm generated from the regression model demonstrated good diagnostic accuracy (area under curve 0.81), with a score of more than 128 points demonstrating 100% specificity for clinical improvement (CCOS score of 11 or greater). The model had excellent reliability (κ = 0.85) and was validated with fair accuracy in the validation cohort (area under the curve 0.75). CONCLUSIONS The presence of gait imbalance and motor deficits independently predict worse clinical and radiological outcomes, respectively, after decompressive surgery for CMI with altered hindbrain CSF flow. Caudal displacement of the obex and a shorter M-line-FVV distance correlated with good CCOS scores, indicating that patients with a greater degree of hindbrain pathology respond better to surgery. The proposed points-based algorithm has good predictive value for postoperative multifactorial outcome in these patients.