Colonic Pseudo-obstruction as a Rare Complication of Pheochromocytoma

Colonic pseudo-obstruction is characterized by dilatation of the colon without a structural lesion causing the obstruction. It usually involves the caecum and right side of the colon and is commonly observed in patients with severe illness or after surgery; it is rarely caused by pheochromocytoma. The diagnosis of colonic pseudo-obstruction can be established by abdominal imaging including computed tomography (CT) of the abdomen or use of a water-soluble contrast enema. In additional to conservative or surgical treatment, alpha-blockers can be used in this setting to relieve the pseudo-obstruction.

Data-Driven Definitions for Active and Structural MRI Lesions in the Sacroiliac Joint in Spondyloarthritis and their Predictive Utility

Objectives To determine quantitative sacroiliac joint (SIJ) MRI lesion cut-offs that optimally define a positive MRI for inflammatory and structural lesions typical of axial spondyloarthritis (axSpA) and that predict clinical diagnosis. Methods The ASAS MRI group assessed MRIs from the ASAS Classification Cohort in two reading exercises: A. 169 cases and 7 central readers; B. 107 cases and 8 central readers. We calculated sensitivity/specificity for the number of SIJ quadrants or slices with bone marrow edema (BME), erosion, fat lesion, where a majority of central readers had high confidence there was a definite active or structural lesion. Cut-offs with ≥95% specificity were analyzed for their predictive utility for follow-up rheumatologist diagnosis of axSpA by calculating positive/negative predictive values (PPV/NPV) and selecting cut-offs with PPV≥95%. Results Active or structural lesions typical of axSpA on MRI had PPV≥95% for clinical diagnosis of axSpA. Cut-offs that best reflect definite active lesion typical of axSpA were either ≥4 SIJ quadrants with BME at any location or at the same location in ≥ 3 consecutive slices. For definite structural lesion, the optimal cut-offs were any one of ≥ 3 SIJ quadrants with erosion or ≥ 5 with fat lesion, erosion at the same location for ≥2 consecutive slices, fat lesion at the same location for ≥3 consecutive slices, or presence of a ‘deep’ (>1cm) fat lesion. Conclusion We propose cut-offs for definite active and structural lesions typical of axSpA that have high PPV for a long-term clinical diagnosis of axSpA for application in disease classification and clinical research.

Structural changes in the sacroiliac joint on MRI and relationship to ASDAS inactive disease in axial spondyloarthritis: a 2-year study comparing treatment with etanercept in EMBARK to a contemporary control cohort in DESIR

Background Limited information is available on the impact of treatment with a tumor necrosis factor inhibitor (TNFi) on structural lesions in patients with recent-onset axial spondyloarthritis (axSpA). We compared 2-year structural lesion changes on magnetic resonance imaging (MRI) in the sacroiliac joints (SIJ) of patients with recent-onset axSpA receiving etanercept in a clinical trial (EMBARK) to similar patients not receiving biologics in a cohort study (DESIR). We also evaluated the relationship between the Ankylosing Spondylitis Disease Activity Score (ASDAS) and change in MRI structural parameters. Methods The difference between etanercept (EMBARK) and control (DESIR) in the net percentage of patients with structural lesion change was determined using the SpondyloArthritis Research Consortium of Canada SIJ Structural Score, with and without adjustment for baseline covariates. The relationship between sustained ASDAS inactive disease, defined as the presence of ASDAS < 1.3 for at least 2 consecutive time points 6 months apart, and structural lesion change was evaluated. Results This study included 163 patients from the EMBARK trial and 76 from DESIR. The net percentage of patients with erosion decrease was significantly greater for etanercept vs control: unadjusted: 23.9% vs 5.3%; P = 0.01, adjusted: 23.1% vs 2.9%; P = 0.01. For the patients attaining sustained ASDAS inactive disease on etanercept, erosion decrease was evident in significantly more than erosion increase: 34/104 (32.7%) vs 5/104 (4.8%); P < 0.001. A higher proportion had erosion decrease and backfill increase than patients in other ASDAS status categories. However, the trend across ASDAS categories was not significant and decrease in erosion was observed even in patients without a sustained ASDAS response. Conclusions These data show that a greater proportion of patients achieved regression of erosion with versus without etanercept. However, the link between achieving sustained ASDAS inactive disease and structural lesion change on MRI could not be clearly established. Trial registration EMBARK: ClinicalTrials.gov identifier: NCT01258738, Registered 13 December 2010; DESIR: ClinicalTrials.gov identifier: NCT01648907, Registered 24 July 2012.

Blood Pressure and Outcomes in Patients With Different Etiologies of Intracerebral Hemorrhage: A Multicenter Cohort Study

Background We aimed to investigate the association between blood pressure (BP) and outcomes in intracerebral hemorrhage (ICH) subtypes with different etiologies. Methods and Results A total of 5656 in‐hospital patients with spontaneous ICH were included between January 2012 and December 2016 in a prospective multicenter cohort study. Etiological subtypes of ICH were assigned using SMASH‐U (structural lesion, medication, amyloid angiopathy, systemic/other disease, hypertension, undetermined) classification. Elevated systolic BP was defined as ≥140 mm Hg. Hypertension was defined as elevated BP for >1 month before the onset of ICH. The primary outcomes were measured as 1‐month survival rate and 3‐month mortality. A total of 5380 patients with ICH were analyzed, of whom 4052 (75.3%) had elevated systolic BP on admission and 3015 (56.0%) had hypertension. In multinomial analysis of patients who passed away by 3 months, systolic BP on admission was significantly different in cerebral amyloid angiopathy ( P <0.001), structural lesion ( P <0.001), and undetermined subtypes ( P =0.003), compared with the hypertensive angiopathy subtype. Elevated systolic BP was dose‐responsively associated with higher 3‐month mortality in hypertensive angiopathy ( P trend =0.013) and undetermined ( P trend =0.005) subtypes. In cerebral amyloid angiopathy, hypertension history had significant inverse association with 3‐month mortality (adjusted odds ratio, 0.37, 95% CI, 0.20–0.65; P <0.001). Similarly, adjusted Cox regression indicated decreased risk of 1‐month survival rate in the presence of hypertension in patients with cerebral amyloid angiopathy (adjusted hazard ratio, 0.47; 95% CI, 0.24–0.92; P =0.027). Conclusions This study suggests that the association between BP and ICH outcomes might specifically depend on its subtypes, and cerebral amyloid angiopathy might be pathologically distinctive from the others. Future studies of individualized BP‐lowering strategy are needed to validate our findings.

Hemiplegic cerebral palsy complicated by acute hemidystonia in adulthood

Practical Implication:Dystonia following a perinatal injury can have a long latency period in decades, sometimes difficult to treat and may not respond to medical therapy, where deep brain stimulation is another treatment option.Background:Dystonia is defined as a movement disorder characterized by sustained or intermittent muscle contractions causing abnormal, often repetitive, movements, postures, or both, and hemidystonia is dystonic posturing involving one side. (1) Our patient had adolescent-onset, persistent, progressive hemidystonia ( Axis 1 ) secondary to contralateral basal ganglia injury at birth (Axis 2 ) according to recent consensus. (1) He presented as an acute hemidystonic attack with rhabdomyolysis (CPK = 6500 IU/L) and involvement of bulbar and respiratory system with no new structural lesion which is a rare presentation of delayed hemidystonia following perinatal injury.

Lost in Transition: The Long and Winding Road Toward Epilepsy Surgery—An Analysis of Obstacles Prior to Surgery and Call for Orchestrated Health Care Efforts in Epilepsy

Difficult-to-treat epilepsy is defined as ongoing seizures despite adequate pharmacological treatment. This condition is affecting a significant percentage of epilepsy patients and is estimated to be as high as one-third of all patients. Epilepsy surgery, targeting the removal of the key parts of cerebral convolutions responsible for seizure generation and often including a structural lesion, can be a very successful approach. However, this necessitates careful patient selection by comprehensive investigations, proving the localization of the epileptogenic zone as well as measures to make such surgeries safe. With careful selection as a prerequisite, the percentage of patients achieving seizure freedom by neurosurgical intervention is high, approximating two-thirds of all epilepsy surgeries performed. In contrast, the average duration of a patient's pharmacoresistant focal epilepsy prior to surgery anywhere around the globe is around 20 years. Given that typical patients are ∼30 to 40 years of age at the time of surgery, many patients have been living with chronic seizures since childhood or adolescence. This means that most of these patients have been going through several stages of medical care for years or even decades, both as children and adults, without ever being fully investigated and/or selected for surgery which is concerning. Yet, there is no set standard for a timeline leading toward successful surgery in epilepsy. It is obvious that the average transit period from the moment of first seizure manifestation until the day of successful surgery takes much too long. This is the reason why we see these patients lost in transition.

Cough headache

Cough headache exists in a primary and secondary form. The latter is due to tonsillar descent or, more rarely, to other space-occupying lesions in the posterior fossa/foramen magnum. Up to 40% of patients have an underlying structural lesion. Most patients with primary cough headache respond to indomethacin and suboccipital craniectomy with posterior fossa reconstruction can relieve cough headache in Chiari type I malformation.

Current understanding of hypothalamic amenorrhoea

Hypothalamic amenorrhoea (HA) accounts for approximately 30% of cases of secondary amenorrhoea in women of reproductive age. It is caused by deficient secretion of hypothalamic gonadotrophin-releasing hormone, which in turn leads to failure of pituitary gonadotrophin and gonadal steroid release. Functional HA (FHA) is defined as HA occurring in the absence of a structural lesion and is predominantly caused by significant weight loss, intense exercise or stress. Treatment of FHA is crucial in avoiding the long-term health consequences on fertility and bone health, in addition to reducing psychological morbidity. This article summarises our understanding of the mechanisms underlying FHA, the evidence base for its clinical management and emerging therapies.

Acute Concussion in the Emergency Department

Concussion evaluations are very common in the Emergency Department, but there is no standardized protocol to evaluate and manage concussions. Triage to rule out more severe brain injury is becoming more uniform. For example, there are emerging guidelines and biomarkers for when a brain CT scan should be ordered for a suspected intracranial bleed or other brain structural lesion. However, the nuance of concussion diagnosis and management is less uniform, leaving many patients with a diagnosis and no clear plan for management and recovery—and this fallout can negatively impact recovery. Following concussion diagnosis, discharge instructions are key, as delayed concussion management and lack of follow-up can lead to improper early management, which too often leads to prolonged, persistent symptoms.