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Aria Nouri, Allan R. Martin, David Mikulis, and Michael G. Fehlings

Degenerative cervical myelopathy encompasses a spectrum of age-related structural changes of the cervical spine that result in static and dynamic injury to the spinal cord and collectively represent the most common cause of myelopathy in adults. Although cervical myelopathy is determined clinically, the diagnosis requires confirmation via imaging, and MRI is the preferred modality. Because of the heterogeneity of the condition and evolution of MRI technology, multiple techniques have been developed over the years in an attempt to quantify the degree of baseline severity and potential for neurological recovery. In this review, these techniques are categorized anatomically into those that focus on bone, ligaments, discs, and the spinal cord. In addition, measurements for the cervical spine canal size and sagittal alignment are also described briefly. These tools have resulted collectively in the identification of numerous useful parameters. However, the development of multiple techniques for assessing the same feature, such as cord compression, has also resulted in a number of challenges, including introducing ambiguity in terms of which methods to use and hindering effective comparisons of analysis in the literature. In addition, newer techniques that use advanced MRI are emerging and providing exciting new tools for assessing the spinal cord in patients with degenerative cervical myelopathy.

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Nisha Giridharan, Smruti K. Patel, Amanda Ojugbeli, Aria Nouri, Peyman Shirani, Aaron W. Grossman, Joseph Cheng, Mario Zuccarello, and Charles J. Prestigiacomo

Idiopathic intracranial hypertension (IIH) is a disease defined by elevated intracranial pressure without established etiology. Although there is now consensus on the definition of the disorder, its complex pathophysiology remains elusive. The most common clinical symptoms of IIH include headache and visual complaints. Many current theories regarding the etiology of IIH focus on increased secretion or decreased absorption of cerebrospinal fluid (CSF) and on cerebral venous outflow obstruction due to venous sinus stenosis. In addition, it has been postulated that obesity plays a role, given its prevalence in this population of patients. Several treatments, including optic nerve sheath fenestration, CSF diversion with ventriculoperitoneal or lumboperitoneal shunts, and more recently venous sinus stenting, have been described for medically refractory IIH. Despite the availability of these treatments, no guidelines or standard management algorithms exist for the treatment of this disorder. In this paper, the authors provide a review of the literature on IIH, its clinical presentation, pathophysiology, and evidence supporting treatment strategies, with a specific focus on the role of venous sinus stenting.

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Lindsay Tetreault, Jefferson R. Wilson, Mark R. N. Kotter, Aria Nouri, Pierre Côté, Branko Kopjar, Paul M. Arnold, and Michael G. Fehlings

OBJECTIVE

The minimum clinically important difference (MCID) is defined as the minimum change in a measurement that a patient would identify as beneficial. Before undergoing surgery, patients are likely to inquire about the ultimate goals of the operation and of their chances of experiencing meaningful improvements. The objective of this study was to define significant predictors of achieving an MCID on the modified Japanese Orthopaedic Association (mJOA) scale at 2 years following surgery for the treatment of degenerative cervical myelopathy (DCM).

METHODS

Seven hundred fifty-seven patients were prospectively enrolled in either the AOSpine North America or International study at 26 global sites. Fourteen patients had a perfect preoperative mJOA score of 18 and were excluded from this analysis (n = 743). Data were collected for each participating subject, including demographic information, symptomatology, medical history, causative pathology, and functional impairment. Univariate log-binominal regression analyses were conducted to evaluate the association between preoperative clinical factors and achieving an MCID on the mJOA scale. Modified Poisson regression using robust error variances was used to create the final multivariate model and compute the relative risk for each predictor.

RESULTS

The sample consisted of 463 men (62.31%) and 280 women (37.69%), with an average age of 56.48 ± 11.85 years. At 2 years following surgery, patients exhibited a mean change in functional status of 2.71 ± 2.89 points on the mJOA scale. Of the 687 patients with available follow-up data, 481 (70.01%) exhibited meaningful gains on the mJOA scale, whereas 206 (29.98%) failed to achieve an MCID. Based on univariate analysis, significant predictors of achieving the MCID on the mJOA scale were younger age; female sex; shorter duration of symptoms; nonsmoking status; a lower comorbidity score and absence of cardiovascular disease; and absence of upgoing plantar responses, lower-limb spasticity, and broad-based unstable gait. The final model included age (relative risk [RR] 0.924, p < 0.0001), smoking status (RR 0.837, p = 0.0043), broad-based unstable gait (RR 0.869, p = 0.0036), and duration of symptoms (RR 0.943, p = 0.0003).

CONCLUSIONS

In this large multinational prospective cohort, 70% of patients treated surgically for DCM exhibited a meaningful functional gain on the mJOA scale. The key predictors of achieving an MCID on the mJOA scale were younger age, shorter duration of symptoms, nonsmoking status, and lack of significant gait impairment.

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Kishan Patel, Hardy Evans, Samuel Sommaruga, Pia Vayssiere, Tariq Qureshi, Luis Kolb, Michael G. Fehlings, Joseph S. Cheng, Enrico Tessitore, Karl Schaller, and Aria Nouri

OBJECTIVE

Klippel-Feil syndrome (KFS) is characterized by congenital fusion of the cervical vertebrae. Due to its rarity, minimal research has been done to assess the quality and management of pain associated with this disorder. Using a large global database, the authors report a detailed analysis of the type, location, and treatment of pain in patients with KFS.

METHODS

Data were obtained from the Coordination of Rare Diseases at Stanford registry and Klippel-Feil Syndrome Freedom registry. The cervical fusions were categorized into Samartzis type I, II, or III. The independent-sample t-test, Wilcoxon rank-sum test, and Friedman test were conducted, with significance set at p < 0.05.

RESULTS

Seventy-five patients (60 female, 14 male, and 1 unknown) were identified and classified as having the following types of Samartzis fusion: type I, n = 21 (28%); type II, n = 15 (20%); type III, n = 39 (52%). Seventy participants (93.3%) experienced pain associated with their KFS. The median age of patients at pain onset was 16.0 years (IQR 6.75–24.0 years), and the median age when pain worsened was 28.0 years (IQR 15.25–41.5 years). Muscle, joint, and nerve pain was primarily located in the shoulders/upper back (76%), neck (72%), and back of head (50.7%) and was characterized as tightness (73%), dull/aching (67%), and tingling/pins and needles (49%). Type III fusions were significantly associated with greater nerve pain (p = 0.02), headache/migraine pain (p = 0.02), and joint pain (p = 0.03) compared to other types of fusion. Patients with cervical fusions in the middle region (C2–6) tended to report greater muscle, joint, and nerve pain (p = 0.06). Participants rated the effectiveness of oral over-the-counter medications as 3 of 5 (IQR 1–3), oral prescribed medications as 3 of 5 (IQR 2–4), injections as 2 of 5 (IQR 1–4), and surgery as 3 of 5 (IQR 1–4), with 0 indicating the least pain relief and 5 the most pain relief. Participants who pursued surgical treatment reported significantly more comorbidities (p = 0.02) and neurological symptoms (p = 0.01) than nonsurgically treated participants and were significantly older when pain worsened (p = 0.03), but there was no difference in levels of muscle, joint, or nerve pain (p = 0.32); headache/migraine pain (p = 0.35); total number of cervical fusions (p = 0.77); location of fusions; or age at pain onset (p = 0.16).

CONCLUSIONS

More than 90% of participants experienced pain. Participants with an increased number of overall cervical fusions or multilevel, contiguous fusions reported greater levels of muscle, joint, and nerve pain. Participants who pursued surgery had more comorbidities and neurological symptoms, such as balance and gait disturbances, but did not report more significant pain than nonsurgically treated participants.

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Allan R. Martin, Sukhvinder Kalsi-Ryan, Muhammad A. Akbar, Anna C. Rienmueller, Jetan H. Badhiwala, Jefferson R. Wilson, Lindsay A. Tetreault, Aria Nouri, Eric M. Massicotte, and Michael G. Fehlings

OBJECTIVE

Degenerative cervical myelopathy (DCM) is among the most common pathologies affecting the spinal cord but its natural history is poorly characterized. The purpose of this study was to investigate functional outcomes in patients with DCM who were managed nonoperatively as well as the utility of quantitative clinical measures and MRI to detect deterioration.

METHODS

Patients with newly diagnosed DCM or recurrent myelopathic symptoms after previous surgery who were initially managed nonoperatively were included. Retrospective chart reviews were performed to analyze clinical outcomes and anatomical MRI scans for worsening compression or increased signal change. Quantitative neurological assessments were collected prospectively, including modified Japanese Orthopaedic Association (mJOA) score; Quick-DASH; graded redefined assessment of strength, sensation, and prehension–myelopathy version (GRASSP–M: motor, sensory, and dexterity); grip dynamometer; Berg balance scale score; gait stability ratio; and gait variability index. A deterioration of 10% was considered significant (e.g., a 2-point decrease in mJOA score).

RESULTS

A total of 117 patients were included (95 newly diagnosed, 22 recurrent myelopathy), including 74 mild, 28 moderate, and 15 severe cases. Over a mean follow-up of 2.5 years, 57% (95% CI 46%–67%) of newly diagnosed patients and 73% (95% CI 50%–88%) of patients with recurrent DCM deteriorated neurologically. Deterioration was best detected with grip strength (60%), GRASSP dexterity (60%), and gait stability ratio (50%), whereas the mJOA score had low sensitivity (33%) in 50 patients. A composite score had a sensitivity of 81% and a specificity of 82%. The sensitivity of anatomical MRI was 28% (83 patients).

CONCLUSIONS

DCM appears to have a poor natural history; however, prospective studies are needed for validation. Serial assessments should include mJOA score, grip strength, dexterity, balance, and gait analysis. The absence of worsening on anatomical MRI or in mJOA scores is not sufficient to determine clinical stability.