Citations

ObjectiveTo compare longitudinally extensive myelitis in neuromyelitis optica spectrum disorders (NMOSD) and spinal cord sarcoidosis (SCS).MethodsWe identified adult patients evaluated between 1996 and 2015 with SCS or NMOSD whose first myelitis episode was accompanied by a spinal cord lesion spanning ≥3 vertebral segments. All NMOSD patients were positive for aquaporin‐4–immunoglobulin G, and all sarcoidosis cases were pathologically confirmed. Clinical characteristics were evaluated. Spine magnetic resonance imaging was reviewed by 2 neuroradiologists.ResultsWe studied 71 patients (NMOSD, 37; SCS, 34). Sixteen (47%) SCS cases were initially diagnosed as NMOSD or idiopathic transverse myelitis. Median delay to diagnosis was longer for SCS than NMOSD (5 vs 1.5 months, p < 0.01). NMOSD myelitis patients were more commonly women, had concurrent or prior optic neuritis or intractable vomiting episodes more frequently, had shorter time to maximum deficit, and had systemic autoimmunity more often than SCS (p < 0.05). SCS patients had constitutional symptoms, cerebrospinal fluid (CSF) pleocytosis, and hilar adenopathy more frequently than NMOSD (p < 0.05); CSF hypoglycorrhachia (11%, p = 0.25) and elevated angiotensin‐converting enzyme (18%, p = 0.30) were exclusive to SCS. Dorsal cord subpial gadolinium enhancement extending ≥2 vertebral segments and persistent enhancement >2 months favored SCS, and ringlike enhancement favored NMOSD (p < 0.05). Maximum disability was similar in both disorders.InterpretationSCS is an under‐recognized cause of longitudinally extensive myelitis that commonly mimics NMOSD. We identified clinical, laboratory, systemic, and radiologic features that, taken together, help discriminate SCS from NMOSD. ANN NEUROL 2016;79:437–447

ObjectiveThe aim of this work was to evaluate the preprogressive phase in subjects with radiologically isolated syndrome (RIS) who evolve to primary progressive multiple sclerosis (PPMS).MethodsA multicenter RIS cohort was previously established. Demographic, clinical, and radiological characteristics of subjects with RIS that evolved directly to PPMS were compared to those that developed a relapsing disease course from onset (clinically isolated syndrome [CIS] or relapsing‐remitting MS) and were also compared to two other population‐ and clinic‐based PPMS cohorts.ResultsOf the 453 subjects with RIS, 128 evolved to symptomatic MS during the follow‐up (113 developed a first acute clinical event consistent with CIS/MS, 15 evolved to PPMS). PPMS prevalence (11.7%) and onset age (mean ± standard deviation; 49.1 ± 12.1) in the RIS group were comparable to other PPMS populations (p > 0.05). Median time to PPMS was 3.5 years (range, 1.6–5.4). RIS evolved to PPMS more commonly in men (p = 0.005) and at an older age (p < 0.001) when compared to CIS/MS, independent of follow‐up duration. Subjects who evolved to PPMS had more spinal cord lesions (100%) before symptomatic evolution than those that developed CIS/MS (64%) and those that remained asymptomatic (23%) within the follow‐up period (P = 0.005). Other MRI characteristics in the preprogressive phase of PPMS were indistinguishable from CIS/MS.InterpretationSubjects with RIS evolve to PPMS at the same frequency as expected from general MS populations in an age‐dependent manner. Besides age, unequivocal presence of spinal cord lesions and being male predicted evolution to PPMS. Our findings further suggest that RIS is biologically part of the MS spectrum. Ann Neurol 2016;79:288–294

Objective To highlight a specific under‐recognized radiological feature of spondylotic myelopathy often resulting in misdiagnosis. Methods Patients evaluated between January 1, 1996 and December 31, 2012 who met the following criteria were included: (1) spondylotic myelopathy was suspected, (2) gadolinium enhancement was detected, and (3) spinal surgery was performed. Results Fifty‐six patients (70% men) whose median age was 53.5 years (range = 24–80) were included. Spinal cord magnetic resonance imaging (cervical in 52; thoracic in 4) revealed longitudinal spindle‐shaped T2‐signal hyperintensity (100%) and cord enlargement (79%) accompanied by a characteristic pancakelike transverse band of gadolinium enhancement in 41 (73%), typically immediately caudal to the site of maximal spinal stenosis. Forty (71%) patients were initially diagnosed with neoplastic or inflammatory myelopathies, and decompressive surgery was delayed by a median of 11 months (range = 1–64). Spinal cord biopsy in 6 did not reveal any alternative diagnosis. Ninety‐five percent were stable or improved. Gadolinium enhancement persisted in 75% at 12 months, raising concern about the accuracy of the initial diagnosis. Twenty patients required a gait aid (36%) at last follow‐up (median = 60 months, range = 10–172). The need for a gait aid preoperatively ( p = 0.005), but not delay of surgery, predicted the need for gait aid at final follow‐up. Interpretation Transverse pancakelike gadolinium enhancement associated with and just caudal to the site of maximal stenosis and at the rostrocaudal midpoint of a spindle‐shaped T2 hyperintensity suggests that spondylosis is the cause of the myelopathy. Persistent enhancement for months to years following decompressive surgery is common. Recognition is important to prevent inappropriate interventions or delay in consideration of a potentially beneficial decompressive surgery. Ann Neurol 2014;76:54–65

Patients with dysglycemia related to known or unrecognized diabetes, stress hyperglycemia, or hypoglycemia in the presence or absence of exogenous insulin routinely require care during the perioperative period or critical illness. Recent single and multicenter studies, a large multinational study, and three meta-analyses evaluated the safety of routine tight glycemic control (80–110 mg/dl) in critically ill adults. Results led to a call for more modest treatment goals (initiation of insulin at a blood glucose >180 mg/dl with a goal of ∼150 mg/dl). In this symposium, an international group of multidisciplinary experts discusses the role of tight glycemic control, glucose measurement technique and its accuracy, glucose variability, hypoglycemia, and innovative methods to facilitate glucose homeostasis in this heterogeneous patient population.