Botulinum Toxin Treatment of Movement Disorders

Abstract

Botulinum neurotoxins (BoNTs) are now among the most widely used therapeutic agents in clinical medicine with indications applied to the fields of movement disorders, pain disorders, and autonomic dysfunction. In this literature review, the efficacy and utility of BoNTs in the field of movement disorders are assessed using the criteria of the Guideline Development Subcommittee of the American Academy of Neurology. The literature supports a level A efficacy (established) for BoNT therapy in cervical dystonia and a level B efficacy (probably effective) for blepharospasm, hemifacial spasm, laryngeal dystonia (spasmodic dysphonia), task-specific dystonias, essential tremor, and Parkinson rest tremor. It is the view of movement disorder experts, however, that despite the level B efficacy, BoNTs should be considered treatment of first choice for blepharospasm, hemifacial spasm, laryngeal, and task-specific dystonias. The emerging data on motor and vocal tics of Tourette syndrome and oromandibular dystonias are encouraging but the current level of efficacy is U (undetermined) due to lack of published high-quality studies.

http://ift.tt/2EROSyk

Novel Method for the Synthesis of Cholesteryl Glucosides starting from Disaccharides

Abstract

Steryl glucosides (SG) and acylated steryl glucosides (ASG) are natural components of plant cell membranes and present in different concentrations in various plant foods. Currently, their positive effects on human health are under investigation. The present work presents a new and efficient synthesis method for cholesteryl glucosides starting from disaccharides. A five-step synthesis protocol was done to obtain the desired product in 35% overall yield. In the first step, the hydroxy groups of the starting material sucrose are protected using benzyl ethers. After the subsequent acidic hydrolysis the obtained pyranosyl moiety of the disaccharide is transformed to its trichloroacetimidate derivative. Next, the formation of the glycosidic bond to cholesterol is performed and catalytic transfer hydrogenation in order to remove the protecting groups leads to the desired product. In this context, APCI-MS-TOF has turned out to be an excellent analytical tool for the high sensitive analysis of SG as well as intermediates.

Practical applications: Due to the comparatively high amounts of SG and ASG in seeds and oils, not only the food industry but also in biodiesel production, these natural compounds are of increasing interest. However, analysis of the compounds is difficult, commercially available pure standard materials are costly and their synthesis often requires time-consuming work-up procedures. The described preparation method allows the synthesis of cholesteryl glucosides which can be used as reference or standard material for the quantitative analysis of phytosteryl glucosides in plant derived samples. The general synthesis method could be also applied to other SG and ASG derivatives.

http://ift.tt/2EPBoYa

Herpes simplex virus (HSV) encephalitis in a young man: an unusual course

We present a case of cerebral venous sinus thrombosis (CVST) as a rare complication of herpes simplex virus (HSV) encephalitis. A young man with no pertinent medical history was diagnosed with HSV encephalitis. After initial treatment, he showed improvement in symptomatology until day 6 when he acutely developed new neurological deficits. An urgent MRI brain showed changes in left temporal lobe consistent with HSV encephalitis and lack of flow void in superior sagittal sinus. Subsequent magnetic resonance venography confirmed the diagnosis of superior sagittal sinus thrombosis along with thrombosis of bilateral frontoparietal cortical draining veins. Anticoagulation was immediately initiated and oral anticoagulation was continued for 1 year. He made complete recovery subsequently. Our case serves as a reminder for the treating clinicians to consider CVST in patients with HSV encephalitis who develop an unexpected new neurological deficits during early phase of appropriate treatment.

http://ift.tt/2FrCAhm

Pleuroparenchymal fibroelastosis with positive MPO-ANCA diagnosed with a CT-guided percutaneous needle biopsy

A 67-year-old woman was referred to our hospital because of gradually increasing dyspnoea on exertion for 6 months. Chest CT scan showed subpleural parenchymal fibrotic opacities with traction bronchiectasis in the bilateral upper lung fields. Serum rheumatoid factor and myeloperoxidase-antineutrophil cytoplasmic antibody (MPO-ANCA) were positive. There was no evident reason to suspect connective tissue disease such as ANCA-associated vasculitis or rheumatoid arthritis. We performed a CT-guided percutaneous needle biopsy of the subpleural lesion that showed slight uptake on the fluorodeoxyglucose-positron emission tomography (FDG-PET) CT scan. This specimen showed subpleural fibrosis as evidenced by an abnormal increase of elastic tissue and minimal collagen deposition, which indicated pleuroparenchymal fibroelastosis (PPFE). Although PPFE can be associated with a variety of causes, its association with MPO-ANCA is unknown. A CT-guided transthoracic lung biopsy caused no adverse events and was useful in the diagnosis of PPFE in our patient.

http://ift.tt/2EOWvWp

Blueberry eye: acquired total anterior staphyloma

Description

A 51-year-old woman presented to the ophthalmology emergency room with inability to close the eyelid, severe pain and light sensitivity in the left eye. Ocular examination showed severe conjunctival injection, deep corneal vascularisation, and extremely thin and protruded cornea, measuring 12x14 mm, with iridocorneal adhesions (figure 1A). The protruded lesion had a typical blueberry-like external appearance, due to the iris plugging the perforated ulcer. The patient's best corrected visual acuity (BCVA) was reduced to hand motion in the left eye and was 20/40 in the right eye, with the intraocular pressure within the normal range in both eyes. The patient was previously diagnosed with fungal keratitis following trauma but was non-compliant with topical antifungal agents and did not maintain a proper follow-up. A sclerokeratoplasty using a 14 mm corneal graft, as well as iridectomy and lensectomy, was performed in her left eye. The corneal graft remained clear...

http://ift.tt/2Ftpctb

Increased incidence of inguinal hernia in offspring of female survivors of childhood central nervous system tumors

Abstract

Female survivors of childhood central nervous system (CNS) tumors experience an increased risk of infertility and various pregnancy complications. We aimed to explore whether the incidence of inguinal hernia could be higher in offspring of female CNS tumor survivors as compared to the general population. Girls who were younger than 15 years of age and diagnosed with CNS tumors between 1958 and 2013 were identified from the Swedish Cancer Registry. By linking with the Swedish Medical Birth Registry, we identified 580 children who were born to mothers of CNS tumor survivors. After 9662 person-years of follow-up, 23 of them were diagnosed with inguinal hernia, giving an incidence rate (IR) of 2.4 per 1000 person-years, whereas the IR was 1.2 for the matched controls. Cox regression analysis was used to calculate the hazard ratio (HR) of inguinal hernia.After adjusting for some confounding factors, the incidence of inguinal hernia was significantly increased with a HR of 1.92 (95%CI 1.08-3.41). The association was predominant in offspring whose mothers were diagnosed with CNS tumors at the age of 10 and older, and with CNS tumors located in the brain. This article is protected by copyright. All rights reserved.

http://ift.tt/2GFnHY3

Parent emotion regulation socializes children's adaptive physiological regulation

Abstract

Parenting practices play a major role in socializing children's developing regulatory abilities, but less is known about how parents' regulatory abilities relate to children's healthy functioning. This study examined whether parents' physiological and emotion regulation abilities corresponded to children's physiological and emotional responding to a structured laboratory-based disappointment task. Ninety-seven 3- to 7-year-olds (56 girls; M = 5.79 years) and one parent participated in a multi-method assessment of parents' and children's regulatory functioning. Direct (coaching children to use reappraisal) and indirect (resting physiology, dispositional use of reappraisal) aspects of parents' regulatory abilities were assessed. As expected, an adaptive pattern of parent regulatory abilities composed of higher resting respiratory sinus arrhythmia, use of reappraisal, and coaching reappraisal was associated with children's physiological reactivity after a disappointment indicative of more effective physiological calming in a recovery context (increased parasympathetic activation). In contrast, parents' regulatory abilities did not relate to changes in children's expressions of emotional distress.

http://ift.tt/2FrNvrg