To report a rare case series of 14 patients of the Marcus-Gunn jaw-winking phenomenon (MGJWP) without ptosis.
This was a retrospective noninterventional case series. The medical records of all patients diagnosed with MGJWP over the past 10 years were retrieved. Patients with documented evidence of absence of ptosis were segregated and analyzed for visual acuity, the severity of Marcus-Gunn, the presence of squint and amblyopia, and the presence of other aberrant regenerations.
A total of 207 patients were diagnosed with MGJWP, out of which 14 (6.76%) patients had isolated MGJWP without blepharoptosis. The mean age of presentation was 9.5 years and males and females were equally affected. The left eye was involved more commonly (57.2%) than the right eye. Twelve patients were congenital and two were presumed to be of traumatic origin. The most common refractive error in this cohort was astigmatism (10, 71.42%), followed by hyperopia (5, 35.71%). One patient had anisometropic amblyopia. Marcus-Gunn was found to be mild (≤2 mm of lid excursion) in all cases. None of the patients had strabismus or any other aberrant innervations. None of the patients underwent surgery and did not develop ptosis or worsening or improvement of Marcus-Gunn after a mean follow-up period of 2.3 years.
Isolated MGJWP in the absence of ptosis is a very rare entity and this is the largest series to date to report such an occurrence. All patients had a mild form of MGJWP with no intervention required in any of the cases.

Areflexia or hyporeflexia is a mandatory clinical criterion for the diagnosis of Guillain-Barré syndrome (GBS). A systematic review of the literature from 1 January 1993 to 30 August 2019 revealed 44 sufficiently detailed patients with GBS and hyper-reflexia, along with one we describe. 73.3% of patients were from Japan, 6.7% from the USA, 6.7% from India, 4.4% from Italy, 4.4% from Turkey, 2.2% from Switzerland and 2.2% from Slovenia, suggesting a considerable geographical variation. Hyper-reflexia was more frequently associated with antecedent diarrhoea (56%) than upper respiratory tract infection (22.2%) and the electrodiagnosis of acute motor axonal neuropathy (56%) than acute inflammatory demyelinating polyneuropathy (4.4%). Antiganglioside antibodies were positive in 89.7% of patients. Hyper-reflexia was generalised in 90.7% of patients and associated with reflex spread in half; it was present from the early progressive phase in 86.7% and disappeared in a few weeks or persisted until 18 months. Ankle clonus or Babinski signs were rarely reported (6.7%); spasticity never developed. 53.3% of patients could walk unaided at nadir, none needed mechanical ventilation or died. 92.9% of patients with limb weakness were able to walk unaided within 6 months. Electrophysiological studies showed high soleus maximal H-reflex amplitude to maximal compound muscle action potential amplitude ratio, suggestive of spinal motoneuron hyperexcitability, and increased central conduction time, suggestive of corticospinal tract involvement, although a structural damage was never demonstrated by MRI. Hyper-reflexia is not inconsistent with the GBS diagnosis and should not delay treatment. All GBS variants and subtypes can present with hyper-reflexia, and this eventuality should be mentioned in future diagnostic criteria for GBS.

Background: Electrophysiological measures are being increasingly utilized due to their ability to provide objective measurements with minimal bias and to detect subtle changes with quantitative data on neural function. Heterogeneous reporting of trial outcomes limits effective interstudy comparison and optimization of treatment. Objective: The objective of this systematic review is to describe the reporting of electrophysiological outcome measures in spinal cord injury (SCI) clinical trials in order to inform a subsequent consensus study. Methods: A systematic search of PubMed and EMBASE databases was conducted according to PRISMA guidelines. Adult human SCI clinical trials published in English between January 1, 2008 and September 15, 2018 with at least one electrophysiological outcome measure were eligible. Findings were reviewed by all authors to create a synthesis narrative describing each outcome measure. Results: Sixty-four SCI clinical trials were included in this review. Identified electrophysiological outcomes included electromyography activity (44%), motor evoked potentials (33%), somatosensory evoked potentials (33%), H-reflex (20%), reflex electromyography activity (11%), nerve conduction studies (9%), silent period (3%), contact heat evoked potentials (2%), and sympathetic skin response (2%). Heterogeneity was present in regard to both methods of measurement and reporting of electrophysiological outcome measures. Conclusion: This review demonstrates need for the development of a standardized reporting set for electrophysiological outcome measures. Limitations of this review include exclusion of non-English publications, studies more than 10 years old, and an inability to assess methodological quality of primary studies due to a lack of guidelines on reporting of systematic reviews of outcome measures.

Fibrocartilaginous embolism is assumed to be caused by fibrocartilaginous nucleus pulposus component migration through retrograde embolization to the spinal cord artery. Fibrocartilaginous embolism is currently not well recognized among pediatricians because of its rarity. We describe a previously healthy 15-year-old soccer player who, after kicking a ball, developed progressive weakness in both legs and urinary retention the next day. Magnetic resonance imaging revealed T2 hyperintensity in the anterior horn of the spinal cord at the Th12/L1 level with Schmorl node at the level of L1/2. We also review the previous literature on fibrocartilaginous embolism of the spinal cord in children (less than18 years age); a total of 25 pediatric patients, including our patient, were identified. The median age was 14 years, and 64% of the reviewed patients were female. The most common trigger event was intense exercise or sports. The neurological symptoms started within one day in most cases, and the time to symptom peak varied from a few hours to two weeks. The most common initial neurological symptoms were weakness or plegia (100%), followed by paresthesia or numbness (48%). Affected areas of the spinal cord were distributed evenly from the cervical to thoracolumbar regions. Although steroids and anticoagulants were most commonly used, the prognosis was quite poor (mild to severe sequelae with three deaths). Although fibrocartilaginous embolism is a very rare condition, physicians should be aware of the characteristics and include fibrocartilaginous embolism of the spinal cord in their differential diagnosis, especially for physically active patients.

METHODS: Systematic review and meta-analysis.
OBJECTIVE: To summarize the available evidence regarding the effects of trans-spinal direct current stimulation (tsDCS) on spinal monosynaptic circuit excitability in healthy individuals.
METHODS: Applied Neuroscience Laboratory, Brazil.
METHODS: Abstract screening was performed independently by two authors for studies found in the following databases: PubMed, CINAHL, PsycINFO, Web of Science, and LILACS. If the authors were unable to agree, a third reviewer was consulted. Randomized clinical trials that reported monosynaptic reflex measures were included. Methodological quality was assessed using the Cochrane tool for assessing the risk of bias, and information extracted about the spinal neurophysiological and stimulation protocols and their results.
RESULTS: The initial search identified 538 studies. After applying the inclusion criteria and excluding duplicates, seven crossover studies were included in the risk of bias assessment, and six studies in the meta-analyses. The meta-analysis results did not show any significant differences between anodal (pooled standardized mean difference (SMD) = -0.09, 95% CI = -0.72 to 0.55, p = 0.79, I2 = 67%) or cathodal tsDCS (pooled SMD = 0.28, 95% CI = -0.07 to 0.63, p = 0.11, I2 = 0%) and sham tsDCS for Hoffmann reflex modulation.
CONCLUSIONS: tsDCS did not affect the Hoffmann reflex, as shown in six studies. However, these findings come from studies with selection, performance and detection bias, and further research is needed to examine the effect of this intervention.

Systematic review.
To determine the validity of the Hoffmann sign for the detection of degenerative cervical myelopathy (DCM) for patients presenting with cervical complaints.
While physical examination maneuvers are often used to diagnose DCM, no previous review has synthesized diagnostic accuracy data.
Medline, Embase, and HealthStar were searched for articles from January 1, 1947 to March 1, 2017 using the following terms: Spinal Cord Diseases, Spinal Cord Compression, Cervical Vertebrae, Signs and Symptoms, Physical Examination, Epidemiologic studies, Epidemiologic Research Design, Predictive Value of Tests, and Myelopathy. The Quality Assessment of Diagnostic Accuracy Studies (QUADAS) checklist was applied to determine the level of evidence. Articles included were published in English or French language, rated as QUADAS level 3 or higher with a minimum 10 patients presenting with cervical complaints having undergone the Hoffman sign. Excluded studies recruited patients with a nondegenerative type of cervical myelopathy, and/or no evaluation with magnetic resonance imaging.
A total of 589 articles were selected for review. Following the application of inclusion and exclusion criteria, 45 articles were analyzed using the QUADAS checklist. Only of three articles were of QUADAS quality 3 or higher. Analysis of combined data from 2/3 studies indicated that the Hoffman sign has a positive likelihood ratio of 2.2 (95% CI 1.5-3.3) and a negative likelihood ratio of 0.63 (95% CI 0.5-0.8).
A positive Hoffman alone is unlikely to lead to more than a small change in estimated probability of DCM as compared with the gold standard test (magnetic resonance imaging). Variability in results across individual studies may result from differences in study design. There are insufficient data to support use of the Hoffman sign alone to confirm or refute a diagnosis of DCM.
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BACKGROUND: The vagus nerve has sensory and motor function in the larynx, as well as parasympathetic function in the thorax and abdomen. Stimulation of the superior laryngeal nerve can cause reflexive bradycardia.
METHODS: We describe a case of a 45-year-old man with pre-syncopal symptoms while exercising, and bradycardia found during cardiology workup. Radiography and flexible laryngoscopy showed evidence of a right-sided, vascular laryngeal mass. Exercise testing before and after superior laryngeal nerve block showed reversal of the symptoms with the block. Subsequent resection of the lymphovascular malformation with CO2 laser eliminated the patient\'s symptoms.
CONCLUSIONS: This is the first case reported of the laryngocardiac reflex producing symptomatic bradycardia as a result of exercise-induced engorgement of a supraglottic lymphovascular malformation, which was then cured by surgical excision. We discuss this case and the literature regarding lymphovascular malformations in the airway and the neural pathways of the laryngocardiac reflex.