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Purpose: We describe a case of Parry-Romberg syndrome (PRS) presenting with panuveitis and retinal vasculitis.Methods: We conducted a retrospective review of our patient's case and related literature published through May 2019.Results: A 26-year-old woman with history of PRS was diagnosed with panuveitis and retinal vasculitis. Intraocular inflammation was controlled with local and systemic corticosteroids. The relationship between PRS and intraocular inflammation is discussed with references to the relevant on literature.Conclusions: Our findings and the accompanying literature review suggest that the patient's ocular involvement included multiple fundus lesions, retinal vascular disorder, and unilateral poliosis - all of which may be attribute to trigeminal neuro vasculitis. As the Varicella-zoster virus may contribute to the onset of the autoimmune processes associated with PRS, this requires further exploration. This report confirms the utility of multimodal imaging in the study, screening, and follow-up of intraocular inflammation in patients with PRS. [ABSTRACT FROM AUTHOR]

Background: It is necessary to understand the mechanism of trigeminal neuralgia (TN) and hemifacial spasm (HFS) in order to seek for an effective noninvasive remedy. As previous studies implied that inflammatory cytokines induced by demyelination following the nerve injury may be the initiated factor causing neuropathic pain, we attempt to analyze the correlation between cytokines and these hyperactive cranial nerve disorders. Method: The consecutive patients whose diagnosis were confirmed by microvascular decompression surgery as primary TN or HFS caused by vascular compression and healthy volunteers between March and May 2018 in XinHua Hospital Shanghai JiaoTong University School of Medicine were recruited. Preoperatively, venous blood was collected and the protein concentrations of IL-1β, IL-2, IL-6, IL-8, IL-10, TNF-α and IFN-γ were determined with ELISA. Each cytokine was compared between the patients and healthy volunteers. Results: Ultimately, 28 healthy volunteers as well as 44 TN and 47 HFS patients were enrolled in this investigation. The serum levels of IL-1β, IL-6, IL-8 and TNF-α in either HFS or TN patients were significantly higher than that in healthy volunteers (p < 0.05), yet which were similar between TN and HFS patients (p > 0.05). Besides, there was a significantly correlation between IL-6 concentration and severity of HFS (r = 0.933, p < 0.05) or TN (r = 0.943, p < 0.05). Discussion: Vascular compression of trigeminal or facial nerve roots may induce a rise in variety of cytokines, and IL-6 may play an important role in the signaling pathways to generate ectopic impulses from these cranial nerves. [ABSTRACT FROM AUTHOR]

Introduction: Localized scleroderma (LoS) of the face and head is often associated with neurological manifestations and/or imaging abnormalities in the central nervous system (CNS).Case series: We present an analysis of 20 cases of LoS affecting the face and head. The CNS symptoms and/or abnormalities in high-resolution computed tomography (HRCT) and/or magnetic resonance imaging (MRI) were observed in 12 patients (60%). In addition to the mild and unspecific disorders (e.g. headaches), serious neurological complications probably in the course of vasculitis were revealed: epilepsy (in two patients), epilepsy and pyramidal sings (in one patient). Neurological disorders and LoS occurred at the same time (in three patients) or at the course of the disease (nine patients) and no later than 29 years since the onset of the disease. No link between neurological disorders and the LoS clinical morphology, immunological and other laboratory parameters has been established.Conclusions: CNS involvement is not correlated with the clinical course of the facial and head LoS and may occur years after the disease initial symptomatology. Imaging follow-up is not required if there is not any emerging neurological symptom. In some cases, however, both HRCT and MRI are useful for monitoring disease evolution and addressing therapeutic choices. [ABSTRACT FROM PUBLISHER]

Conclusion: Wider-area botulinum toxin (BT) injection with the dosage depending on specific pathology is a promising approach for the treatment of hemifacial spasm (HFS), resulting in effective and long-lasting control of HFS with fewer side-effects.Objective: The aim of this study was to develop a BT injection strategy that minimizes complications by considering the causes, duration, and electrophysiologic features of the disease, as well as the patient’s age.Methods: From July 2011 to July 2015, 26 patients were included in the strategy. The mean age was 61.8 ± 14.6. In the case of essential HFS, 2 units/injection site was the standard dosage. If a patient was aged over 60 and had a reduced electromyogram (EMG) amplitude, the dosage was reduced by 0.5 units/site. In the case of post-paralytic and tumor-induced HFS, 1.5 units/site was the standard dosage. All cases were managed by alternating injections of Botox and Dysport with no physical therapy.Results: Most HFS patients treated with the injection strategy had complete remission of HFS within 1 week. The average number of BT injection sites was 22.6 ± 6.7. The mean total BT dosage on the affected side was 28.6 ± 4.9 units. The mean duration of BT efficacy was 28.6 ± 7.7 weeks. [ABSTRACT FROM PUBLISHER]

Left hemiface biases observed within the Emotional Chimeric Face Task (ECFT) support emotional face perception models whereby all expressions are preferentially processed by the right hemisphere. However, previous research using this task has not considered that the visible midline between hemifaces might engage atypical facial emotion processing strategies in upright or inverted conditions, nor controlled for left visual field (thus right hemispheric) visuospatial attention biases. This study used novel emotional chimeric faces (blended at the midline) to examine laterality biases for all basic emotions. Left hemiface biases were demonstrated across all emotional expressions and were reduced, but not reversed, for inverted faces. The ECFT bias in upright faces was significantly increased in participants with a large attention bias. These results support the theory that left hemiface biases reflect a genuine bias in emotional face processing, and this bias can interact with attention processes similarly localized in the right hemisphere. [ABSTRACT FROM PUBLISHER]

Although neurovascular confliction was believed to be the cause of hemifacial spasm (HFS), the mechanism of the disorder remains unclear to date. Current theories, merely focusing on the facial nerve, have failed to explain the clinical phenomenon of immediate relief following a successful microvascular decompression surgery (MVD). With the experience of thousands of microvascular decompression surgeries and preliminary investigations, we have learned that the offending artery may play a more important role than the effect of merely mechanical compression in the pathogenesis of the disease. We believe that the attrition of neurovascular interface is the essence of the etiology, and the substance of the disease is emersion of ectopic action potentials from the demyelinated facial nerve fibers, which were triggered by the sympathetic endings from the offending artery wall. In this paper, we put forward evidence to support this hypothesis, both logically and theoretically. [ABSTRACT FROM AUTHOR]

Facial hemiatrophies are anomalies of the first branchial arch and affect one in 4000-5000 newborns. Bone distraction is the technique of choice for the treatment of these dysmorphoses. Mandibular osteodistraction requires prior determination of the characteristics of the distraction vector whose three components will serve to activate the distractor. The patient, aged 5 years, presented with a right facial hemiatrophy, Grade IB according to the classification of Pruzansky. Tomodensitometric acquisition was obtained with a CT scanner. Software specifically designed for this application allows segmentation of the anatomical elements by a region-growing algorithm. The 3D representation of each element is added to a 3D scene, in which are placed the built-up landmarks necessary for the surgical simulation after 3D cephalometric analysis. The surgical cleavage plane is oriented according to the surgeon's requirements while preserving the predominant anatomical elements. The software allows performance of rotations and translations of the bone segments rendered independently from the cleavage plane. The distances and angles covered during the virtual movement are measured at its conclusion. The aim of moving the bone segments is to render the mandibular occlusion plane parallel to the reference occlusion plane. The vertical growth of the maxilla is realized by secondary recuperation. The distractor used was of an external multidirectional type allowing elongation of the mandibular ramus and mandibular corpus, closure of the goniac angle, and lateralization or medialization of the ramus. On the 15th day, the mandibular angle was reduced by 10°, which allowed closure of the anterior gap and recentering of the incisive areas by a half-cuspid. The patient presented with a complex bone deficit in the three spatial directions, which allowed the development of software for modeling the distraction. Other clinical cases will be necessary to validate this 3D imaging-based technique. [ABSTRACT FROM AUTHOR]

The authors present an image of the Madonna and Child painted by one of the less well-known Italian artists of the Renaissance. The appearance of the child in this work raises the question of whether his obvious physical deformities were deliberately portrayed as an empathic gesture toward children with disabilities. Various retrospective diagnoses of the child’s condition are enumerated, one of which is considered more probable. [ABSTRACT FROM PUBLISHER]