UNASSIGNED: To identify a rationale for treatment of patients with Marcus Gunn jaw winking syndrome (MGJWS).
UNASSIGNED: Retrospective review of 38 consecutive patients with MGJWS referred to a single tertiary institution. Clinical data included visual acuity, ocular motility, side of jaw-wink, presence or absence of ptosis, levator function, clinical photographs, and management undertaken. Thirty-two patients were operated on with customized surgery by a senior surgeon (FQL).
UNASSIGNED: Cases with no ptosis or mild ptosis were managed conservatively. Levator advancement (LA) was successful in case of moderate ptosis and negligible synkynesis but resulted in a more evident synkinesis. Levator resection (LR) in patients with severe ptosis was associated with high rate of ptosis recurrence. Ptosis was adequately corrected in all patients submitted to uni- or bilateral levator excision (LE) and bilateral frontalis suspension (FS) or unilateral frontalis flap (FF). Jaw winking resolved in all patients submitted to LE but recurred in three cases at a later stage. Strabismus surgery was performed simultaneously in case of associated esotropia or hypotropia.
UNASSIGNED: Moderate ptosis can be corrected with LA, but success is not related to levator function and synkinesis becomes more evident postoperatively. In severe ptosis, LR showed unpredictable results. In case of severe ptosis and severe synkinesis, uni- or bilateral LE and bilateral FS are recommended; unilateral FF is an alternative in patients who refuse bilateral treatment, as the cosmetic outcome is usually better than after unilateral FS.

The aim of the pilot project was to research relationships between the occurrence and level of intensity of primitive reflexes in primary school children, the ability to read an analogue clock and to tell the time. A group of 28 children (14 girls and 14 boys) who attended Montessori Primary School was examined. In the first stage, participants were assessed for the presence of five primitive reflexes (PR): the asymmetrical tonic neck reflex (ATNR), symmetrical tonic neck reflex (STNR), spinal Galant reflex, tonic labyrinthine reflex (TLR) and Palmar grasp reflex. Romberg\'s test was employed to identify signs of difficulties with control of balance and/or proprioception. In the second stage, pupils underwent tests that challenged their ability to read a clock and calculate passing time. After summing up points obtained for all tests, a correlation coefficient was made from which the results were derived. There is a negative correlation between the ability to read an analogue clock and the continued presence of some primitive reflexes. Lower neuromotor maturity (higher points of PR) correlates with lower ability to read a clock. The highest correlations between difficulty with telling the time were found with persistence of the STNR, ATNR and Romberg\'s test.

Accumulating evidence demonstrates a role of the cerebellum in nociception. Some studies suggest that this is mediated via endogenous pain modulation. Here, we used t-DCS to test the effects of modulation of cerebellar function on nociception and endogenous pain modulation. Anodal, cathodal, and sham cerebellar t-DCS were investigated in a cross-over design in 21 healthy subjects. The nociceptive flexor (RIII) reflex, conditioning pain modulation (CPM), and offset analgesia (OA) paradigms were used to assess endogenous pain modulation. Somatosensory evoked potentials (SEPs) and pain ratings were used to assess supraspinal nociception and pain perception, respectively. No significant t-DCS effects were detected when including all t-DCS types and time points (baseline, 0, 30, 60 min post t-DCS) in the analysis. Exploratory analysis revealed an increased RIII reflex size immediately after cathodal t-DCS (compared to sham, P = 0.046, η2p = 0.184), in parallel with a trend for a decrease in electrical pain thresholds (P = 0.094, η2p = 0.134), and increased N120 SEP amplitudes 30 min after cathodal compared to anodal t-DCS (P = 0.007, η2p = 0.374). OA was increased after anodal compared to sham stimulation (P = 0.023, η2p = 0.232). Exploratory results suggested that cathodal (inhibitory) cerebellar t-DCS increased pain perception and reduced endogenous pain inhibition while anodal (excitatory) t-DCS increased endogenous pain inhibition. Results are principally compatible with activation of endogenous pain inhibition by cerebellar excitation. However, maybe due to limited t-DCS skull penetration, effects were small and unlikely to be clinically significant.

BACKGROUND: We previously found that atypical responses to the knee jerk reflex, i.e., tonic responses (TRs), clonus and contralateral responses in very high-risk (VHR) infants were associated with cerebral palsy (CP) at 21 months. The current study aimed for a better understanding of pathophysiology of atypical knee jerk responses by evaluating whether infant atypical knee jerk responses are associated with CP and atypical knee jerk responses at school-age.
METHODS: 31 VHR-children, who had also been assessed longitudinally during infancy, and 24 typically developing children, were assessed at 7-10 years (school-age). We continuously recorded surface EMG of thigh muscles during knee jerk responses longitudinally during infancy and once at school-age. Neurological condition was assessed with age-appropriate neurological examinations. It included the diagnosis of CP at 21 months corrected age and school-age. CP\'s type and severity (Gross Motor Function Classification System (GMFCS)) were reported.
RESULTS: Persistent TRs in infancy were associated with CP at school-age. TR prevalence decreased from infancy to childhood. At school-age it was no longer associated with CP. Clonus prevalence in VHR-children did not change with increasing age; it was significantly higher in children without than those with CP. Reflex irradiation was common in all school-age children, and its prevalence in contralateral muscles in VHR-children decreased between infancy and childhood.
CONCLUSIONS: In infancy, TRs indicated an increased risk of CP, but at school-age TRs were not associated with CP. In general, spinal hyperexcitability, expressed as reflex irradiation and TRs, decreased between infancy and school-age.

To evaluate the correlations between relative afferent pupillary defect (RAPD) magnitude, assessed using the clinical plus scale and neutral density filters, and visual field parameters in patients with unilateral or asymmetrical bilateral optic neuropathy or retinopathy.
Fifty-two patients with RAPD, graded by the swinging flashlight test and neutral density filters, were analyzed in this cross-sectional trial. The RAPD clinical plus scale was divided into grade 1+, initial weak constriction; grade 2+, initial stall then dilatation; grade 3+, immediate dilatation; and grade 4+, fixed amaurotic pupil. Patients with positive RAPD underwent a visual field examination with Humphrey automated perimetry that included visual field index (VFI), mean deviation (MD), and pattern standard deviation (PSD). Spearman\'s rank correlation coefficients and linear regression were used to analyze the association between RAPD grades and visual field parameters.
RAPD clinical plus grades were correlated with interocular VFI (r = 0.55, P < 0.001) and MD (r = 0.48, P = 0.004) differences. Average interocular VFI differences were estimated as follows: 16.75 × RAPD plus grade- 7.53. RAPD, graded by neutral density filters, was correlated with VFI (r = 0.59, P < 0.001), MD (r = 0.54, P < 0.001), and PSD (r = 0.34, P = 0.01).
The RAPD plus scale and neutral density filter grading systems were associated with quantitative visual field defect parameters, with VFI showing the strongest association. RAPD clinical grading could substitute more sophisticated central visual field evaluation methods as a low-cost, low-tech, and widely available approach.

Many patients with severe stroke have impaired airway protective reflexes, resulting in prolonged invasive mechanical ventilation.
To test whether early vs standard tracheostomy improved functional outcome among patients with stroke receiving mechanical ventilation.
In this randomized clinical trial, 382 patients with severe acute ischemic or hemorrhagic stroke receiving invasive ventilation were randomly assigned (1:1) to early tracheostomy (≤5 days of intubation) or ongoing ventilator weaning with standard tracheostomy if needed from day 10. Patients were randomized between July 28, 2015, and January 24, 2020, at 26 US and German neurocritical care centers. The final date of follow-up was August 9, 2020.
Patients were assigned to an early tracheostomy strategy (n = 188) or to a standard tracheostomy (control group) strategy (n = 194).
The primary outcome was functional outcome at 6 months, based on the modified Rankin Scale score (range, 0 [best] to 6 [worst]) dichotomized to a score of 0 (no disability) to 4 (moderately severe disability) vs 5 (severe disability) or 6 (death).
Among 382 patients randomized (median age, 59 years; 49.8% women), 366 (95.8%) completed the trial with available follow-up data on the primary outcome (177 patients [94.1%] in the early group; 189 patients [97.4%] in the standard group). A tracheostomy (predominantly percutaneously) was performed in 95.2% of the early tracheostomy group in a median of 4 days after intubation (IQR, 3-4 days) and in 67% of the control group in a median of 11 days after intubation (IQR, 10-12 days). The proportion without severe disability (modified Rankin Scale score, 0-4) at 6 months was not significantly different in the early tracheostomy vs the control group (43.5% vs 47.1%; difference, -3.6% [95% CI, -14.3% to 7.2%]; adjusted odds ratio, 0.93 [95% CI, 0.60-1.42]; P = .73). Of the serious adverse events, 5.0% (6 of 121 reported events) in the early tracheostomy group vs 3.4% (4 of 118 reported events) were related to tracheostomy.
Among patients with severe stroke receiving mechanical ventilation, a strategy of early tracheostomy, compared with a standard approach to tracheostomy, did not significantly improve the rate of survival without severe disability at 6 months. However, the wide confidence intervals around the effect estimate may include a clinically important difference, so a clinically relevant benefit or harm from a strategy of early tracheostomy cannot be excluded.
ClinicalTrials.gov Identifier: NCT02377167.

Background: Primitive reflexes (PRs) are observed as an automatic response to a specific stimulus. They are vivid from intrauterine life to 6 months postnatal. The reactions are inhibited with the growing maturation of the central nervous system (CNS). In some cases, when the natural process of development is incorrect, PRs manifest later. The analysis of differentiation in gait parameters in children with persistent PRs is important for better understanding their specific behaviour and movement. This study’s aim was to investigate the influence of active PRs on the gait parameters of preschool children. Methods: There were 50 children examined, 30 girls and 20 boys. They were 3.5−6 years old. The children had persistent PRs in the trace form. Each child was examined by S. Goddard’s Battery Test. The acquisition of the spatial-temporal gait parameters was performed using a BTS G-SENSOR measurement instrument. Participants walked barefoot, in the most natural way for them, at a self-selected speed on a 5 m walkway, then turned around and went back. They performed this twice. Results: The reflex activity influences gait cycle duration (p = 0.0099), the left step length (p = 0.0002), the left double support phase (p = 0.0024), the right double support phase (p = 0.0258) and the right single phase. Difficulties in recreating the crawling pattern and GRASP reflex influence gait cadence (p < 0.05). The left GRASP reflex corresponds to step length (p < 0.05). The activeness of the symmetrical tonic neck reflex correlates with the right single support (p < 0.05). Conclusion: The presence of PRs affect walking gait in preschool children.

To quantify interictal photophobia in migraine with and without aura using reflexive eye closure as an implicit measure of light sensitivity and to assess the contribution of melanopsin and cone signals to these responses.
Participants were screened to meet criteria for 1 of 3 groups: headache-free (HF) controls, migraine without aura (MO), and migraine with visual aura (MA). MO and MA participants were included if they endorsed ictal and interictal photophobia. Exclusion criteria included impaired vision, inability to collect usable pupillometry, and history of either head trauma or seizure. Participants viewed light pulses that selectively targeted melanopsin, the cones, or their combination during recording of orbicularis oculi EMG (OO-EMG) and blinking activity.
We studied 20 participants in each group. MA and MO groups reported increased visual discomfort to light stimuli (discomfort rating, 400% contrast, MA: 4.84 [95% confidence interval 0.33, 9.35]; MO: 5.23 [0.96, 9.50]) as compared to HF controls (2.71 [0, 6.47]). Time course analysis of OO-EMG and blinking activity demonstrated that reflexive eye closure was tightly coupled to the light pulses. The MA group had greater OO-EMG and blinking activity in response to these stimuli (EMG activity, 400% contrast: 42.9%Δ [28.4, 57.4]; blink activity, 400% contrast: 11.2% [8.8, 13.6]) as compared to the MO (EMG activity, 400% contrast: 9.9%Δ [5.8, 14.0]; blink activity, 400% contrast: 4.7% [3.5, 5.9]) and HF control (EMG activity, 400% contrast: 13.2%Δ [7.1, 19.3]; blink activity, 400% contrast: 4.5% [3.1, 5.9]) groups.
Our findings suggest that the intrinsically photosensitive retinal ganglion cells (ipRGCs), which integrate melanopsin and cone signals, provide the afferent input for light-induced reflexive eye closure in a photophobic state. Moreover, we find a dissociation between implicit and explicit measures of interictal photophobia depending on a history of visual aura in migraine. This implies distinct pathophysiology in forms of migraine, interacting with separate neural pathways by which the amplification of ipRGC signals elicits implicit and explicit signs of visual discomfort.

The aim of the study was to compare the clinical value of pudendal nerve terminal motor latency in fecal incontinence patients with that of another diagnostic test-anorectal manometry.
This study used a cross-sectional design. Medical records of fecal incontinence patients who underwent pudendal nerve terminal motor latency and anorectal manometry testing were reviewed. Greater than 2.4 ms of pudendal nerve terminal motor latency was determined to be abnormal. Anorectal manometry was performed using a station pull-through technique. Mean resting anal pressure, maximal resting anal pressure, mean squeezing anal pressure, and maximal squeezing anal pressure were investigated. For normal and abnormal pudendal nerve terminal motor latency groups, comparative analyses were performed on anorectal manometry results.
A total of 31 patients were included. Thirteen patients showed normal pudendal nerve terminal motor latency. For anorectal manometry results, there was no significant difference between normal and abnormal pudendal nerve terminal motor latency groups. Fourteen patients had diabetes mellitus. Subgroup analysis of the 14 diabetic patients showed no significant difference between normal and abnormal pudendal nerve terminal motor latency groups. For 17 nondiabetic patients, there was a significant difference between the groups with positive correlations with mean/maximal resting anal pressures.
Pudendal nerve terminal motor latency significantly correlates with anorectal manometry in fecal incontinence only in nondiabetic patients.

Clinical outcomes related to congenital Zika syndrome (CZS) include microcephaly accompanied by specific brain injuries. Among several CZS outcomes that have been described, epilepsy and motor impairments are present in most cases. Pharmacological treatment for seizures resulting from epilepsy is performed with anticonvulsant drugs, which in the long term are related to impairments in the child\'s neuropsychomotor development. Here, we describe the results from a two-year follow-up of a cohort of children diagnosed with CZS related to the growth of the head circumference and some neurological and motor outcomes, including the pharmacological approach, and its results in the treatment of epileptic seizures. This paper is part of a prospective cohort study carried out in the state of Mato Grosso Sul, Brazil, based on a Zika virus (ZIKV)-exposed child population. Our data were focused on the assessment of head circumference growth and some neurological and motor findings, including the description of seizure conditions and pharmacological management in two periods. Among the 11 children evaluated, 8 had severe microcephaly associated with motor impairment and/or epilepsy. Seven children were diagnosed with epilepsy. Of these, 3 had West syndrome. In four children with other forms of epilepsy, there was no pharmacological control.