Inter-rater reliability determination for two tests of ulnar nerve conduction across the elbow.

INTRODUCTION: The inter-rater variability in determination of ulnar nerve conduction across the elbow compromises test accuracy. The extent of this variability is unknown. The objective of this study was to determine and compare inter-rater reliability of variables derived from 2 different ulnar nerve conduction studies (NCSs) across the elbow. METHODS: Two investigators performed a standard ulnar NCS and a 6-cm conduction time (Six-Centimeter Conduction Time test, SCCT) on 60 extremities of asymptomatic subjects. In the standard test, below-elbow (BE) and above-elbow (AE) stimulation points were ≥ 10 cm apart, measured along a curved path, to calculate across-elbow NCV. In SCCT, BE and AE were precisely 6 cm apart measured linearly to calculate CTE (conduction time elbow). Inter-rater reliability was assessed by means of intraclass correlation coefficients (ICC). RESULTS: ICC for across-elbow NCV and CTE were 0.726 and 0.801, respectively. CONCLUSIONS: Reliability of CTE and across-elbow NCV are similar. Shorter distances, if measured linearly, can be used to determine across-elbow ulnar nerve conduction. Muscle Nerve 55: 664-668, 2017.

High-resolution ultrasound in combat-related peripheral nerve injuries.

INTRODUCTION: Peripheral nerve injuries (PNI) sustained in combat are typically severe and are frequently associated with marked soft tissue damage, anatomic distortion, and retained metallic fragments. These features complicate clinical and electrodiagnostic assessment and may preclude MRI. METHODS: We describe 4 cases of military personnel who sustained high-velocity gunshot wounds or blasts with metal fragment injuries in which high resolution peripheral nerve ultrasound (US) proved beneficial. RESULTS: In these cases, the clinical and electrodiagnostic exams provided inadequate localization and severity data of the nerve injuries, and MRI was either precluded or provided no additional information. In each case, US disclosed focal nerve segment abnormalities, including regions of focal enlargement and nerve discontinuity with end-bulb neuroma, which guided surgical planning for nerve repair. The findings on US were subsequently confirmed intra-operatively. CONCLUSIONS: High resolution peripheral nerve US is a useful modality in assessment of combat-related PNI. Muscle Nerve, 2016 Muscle Nerve 54: 1139-1144, 2016.

Effects of pontine lesions on REM sleep.

Over the past several decades, the neural mechanisms underlying REM sleep have become increasingly understood. A more detailed understanding of the respective roles of the pontine nuclei in the generation of REM sleep and its related phenomenon has allowed for the recognition of specific effects that brainstem lesions have on sleep. In humans, however, the effects of such lesions are limited to case reports and small case series. This article offers a comprehensive review of the basic neurobiology of REM sleep. In addition, we discuss specific clinical effects that various pontine lesions have with regard to REM sleep and the spectrum of clinical sleep disorders characterized by abnormalities in REM-related phenomena. We review the existing literature detailing the interactions between clinical sleep manifestations and brainstem pathology.

Treatment responsive GABA(B)-receptor limbic encephalitis presenting as new-onset super-refractory status epilepticus (NORSE) in a deployed U.S. soldier.

A 23-year-old, previously healthy, deployed U.S. soldier presented with bilateral temporal lobe seizures recalcitrant to multiple antiepileptic drugs and anti-seizure anaesthetic agents. He received methylprednisolone, intravenous immunoglobulins, plasma exchange, and rituximab for presumed autoimmune encephalitis before achieving seizure freedom. Six weeks after presentation, the aetiology of his refractory seizures was found to be due to autoantibodies targeting the anti-GABA(B)-receptor. This case is noteworthy for being the first reported case of anti-GABA(B)-receptor limbic encephalitis presenting with new-onset refractory status epilepticus (NORSE), a clinical syndrome that often carries a grave prognosis and in which a treatable aetiology is often never discovered. Our case also supports testing for GABA-receptor autoantibodies and the upfront use of multi-modal immunotherapy in patients presenting with limbic encephalitis and new refractory seizures.

Factors Governing the Erythropoietic Response to Intravenous Iron Infusion in Patients with Chronic Kidney Disease: A Retrospective Cohort Study.

BACKGROUND: Limited knowledge exists about factors affecting parenteral iron response. A study was conducted to determine the factors influencing the erythropoietic response to parenteral iron in iron-deficient anaemic patients whose kidney function ranged from normal through all stages of chronic kidney disease (CKD) severity. METHODS: This retrospective cohort study included parenteral iron recipients who did not receive erythropoiesis-stimulating agents (ESA) between 2017 and 2019. The study cohort was derived from two groups of patients: those managed by the CKD team and patients being optimised for surgery in the pre-operative clinic. Patients were categorized based on their kidney function: Patients with normal kidney function [estimated glomerular filtration rate (eGFR) ≥ 60 mL/min/1.73 m2] were compared to those with CKD stages 3-5 (eGFR < 60 mL/min/1.73 m2). Patients were further stratified by the type of iron deficiency [absolute iron deficiency (AID) versus functional iron deficiency (FID)]. The key outcome was change in hemoglobin (∆Hb) between pre- and post-infusion haemoglobin (Hb) values. Parenteral iron response was assessed using propensity-score matching and multivariate linear regression. The impact of kidney impairment versus the nature of iron deficiency (AID vs. FID) in response was explored. RESULTS: 732 subjects (mean age 66 ± 17 years, 56% females and 87% White) were evaluated. No significant differences were observed in the time to repeat Hb among CKD stages and FID/AID patients. The Hb rise was significantly lower with lower kidney function (non-CKD and CKD1-2; 13 g/L, CKD3-5; 7 g/L; p < 0.001). When groups with different degrees of renal impairment were propensity-score matched according to whether iron deficiency was due to AID or FID, the level of CKD was found not to be relevant to Hb responses [unmatched (∆Hb) 12.1 vs. 8.7 g/L; matched (∆Hb) 12.4 vs. 12.1 g/L in non-CKD and CKD1-2 versus CKD3-5, respectively]. However, a comparison of patients with AID and FID, while controlling for the degree of CKD, indicated that patients with FID exhibited a diminished Hb response regardless of their level of kidney impairment. CONCLUSION: The nature of iron deficiency rather than the severity of CKD has a stronger impact on Hb response to intravenous iron with an attenuated response seen in functional iron deficiency irrespective of the degree of renal impairment.

Myasthenia gravis--an unexpected cause of respiratory failure and reversible left ventricular dysfunction after cardiac surgery.

We report a case of postpericardiotomy myasthenia gravis. A 68-year-old male patient without prior history of neuromuscular or autoimmune disorders presented with respiratory failure and severe left ventricular dysfunction four weeks after mitral valve replacement. Markedly elevated acetylcholine receptor antibodies were noted, and the patient responded promptly to immunologic therapy. Awareness of this rare but potentially fatal consequence of cardiac surgery may allow the early institution of specific treatment.

The safety, efficacy, and cost-effectiveness of intraoperative cell salvage in metastatic spine tumor surgery.

BACKGROUND CONTEXT: Metastatic spine tumor surgery (MSTS) is associated with substantial blood loss, therefore leading to high morbidity and mortality. Although intraoperative cell salvage with leukocyte depletion filter (IOCS-LDF) has been studied as an effective means of reducing blood loss in other surgical settings, including the spine, no study has yet analyzed the efficacy of reinfusion of salvaged blood in reducing the need for allogenic blood transfusion in patients who have had surgery for MSTS. PURPOSE: This study aimed to analyze the efficacy, safety, and cost-effectiveness of using IOCS-LDF in MSTS. STUDY DESIGN: This is a retrospective controlled study. PATIENT SAMPLE: A total of 176 patients undergoing MSTS were included in the study. METHODS: All patients undergoing MSTS at a single center between February 2010 and December 2014 were included in the study. The primary outcome measure was the use of autologous blood transfusion. Secondary outcome measures included hospital stay, survival time, complications, and procedural costs. The key predictor variable was whether IOCS-LDF was used during surgery. Logistic and linear regression analyses were conducted by controlling variables such as tumor type, number of diseased vertebrae, approach, number and site of stabilized segments, operation time, preoperative anemia, American Society of Anesthesiologists (ASA) grade, age, gender, and body mass index (BMI). No funding was obtained and there are no conflicts of interest to be declared. RESULTS: Data included 63 cases (IOCS-LDF) and 113 controls (non-IOCS-LDF). Intraoperative cell salvage with LDF utilization was substantively and significantly associated with a lower likelihood of allogenic blood transfusion (OR=0.407, p=.03). Intraoperative cell salvage with LDF was cost neutral (p=.88). Average hospital stay was 3.76 days shorter among IOCS-LDF patients (p=.03). Patient survival and complication rates were comparable in both groups. CONCLUSIONS: We have demonstrated that the use of IOCS-LDF in MSTS reduces the need for postoperative allogenic blood transfusion while maintaining satisfactory postoperative hemoglobin. We recommend routine use of IOCS-LDF in MSTS for its safety, efficacy, and potential cost benefit.

Post-traumatic shoulder dystonia in an active duty soldier.

Acquired focal dystonia is often precipitated by minor local traumatic injury, resulting in severe pain and disability. An active duty soldier with shoulder dystonia, after a fall, that responded partially to botulinum toxin A is described. Post-traumatic dystonia as a neurological illness is discussed, with emphasis on mechanisms, precipitating causes, differential diagnosis, and treatment implications for battlefield clinicians.

Ulnar neuropathy at the elbow: Five new things.

Ulnar neuropathy at the elbow (UNE) is the second most frequent compression neuropathy. While other diagnostic imaging tools are emerging to assist in the diagnosis of UNE, electrodiagnosis remains the gold standard. However, the electrodiagnostic approach to UNE presents unique challenges limiting its diagnostic accuracy. We review advances in 5 areas relevant to the diagnosis of UNE: technologic advancements with modern EMG machines have allowed for reconsideration of the question of experimental error and lesion detection; how temperature effects can lead to misdiagnosis; the effect of body mass index on the electrodiagnosis of UNE; the validation of short segment studies; and the emerging role of high-resolution sonography as a diagnostic tool.

Improving awareness of nonanesthesia-related malignant hyperthermia presentations: a tale of two brothers.

A 30-year-old man developed unexplained rhabdomyolysis, persistently increased creatine kinase and severe debilitating muscle cramps. After a nondiagnostic neurologic evaluation, he was referred for a muscle biopsy, to include histology/histochemistry, a myoglobinuria panel, and a caffeine halothane contracture test. Only the caffeine halothane contracture test was positive, and a subsequent ryanodine receptor type 1 gene evaluation revealed a mutation functionally causative for malignant hyperthermia. His identical twin brother, who was suffering from similar complaints, was found to share the same mutation. They each require oral dantrolene therapy to control symptoms, despite difficulty in identifying health care providers familiar with treating this disorder.

Multiple cranial neuropathies.

Patients presenting with multiple cranial neuropathies are not uncommon in neurologic clinical practice. The evaluation of these patients can often be overwhelming due to the vast and complicated etiologies as well as the potential for devastating neurologic outcomes. Dysfunction of the cranial nerves can occur anywhere in their course from intrinsic brainstem dysfunction to their peripheral courses. The focus of this review will be on the extramedullary causes of multiple cranial neuropathies as discussion of the brainstem syndromes is more relevant when considering intrinsic disorders of the brainstem. The goals are to provide the reader with an overview of those extramedullary conditions that have a predilection for causing multiple cranial nerve palsies. In turn, this will serve to provide a practical and systematic approach to allow for a more targeted diagnostic evaluation of this, often cumbersome, presentation.