In vivo confocal microscopy of meibomian glands in primary blepharospasm: A prospective case-control study in a Chinese population.

PubMed

Lin, Tong; Gong, Lan

2016-06-01

The aim of the study was to evaluate the morphological changes of meibomian glands (MGs) in primary blepharospasm (PBS) by in vivo laser scanning confocal microscopy (LSCM) and to investigate the correlations between clinical data of PBS and LSCM parameters of MGs. This prospective and case-control study recruited 30 consecutive PBS patients and 30 age- and gender-matched healthy controls. After questionnaire assessments of ocular surface disease index (OSDI), Jankovic rating scale, and blepharospasm disability index, all subjects underwent blink rate evaluation, tear film break-up time (TBUT), corneal fluorescein staining (CFS), Schirmer test, MG expressibility, meibum quality, MG dropout, and LSCM examination of the MGs. The main LSCM outcomes included the mean MG acinar area and density, orifice diameter, meibum secretion reflectivity, acinar irregularity, and inhomogeneity of interstice and acinar wall. The PBS patients had significantly higher blink rate, higher OSDI and CFS scores, lower TBUT and Schirmer test value, and worse MG expressibility than the controls (All P < 0.05), whereas meibum quality showed no difference (P > 0.05). The PBS patients showed lower values of MG acinar area, orifice diameter and meibum secretion reflectivity, and higher scores of acinar irregularity and inhomogeneity of interstices than the controls (All P < 0.05). For the PBS patients, the severity of blepharospasm evaluated by JCR scale was strong correlated with MG acinar area (P < 0.001), orifice diameter (P = 0.002), meibum secretion reflectivity (P = 0.002), and MG acinar irregularity (P = 0.013). The MG expressibility was significantly correlated to MG acinar area (P = 0.039), orifice diameter (P < 0.001), and MG acinar irregularity (P = 0.014). The OSDI score was moderate correlated with MG acinar irregularity (P = 0.016), whereas the TBUT value was positively correlated with MG acinar area (P = 0.045) and negatively correlated to MG acinar irregularity (P = 0.016). The CFS score was negatively correlated to MG orifice diameter (P = 0.008). The LSCM provided a noninvasive tool for in vivo histopathologic studies of MGs in PBS patients. The excessive constriction of lid muscles closely related to MG morphological alterations of PBS, which offered a new research approach to interpret the interactional mechanism between dry eye and PBS.

In vivo confocal microscopy of meibomian glands in primary blepharospasm

PubMed Central

Lin, Tong; Gong, Lan

2016-01-01

Abstract The aim of the study was to evaluate the morphological changes of meibomian glands (MGs) in primary blepharospasm (PBS) by in vivo laser scanning confocal microscopy (LSCM) and to investigate the correlations between clinical data of PBS and LSCM parameters of MGs. This prospective and case–control study recruited 30 consecutive PBS patients and 30 age- and gender-matched healthy controls. After questionnaire assessments of ocular surface disease index (OSDI), Jankovic rating scale, and blepharospasm disability index, all subjects underwent blink rate evaluation, tear film break-up time (TBUT), corneal fluorescein staining (CFS), Schirmer test, MG expressibility, meibum quality, MG dropout, and LSCM examination of the MGs. The main LSCM outcomes included the mean MG acinar area and density, orifice diameter, meibum secretion reflectivity, acinar irregularity, and inhomogeneity of interstice and acinar wall. The PBS patients had significantly higher blink rate, higher OSDI and CFS scores, lower TBUT and Schirmer test value, and worse MG expressibility than the controls (All P < 0.05), whereas meibum quality showed no difference (P > 0.05). The PBS patients showed lower values of MG acinar area, orifice diameter and meibum secretion reflectivity, and higher scores of acinar irregularity and inhomogeneity of interstices than the controls (All P < 0.05). For the PBS patients, the severity of blepharospasm evaluated by JCR scale was strong correlated with MG acinar area (P < 0.001), orifice diameter (P = 0.002), meibum secretion reflectivity (P = 0.002), and MG acinar irregularity (P = 0.013). The MG expressibility was significantly correlated to MG acinar area (P = 0.039), orifice diameter (P < 0.001), and MG acinar irregularity (P = 0.014). The OSDI score was moderate correlated with MG acinar irregularity (P = 0.016), whereas the TBUT value was positively correlated with MG acinar area (P = 0.045) and negatively correlated to MG acinar irregularity (P = 0.016). The CFS score was negatively correlated to MG orifice diameter (P = 0.008). The LSCM provided a noninvasive tool for in vivo histopathologic studies of MGs in PBS patients. The excessive constriction of lid muscles closely related to MG morphological alterations of PBS, which offered a new research approach to interpret the interactional mechanism between dry eye and PBS. PMID:27281086

Dysport (botulinum toxin type A) in routine therapeutic usage: a telephone needs assessment survey of European physicians to evaluate current awareness and adherence to product labeling changes.

PubMed

Hubble, Jean; Schwab, Joseph; Hubert, Catherine; Abbott, Chandra Coleman

2013-01-01

Botulinum neurotoxin type A is a well-established treatment for a number of conditions involving muscle hyperactivity. Dysport (Ipsen Ltd, Wrexham, United Kingdom) is a botulinum neurotoxin type A preparation that has been available for a number of therapeutic uses for over 20 years in the European Union (EU). This survey was part of the EU botulinum toxin risk management plan to identify potential educational needs of injectors by collecting data on their routine practice administration of Dysport and their awareness of potential adverse events (AEs) that are included in the current product labeling. Dysport-experienced injectors in 5 EU countries were surveyed via telephone about their experience of Dysport in patients with cervical dystonia, adult upper and lower limb spasticity, pediatric cerebral palsy, and blepharospasm/hemifacial spasm. The reconstitution dilution volume most often used was 2.5 mL per 500 U for all indications. The mean total dose ranged from 387 to 530 U for cervical dystonia, 508 to 773 U for upper limb spasticity, 600 to 832 U for lower limb spasticity, 375 to 700 U for pediatric cerebral palsy, and 54 to 213 U for blepharospasm/hemifacial spasm. The potential AEs most commonly mentioned by surveyed physicians were dysphagia for cervical dystonia, arm muscle weakness for upper limb spasticity, leg muscle weakness for lower limb spasticity, and pediatric cerebral palsy and ptosis for blepharospasm/hemifacial spasm. The results indicate that product-labeling recommendations are generally applied in clinical practice and that there is a good familiarity with potential AEs based on clinical condition. Nevertheless, the survey shows that experienced injectors do sometimes deviate from the manufacturers labeling recommendations, highlighting the importance of ongoing education.

Crotoxin in humans: analysis of the effects on extraocular and facial muscles.

PubMed

Ribeiro, Geraldo de Barros; Almeida, Henderson Celestino de; Velarde, David Toledo

2012-01-01

Crotoxin is the main neurotoxin of South American rattlesnake Crotalus durissus terrificus. The neurotoxic action is characterized by a presynaptic blockade. The purpose of this research is to assess the ability of crotoxin to induce temporary paralysis of extraocular and facial muscles in humans. Doses of crotoxin used ranged from 2 to 5 units (U), each unit corresponding to one LD50. We first applied 2U of crotoxin in one of the extraocular muscles of 3 amaurotic individuals to be submitted to ocular evisceration. In the second stage, we applied crotoxin in 12 extraocular muscles of 9 patients with strabismic amblyopia. In the last stage, crotoxin was used in the treatment of blepharospasm in another 3 patients. No patient showed any systemic side effect or change in vision or any eye structure problem after the procedure. The only local side effects observed were slight conjunctival hyperemia, which recovered spontaneously. In 2 patients there was no change in ocular deviation after 2U crotoxin application. Limitation of the muscle action was observed in 8 of the 12 applications. The change in ocular deviation after application of 2U of crotoxin (9 injections) was in average 15.7 prism diopters (PD). When the dose was 4U (2 applications) the change was in average 37.5 PD and a single application of 5U produced a change of 16 PD in ocular deviation. This effect lasted from 1 to 3 months. Two of the 3 patients with blepharospasm had the hemifacial spasm improved with crotoxin, which returned after 2 months. This study provides data suggesting that crotoxin may be a useful new therapeutic option for the treatment of strabismus and blepharospasm. We expect that with further studies crotoxin could be an option for many other medical areas.

A Dynamic Circuit Hypothesis for the Pathogenesis of Blepharospasm.

PubMed

Peterson, David A; Sejnowski, Terrence J

2017-01-01

Blepharospasm (sometimes called "benign essential blepharospasm," BEB) is one of the most common focal dystonias. It involves involuntary eyelid spasms, eye closure, and increased blinking. Despite the success of botulinum toxin injections and, in some cases, pharmacologic or surgical interventions, BEB treatments are not completely efficacious and only symptomatic. We could develop principled strategies for preventing and reversing the disease if we knew the pathogenesis of primary BEB. The objective of this study was to develop a conceptual framework and dynamic circuit hypothesis for the pathogenesis of BEB. The framework extends our overarching theory for the multifactorial pathogenesis of focal dystonias (Peterson et al., 2010) to incorporate a two-hit rodent model specifically of BEB (Schicatano et al., 1997). We incorporate in the framework three features critical to cranial motor control: (1) the joint influence of motor cortical regions and direct descending projections from one of the basal ganglia output nuclei, the substantia nigra pars reticulata, on brainstem motor nuclei, (2) nested loops composed of the trigeminal blink reflex arc and the long sensorimotor loop from trigeminal nucleus through thalamus to somatosensory cortex back through basal ganglia to the same brainstem nuclei modulating the reflex arc, and (3) abnormalities in the basal ganglia dopamine system that provide a sensorimotor learning substrate which, when combined with patterns of increased blinking, leads to abnormal sensorimotor mappings manifest as BEB. The framework explains experimental data on the trigeminal reflex blink excitability (TRBE) from Schicatano et al. and makes predictions that can be tested in new experimental animal models based on emerging genetics in dystonia, including the recently characterized striatal-specific D1R dopamine transduction alterations caused by the GNAL mutation. More broadly, the model will provide a guide for future efforts to mechanistically link multiple factors in the pathogenesis of BEB and facilitate simulations of how exogenous manipulations of the pathogenic factors could ultimately be used to prevent and reverse the disorder.

A Dynamic Circuit Hypothesis for the Pathogenesis of Blepharospasm

PubMed Central

Peterson, David A.; Sejnowski, Terrence J.

2017-01-01

Blepharospasm (sometimes called “benign essential blepharospasm,” BEB) is one of the most common focal dystonias. It involves involuntary eyelid spasms, eye closure, and increased blinking. Despite the success of botulinum toxin injections and, in some cases, pharmacologic or surgical interventions, BEB treatments are not completely efficacious and only symptomatic. We could develop principled strategies for preventing and reversing the disease if we knew the pathogenesis of primary BEB. The objective of this study was to develop a conceptual framework and dynamic circuit hypothesis for the pathogenesis of BEB. The framework extends our overarching theory for the multifactorial pathogenesis of focal dystonias (Peterson et al., 2010) to incorporate a two-hit rodent model specifically of BEB (Schicatano et al., 1997). We incorporate in the framework three features critical to cranial motor control: (1) the joint influence of motor cortical regions and direct descending projections from one of the basal ganglia output nuclei, the substantia nigra pars reticulata, on brainstem motor nuclei, (2) nested loops composed of the trigeminal blink reflex arc and the long sensorimotor loop from trigeminal nucleus through thalamus to somatosensory cortex back through basal ganglia to the same brainstem nuclei modulating the reflex arc, and (3) abnormalities in the basal ganglia dopamine system that provide a sensorimotor learning substrate which, when combined with patterns of increased blinking, leads to abnormal sensorimotor mappings manifest as BEB. The framework explains experimental data on the trigeminal reflex blink excitability (TRBE) from Schicatano et al. and makes predictions that can be tested in new experimental animal models based on emerging genetics in dystonia, including the recently characterized striatal-specific D1R dopamine transduction alterations caused by the GNAL mutation. More broadly, the model will provide a guide for future efforts to mechanistically link multiple factors in the pathogenesis of BEB and facilitate simulations of how exogenous manipulations of the pathogenic factors could ultimately be used to prevent and reverse the disorder. PMID:28326032

Pilot Study of Topical Acetyl Hexapeptide-8 in Treatment of Blepharospasm in Patients Receiving Botulinum Neurotoxin Therapy

PubMed Central

Lungu, Codrin; Considine, Elaine; Zahir, Sana; Ponsati, Berta; Arrastia, Silvia; Hallett, Mark

2016-01-01

Background Injectable botulinum neurotoxin (BoNT) is the principal effective treatment for blepharospasm (BSP). This trial explores the safety and efficacy of topical Acetyl Hexapeptide-8 (AH8), a competitive SNAP25 inhibitor, as a potential new therapy in BSP. Methods Double-blind, placebo-controlled, randomized trial of daily topical application of AH8 in 24 patients with BSP. The primary outcome was time to return to baseline Jankovic Rating Scale (JBRS) after a BoNT injection simultaneously with initiation of AH8. Patients displaying a strictly regular pattern of response to 3-monthly injections of BoNT were included. Results There were no significant adverse events. There was a trend for longer time until return to baseline JBRS after injection in the active group compared to placebo (3.7 vs 3.0 months), and for better scores in the active group. One third (4/12) of the patients in the active group had a significant extension of symptom control after BoNT (range: 3.3-7.1 months). Conclusions Topical AH8 is safe and promising for extending the duration of action of BoNT therapy for BSP. PMID:23146065

Effectiveness and cost-effectiveness of a patient-initiated botulinum toxin treatment model for blepharospasm and hemifacial spasm compared to standard care: study protocol for a randomised controlled trial.

PubMed

Wickwar, Sadie; McBain, Hayley; Newman, Stanton P; Hirani, Shashivadan P; Hurt, Catherine; Dunlop, Nicola; Flood, Chris; Ezra, Daniel G

2016-03-09

Blepharospasm and hemifacial spasm are debilitating conditions that significantly impact on patient quality of life. Cyclical treatment with botulinum toxin injections offers temporary relief, but the duration of treatment efficacy is variable. The standard model of patient care defines routine fixed-time based scheduled treatment cycles which may lead to unnecessarily frequent treatment for some patients and experience of distressing symptoms in others, if symptoms return before the scheduled follow-up period. A randomised controlled trial will compare a patient-initiated model of care, where patients determine botulinum toxin treatment timing, to the standard model of care in which care is scheduled by the clinical team. A sample of 266 patients with blepharospasm or hemifacial spasm will be recruited from Moorfields Eye Hospital (MEH), London. The trial will be accompanied by a mixed-methods evaluation of acceptability of the new service. Patients who meet eligibility criteria will be assessed at baseline and those in the intervention group will be provided with instructions on how to book their own treatment appointments. Patients in both groups will be followed up 3 and 9 months into the trial and all patients will be returned to usual care after 9 months to meet safety protocols. Primary outcome measures include disease severity (questionnaire), functional disability (questionnaire) and patient satisfaction with care (questionnaire). Secondary outcomes include disease-specific quality of life (questionnaire), mood (questionnaire), illness and treatment perceptions (questionnaire and semi-structured interviews), economic impact (questionnaire) and acceptability (questionnaire and semi-structured interviews). This trial will assess the effectiveness and cost-effectiveness of a patient-led care model for botulinum toxin therapy. If the new model is shown to be effective in reducing distress and disability in these populations and is found to be acceptable to patients, whilst being cost-effective, this will have significant implications for service organisation across the NHS. UK Clinical Research Network (UKCRN) Portfolio 18660. Clinicaltrials.gov ID NCT102577224 (registered 29 October 2015).

The Changes in Corneal Astigmatism after Botulinum Toxin-A Injection in Patients with Blepharospasm

PubMed Central

Moon, Nam Ju; Lee, Hyeon Il

2006-01-01

To determine if the involuntary contractions of eyelids may have any effects on the development of corneal astigmatism, we performed this prospective study which includes 19 patients with either essential blepharospasm or hemifacial spasm. In hemifacial spasm, the degree of corneal astigmatism was evaluated between two eyes. Then the topographic changes were checked using vector analysis technique before and after passively opening the eyelids. They were also measured before and at 1 and 6 months after the injection of Botulinum toxin. Resultantly, 20 eyes had the with-the-rule (group1) and 9 eyes against-the-rule (group2) astigmatism. In hemifacial spasm, significantly more astigmatism was found at spastic eyes. The corneal topographic changes after passively opening the eyelids showed 10 eyes with the astigmatic shift to the with-the-rule, while the remaining 19 to the against-the-rule. At 1 month after injection of Botulinum toxin, group 1 showed reduced average corneal astigmatism, whereas group 2 showed increased astigmatism. The astigmatic change vector showed significantly more against-the-rule. In the contrary, 6 months after treatment, corneal astigmatism again increased in group 1 and decreased in group 2. So they took on the appearance of pretreatment astigmatic status eventually. Conclusively eyelids may play an important role in corneal curvature. PMID:16479079

Topical anesthesia without intracameral lidocaine in cataract surgery.

PubMed

Spiritus, A; Huygens, M; Callebaut, F

2000-01-01

To evaluate the efficacy of topical anesthesia with oxybuprocaïne 0.4% without intracameral lidocaïne as an alternative to peribulbar or retrobulbar anesthesia in cataract surgery. Fifty-eight patients (eighty-two eyes) were included in this study. All patients received topical anesthesia with oxybuprocaïne 0.4%. No intracameral lidocaïne was used at the start of the intervention. Seventy-five per cent of patients received oral sedation with lorazepam 2.5 mg. All surgery was done using a superior corneal incision and phacoemulsification followed by a foldable IOL implantation. Subjective pain was assessed at 4 intervals during surgery using a 4-point pain scale. All patients were evaluated for intraoperative eye motility and blepharospasm. Patient and surgeon satisfaction was measured with a 4-point satisfaction scale. 15% of patients experienced mild pain during phaco and 43% had mild pain during corneal suturing. No patient had severe pain during the operation. In 4% of patients, intracameral lidocaïne was used to relieve pain. The surgeon and patient satisfaction was high. No eye movements or blepharospasm were recorded in 75% and 62% of cases respectively. No serious complications occurred. Topical anesthesia is a safe and effective alternative to peribulbar and retrobulbar anesthesia in corneal cataract surgery for the experienced surgeon.

Clinical outcomes of individualized botulinum neurotoxin type A injection techniques in patients with essential blepharospasm.

PubMed

Sung, Youngje; Nam, Sang Min; Lew, Helen

2015-04-01

To assess the clinical outcomes following botulinum neurotoxin type A (BoNT-A) treatment with an individualized injection technique based on the types of spasms and to compare the results of the individualized injection technique with those of the conventional injection technique in the same patients. From November 2011 to July 2013, 77 BoNT-A injections were performed in 38 patients. Eighteen patients were treated with conventional BoNT-A injections before 2011, and 20 patients were referred to our hospital for unsatisfactory results after a conventional injection technique. We classified the patients by spasm-dominant sites: the lateral orbital area, representing the orbital orbicularis-dominant group (ODG); the glabella, representing the corrugator-dominant group (CDG); and the ptosis, representing the palpebral part of the orbicularis-dominant group (PDG). We increased the injection dose into the spasm-dominant sites of the blepharospasm groups. We assessed subjective symptom scores (functional disability score, FDS) after treatment. This study included 38 patients (26 women, 12 men; mean age, 60.6 ± 10.9 years). There were 21 patients in the ODG, 10 patients in the CDG, and 7 patients in the PDG. Mean ages were 59.7 ± 12.6, 59.8 ± 8.5, and 66.8 ± 9.0 years, and mean BoNT-A injection dose was 38.8 ± 11.2, 38.8 ± 11.2, and 38.8 ± 10.8 U in each group, respectively (p = 0.44, 0.82 Kruskal-Wallis test). Mean FDS after injection was 1.7 ± 0.7 in the ODG, 1.4 ± 0.8 in the CDG, and 1.2 ± 0.3 in the PDG. There were significant differences in reading and job scale among the three groups. In a comparison between the conventional and individualized injection techniques, there was a significant improvement in mean FDS and in the reading scale in the PDG with the individualized injection technique. The success rate was 92.1% in the conventional injection group and 94.1% in the individualized injection group. The individualized injection technique of BoNT-A according to the spasm-dominant site is an effective and safe treatment method for essential blepharospasm patients.

A novel ANO3 variant identified in a 53-year-old woman presenting with hyperkinetic dysarthria, blepharospasm, hyperkinesias, and complex motor tics.

PubMed

Blackburn, Patrick R; Zimmermann, Michael T; Gass, Jennifer M; Harris, Kimberly G; Cousin, Margot A; Boczek, Nicole J; Ross, Owen A; Klee, Eric W; Brazis, Paul W; Van Gerpen, Jay A; Atwal, Paldeep S

2016-12-05

Cervical dystonias have a variable presentation and underlying etiology, but collectively represent the most common form of focal dystonia. There are a number of known genetic forms of dystonia (DYT1-27); however the heterogeneity of disease presentation does not always make it easy to categorize the disease by phenotype-genotype comparison. In this report, we describe a 53-year-old female who presented initially with hand tremor following a total hip arthroplasty. The patient developed a mixed hyperkinetic disorder consisting of chorea, dystonia affecting the upper extremities, dysarthria, and blepharospasm. Whole exome sequencing of the patient revealed a novel heterozygous missense variant (Chr11(GRCh38): g.26525644C > G; NM_031418.2(ANO3): c.702C > G; NP_113606.2. p.C234W) in exon 7 in the ANO3 gene. ANO3 encodes anoctamin-3, a Ca +2 -dependent phospholipid scramblase expressed in striatal-neurons, that has been implicated in autosomal dominant craniocervical dystonia (Dystonia-24, DYT24, MIM# 615034). To date, only a handful of cases of DYT-24 have been described in the literature. The complex clinical presentation of the patient described includes hyperkinesias, complex motor movements, and vocal tics, which have not been reported in other patients with DYT24. This report highlights the utility of using clinical whole exome sequencing in patients with complex neurological phenotypes that would not normally fit a classical presentation of a defined genetic disease.

A Novel Animal Model for Investigating the Neural Basis of Focal Dystonia

DTIC Science & Technology

2016-09-01

basal ganglia as the predisposing condition and dry eye as an environmental trigger. Based on experiments during the 1st year of the grant, our...experiments and preliminary recordings from the superior colliculus. 15. SUBJECT TERMS Dystonia, benign essential blepharospasm, dry eye , motor...that hypersynchronized, 7 Hz neuronal oscilla‐ tions of the basal ganglia created the predisposing condition and that  eye  irritation from  dry   eye  was

Effect of topical administration of tramadol on corneal wound healing in rats.

PubMed

Cuvas Apan, Ozgun; Ozer, Murat Atabey; Takir, Selcuk; Apan, Alparslan; Sengul, Demet

2016-10-01

In this study, we aimed to investigate the effects of topical tramadol administration on corneal wound healing, and examine ophthalmic structures and intraocular pressure 7 days after tramadol administration. The experiments were conducted on eight male Wistar rats (250-300 g). After ophthalmic examination, epithelial cell layers in the central cornea were wounded. Rats received 30 μL of tramadol hydrochloride in one eye (Group Tramadol) and the same volume of vehicle in the other (Group Control) every 12 h for 7 days. Both eyes were stained with fluorescein dye, photographed, and wound area was calculated every 8 h until complete healing was observed. Eye blink frequency and corneal reflex tests were measured before and after drug administrations. After 7 days, slit lamp biomicroscopy, fundoscopy, Goldmann applanation tonometry, and histological evaluation were performed. There was no difference in the corneal wound healing rates between the tramadol and control groups. Reduction in wound area over time was also similar; group-time interaction was insignificant (F = 738.911; p = 0.225). Tramadol application resulted in blinking and blepharospasm for 30 s, but vehicle did not. Corneal reflex was intact and eye blink frequency test results were similar in all measurement times in both groups. Slit lamp biomicroscopy, fundoscopy, and intraocular pressures were within normal range. Corneal cells appeared unaffected by the repeated doses of tramadol for 7 days. Topical tramadol application on the cornea did not cause any side effect, except for initial temporary blinking and blepharospasm. Corneal wound healing was not affected, either.

Changes in ocular higher-order aberrations following botulinum toxin treatment in patients with blepharospasm : BTX improves dry eye in patients with BEB.

PubMed

Isshiki, Yoshihiko; Ishikawa, Hiroto; Mimura, Osamu

2016-11-01

To evaluate the effects of botulinum toxin type A (BTX-A) treatment in patients with benign essential blepharospasm (BEB) by monitoring the ocular surface and ocular higher-order aberrations (HOAs) before and after treatment. The present study reports a prospective case series of 38 patients (76 eyes, 11 men and 27 women; mean age 66.8 ± 9.8 years) with BEB who underwent BTX-A treatment at Kokura Memorial Hospital between 2013 and 2014. Patients were evaluated for ophthalmoscopic findings, Schirmer I test, tear film break-up time (t-BUT), HOAs, fluctuation index (FI), stability index (SI) using a wavefront aberrometer, 15 subjective symptoms using the Dry Eye-Related Quality of Life Score (DEQS), and complications before and after the treatment. After BTX-A treatment, the Schirmer I test score improved significantly from 5.9 ± 5.4 to 8.7 ± 6.1 mm and t-BUT recovered from 5.2 ± 1.7 to 7.3 ± 1.7 s. HOAs were classified into four patterns: stable (60.5 %), small fluctuation (14.5 %), sawtooth (17.1 %), and reverse sawtooth (7.9 %), and they significantly reduced after the treatment. Only FI (not SI) showed a marked reduction, and the DEQS significantly improved from 44.7 ± 21.6 to 37.6 ± 21.0 after the treatment (p < 0.05). There were no complications during the observations. BTX-A is a treatment with a high potential to improve ocular surface disorders induced by BEB.

Epidemiological, clinical and genetic aspects of adult onset isolated focal dystonia in Ireland.

PubMed

Williams, L; McGovern, E; Kimmich, O; Molloy, A; Beiser, I; Butler, J S; Molloy, F; Logan, P; Healy, D G; Lynch, T; Walsh, R; Cassidy, L; Moriarty, P; Moore, H; McSwiney, T; Walsh, C; O'Riordan, S; Hutchinson, M

2017-01-01

Adult onset idiopathic isolated focal dystonia presents with a number of phenotypes. Reported prevalence rates vary considerably; well-characterized cohorts are important to our understanding of this disorder. To perform a nationwide epidemiological study of adult onset idiopathic isolated focal dystonia in the Republic of Ireland. Patients with adult onset idiopathic isolated focal dystonia were recruited from multiple sources. Diagnosis was based on assessment by a neurologist with an expertise in movement disorders. When consent was obtained, a number of clinical features including family history were assessed. On the prevalence date there were 592 individuals in Ireland with adult onset idiopathic isolated focal dystonia, a point prevalence of 17.8 per 100 000 (95% confidence interval 16.4-19.2). Phenotype numbers were cervical dystonia 410 (69.2%), blepharospasm 102 (17.2%), focal hand dystonia 39 (6.6%), spasmodic dysphonia 18 (3.0%), musician's dystonia 17 (2.9%) and oromandibular dystonia six (1.0%). Sixty-two (16.5%) of 375 consenting index cases had a relative with clinically confirmed adult onset idiopathic isolated focal dystonia (18 multiplex and 24 duplex families). Marked variations in the proportions of patients with tremor, segmental spread, sensory tricks, pain and psychiatric symptoms by phenotype were documented. The prevalence of adult onset idiopathic isolated focal dystonia in Ireland is higher than that recorded in many similar service-based epidemiological studies but is still likely to be an underestimate. The low proportion of individuals with blepharospasm may reflect reduced environmental exposure to sunlight in Ireland. This study will serve as a resource for international comparative studies of environmental and genetic factors in the pathogenesis of the disorder. © 2016 EAN.

Hemifacial Spasm: A Neurosurgical Perspective

PubMed Central

Kong, Doo-Sik

2007-01-01

Hemifacial spasm (HFS) is characterized by tonic clonic contractions of the muscles innervated by the ipsilateral facial nerve. Compression of the facial nerve by an ectatic vessel is widely recognized as the most common underlying etiology. HFS needs to be differentiated from other causes of facial spasms, such as facial tic, ocular myokymia, and blepharospasm. To understand the overall craniofacial abnormalities and to perform the optimal surgical procedures for HFS, we are to review the prevalence, pathophysiology, differential diagnosis, details of each treatment modality, usefulness of brainstem auditory evoked potentials monitoring, debates on the facial EMG, clinical course, and complications from the literature published from 1995 to the present time. PMID:19096569

[Facial tics and spasms].

PubMed

Potgieser, Adriaan R E; van Dijk, J Marc C; Elting, Jan Willem J; de Koning-Tijssen, Marina A J

2014-01-01

Facial tics and spasms are socially incapacitating, but effective treatment is often available. The clinical picture is sufficient for distinguishing between the different diseases that cause this affliction.We describe three cases of patients with facial tics or spasms: one case of tics, which are familiar to many physicians; one case of blepharospasms; and one case of hemifacial spasms. We discuss the differential diagnosis and the treatment possibilities for facial tics and spasms. Early diagnosis and treatment is important, because of the associated social incapacitation. Botulin toxin should be considered as a treatment option for facial tics and a curative neurosurgical intervention should be considered for hemifacial spasms.

Prospective Study Evaluating IncobotulinumtoxinA for Cervical Dystonia or Blepharospasm: Interim Results from the First 145 Subjects with Cervical Dystonia.

PubMed

Fernandez, Hubert H; Pagan, Fernando; Danisi, Fabio; Greeley, David; Jankovic, Joseph; Verma, Amit; Sethi, Kapil; Pappert, Eric J

2013-01-01

We report the interim results from XCiDaBLE, a large, prospective, observational "naturalistic" study evaluating Xeomin® (incobotulinumtoxinA) for Cervical Dystonia or BLEpharospasm in the United States. Subjects (≥ 18 years old) with cervical dystonia (CD) are followed for two treatment cycles and monitored via Interactive Voice/Web Response. The subject's physician must have chosen to treat with incobotulinumtoxinA prior to and independent of enrollment in this study. Subject-reported scales include the Subject Global Impression-Severity and Improvement and Cervical Dystonia Impact Profile (CDIP-58), and Work Productivity and Quality of Life (QoL) are assessed by means of an employment questionnaire and work history and the SF-12v2 Health Survey (SF-12v2). Subjects are seen by the investigator for three visits, which include a baseline visit (including the first injection), a second injection visit, and a final study visit (12 weeks after the second injection). This ongoing study includes 145 subjects with a diagnosis of CD. The majority were female (82.3%) and white (91.0%) and had previously been treated with botulinum toxins (77.2%). There were 106 employed at the time of disease onset, but 12.6 years later only 44% were still employed at the time of enrolment into the study, and 20% were either receiving or seeking disability benefits. The mean total dose/treatment of CD was 225.2 units for the first injection. The CDIP-58 total score was significantly improved 4 weeks after the first injection compared to baseline (p≤0.0001). Most subjects noted improvement in their global impression assessment. No new or unexpected adverse events occurred. The results from these interim analyses confirm previous controlled, single-dose studies of incobotulinumtoxinA in terms of efficacy and safety.

Prospective Study Evaluating IncobotulinumtoxinA for Cervical Dystonia or Blepharospasm: Interim Results from the First 145 Subjects with Cervical Dystonia

PubMed Central

Fernandez, Hubert H.; Pagan, Fernando; Danisi, Fabio; Greeley, David; Jankovic, Joseph; Verma, Amit; Sethi, Kapil; Pappert, Eric J.

2013-01-01

Background We report the interim results from XCiDaBLE, a large, prospective, observational “naturalistic” study evaluating Xeomin® (incobotulinumtoxinA) for Cervical Dystonia or BLEpharospasm in the United States. Methods Subjects (≥ 18 years old) with cervical dystonia (CD) are followed for two treatment cycles and monitored via Interactive Voice/Web Response. The subject's physician must have chosen to treat with incobotulinumtoxinA prior to and independent of enrollment in this study. Subject-reported scales include the Subject Global Impression-Severity and Improvement and Cervical Dystonia Impact Profile (CDIP-58), and Work Productivity and Quality of Life (QoL) are assessed by means of an employment questionnaire and work history and the SF-12v2 Health Survey (SF-12v2). Subjects are seen by the investigator for three visits, which include a baseline visit (including the first injection), a second injection visit, and a final study visit (12 weeks after the second injection). Results This ongoing study includes 145 subjects with a diagnosis of CD. The majority were female (82.3%) and white (91.0%) and had previously been treated with botulinum toxins (77.2%). There were 106 employed at the time of disease onset, but 12.6 years later only 44% were still employed at the time of enrolment into the study, and 20% were either receiving or seeking disability benefits. The mean total dose/treatment of CD was 225.2 units for the first injection. The CDIP-58 total score was significantly improved 4 weeks after the first injection compared to baseline (p≤0.0001). Most subjects noted improvement in their global impression assessment. No new or unexpected adverse events occurred. Discussion The results from these interim analyses confirm previous controlled, single-dose studies of incobotulinumtoxinA in terms of efficacy and safety. PMID:23724362

Sexual Well-Being in Patients with Blepharospasm, Spasmodic Torticollis, and Hemifacial Spasm: A Pilot Study

PubMed Central

Perozzo, Paola; Salatino, Adriana; Cerrato, Paolo; Ricci, Raffaella

2016-01-01

Mood, anxiety, and other psychological symptoms are common in dystonic patients suffering from blepharospasm (BSP) and spasmodic torticollis (ST). Since sexual well-being is an important aspect of mental health, here, we investigated whether these patients may also experience a worsening of their sexual life. In particular, quality of sexual life was evaluated in patients suffering from BSP (N = 30), ST (N = 30), and in a control group of patient with Hemifacial spasm (HFS; N = 30), undergoing botulinum toxin type A therapy. A group of 30 age-matched healthy volunteers constituted an additional control group. Patients were evaluated just before the periodic injection of botulinum toxin. Sexual functioning was assessed using the Sexual Functioning Inventory, a reduced form of the Golombok Rust Inventory, previously employed in patients with Parkinson’s disease. Depression (Beck Depression Inventory) and anxiety (STAI-X1/X2) were also assessed. Results revealed that sexual functioning was significantly affected in patients with BSP, ST, and HFS with respect to healthy controls. Dystonic patients manifested more sexual dysfunction than patients with HFS. Overall, females had a poorer quality of sexual life than males and, among females, women with BSP were the most dysfunctional. Psychological symptoms were present in patients with dystonia, but not in patients with HFS. As discussed in the paper, several factors might be taken into account to explain worse quality of sexual life in patients with dystonia compared to patients with hemifacial spasm. Among them an important role might be played by the central origin of dystonia pathophysiology (i.e., altered activity of cortico-striato-thalamic-cortical circuits). Future investigations are necessary to further explore these preliminary findings, considering that this is the first time that sexual well-being is evaluated in patients with BSP, ST, and HFS, and comparable data are not available. PMID:27761118

How Many Dystonias? Clinical Evidence.

PubMed

Albanese, Alberto

2017-01-01

Literary reports on dystonia date back to post-Medieval times. Medical reports are instead more recent. We review here the early descriptions and the historical establishment of a consensus on the clinical phenomenology and the diagnostic features of dystonia syndromes. Lumping and splitting exercises have characterized this area of knowledge, and it remains largely unclear how many dystonia types we are to count. This review describes the history leading to recognize that focal dystonia syndromes are a coherent clinical set encompassing cranial dystonia (including blepharospasm), oromandibular dystonia, spasmodic torticollis, truncal dystonia, writer's cramp, and other occupational dystonias. Papers describing features of dystonia and diagnostic criteria are critically analyzed and put into historical perspective. Issues and inconsistencies in this lumping effort are discussed, and the currently unmet needs are critically reviewed.

[Contribution of botulinum toxin to maxillo-facial surgery].

PubMed

Batifol, D; de Boutray, M; Goudot, P; Lorenzo, S

2013-04-01

Botulinum toxin has a wide range of use in maxillo-facial surgery due to its action on muscles, on the glandular system, and against pain. It already has been given several market authorizations as indicated for: blepharospasm, spasmodic stiff neck, and glabellar lines. Furthermore, several studies are ongoing to prove its effectiveness and usefulness for many other pathologies: treatment of pain following cervical spine surgery; action on salivary glands after trauma, hypertrophy, or hyper-salivation; analgesic action (acknowledged but still being experimented) on neuralgia, articular pain, and keloids scars due to its anti-inflammatory properties. Botulinum toxin injections in the cervico-facial area are more and more used and should be to be correctly assessed. Copyright © 2013 Elsevier Masson SAS. All rights reserved.

Grey Matter Microstructural Integrity Alterations in Blepharospasm Are Partially Reversed by Botulinum Neurotoxin Therapy

PubMed Central

Chirumamilla, Venkata Chaitanya; Koirala, Nabin; Paktas, Burcu; Deuschl, Günther; Zeuner, Kirsten E.; Groppa, Sergiu

2016-01-01

Objective Benign Essential Blepharospasm (BEB) and hemifacial spasm (HFS) are the most common hyperkinetic movement disorders of facial muscles. Although similar in clinical presentation different pathophysiological mechanisms are assumed. Botulinum Neurotoxin (BoNT) is a standard evidence-based treatment for both conditions. In this study we aimed to assess grey matter microstructural differences between these two groups of patients and compared them with healthy controls. In patients we furthermore tracked the longitudinal morphometric changes associated with BoNT therapy. We hypothesized microstructural differences between the groups at the time point of maximum symptoms representation and distinct longitudinal grey matter dynamics with symptom improvement. Methods Cross-sectional and longitudinal analyses of 3T 3D-T1 MRI images from BEB, HFS patients prior to and one month after BoNT therapy and from a group of age and sex matched healthy controls. Cortical thickness as extracted from Freesurfer was assessed as parameter of microstructural integrity. Results BoNT therapy markedly improved motor symptoms in patients with BEB and HFS. Significant differences of grey matter integrity have been found between the two patients groups. The BEB group showed lower cortical thickness at baseline in the frontal-rostral, supramarginal and temporal regions compared to patients with HFS. In this group BoNT treatment was associated with a cortical thinning in the primary motor cortex and the pre-supplementary motor area (pre-SMA). Contrary patients with HFS showed no longitudinal CT changes. A decreased cortical thickness was attested bilaterally in the temporal poles and in the right superior frontal region in BEB patients in comparison to HC. Patients in the HFS group presented a decreased CT in the left lingual gyrus and temporal pole. Conclusions Although patients with BEB and HFS present clinically with involuntary movements of facial muscles, they exhibited differences in cortical thickness. While BoNT therapy was equally effective in both groups, widespread changes of cortical morphology occurred only in BEB patients. We demonstrated specific disease- and therapy-dependent structural changes induced by BoNT in the studied hyperkinetic conditions. PMID:27992533

Scientific skepticism and new discoveries: an analysis of a report of zinc/phytase supplementation and the efficacy of botulinum toxins in treating cosmetic facial rhytides, hemifacial spasm and benign essential blepharospasm.

PubMed

Cohen, Joel L

2014-10-01

A recent paper in the Journal of Drugs in Dermatology by Koshy and colleagues (2012, 11( 4 ):507-512) report on "Effect of Dietary Zinc and Phytase Supplementation on Botulinum Toxin Treatments" and conclude by claiming the discovery of "a potentially meaningful role for zinc and/or phytase supplementation in increasing the degree and duration of botulinum toxin effect in the treatment of cosmetic facial rhytids, benign essential blepharospasm, and hemifacial spasm". The purpose of this paper is to examine these published claims for possible methodological and design errors and potential sources of bias. The authors evaluated the published results in comparison to the published literature on zinc deficiency, the role of phytase, prior reports of an effect of zinc on activity of botulinum toxin, issues of study design and execution and if the reported results of the study supported the study's conclusions. Multiple issues are present in the reported study, which appear to invalidate its conclusions. These areas include lack of direct evidence for the presence of clinical or subclinical zinc deficiency in the study population or for the level of phytate in the study population sufficient to interfere with zinc absorption in these subjects. Additionally, there is ambiguity as to the actual dose of zinc used as well as in the study design itself. Also there is a failure of the study through the "unmasking" of the crossover design. There is potential financial conflict of interest in the study execution that may have biased the reported results. Finally there is inadequate data presented to evaluate the claims made of a "new discovery" as to the three disease entities reported on and the various botulinum toxins used in each of the three treatment arms of the study. Based on this evaluation, it appears that a high level of clinical and scientific skepticism is warranted concerning any claim of a beneficial effect of zinc and phytase supplementation on the efficacy or potency of any botulinum toxins in the reported conditions.

Whole-exome sequencing for variant discovery in blepharospasm.

PubMed

Tian, Jun; Vemula, Satya R; Xiao, Jianfeng; Valente, Enza Maria; Defazio, Giovanni; Petrucci, Simona; Gigante, Angelo Fabio; Rudzińska-Bar, Monika; Wszolek, Zbigniew K; Kennelly, Kathleen D; Uitti, Ryan J; van Gerpen, Jay A; Hedera, Peter; Trimble, Elizabeth J; LeDoux, Mark S

2018-05-16

Blepharospasm (BSP) is a type of focal dystonia characterized by involuntary orbicularis oculi spasms that are usually bilateral, synchronous, and symmetrical. Despite strong evidence for genetic contributions to BSP, progress in the field has been constrained by small cohorts, incomplete penetrance, and late age of onset. Although several genetic etiologies for dystonia have been identified through whole-exome sequencing (WES), none of these are characteristically associated with BSP as a singular or predominant manifestation. We performed WES on 31 subjects from 21 independent pedigrees with BSP. The strongest candidate sequence variants derived from in silico analyses were confirmed with bidirectional Sanger sequencing and subjected to cosegregation analysis. Cosegregating deleterious variants (GRCH37/hg19) in CACNA1A (NM_001127222.1: c.7261_7262delinsGT, p.Pro2421Val), REEP4 (NM_025232.3: c.109C>T, p.Arg37Trp), TOR2A (NM_130459.3: c.568C>T, p.Arg190Cys), and ATP2A3 (NM_005173.3: c.1966C>T, p.Arg656Cys) were identified in four independent multigenerational pedigrees. Deleterious variants in HS1BP3 (NM_022460.3: c.94C>A, p.Gly32Cys) and GNA14 (NM_004297.3: c.989_990del, p.Thr330ArgfsTer67) were identified in a father and son with segmental cranio-cervical dystonia first manifest as BSP. Deleterious variants in DNAH17, TRPV4, CAPN11, VPS13C, UNC13B, SPTBN4, MYOD1, and MRPL15 were found in two or more independent pedigrees. To our knowledge, none of these genes have previously been associated with isolated BSP, although other CACNA1A mutations have been associated with both positive and negative motor disorders including ataxia, episodic ataxia, hemiplegic migraine, and dystonia. Our WES datasets provide a platform for future studies of BSP genetics which will demand careful consideration of incomplete penetrance, pleiotropy, population stratification, and oligogenic inheritance patterns. © 2018 The Authors. Molecular Genetics & Genomic Medicine published by Wiley Periodicals, Inc.

Curvularia keratomycosis in a dog.

PubMed

Ben-Shlomo, Gil; Plummer, Caryn; Barrie, Kathleen; Brooks, Dennis

2010-03-01

A 10-year-old, spayed female Bichon Frise was referred to the Veterinary Medical Center of the University of Florida with a 6-week history of blepharospasm and a nonhealing ulcer of the left eye. Ophthalmic examination revealed a 3-4 mm diameter corneal ulcer with faint edema at the lesion edge, and a raised grayish area on the paraxial cornea near the 2 o'clock position. Cytological testing revealed fungal hyphae and extracellular cocci. Culture of the lesion found heavy growth of Enterococcus faecalis and Curvularia spp. Treatment with a combination of topical antibiotics (polymyxin B, sulfate/trimetoprim, and 10% sulfacetamide sodium), 1% miconazole, autologous serum, and 5% hypertonic saline was instituted. After 8 days of medical therapy, an improvement of clinical signs was noted, and 15 days after initiation of treatment, the ulcer was healed with minimal corneal scarring.

Involuntary movements in the elderly. Parkinson's disease and other causes.

PubMed

Miller, J Q

1986-03-01

Dyskinesia is usually lifelong and progressive; therefore, physicians generally see the disorder in elderly patients. Medical treatment must be carefully selected on the basis of the cause of the dyskinesia. Parkinsonian dyskinesia is well controlled by drug therapy. However, patients can become less responsive to a drug after years of use and may experience unwelcome side effects. Cerebellar tremor is extremely disabling because it worsens with activity, but no satisfactory therapy is available. Senile, essential, and familial tremors are also intensified by action, but they can often be suppressed with a mild tranquilizer or a beta blocker. Drug treatment of blepharospasm and spastic dysphonia has been disappointing: Facial or laryngeal surgery is sometimes required. Tardive dyskinesia is caused by neuroleptic drugs, so the only therapy for the disorder is withdrawal of the offending drug.

Topical botulinum toxin.

PubMed

Collins, Ashley; Nasir, Adnan

2010-03-01

Nanotechnology is a rapidly growing discipline that capitalizes on the unique properties of matter engineered on the nanoscale. Vehicles incorporating nanotechnology have led to great strides in drug delivery, allowing for increased active ingredient stability, bioavailability, and site-specific targeting. Botulinum toxin has historically been used for the correction of neurological and neuromuscular disorders, such as torticollis, blepharospasm, and strabismus. Recent dermatological indications have been for the management of axillary hyperhydrosis and facial rhytides. Traditional methods of botulinum toxin delivery have been needle-based. These have been associated with increased pain and cost. Newer methods of botulinum toxin formulation have yielded topical preparations that are bioactive in small pilot clinical studies. While there are some risks associated with topical delivery, the refinement and standardization of delivery systems and techniques for the topical administration of botulinum toxin using nanotechnology is anticipated in the near future.

Development of future indications for BOTOX.

PubMed

Brin, Mitchell F

2009-10-01

Since the late 1970s, local injections of BoNT have provided clinical benefit for patients with inappropriately contracting muscles with or without pain or sensory disturbance. Marketing authorization for some BoNTs, depending on country, include core indications of dystonia (blepharospasm and cervical dystonia), large muscle spastic disorders (not yet approved in the United States, e.g., adult post-stroke spasticity and equinus foot deformity), hyperhidrosis and aesthetic. Subsequent development has extended to selected conditions characterized by recurrent or chronic pain (migraine headache), and urologic indications (neurogenic/idiopathic overactive bladder; prostate hyperplasia), with multiple additional opportunities available. Portfolio management requires a careful individual opportunity assessment of scientific and technical aspects (basic science foundation, potential to treat unmet medical need, product-specific risk in specific populations, therapeutic margin/safety profile, and probability of successful registration pathway). This article describes ongoing development targets for BOTOX.

Botulinum Toxin for the Treatment of Tremor and Tics.

PubMed

Lotia, Mitesh; Jankovic, Joseph

2016-02-01

The therapeutic applications of botulinum toxin (BoNT) have grown manifold since its initial approval in 1989 by the U.S. Food and Drug Administration for the treatment of strabismus, blepharospasm, and other facial spasms. Although it is the most potent biologic toxin known to man, long-term studies have established its safety in the treatment of a variety of neurologic and nonneurologic disorders. Despite a paucity of randomized controlled trials, BoNT has been found to be beneficial in treating a variety of tremors and tics when used by clinicians skilled in the administration of the drug for these hyperkinetic movement disorders. Botulinum toxin injections can provide meaningful improvement in patients with localized tremors and tics; in some cases, they may be an alternative to other treatments with more undesirable adverse effects. Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Current and future medical treatment in primary dystonia

PubMed Central

Delnooz, Cathérine C.S.

2012-01-01

Dystonia is a hyperkinetic movement disorder, characterized by involuntary and sustained contractions of opposing muscles causing twisting movements and abnormal postures. It is often a disabling disorder that has a significant impact on physical and psychosocial wellbeing. The medical therapeutic armamentarium used in practice is quite extensive, but for many of these interventions formal proof of efficacy is lacking. Exceptions are the use of botulinum toxin in patients with cervical dystonia, some forms of cranial dystonia (in particular, blepharospasm) and writer’s cramp; deep brain stimulation of the pallidum in generalized and segmental dystonia; and high-dose trihexyphenidyl in young patients with segmental and generalized dystonia. In order to move this field forward, we not only need better trials that examine the effect of current treatment interventions, but also a further understanding of the pathophysiology of dystonia as a first step to design and test new therapies that are targeted at the underlying biologic and neurophysiologic mechanisms. PMID:22783371

Systemic mycosis in three dogs from nonendemic regions.

PubMed

Pratt, Carmela L; Sellon, Rance K; Spencer, Erick S; Johnson, Ty W; Righter, Daniel J

2012-01-01

Three dogs were examined for clinical signs ultimately attributed to systemic fungal infections. One dog was evaluated for chronic, ulcerated dermal lesions and lymphadenomegaly; one dog was examined for acute onset of unilateral blepharospasm; and one dog had diarrhea and hematochezia. Two of the dogs were diagnosed with blastomycosis (one with disseminated disease and the other with the disease localized to the left eye). The third dog was diagnosed with disseminated histoplasmosis. None of the dogs originated from, or had traveled to, typical regions endemic for these fungal diseases. All diagnoses were established from histopathology and either polymerase chain reaction (PCR) or cytology and culture. The two dogs diagnosed with blastomycosis were treated with either itraconazole or ketoconazole with apparent resolution of the infections. The dog with ocular involvement had an enucleation prior to beginning therapy. The dog diagnosed with histoplasmosis was euthanized without treatment. In patients with characteristic clinical features, systemic fungal infections should still be considered as differential diagnoses regardless of their travel history.

Actinomyces bowdenii ulcerative keratitis in a dog.

PubMed

Sherman, Amanda; Daniels, Joshua B; Wilkie, David A; Lutz, Elizabeth

2013-09-01

A 5-year-old spayed female diabetic mixed-breed dog underwent phacoemulsification and intraocular lens implantation to correct bilateral hypermature cataracts. Two months postsurgery, the patient presented with ulcerative keratitis and multifocal stromal abscessation OD, which was controlled, but never resolved, with topical fluoroquinolone therapy. The patient re-presented 2 months later with a new, raised, white gritty corneal opacity associated with hyperemia, chemosis, and blepharospasm OD. Cytology of the right cornea revealed filamentous bacteria, suggestive of Actinomyces spp. Actinomyces bowdenii was subsequently isolated in pure culture and identified via 16s rDNA sequencing. Actinomyces bowdenii has never before been described as a cause of ocular infection. An immunosuppressed corneal environment likely contributed to this opportunistic Actinomycosis. The infection was not controlled with fluoroquinolone therapy, and the isolate, in vitro, was resistant to three fluoroquinolones (ciprofloxacin, ofloxacin, and levofloxacin), which also has not been previously reported for this species of Actinomyces. A superficial keratectomy with conjunctival graft was employed to successfully manage the infection. © 2012 American College of Veterinary Ophthalmologists.

Intraerythrocytic iridovirus in central bearded dragons (Pogona vitticeps).

PubMed

Grosset, Claire; Wellehan, James F X; Owens, Sean D; McGraw, Sabrina; Gaffney, Patricia M; Foley, Janet; Childress, April L; Yun, Susan; Malm, Kirsten; Groff, Joseph M; Paul-Murphy, Joanne; Weber, E Scott

2014-05-01

Three adult central bearded dragons (Pogona vitticeps) originating from a commercial breeding facility presented with clinical signs, including anorexia, dehydration, white multifocal lesions on the dorsal aspect of the tongue, blepharospasm, and weight loss. In 1 of 3 lizards, a marked regenerative anemia was noted, and all 3 bearded dragons had erythrocytic intracytoplasmic inclusion bodies. Nine bearded dragons housed in contact also had identical, but fewer intraerythrocytic inclusions. Inclusion bodies examined by electron microscopy had particles consistent with iridoviruses. Attempts to culture the virus were unsuccessful; however, amplification and sequencing of regions of the viral DNA polymerase by polymerase chain reaction confirmed the presence of an iridovirus. One of the bearded dragons died, while the 2 others showing clinical signs were euthanized. The remaining 9 infected bearded dragons of the teaching colony were also euthanized. Postmortem examination revealed a moderate, multifocal, lymphoplasmacytic or mononuclear adenitis of the tongue in the 3 bearded dragons, and a lymphohistiocytic hepatitis with bacterial granulomas in 2 lizards. © 2014 The Author(s).

Globe penetration in a cat following maxillary nerve block for dental surgery.

PubMed

Perry, Rachel; Moore, Denise; Scurrell, Emma

2015-01-01

Dental treatment was carried out in an 8.5-year-old castrated male domestic shorthair cat found to have tooth resorption. Right mandibular, and right and left maxillary nerve blocks were administered using a 1 ml syringe attached to a 25 G x 5/8 inch needle and an intraoral technique. The following day the cat displayed blepharospasm of the right eye. The ocular signs progressed and 5 days later an ophthalmologist confirmed a blind, glaucomatous right eye. It was suspected that the eye had suffered a penetrating injury during dental surgery. Enucleation of the right eye was performed and gross and histopathological examination revealed a penetrating wound consistent with a needle tract injury. Complications arising from veterinary dental regional anaesthesia appear to be rare; however, it may be that they are under-reported. This case report highlights the risks involved and reviews the safest and most efficacious regional anaesthesia technique for the feline maxilla. © ISFM and AAFP 2015.

Surgical correction of congenital entropion in related Boer goat kids using a combination Hotz-Celsus and lateral eyelid wedge resection procedure.

PubMed

Donnelly, Kevin S; Pearce, Jacqueline W; Giuliano, Elizabeth A; Fry, Pamela R; Middleton, John R

2014-11-01

Five related Boer goat kids (≤4 months of age) were presented to the University of Missouri, Veterinary Teaching Hospital (MU-VMTH) with epiphora and blepharospasm of several weeks duration and commencing prior to 1 month of age in all animals. Clinical examination confirmed euryblepharon and entropion bilaterally in two females and one male and unilaterally in two female kids. Deep stromal corneal ulceration was present in two eyes, and corneal granulation tissue and fibrosis were present in half (5/10) the affected eyes. A combination Hotz-Celsus and lateral eyelid wedge resection procedure was performed on all affected eyelids. Recheck examinations and long-term follow-up confirmed resolution of the entropion, preservation of normal eyelid conformation, and restoration of ocular comfort. Pedigree analysis ruled out sex-linked and autosomal dominant inheritance patterns; a specific mode of inheritance could not be determined. The Boer goat breed may be at increased risk for the development of entropion. This cases series represents the first report of entropion in the caprine species. © 2014 American College of Veterinary Ophthalmologists.

Histiocytic Sarcoma and Bilateral Facial Vein Thrombosis in a Siberian Hamster (Phodopus sungorus).

PubMed

Coble, Dondrae J; Shoemaker, Margaret; Harrington, Bonnie; Dardenne, Adrienne D; Bolon, Brad

2015-04-01

A 21-mo-old, male Siberian hamster (Phodopus sungorus) presented with left-sided facial swelling, proptosis of the left eye, and blepharospasm of the right eye. The hamster had been used only for breeding. Because of the poor prognosis, the hamster was euthanized without additional diagnostic assays or treatments. Routine gross pathologic evaluation demonstrated exophthalmos and presumptive hyphema of the left eye, bilateral facial edema, freely movable nodules within the mesentery, white foci within the liver, and a large mass effacing the cranial pole of the right kidney. On histologic evaluation, the mesenteric nodules and liver foci expressed histiocytic marker CD163 and thus were diagnosed as sites of histiocytic sarcoma, whereas the kidney mass was a well-differentiated renal cell carcinoma. The facial swelling resulted from bilateral, chronic, severe, branching thrombi in many facial veins. Additional age-related histopathologic findings were observed in other organs, including diffuse glomerulopathy, nesidioblastosis (pancreatic islet neoformation), and multiple foci of severe cartilage degeneration in the axial skeleton. To our knowledge, this report provides the first description of histiocytic sarcoma in a Siberian hamster.

[Botulinum toxin--the dose controls the poison. A historical sketch].

PubMed

Homann, C N; Wenzel, K; Kriechbaum, N; Suppan, K; Crevenna, R; Ivanic, G; Dressler, D

2002-06-01

Botulism, a potentially lethal form of paralytic food poisoning, was described as early as 1793. Basic research, especially in the late nineteenth and early twentieth centuries, revealed that botulism is caused by exotoxins. Further biochemical work around and after the Second World War gave insight into the molecular structure of seven different serotypes of botulinum toxin (BT/A-G) as well as into its acetylcholine blocking mode of action. In 1977, Scott treated patients with strabism by injecting minute amounts of purified BT/A. In short sequence, BT proved effective in blepharospasm, cervical dystonia, and various off-label indications. In the near future, registration of these new indications, marketing of new serotypes (BT/B), and availability of more practical antibody tests can be expected. The first applications of BT were performed by Roggenkaemper, Dressler, and Benecke in Germany and by Poewe and Auff in Austria. According to a worldwide trend, a rapid expansion regarding BT users and indications followed. Formation of BT competence centers in both countries aims at maintaining high standards in BT research and education.

A 14-year-old girl who regained normal vision after bilateral visual impairment following hot water injury to the eyes.

PubMed

Monsudi, Kehinde F; Ayanniyi, Abdulkabir A

2011-04-01

A 14-year-old girl presented with bilateral visual impairment following hot water injury to the eyes. The patient was admitted for a week and managed with guttae tropicamide 0.5% 8 h, diclofenac sodium 0.1% 4 h, and fluoroquinolone 0.3% 6 h and ointment chloramphenicol 8 h. Also, the patient was managed with capsule doxycycline 100 mg 12 h for 10 days, tablet cataflam 50 mg 12 h for 7 days and intramuscular tetanus toxoid 0.5 mg stat and dermacine cream for facial scald. The visual acuities improved from 3/60 (right eye) and 6/24 (left eye) to 6/6 in both eyes. There were resolutions of facial/eye pain, tearing, photophobia, lid edema, blepharospasm, and conjunctival hyperemia. There was complete healing of facial wounds and corneal ulcers. She was discharged from hospital on the 7th day of admission. Prompt presentation, degree of scald sustained, and appropriate medical intervention enhanced visual recovery and wound healing in the patient.

Successful medical treatment for globe penetration following tooth extraction in a dog.

PubMed

Guerreiro, Cleo E; Appelboam, Helen; Lowe, Robert C

2014-03-01

A five-year-old entire male Tibetan Terrier was referred for left-sided periorbital swelling and blepharospasm 4 days following ipsilateral maxillary tooth extraction. Examination of the left eye revealed mild exophthalmos, pain on retropulsion, and absent menace response and pupillary light reflexes. Examination of the posterior segment was not possible owing to the anterior segment pathology. Differential diagnoses considered were iatrogenic globe penetration and peribulbar abscess/cellulitis. Ocular ultrasound was consistent with a penetrating wound to the globe. Treatment with systemic prednisolone and marbofloxacin, and topical atropine sulfate 1%, prednisolone acetate, and brinzolamide was started. Marked clinical improvement allowed visual confirmation of the perforation. Oral prednisolone was tapered over the following 10 weeks. At final re-examination (10 months), the patient was visual, and fundic examination revealed an additional chorioretinal scar, most likely an exit wound that was obscured by vitreal debris on initial examinations. Neither scar was associated with retinal detachment. To the authors' knowledge, this is the first reported case of successful medical management of iatrogenic globe penetration following exodontic procedures. © 2013 American College of Veterinary Ophthalmologists.

[Sotos syndrome associated with focal dystonia].

PubMed

Bravo, M; Chacón, J; Bautista, E; Pérez-Camacho, I; Trujillo, A; Grande, M A

Sotos syndrome is a form of infantile gigantism characterized by excessive body size from the time of birth, particular facies, acromegalic changes and signs of non-progressive cerebral involvement. The etiology is unknown. Diagnosis is based on somatometric data and the particular phenotype traits. Biochemical and endocrine studies are normal. Torticollis is a focal dystonia and therefore more common in adults. A 20 year old woman with macrosomic features since birth presented with: weight 104 kg, height 182 cm; prognathism, hypertelorism, a broad over hanging forehead with a high hair line; large ears, hands and feet; torticollis towards the right with elevation and anteroversion of the right shoulder which caused symptomatic scoliosis. She was bradypsychic and rather slow in speech. The complementary tests done (cerebral and cervical CT and MR, bone gammography, evoked potentials, EMG-ENG, sural nerve biopsy, biopsy of skin and muscle, EEG and hormone and biochemistry studies) were normal. The torticollis was treated with botulinus toxin and improved considerably, as did the scoliosis. To date, dystonia has not been described in association with Sotos syndrome. This may be a causal association, or even perhaps hereditary, since the patient's mother had dystonia (in the form of blepharospasm).

A novel automated instrument designed to determine photosensitivity thresholds (Conference Presentation)

NASA Astrophysics Data System (ADS)

Aguilar, Mariela C.; Gonzalez, Alex; Rowaan, Cornelis; De Freitas, Carolina; Rosa, Potyra R.; Alawa, Karam; Lam, Byron L.; Parel, Jean-Marie A.

2016-03-01

As there is no clinically available instrument to systematically and reliably determine the photosensitivity thresholds of patients with dry eyes, blepharospasms, migraines, traumatic brain injuries, and genetic disorders such as Achromatopsia, retinitis pigmentosa and other retinal dysfunctions, a computer-controlled optoelectronics system was designed. The BPEI Photosensitivity System provides a light stimuli emitted from a bi-cupola concave, 210 white LED array with varying intensity ranging from 1 to 32,000 lux. The system can either utilize a normal or an enhanced testing mode for subjects with low light tolerance. The automated instrument adjusts the intensity of each light stimulus. The subject is instructed to indicate discomfort by pressing a hand-held button. Reliability of the responses is tracked during the test. The photosensitivity threshold is then calculated after 10 response reversals. In a preliminary study, we demonstrated that subjects suffering from Achromatopsia experienced lower photosensitivity thresholds than normal subjects. Hence, the system can safely and reliably determine the photosensitivity thresholds of healthy and light sensitive subjects by detecting and quantifying the individual differences. Future studies will be performed with this system to determine the photosensitivity threshold differences between normal subjects and subjects suffering from other conditions that affect light sensitivity.

Effect of Botulinum Toxin A on Muscle Healing and its Implications in Aesthetic and Reconstructive Surgery.

PubMed

Silberstein, Eldad; Maor, Ehud; Sukmanov, Oleg; Bogdanov Berezovsky, Alexander; Shoham, Yaron; Krieger, Yuval

2018-04-06

Muscle activity contributes to the enhancement of facial aging deformity, blepharospasm, cerebral palsy spasticity, trismus, torticollis, and other conditions. Myotomy of the involved muscles in order to reduce the deformity has variable success rates due to muscle healing and regeneration of activity. The goal of this study was to investigate whether blocking striated muscle activity with Botulinum toxin (BtxA) during the healing time after myotomy alters the healing process and reduces long-term muscle activity. Eighteen Sprague Dawley rats where divided into 3 groups: group A (n = 7) underwent myotomy of their Latisimus Dorsi muscle; group B (n = 7) underwent myotomy and injection of BtxA into their severed muscle; group C (n = 4) injection of BtxA only. Muscle strength was tested periodically using a grip test. Starting at week 16 and until the termination of study at week 22, group B (Myotomy + BtxA) showed significant reduction in muscle power compared to the two control groups. Addition of BtxA injection into a muscle immediately after myotomy may interfere with muscle healing and contribute to a more successful long-term result.

[Myths and evidence on the use of botulinum toxin: neuropharmacology and dystonia].

PubMed

Garcia-Ruiz, P J; Sanz-Cartagena, P; Martinez-Castrillo, J C; Ares-Pensado, B; Aviles-Olmos, I; Blazquez-Estrada, M; Fanjul-Arbos, S; Garcia-Caldentey, J; Gazulla, J; Gutierrez-Garcia, J; Huete-Anton, B; Lucas-Rodenas, C; Luquin, M R; Martinez-Torres, I; Medialdea-Natera, P; Mendoza-Rodriguez, A; Mir-Rivera, P; Posada, I J; Ruiz-Martinez, J; Sanchez-Alonso, P; Trejo-Gabriel Y Galan, J M; Vela, L; Pena-Segura, J L

2018-03-01

Botulinum toxin type A (BTA) is a bacterial endotoxin, whose therapeutic use has had a dramatic impact on different neurological disorders, such as dystonia and spasticity. To analyze and summarize different questions about the use of BTA in our clinical practice. A group of experts in neurology developed a list of topics related with the use of BTA. Two groups were considered: neuropharmacology and dystonia. A literature search at PubMed, mainly for English language articles published up to June 2016 was performed. The manuscript was structured as a questionnaire that includes those questions that, according to the panel opinion, could generate more controversy or doubt. The initial draft was reviewed by the expert panel members to allow modifications, and after subsequent revisions for achieving the highest degree of consensus, the final text was then validated. Different questions about diverse aspects of neuropharmacology, such as mechanism of action, bioequivalence of the different preparations, immunogenicity, etc. were included. Regarding dystonia, the document included questions about methods of evaluation, cervical dystonia, blepharospasm, etc. This review does not pretend to be a guide, but rather a tool for continuous training of residents and specialists in neurology, about different specific areas of the management of BTA.

Episcleral cyclosporine A implants for the management of unilateral keratoconjunctivitis sicca in an 8-year-old mare.

PubMed

Mackenzie, Catriona J; Carslake, Harry B; Robin, Matthew; Kent, Rebecca J; Malalana, Fernando

2017-01-01

An 8-year-old mare was presented for investigation of a 1-month history of blepharospasm, eyelid swelling, corneal edema, and ocular discharge of the right eye (OD). Ophthalmic examination confirmed mucopurulent ocular discharge, conjunctival hyperemia, and a dry, dull appearance to the cornea OD. Schirmer tear test results confirmed an absence of tear production OD (0 mm/min) consistent with keratoconjunctivitis sicca. Treatment with topical 0.2% cyclosporine A resulted in an improvement in clinical signs. An episcleral cyclosporine A implant was placed under standing sedation 5 days after initial presentation. Re-examination 9 days post-operatively confirmed that the mare's tear production in the right eye had improved and no further clinical signs had been observed. Topical medications were gradually discontinued. Re-examinations performed up to 12 months postsurgery showed no recurrence of clinical signs and no adverse effects of the implant. To our knowledge, this is the first report of the use of a cyclosporine A implant in the management of KCS in a horse and highlights its potential as an effective, alternative therapy in the management of KCS in horses. © 2016 American College of Veterinary Ophthalmologists.

Saccade and cognitive impairment associated with kava intoxication.

PubMed

Cairney, Sheree; Maruff, Paul; Clough, Alan R; Collie, Alex; Currie, Jon; Currie, Bart J

2003-10-01

Kava is an extract from the Piper methysticum Forst. f. plant that has social and spiritual importance in Pacific islands societies. Herbal remedies that contain kava are used for the psychiatric treatment of anxiety and insomnia. Laboratory studies have found only subtle, if any, changes on cognitive or motor functions from the acute effects of consuming small clinical doses of kava products. Intoxication from recreational doses of kava has not been studied. The performance of individuals intoxicated from drinking kava (n=11) was compared with a control group (n=17) using saccade and cognitive tests. On average, intoxicated individuals had consumed 205 g of kava powder each (approximately 150 times clinical doses) in a group session that went for 14.4 h and ended 8 h prior to testing. Intoxicated kava drinkers showed ataxia, tremors, sedation, blepharospasm and elevated liver enzymes (GGT and ALP), together with saccadic dysmetria, saccadic slowing and reduced accuracy performing a visual search task that only became evident as the task complexity increased. Kava intoxication is characterized by specific abnormalities of movement coordination and visual attention but normal performance of complex cognitive functions. Saccade abnormalities suggest disruption of cerebellar and GABAergic functions. Copyright 2003 John Wiley & Sons, Ltd.

Efficacy of Zolpidem for Dystonia: A Study Among Different Subtypes

PubMed Central

Miyazaki, Yoshimichi; Sako, Wataru; Asanuma, Kotaro; Izumi, Yuishin; Miki, Tetsuro; Kaji, Ryuji

2012-01-01

Although there are some newly developed options to treat dystonia, its medical treatment is not always satisfactory. Zolpidem, an imidazopyridine agonist with a high affinity on benzodiazepine subtype receptor BZ1 (ω1), was found to improve clinical symptoms of dystonia in a limited number of case reports. To investigate what subtype of dystonia is responsive to the therapy, we conducted an open label study to assess the efficacy of zolpidem (5–20 mg) in 34 patients suffering from miscellaneous types of dystonia using the Burke–Fahn–Marsden Dystonia Rating Scale (BFMDRS). Patients were entered into the study if they had been refractory to other medications as evaluated by BFMDRS (no change in the previous two successive visits). After zolpidem therapy, the scores in the patients as a whole were decreased from 7.2 ± 7.9 to 5.5 ± 5.0 (P = 0.042). Patients with generalized dystonia, Meige syndrome/blepharospasm, and hand dystonia improved in the scale by 27.8, 17.8, and 31.0%, respectively, whereas no improvement was found in cervical dystonia patients. Overall response rate among patients were comparable to that of trihexyphenidyl. Zolpidem may be a therapeutic option for generalized dystonia, Meige syndrome, and hand dystonia including musician’s. Drowsiness was the dose-limiting factor. PMID:22529836

Topical anesthesia in phacoemulsification.

PubMed

Waheeb, Saad

2010-09-01

To evaluate the efficacy of topical anesthesia; topical Benoxinate 0.4% (Oxybuprocaine) and Xylocaine (Lidocaine) gel, in selected cataract patients as an alternative to peribulbar or retrobulbar block anesthesia during cataract surgery. Prospective non-comparative evaluation of patients' and surgeon's satisfaction at the end of the procedure. Three hundred patients (300 eyes) were included in the study. The procedure was explained to patients with details regarding what will happen and what to expect during surgery. All patients received topical anesthesia with Benoxinate 0.4% eye drops and Xylocaine gel 2%. All surgeries were done by the same surgeon using the same machine (updated LEGACY phacoemulsifier, Alcon) and approach (clear corneal incision) and followed by a foldable intraocular lens (IOL) implantation. None of the patients had severe pain during the procedure; only 2% (six of 300) required use of intravenous sedation (Propofol), both the surgeon's and the patients' satisfaction were high. Eye movements and blepharospasm were not significant problems, and no serious complications occurred. Rate of vitreous loss due to posterior capsule tear/rupture was within literature reported range and not different from our previous experience. Topical anesthesia is a satisfactory and safe alternative to retrobulbar and peribulbar anesthesia for clear corneal phacoemulsification and intraocular lens implantation in selected cataract patients in the hands of experienced cataract surgeon.

Topical anesthesia in phacoemulsification

PubMed Central

Waheeb, Saad

2010-01-01

Purpose: To evaluate the efficacy of topical anesthesia; topical Benoxinate 0.4% (Oxybuprocaine) and Xylocaine (Lidocaine) gel, in selected cataract patients as an alternative to peribulbar or retrobulbar block anesthesia during cataract surgery. Materials and Methods: Prospective non-comparative evaluation of patients’ and surgeon’s satisfaction at the end of the procedure. Three hundred patients (300 eyes) were included in the study. The procedure was explained to patients with details regarding what will happen and what to expect during surgery. All patients received topical anesthesia with Benoxinate 0.4% eye drops and Xylocaine gel 2%. All surgeries were done by the same surgeon using the same machine (updated LEGACY phacoemulsifier, Alcon) and approach (clear corneal incision) and followed by a foldable intraocular lens (IOL) implantation. Results: None of the patients had severe pain during the procedure; only 2% (six of 300) required use of intravenous sedation (Propofol), both the surgeon’s and the patients’ satisfaction were high. Eye movements and blepharospasm were not significant problems, and no serious complications occurred. Rate of vitreous loss due to posterior capsule tear/rupture was within literature reported range and not different from our previous experience. Conclusion: Topical anesthesia is a satisfactory and safe alternative to retrobulbar and peribulbar anesthesia for clear corneal phacoemulsification and intraocular lens implantation in selected cataract patients in the hands of experienced cataract surgeon. PMID:21120050

Risk and protective factors for spasmodic dysphonia: a case-control investigation.

PubMed

Tanner, Kristine; Roy, Nelson; Merrill, Ray M; Kimber, Kamille; Sauder, Cara; Houtz, Daniel R; Doman, Darrin; Smith, Marshall E

2011-01-01

Spasmodic dysphonia (SD) is a chronic, incurable, and often disabling voice disorder of unknown pathogenesis. The purpose of this study was to identify possible endogenous and exogenous risk and protective factors uniquely associated with SD. Prospective, exploratory, case-control investigation. One hundred fifty patients with SD and 150 medical controls (MCs) were interviewed regarding their personal and family histories, environmental exposures, illnesses, injuries, voice use patterns, and general health using a previously vetted and validated epidemiologic questionnaire. Odds ratios and multiple logistic regression analyses (α<0.15) identified several factors that significantly increased the likelihood of having SD. These factors included (1) a personal history of mumps, blepharospasm, tremor, intense occupational and avocational voice use, and a family history of voice disorders; (2) an immediate family history of meningitis, tremor, tics, cancer, and compulsive behaviors; and (3) an extended family history of tremor and cancer. SD is likely multifactorial in etiology, involving both genetic and environmental factors. Viral infections/exposures, along with intense voice use, may trigger the onset of SD in genetically predisposed individuals. Future studies should examine the interaction among genetic and environmental factors to determine the pathogenesis of SD. Copyright © 2011 The Voice Foundation. Published by Mosby, Inc. All rights reserved.

Postanaesthetic tear production and ocular irritation in cats.

PubMed

Peche, N; Köstlin, R; Reese, S; Pieper, K

2015-01-01

General anaesthesia significantly reduces tear production and normal values are not immediately re-established on ending anaesthesia. Therefore, adequate protection of the cornea has to be assured during the perianaesthetic period. There are various methods available, including taping of the eyelids and the application of eye ointments, gels and drops. In human medicine studies, different formulations were found to induce signs of ocular irritation. The aim of the present study was to determine tear production in cats after general anaesthesia, and to identify possible causes of irritation. Tear production was determined in 41 cats after general anaesthesia and eyes were examined for signs of irritation. Two different anaesthetic protocols were used. To protect the cornea, an ointment and gel were applied to the right and left eyes, respectively. Postoperatively, tear production was significantly reduced for 6 hours and 18 hours in the right and left eyes, respectively. Two hours after anaesthesia, blepharospasm of the right eye was observed in 92.7% (n = 38) of the cats. In contrast, the left eye was always held open. This study demonstrated that tear production in cats is significantly decreased both during and after anaesthesia. The degree of reduction was independent of the anaesthetic protocol. Both the eye ointment and gel proved effective in protecting the corneal surface. However, eye gel use is recommended because the eye ointment consistently caused an irritation comparable to the foreign-body sensation reported in humans.

Conjunctivitis and ulcerative keratitis secondary to conjunctival plant foreign bodies in a herd of alpacas (Lama Pacos).

PubMed

Fischer, Kristin; Hendrix, Diane

2012-03-01

To describe six cases of conjunctivitis and ulcerative keratitis secondary to plant foreign bodies from weed-contaminated grass hay which were lodged within the temporal conjunctival fornix in a herd of alpacas. A total of 21 alpacas from the same farm developed blepharospasm and mucopurulent ocular discharge. Six of the 21 were evaluated at the University of Tennessee College of Veterinary Medicine within a 2-week period. The six animals evaluated received an ophthalmic examination including slit-lamp biomicroscopy, fluorescein staining, and topical corneal anesthesia for foreign body removal when necessary. Six animals had conjunctivitis characterized by hyperemia and mucopurulent discharge. Five animals had superficial ulcerative keratitis with intense corneal neovascularization and edema. In all cases, the keratitis was located at the temporal limbus. Seven plant foreign bodies were observed and were removed following topical corneal anesthesia in five animals. The plant seeds were identified as three common weed species found in orchard grass hay. Complete resolution of all clinical signs was achieved with foreign body removal and medical therapy. Fan-shaped temporal corneal ulcers may signify the presence of conjunctival foreign bodies in alpacas. Plant foreign bodies should be considered in addition to ocular infection when multiple animals of a herd are affected as outbreaks may occur with weed-contaminated hay. © 2011 American College of Veterinary Ophthalmologists.

Botulinum toxin: A review of the mode of action in migraine.

PubMed

Do, T P; Hvedstrup, J; Schytz, H W

2018-05-01

Botulinum toxin serotype A (BoNT/A) was originally used in neurology for the treatment of dystonia and blepharospasms, but is now clinically used worldwide for the treatment of chronic migraine. Still, the possible mode of action of BoNT/A in migraine is not fully known. However, the mode of action of BoNT/A has been investigated in experimental pain as well as migraine models, which may elucidate the underlying mechanisms in migraine. The aim of this study was to review studies on the possible mode of action of BoNT/A in relation to chronic migraine treatment. Observations suggest that the mode of action of BoNT/A may not be limited to the injection site, but also includes anatomically connected sites due to axonal transport. The mechanisms behind the effect of BoNT/A in chronic migraine may also include modulation of neurotransmitter release, changes in surface expression of receptors and cytokines as well as enhancement of opioidergic transmission. Clinical and experimental studies with botulinum toxin in the last decade have advanced our understanding of headache and other pain states. More research into botulinum toxin as treatment for headache is warranted as it can be an attractive alternative for patients who do not respond positively to other drugs. © 2018 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

Clinical and ultrasonographic findings of some ocular conditions in sheep and goats

PubMed Central

El-Tookhy, O.; Tharwat, M.

2013-01-01

This study was carried out to describe the ultrasonographic findings in relation to the clinical symptoms of some common ocular conditions in sheep and goats. Fifty animals (32 goats and 18 sheep) with different ocular problems were examined. Ultrasonographic examination was performed using a B-mode ocular ultrasound unit, and the structure of the globe was evaluated at a depth of 4-6 cm. Early cases (n=35, 70%) showed varying ocular conditions; hypopyon, (n=8, 16%), stromal abscesses, (n=4, 8%), and anterior uveitis (n=23, 46%). Hypopyon appeared clinically as a white or yellowish material in the anterior chamber, and ultrasonographically as a hyperechoic mass in the anterior chamber. Severe iridocyclitis was noticed in acute cases of infectious keratoconjunctivitis (IKC) accompanied by blepharospasm, photophobia, excessive tearing and eyelid margin crust formation. Ultrasonographically, the pupil appeared constricted with increased hyperechoic thickening of the ciliary body. In chronic cases of IKC, corneal pigmentation (n=5, 10%) and cataract (n=10, 20%) were seen. Ultrasonographically the type and degree of cataract were diagnosed. The present study provides an inside view of the inner ocular structures during the course of certain eye diseases where ophthalmoscopic examination is not possible. Our findings, although preliminary, are relevant for the more complete diagnosis of certain external ocular conditions in sheep and goat herds. PMID:26623306

An unusual case of feline acute corneal hydrops: atypical disease presentation and possible in vivo detection of Descemet's membrane detachment in the cat's unaffected eye.

PubMed

Schlesener, Brittany N; Scott, Erin M; Vallone, Lucien V

2017-11-07

A 1-year-old, female spayed, domestic shorthair cat presented for blepharospasm of the right eye. Slit-lamp biomicroscopic examination showed focal corneal ulceration and presumptive keratomalacia of the right eye. Examination of the left eye was normal apart from a focal endothelial opacity. Within the first 24 h of medical management, the right eye developed marked corneal edema and globular anterior protrusion of the corneal surface consistent with feline acute corneal hydrops (FACH). Surgical management consisted of a bridge conjunctival graft, nictitating membrane flap, and temporary tarsorrhaphy. Resolution of corneal edema and pain occurred in the right eye within 24 days. Spectral domain optical coherence tomography (SD-OCT) of the anterior segment was performed in both eyes. Conjunctival tissue from the bridge graft precluded examination of deeper corneal structures in the right eye. The left eye displayed a focal separation of the corneal endothelium and Descemet's membrane from the overlying stroma. These SD-OCT findings are similar to the analogous syndrome found in humans and represent a potential etiology for FACH of the right eye in the case presented here. Unfortunately, the cat was lost to follow-up and the progression of this lesion to FACH in the left eye could not be determined. © 2017 American College of Veterinary Ophthalmologists.

Diagnosis of intraocular lymphosarcoma in a dog by use of a polymerase chain reaction assay for antigen receptor rearrangement.

PubMed

Pate, Diana O; Gilger, Brian C; Suter, Steven E; Clode, Alison B

2011-03-01

A 12-year-old castrated male mixed-breed dog was evaluated because of blepharospasm and blindness affecting both eyes. During examination and diagnostic testing of the dog, fine-needle aspirates of splenic nodules were examined microscopically and stage Vb multicentric large-cell lymphosarcoma was identified. Aqueocentesis was performed, and sample analysis revealed intraocular lymphosarcoma; B-cell neoplasia was confirmed by use of a PCR assay for antigen receptor rearrangement (PARR) performed on samples of aqueous humor. Secondary uveitis and glaucoma were detected bilaterally in addition to chronic superficial corneal ulcerations in the left eye. Treatment for abdominal and intraocular lymphosarcoma involving administration of vincristine, l-asparaginase, cyclophosphamide, doxorubicin, and prednisone was initiated. Secondary uveitis and glaucoma were controlled with topical treatment; however, the corneal ulceration did not resolve. Seven weeks following diagnosis, the dog died as a result of complications related to systemic neoplasia and chemotherapy. In the dog of this report, intraocular lymphosarcoma was diagnosed via PARR performed on samples of aqueous humor. Moreover, the immunophenotype of the neoplastic cells was determined by use of that diagnostic technique. Because secondary uveitis is a common finding in dogs and cats with systemic lymphosarcoma, intraocular lymphosarcoma should be considered as a differential diagnosis; furthermore, investigation (eg, PARR performed on aqueous humor samples) to identify the presence of intraocular lymphosarcoma is warranted, thereby allowing targeted interventions to be considered in management of those patients.

Computer vision syndrome: a study of the knowledge, attitudes and practices in Indian ophthalmologists.

PubMed

Bali, Jatinder; Navin, Neeraj; Thakur, Bali Renu

2007-01-01

To study the knowledge, attitude and practices (KAP) towards computer vision syndrome prevalent in Indian ophthalmologists and to assess whether 'computer use by practitioners' had any bearing on the knowledge and practices in computer vision syndrome (CVS). A random KAP survey was carried out on 300 Indian ophthalmologists using a 34-point spot-questionnaire in January 2005. All the doctors who responded were aware of CVS. The chief presenting symptoms were eyestrain (97.8%), headache (82.1%), tiredness and burning sensation (79.1%), watering (66.4%) and redness (61.2%). Ophthalmologists using computers reported that focusing from distance to near and vice versa (P =0.006, chi2 test), blurred vision at a distance (P =0.016, chi2 test) and blepharospasm (P =0.026, chi2 test) formed part of the syndrome. The main mode of treatment used was tear substitutes. Half of ophthalmologists (50.7%) were not prescribing any spectacles. They did not have any preference for any special type of glasses (68.7%) or spectral filters. Computer-users were more likely to prescribe sedatives/anxiolytics (P = 0.04, chi2 test), spectacles (P = 0.02, chi2 test) and conscious frequent blinking (P = 0.003, chi2 test) than the non-computer-users. All respondents were aware of CVS. Confusion regarding treatment guidelines was observed in both groups. Computer-using ophthalmologists were more informed of symptoms and diagnostic signs but were misinformed about treatment modalities.

Topical delivery of acetyl hexapeptide-8 from different emulsions: influence of emulsion composition and internal structure.

PubMed

Hoppel, Magdalena; Reznicek, Gottfried; Kählig, Hanspeter; Kotisch, Harald; Resch, Günter P; Valenta, Claudia

2015-02-20

Acetyl hexapeptide-8 (AH-8) is a well-known component of anti-aging products and was recently explored as a promising topical treatment of blepharospasm. Although AH-8 appears in a variety of cosmetic products, its skin penetration is sparsely studied and controversially discussed. Therefore, the aim of the present study was to investigate the influence of the vehicle type on the AH-8 delivery to the skin. Besides skin permeation experiments with Franz type diffusion cells, the spatial distribution of AH-8 in the stratum corneum after a real in-use application was investigated by in vitro tape stripping on porcine ear skin. By applying LC-MS/MS for quantification of AH-8, we demonstrated that a multiple water-in-oil-in-water (W/O/W) emulsion can significantly increase penetration of AH-8 into porcine skin compared to simple O/W and W/O emulsions. The internal structure of the developed multiple emulsion was confirmed by electron microscopic investigations and NMR self diffusion studies. In general, a clear superiority of water-rich W/O/W and O/W emulsions over an oil-rich W/O emulsion in terms of dermal delivery of AH-8 was found. This enhanced delivery of AH-8 could be explained by an increased absorption of the water-rich emulsions into the skin, confirmed by combined ATR-FTIR and tape stripping experiments. Copyright © 2014 Elsevier B.V. All rights reserved.

The effect of UV-blocking contact lenses as a therapy for canine chronic superficial keratitis.

PubMed

Denk, Nora; Fritsche, Jens; Reese, Sven

2011-05-01

To evaluate the effect of UV-blocking soft contact lenses in treatment for chronic superficial keratitus (CSK). Twenty six dogs with CSK were treated continuously with UV-blocking contact lenses for 6 months. A contact lens was placed on one eye of each dog; the other eye remained without a lens as a control eye. After this primary study, five of the dogs were further treated and they wore then contact lenses in both eyes. Continuously, all patients were concurrently treated topically with cyclosporine. The contact lenses were changed every 4 weeks and an ophthalmic examination performed. Evaluation criteria included corneal alterations as pigmentation, edema, pannus and vascularization. To determine the transmittance characteristics of the contact lenses before and after use, 32 contact lenses were measured with a UV-vis-NIR spectrophotometer. Pigmentation increased in eyes wearing lenses and in control eyes over the evaluation period of 6 months. Corneal edema increased in the eyes wearing lenses, but remained unaffected in the control eyes. A significant difference in the incidence of pannus and the extent of corneal vascularisation could not be evaluated. Adverse effects were noted in six cases (corneal edema and vascularisation, conjunctivitis, blepharospasm). All new lenses studied reduced UV-radiation to a safe level, whereas used lenses did not maintain their transmittance characteristics. No positive effect of UV-blocking contact lenses could be proven with the study design used. © 2011 American College of Veterinary Ophthalmologists.

The Mayo Clinic Arizona Spasmodic Dysphonia Experience: A Demographic Analysis of 718 Patients.

PubMed

Patel, Alpen B; Bansberg, Stephen F; Adler, Charles H; Lott, David G; Crujido, Lisa

2015-11-01

Analyze demographic data collected over a 25-year experience of 718 patients with spasmodic dysphonia (SD) who have been treated with botulinum toxin-A (BoNT-A) and compare our data with previously published studies. Seven hundred eighteen patients with SD were treated with 6621 BoNT-A injections at Mayo Clinic Arizona between 1989 and 2014. All patients were treated by the same physician team. Background demographic data for each patient were recorded. Of 718 patients, 557 patients were female (77.6%). Six hundred sixty of 718 (91.8%) patients had adductor SD (AdSD), and 58 of 718 (8.1%) patients had abductor SD (AbSD). Average age of onset was 51 years. Of 718 patients, 378 (52.6%) had vocal tremor (VT); VT was present in 54.4% of AdSD patients and 32.1% of AbSD patients. Thirty-seven of 718 (5.2%) patients had other dystonias, including cervical dystonia (2.3%), blepharospasm (1.4%), limb dystonia (1.1%), and oromandibular dystonia (0.3%). A positive family history of SD was present in only 6 of 718 patients (0.8%) and of other dystonias in 11 of 718 patients (1.5%). Spasmodic dysphonia is a chronic and potentially disabling focal laryngeal dystonia. The Mayo Clinic Arizona SD experience compares to prior reports and reveals a female preponderance, onset in middle age, infrequent hereditary pattern, high co-occurrence of VT, and low co-occurrence of other dystonias. © The Author(s) 2015.

A Novel Mutation in CLCN1 Associated with Feline Myotonia Congenita

PubMed Central

Gandolfi, Barbara; Daniel, Rob J.; O'Brien, Dennis P.; Guo, Ling T.; Youngs, Melanie D.; Leach, Stacey B.; Jones, Boyd R.; Shelton, G. Diane; Lyons, Leslie A.

2014-01-01

Myotonia congenita (MC) is a skeletal muscle channelopathy characterized by inability of the muscle to relax following voluntary contraction. Worldwide population prevalence in humans is 1∶100,000. Studies in mice, dogs, humans and goats confirmed myotonia associated with functional defects in chloride channels and mutations in a skeletal muscle chloride channel (CLCN1). CLCN1 encodes for the most abundant chloride channel in the skeletal muscle cell membrane. Five random bred cats from Winnipeg, Canada with MC were examined. All cats had a protruding tongue, limited range of jaw motion and drooling with prominent neck and proximal limb musculature. All cats had blepharospasm upon palpebral reflex testing and a short-strided gait. Electromyograms demonstrated myotonic discharges at a mean frequency of 300 Hz resembling the sound of a ‘swarm of bees’. Muscle histopathology showed hypertrophy of all fiber types. Direct sequencing of CLCN1 revealed a mutation disrupting a donor splice site downstream of exon 16 in only the affected cats. In vitro translation of the mutated protein predicted a premature truncation and partial lack of the highly conserved CBS1 (cystathionine β-synthase) domain critical for ion transport activity and one dimerization domain pivotal in channel formation. Genetic screening of the Winnipeg random bred population of the cats' origin identified carriers of the mutation. A genetic test for population screening is now available and carrier cats from the feral population can be identified. PMID:25356766

Functional Aspects of Gait in Essential Tremor: A Comparison with Age-Matched Parkinson's Disease Cases, Dystonia Cases, and Controls.

PubMed

Louis, Elan D; Rao, Ashwini K

2015-01-01

An understanding of the functional aspects of gait and balance has wide ramifications. Individuals with balance disorders often restrict physical activity, travel, and social commitments to avoid falling, and loss of balance confidence, itself, is a source of disability. We studied the functional aspects of gait in patients with essential tremor (ET), placing their findings within the context of two other neurological disorders (Parkinson's disease [PD] and dystonia) and comparing them with age-matched controls. We administered the six-item Activities of Balance Confidence (ABC-6) Scale and collected data on number of falls and near-falls, and use of walking aids in 422 participants (126 ET, 77 PD, 46 dystonia, 173 controls). Balance confidence was lowest in PD, intermediate in ET, and relatively preserved in dystonia compared with controls. This ordering reoccurred for each of the six ABC-6 items. The number of near-falls and falls followed a similar ordering. Use of canes, walkers, and wheelchairs was elevated in ET and even greater in PD. Several measures of balance confidence (ABC-6 items 1, 4, 5, and 6) were lower in torticollis cases than in those with blepharospasm, although the two groups did not differ with respect to falls or use of walking aids. Lower balance confidence, increased falls, and greater need for walking aids are variably features of a range of movement disorder patients compared to age-matched controls. While most marked among PD patients, these issues affected ET patients as well and, to a small degree, some patients with dystonia.

What’s special about task in dystonia? A voxel-based morphometry and diffusion weighted imaging study

PubMed Central

Ramdhani, Ritesh A.; Kumar, Veena; Velickovic, Miodrag; Frucht, Steven J.; Tagliati, Michele; Simonyan, Kristina

2014-01-01

Background Numerous brain imaging studies have demonstrated structural changes in the basal ganglia, thalamus, sensorimotor cortex and cerebellum across different forms of primary dystonia. However, our understanding of brain abnormalities contributing to the clinically well-described phenomenon of task-specificity in dystonia remained limited. Methods We used high-resolution MRI with voxel-based morphometry and diffusion tensor imaging with tract-based spatial statistics of fractional anisotropy to examine gray and white matter organization in two task-specific dystonia forms, writer’s cramp and laryngeal dystonia, and two non-task-specific dystonia forms, cervical dystonia and blepharospasm. Results A direct comparison between the both dystonia forms revealed that characteristic gray matter volumetric changes in task-specific dystonia involve the brain regions responsible for sensorimotor control during writing and speaking, such as primary somatosensory cortex, middle frontal gyrus, superior/inferior temporal gyrus, middle/posterior cingulate cortex, occipital cortex as well as the striatum and cerebellum (lobules VI-VIIa). These gray matter changes were accompanied by white matter abnormalities in the premotor cortex, middle/inferior frontal gyrus, genu of the corpus callosum, anterior limb/genu of the internal capsule, and putamen. Conversely, gray matter volumetric changes in non-task-specific group were limited to the left cerebellum (lobule VIIa) only, while white matter alterations were found to underlie the primary sensorimotor cortex, inferior parietal lobule and middle cingulate gyrus. Conclusion Distinct microstructural patterns in task-specific and non-task-specific dystonias may represent neuroimaging markers and provide evidence that these two dystonia subclasses likely follow divergent pathophysiological mechanisms precipitated by different triggers. PMID:24925463

Genotype-phenotype correlations in THAP1 dystonia: molecular foundations and description of new cases

PubMed Central

LeDoux, Mark S.; Xiao, Jianfeng; Rudzińska, Monika; Bastian, Robert W.; Wszolek, Zbigniew K.; Van Gerpen, Jay A.; Puschmann, Andreas; Momčilović, Dragana; Vemula, Satya R.; Zhao, Yu

2012-01-01

An extensive variety of THAP1 sequence variants have been associated with focal, segmental and generalized dystonia with age of onset ranging from 3 to over 60 years. In previous work, we screened 1,114 subjects with mainly adult-onset primary dystonia (Neurology 2010;74:229-238) and identified 6 missense mutations in THAP1. For this report, we screened 750 additional subjects for mutations in coding regions of THAP1 and interrogated all published descriptions of THAP1 phenotypes (gender, age of onset, anatomical distribution of dystonia, family history and site of onset) to explore the possibility of THAP1 genotype-phenotype correlations and facilitate a deeper understanding of THAP1 pathobiology. We identified 5 additional missense mutations in THAP1 (p.A7D, p.K16E, p.S21C, p.R29Q, and p.I80V). Three of these variants are associated with appendicular tremors, which were an isolated or presenting sign in some of the affected subjects. Abductor laryngeal dystonia and mild blepharospasm can be manifestations of THAP1 mutations in some individuals. Overall, mean age of onset for THAP1 dystonia is 16.8 years and the most common sites of onset are the arm and neck, and the most frequently affected anatomical site is the neck. In addition, over half of patients exhibit either cranial or laryngeal involvement. Protein truncating mutations and missense mutations within the THAP domain of THAP1 tend to manifest at an earlier age and exhibit more extensive anatomical distributions than mutations localized to other regions of THAP1. PMID:22377579

[Treatment of keratitis superficialis chronica of the dog with strontium 90].

PubMed

Höcht, Stefan; Grüning, Georg; Allgoewer, Ingrid; Nausner, Martin; Brunnberg, Leo; Hinkelbein, Wolfgang

2002-02-01

Corneal pannus is a disease which, if untreated, nearly always is progressive and may lead to blindness of the affected dog. A therapeutic standard is yet to be defined. Beta-ray irradiation with Sr-90 is often recommended on a casuistic basis, but systematic studies are sparse. The aim of the present study was to evaluate efficacy and to document side effects of radiotherapy with Sr-90. 17 animals were treated. 13 of them received treatment of 15 Gy surface dose twice within 2 days with additional medical therapy with ciclosporin and prednisolon. Only the more affected eye was treated with radiation which was applied with an eye-applicator, the other eye served as control. Four animals with already advanced impairment of vision received keratectomy, afterwards radiation was applied on both sides. Medical treatment alone led to deterioration in vascularization and spread of pigmentation in eleven of 13 (85%) of the control-eyes, density of pigmentation increased in eight of 13 (62%). After radiation therapy, almost all animals showed a marked initial improvement. Even if progressive disease occurred later on, further worsening as it happened in the control-eyes could be stopped in nine resp. ten of 13 eyes (69% and 77%). All animals with keratectomy and radiotherapy regained and preserved adequate vision. Besides short-term blepharospasm, no side effects were recorded. Corneal pannus is responsive to radiation therapy with Sr-90 and long-term benefit can be achieved. Side effects are minimal. Optimal sequencing of therapy and dosage still have to be examined.

Clinical, histopathological and immunohistochemical characterization of a novel equine ocular disorder: heterochromic iridocyclitis with secondary keratitis in adult horses.

PubMed

Pinto, Nelson I; McMullen, Richard J; Linder, Keith E; Cullen, John M; Gilger, Brian C

2015-11-01

To describe the clinical, histopathologic and immunohistochemical characteristics of an equine ocular inflammatory disease resulting in anterior uveitis and corneal endothelial inflammation associated with iris pigment dispersion and retrocorneal fibrous membrane (RFM) formation. Retrospective study. Sixteen horses with evidence of pigmented keratic precipitates (KPs), corneal edema, and/or iris depigmentation. Information collected from the medical records included signalment, clinical signs, prereferral treatment duration and response to therapy, ophthalmic examination findings, postreferral treatment, response to therapy, and outcome. Twenty-one eyes from 16 horses were affected. Age ranged between 9 and 25 years (Average 16.1 years). Blepharospasm, epiphora, and/or corneal opacification were the first clinical signs noted. At the time of referral pigmented KPs, corneal edema, iridal depigmentation, and retrocorneal membranes were commonly seen. Treatment included topical and/or systemic anti-inflammatories and antibiotics with variable response. Reduction or cessation of anti-inflammatory therapy resulted in worsening of clinical signs and disease progression. Eight eyes ultimately required enucleation. Histopathology changes include iridal pigment loss and dispersion, RFM formation, and keratitis. Variable degrees of lymphoplasmacytic inflammation were dominated by T-cells within the corneal stroma, RFM, iris, and ciliary body with occasional multinucleated giant cells. Heterochromic iridocyclitis with secondary keratitis (HIK) is characterized by uveal inflammation with pigment dispersion and suspected corneal endothelial dysfunction. Horses being treated for HIK require diligent and frequent follow-up examinations in combination with aggressive local immune suppression to control the disease. However, HIK may not respond to therapy and enucleation may ultimately be required to ensure the horse's comfort. © 2014 American College of Veterinary Ophthalmologists.

Neuro-Ophthalmology at a Tertiary Eye Care Centre in India.

PubMed

Dhiman, Rebika; Singh, Digvijay; Gantayala, Shiva P; Ganesan, Vaitheeswaran L; Sharma, Pradeep; Saxena, Rohit

2017-11-09

Neuro-ophthalmology as a specialty is underdeveloped in India. The aim of our study was to determine the spectrum and profile of patients presenting to a tertiary eye care center with neuro-ophthalmic disorders. A retrospective hospital-based study was conducted, and records of all patients seen at the neuro-ophthalmology clinic of Dr. Rajendra Prasad Centre for Ophthalmic Sciences, All India Institute of Medical Sciences, New Delhi, India, over a 1-year period were retrieved and evaluated. Of a total of 30,111 patients referred to various specialty clinics in a span of 1 year, 1597 (5%) were referred for neuro-ophthalmology evaluation. The mean patient age was 30.8 ± 19.5 years, with a male dominance (M:F = 2.02:1). Among these patients, optic nerve disorders were noted in 63.8% (n = 1,020), cranial nerve palsy in 7% (n = 114), cortical visual impairment in 6.5% (n = 105), and others (eye/optic nerve hypophasia, blepharospasm, and optic disc drusen) in 6% (n = 95). Among the patients with optic nerve disorders, optic neuropathy without disc edema/(traumatic optic neuropathy, hereditary, tumor-related, retrobulbar neuritis, toxic, and idiopathic) was noted in 42.8% (n = 685) and optic neuropathy with disc edema (ischemic optic neuropathy, papilledema, post-papilledema optic atrophy, papillitis, neuroretinitis, and inflammatory optic neuropathy) in 20.9% (n = 335). Sixteen percent of patients (n = 263) were incorrect referrals. The neuro-ophthalmic clinic constitutes a significant referral unit in a tertiary eye care center in India. Traumatic and ischemic optic neuropathies are the most common diagnoses. Neuro-ophthalmology requires further development as a subspecialty in India to better serve the nation's population.

Autoimmune post-herpes simplex encephalitis of adults and teenagers.

PubMed

Armangue, Thaís; Moris, Germán; Cantarín-Extremera, Verónica; Conde, Carlos Enrique; Rostasy, Kevin; Erro, Maria Elena; Portilla-Cuenca, Juan Carlos; Turón-Viñas, Eulàlia; Málaga, Ignacio; Muñoz-Cabello, Beatriz; Torres-Torres, Carmen; Llufriu, Sara; González-Gutiérrez-Solana, Luis; González, Guillermo; Casado-Naranjo, Ignacio; Rosenfeld, Myrna; Graus, Francesc; Dalmau, Josep

2015-11-17

To report 14 patients with immune-mediated relapsing symptoms post-herpes simplex encephalitis (HSE) and to compare the clinical and immunologic features of the teenage and adult group with those of young children. Prospective observational study of patients diagnosed between June 2013 and February 2015. Immunologic techniques have been reported previously. Among the teenage and adult group (8 patients, median age 40 years, range 13-69; 5 male), 3 had an acute symptom presentation suggesting a viral relapse, and 5 a presentation contiguous with HSE suggesting a recrudescence of previous deficits. Seven patients developed severe psychiatric/behavioral symptoms disrupting all social interactions, and one refractory status epilepticus. Blepharospasm occurred in one patient. Five patients had CSF antibodies against NMDA receptor (NMDAR) and 3 against unknown neuronal cell surface proteins. In 5/6 patients, the brain MRI showed new areas of contrast enhancement that decreased after immunotherapy and clinical improvement. Immunotherapy was useful in 7/7 patients, sometimes with impressive recoveries, returning to their baseline HSE residual deficits. Compared with the 6 younger children (median age 13 months, range 6-20, all with NMDAR antibodies), the teenagers and adults were less likely to develop choreoathetosis (0/8 vs 6/6, p < 0.01) and decreased level of consciousness (2/8 vs 6/6, p < 0.01) and had longer delays in diagnosis and treatment (interval relapse/antibody testing 85 days, range 17-296, vs 4 days, range 0-33, p = 0.037). In teenagers and adults, the immune-mediated relapsing syndrome post-HSE is different from that known in young children as choreoathetosis post-HSE and is underrecognized. Prompt diagnosis is important because immunotherapy can be highly effective. © 2015 American Academy of Neurology.

The clinical spectrum of laryngeal dystonia includes dystonic cough: observations of a large series.

PubMed

Payne, Susannah; Tisch, Stephen; Cole, Ian; Brake, Helen; Rough, Judy; Darveniza, Paul

2014-05-01

Laryngeal dystonia is a movement disorder of the muscles within the larynx, which most commonly manifests as spasmodic dysphonia (SD). Rarer reported manifestations include dystonic respiratory stridor and dyscoordinate breathing. Laryngeal dystonia has been treated successfully with botulinum neurotoxin (BTX) injections since 1984. We reviewed prospectively collected data in a consecutive series of 193 patients with laryngeal dystonia who were seen at St. Vincent's Hospital between 1991 and 2011. Patient data were analyzed in Excel, R, and Prism. Laryngeal dystonia manifested as SD (92.7%), stridor (11.9%), dystonic cough (6.2%), dyscoordinate breathing (4.1%), paroxysmal hiccups (1.6%), and paroxysmal sneezing (1.6%). There were more women (68.4%) than men (31.6%), and the average age at onset was 47 years. A positive family history of dystonia was present in 16.1% of patients. A higher incidence of extra-laryngeal dystonia (ie, torticollis and blepharospasm) and concurrent manifestations of laryngeal dystonia were present in patients with dystonic cough, dyscoordinate breathing, paroxysmal sneezing, and hiccups than in other patients (P = 0.003 and P < 0.0001, respectively). The average starting dose of BTX decreased from 2.3 to 0.5 units between 1991 and 2011. The median treatment rating was excellent across all subgroups. Patients with adductor SD, stridor, extra-laryngeal dystonia and male patients had relatively better treatment outcomes. Technical failures were rare (1.1%). Dysphonia secondary to vocal cord paresis followed 38.7% of treatments. Laryngeal dystonia manifests predominantly as SD, but other manifestations include stridor, dyscoordinate breathing, paroxysmal cough, hiccups, and sneezing. BTX injections are very effective across all subgroups. Severe adverse events are rare. © 2014 International Parkinson and Movement Disorder Society.

Clinical experience with patients with spasmodic dysphonia and primary Meige syndrome.

PubMed

Pedrero-Escalas, María Fernanda; García-López, Isabel; Santiago-Pérez, Susana; Vivancos, Francisco; Gavilán, Javier

2018-04-28

Meige syndrome (MS) is part of the group of segmental cranial dystonias, which affect more than two cranial muscle groups. Specifically, blepharospasm is associated with another cranial dystonia (oromandibular, cervical or laryngeal). The aim of this paper was to report our experience in patients with spasmodic dysphonia (SD) associated with primary MS. A retrospective study involving 8 patients between May 2010 and June 2015. Variables recorded were: age, sex, associated dystonia, electromyographic pattern in laryngeal muscles and treatment given. Outcomes after treatment were assessed using GRBAS(i) scale and VHI-30 questionnaire, always provided by the same examiner. Fifty-six patients with MS were treated in the Neurology Department. Eight patients of 56 were diagnosed with SD (prevalence of 14%). All of our patients had adductor SD. The median age was 71years. All the patients were treated with intralaryngeal botulinum toxin under electromyographic control. Clinically relevant improvements were found after treatment on both the GRBAS(i) scale and the VHI-30 questionnaire. In the study of SD, we should always rule out an association with MS. From the point of view of otorhinolaryngology, the joint use of the GRBAS(i) scale and the VHI-30 questionnaire are useful, reliable and efficient methods for assessing progress and response to treatment. Laryngeal infiltration under electromyographic control with botulinum toxin is the therapeutic alternative that provides better results. The management of SD associated with MS does not differ from isolated SD. Copyright © 2018 Sociedad Española de Otorrinolaringología y Cirugía de Cabeza y Cuello. Publicado por Elsevier España, S.L.U. All rights reserved.

Trigeminal Proprioception Evoked by Strong Stretching of the Mechanoreceptors in Müller's Muscle Induces Reflex Contraction of the Orbital Orbicularis Oculi Slow-Twitch Muscle Fibers.

PubMed

Matsuo, Kiyoshi; Ban, Ryokuya; Ban, Midori; Yuzuriha, Shunsuke

2014-01-01

The mixed orbicularis oculi muscle lacks an intramuscular proprioceptive system such as muscle spindles, to induce reflex contraction of its slow-twitch fibers. We evaluated whether the mechanoreceptors in Müller's muscle function as extrinsic mechanoreceptors to induce reflex contraction of the slow-twitch fibers of the orbicularis oculi in addition to those of the levator and frontalis muscles. We evaluated in patients with aponeurosis-disinserted blepharoptosis whether strong stretching of the mechanoreceptors in Müller's muscle from upgaze with unilateral lid load induced reflex contraction of the orbicularis oculi slow-twitch fibers and whether anesthesia of Müller's muscle precluded the contraction. We compared the electromyographic responses of the bilateral orbicularis oculi muscles to unilateral intraoperative direct stimulation of the trigeminal proprioceptive nerve with those to unilateral transcutaneous electrical stimulation of the supraorbital nerve. Upgaze with a unilateral 3-g lid load induced reflex contraction of the bilateral orbicularis oculi muscles with ipsilateral dominance. Anesthesia of Müller's muscle precluded the reflex contraction. The orbicularis oculi reflex evoked by stimulation of the trigeminal proprioceptive nerve differed from that by electrical stimulation of the supraorbital nerve in terms of the intensity of current required to induce the reflex, the absence of R1, and duration. The mechanoreceptors in Müller's muscle functions as an extramuscular proprioceptive system to induce reflex contraction of the orbital orbicularis oculi slow-twitch fibers. Whereas reflex contraction of the pretarsal orbicularis fast-twitch fibers functions in spontaneous or reflex blinking, that of the orbital orbicularis oculi slow-twitch fibers may factor in grimacing and blepharospasm.

Trigeminal Proprioception Evoked by Strong Stretching of the Mechanoreceptors in Müller's Muscle Induces Reflex Contraction of the Orbital Orbicularis Oculi Slow-Twitch Muscle Fibers

PubMed Central

Ban, Ryokuya; Ban, Midori; Yuzuriha, Shunsuke

2014-01-01

Objective: The mixed orbicularis oculi muscle lacks an intramuscular proprioceptive system such as muscle spindles, to induce reflex contraction of its slow-twitch fibers. We evaluated whether the mechanoreceptors in Müller's muscle function as extrinsic mechanoreceptors to induce reflex contraction of the slow-twitch fibers of the orbicularis oculi in addition to those of the levator and frontalis muscles. Methods: We evaluated in patients with aponeurosis-disinserted blepharoptosis whether strong stretching of the mechanoreceptors in Müller's muscle from upgaze with unilateral lid load induced reflex contraction of the orbicularis oculi slow-twitch fibers and whether anesthesia of Müller's muscle precluded the contraction. We compared the electromyographic responses of the bilateral orbicularis oculi muscles to unilateral intraoperative direct stimulation of the trigeminal proprioceptive nerve with those to unilateral transcutaneous electrical stimulation of the supraorbital nerve. Results: Upgaze with a unilateral 3-g lid load induced reflex contraction of the bilateral orbicularis oculi muscles with ipsilateral dominance. Anesthesia of Müller's muscle precluded the reflex contraction. The orbicularis oculi reflex evoked by stimulation of the trigeminal proprioceptive nerve differed from that by electrical stimulation of the supraorbital nerve in terms of the intensity of current required to induce the reflex, the absence of R1, and duration. Conclusions: The mechanoreceptors in Müller's muscle functions as an extramuscular proprioceptive system to induce reflex contraction of the orbital orbicularis oculi slow-twitch fibers. Whereas reflex contraction of the pretarsal orbicularis fast-twitch fibers functions in spontaneous or reflex blinking, that of the orbital orbicularis oculi slow-twitch fibers may factor in grimacing and blepharospasm. PMID:25210572

The Ex vivo Eye Irritation Test (EVEIT) model as a mean of improving venom ophthalmia understanding.

PubMed

Delafontaine, Marie; Panfil, Claudia; Spöler, Felix; Kray, Stefan; Burgher, François; Mathieu, Laurence; Blomet, Joël; Schrage, Norbert F; Tambourgi, Denise V

2018-06-08

Snakes belonging to the genus Naja (Elapid family), also known as "spitting cobras", can spit venom towards the eyes of the predator as a defensive strategy, causing painful and potentially blinding ocular envenoming. Venom ophthalmia is characterized by pain, hyperemia, blepharitis, blepharospasm and corneal erosions. Elapid venom ophthalmia is not well documented and no specific treatment exists. Furthermore, accidental ejection of venom by non-spitting vipers, as Bothrops, also occurs. The Ex vivo Eye Irritation Test model (EVEIT) has enabled important progress in the knowledge of chemical ocular burns. Considering the lack of experimental animal model, we adapted the EVEIT to study venom ophthalmia mechanisms. Ex vivo rabbit corneas were exposed to venoms from spitting (Naja mossambica, Naja nigricollis) and non-spitting (Naja naja, Bothrops jararaca and Bothrops lanceolatus) snakes, and rinsed or not with water. The corneal thickness and the depth of damage were assessed using high-resolution optical coherence tomography (HR-OCT) imaging and histological analysis. All Naja venoms induced significant corneal edema, collagen structure disorganization and epithelial necrosis. Corneas envenomed by African N. mossambica and N. nigricollis venoms were completely opaque. Opacification was not observed in corneas treated with venoms from non-spitting snakes, such as the Asian cobra, N. naja, and the vipers, B. jararaca and B. lanceolatus. Moreover, Bothrops venoms were able to damage the epithelium and cause collagen structure disorganization, but not edema. Immediate water rinsing improved corneal status, though damage and edema could still be observed. In conclusion, the present study shows that the EVEIT model was successfully adapted to set a new experimental ex vivo animal model of ophthalmia, caused by snake venoms, which will enable to explore new therapies for venom ophthalmia. Copyright © 2018. Published by Elsevier Ltd.

Acute effects of sulfur mustard injury--Munich experiences.

PubMed

Kehe, K; Thiermann, H; Balszuweit, F; Eyer, F; Steinritz, D; Zilker, T

2009-09-01

Sulfur mustard (SM) is a strong vesicant agent which has been used in several military conflicts. Large stockpiles still exist to the present day. SM is believed to be a major threat to civilian populations because of the persistent asymmetric threat by non-state actors, such as terrorist groups, its easy synthesis and handling and the risk of theft from stockpiles. Following an asymptomatic interval of several hours, acute SM exposure produces subepidermal skin blisters, respiratory tract damage, eye lesions and bone marrow depression. Iranian victims of SM exposure during the Iran-Iraq (1984-1988) war were treated at intensive care units of 3 Munich hospitals. All 12 patients were injured following aerial attacks with SM filled bombs, which exploded in a distance between 5 and 30m. All patients soon noted an offensive smell of garlic, addle eggs or oil roasted vegetables. No individual protective equipment was used. Eye itching and skin blistering started 2h after SM exposure. Some patients complained of nausea, dizziness and hoarseness. 4h after exposure, most patients started vomiting. Eye symptoms worsened and most patients suffered from temporary blindness due to blepharospasm and lid oedema. Additionally, pulmonary symptoms such as productive cough occurred. Patients were transferred to Munich 4-17 days after SM exposure. On admission all patients showed significant skin blistering and pigmentation. Conjunctivitis and photophobia were the major eye symptoms. Pulmonary symptoms, including productive cough were persistent. Bronchoscopy revealed massive inflammation of the trachea with signs of necrosis. 3 patients needed tracheotomy. Chest X-ray did not yield abnormal observations. This presentation summarizes the experience of treating SM victims in Munich and discusses therapeutic implications.

Progressive Supranuclear Palsy and Corticobasal Degeneration: Pathophysiology and Treatment Options.

PubMed

Lamb, Ruth; Rohrer, Jonathan D; Lees, Andrew J; Morris, Huw R

2016-09-01

There are currently no disease-modifying treatments for progressive supranuclear palsy (PSP) or corticobasal degeneration (CBD), and no approved pharmacological or therapeutic treatments that are effective in controlling their symptoms. The use of most pharmacological treatment options are based on experience in other disorders or from non-randomized historical controls, case series, or expert opinion. Levodopa may provide some improvement in symptoms of Parkinsonism (specifically bradykinesia and rigidity) in PSP and CBD; however, evidence is conflicting and where present, benefits are often negligible and short lived. In fact, "poor" response to levodopa forms part of the NINDS-SPSP criteria for the diagnosis of PSP and consensus criteria for the diagnosis of CBD (Lang Mov Disord. 20 Suppl 1:S83-91, 2005; Litvan et al. Neurology. 48:119-25, 1997; Armstrong et al. Neurology. 80(5):496-503, 2013). There is some evidence that intrasalivery gland botulinum toxin is useful in managing problematic sialorrhea and that intramuscular botulinum toxin and baclofen are helpful in reducing dystonia, including blepharospasm. Benzodiazepines may also be useful in managing dystonia. Myoclonus may be managed using levetiracetam and benzodiazepines. Pharmacological agents licensed for Alzheimer's disease (such as acetylcholinesterase inhibitors and N-Methyl-D-aspartate receptor antagonists) have been used off-label in PSP, CBD, and other tauopathies with the aim of improving cognition; however, there is limited evidence that they are effective and risk of adverse effects may outweigh benefits. The use of atypical antipsychotics for behavioural symptoms is not recommended in the elderly or those with demetia associated conditions and most antipsychotics will worsen Parkinsonism. Antidepressants may be useful for behavioral symptoms and depression but are often poorly tolerated due to adverse effects. In the absence of an effective drug treatment to target the underlying cause of CBD and PSP, management should focus on optimizing quality of life, relieving symptoms and assisting patients with their activities of daily living (ADL). Patients should be managed by a multidisciplinary team consisting of neurologists, physiotherapists (PT), occupational therapists (OT), speech and language therapists (SALT), dieticians, ophthalmologists, psychologists, and palliative care specialists.

The comparison between intradermal injection of abobotulinumtoxinA and normal saline for face-lifting: a split-face randomized controlled trial.

PubMed

Wanitphakdeedecha, Rungsima; Ungaksornpairote, Chanida; Kaewkes, Arisa; Rojanavanich, Viboon; Phothong, Weeranut; Manuskiatti, Woraphong

2016-12-01

Botulinum toxin type A (BTA) has been approved for the treatment of strabismus, blepharospasm, muscle spasm, cervical dystonia, pain syndrome, glabella wrinkles, and severe primary axillary hyperhidrosis. Intradermal injection of BTA has been used off-label by many clinicians for the purpose of face-lifting effect. Few studies on onabotulinumtoxinA (ONA) demonstrated no clinical efficacy on face-lifting effect when comparing to normal saline solution (NSS). So far, there is no split-face comparison study on face-lifting effect of abobotulinumtoxinA (ABO). To determine the face-lifting effect of ABO intradermal injection and NSS. Twenty-two subjects with symmetrical faces on both facial expression and expressionless were randomly injected with ABO at 1:7 cc dilution (500 unit or one vial in 7 cc of NSS) on one side and NSS on the other side using intradermal injection technique. Standardized photographic documentation was obtained at baseline, and at 2 weeks after treatment. The face-lifting effect was graded by two blinded dermatologists using photographic comparison and rated by the patients. Side effects were also recorded at the end of the study. Face-lifting effect was demonstrated in 40.9% and 4.5% of patients with ABO and NSS, respectively. There was a statistically significant difference in face-lifting effect when comparing between ABO and NSS (P = 0.021). The face-lifting was reported in 50.0% of patients receiving ABO injection. The patients with oval-face shape tended to respond better with ABO (P = 0.046). The odd of face-lifting effect for patients aged younger than 32 was higher than patients aged older than 32 with the odds ratio of 7.9 and 95% confidence interval of 1.1-56.1. Facial asymmetry was found in 22.7% of subjects. This study demonstrated the face-lifting effect of ABO intradermal injection. Patients with oval-face shape and aged younger than 32 tended to respond better. Therefore, patient selection should be emphasized to improve efficacy of this technique. © 2016 The Authors. Journal of Cosmetic Dermatology Published by Wiley Periodicals, Inc.

Clinicopathological effects of pepper (oleoresin capsicum) spray.

PubMed

Yeung, M F; Tang, William Y M

2015-12-01

Pepper (oleoresin capsicum) spray is one of the most common riot-control measures used today. Although not lethal, exposure of pepper spray can cause injury to different organ systems. This review aimed to summarise the major clinicopathological effects of pepper spray in humans. MEDLINE, EMBASE database, and Cochrane Database of Systematic Reviews were used to search for terms associated with the clinicopathological effects of pepper spray in humans and those describing the pathophysiology of capsaicin. A phone interview with two individuals recently exposed to pepper spray was also conducted to establish clinical symptoms. Major key words used for the MEDLINE search were "pepper spray", "OC spray", "oleoresin capsicum"; and other key words as "riot control agents", "capsaicin", and "capsaicinoid". We then combined the key words "capsaicin" and "capsaicinoid" with the major key words to narrow down the number of articles. A search with other databases including EMBASE and Cochrane Database of Systematic Reviews was also conducted with the above phrases to identify any additional related articles. All article searches were confined to human study. The bibliography of articles was screened for additional relevant studies including non-indexed reports, and information from these was also recorded. Non-English articles were included in the search. Fifteen articles were considered relevant. Oleoresin capsicum causes almost instantaneous irritative symptoms to the skin, eyes, and respiratory system. Dermatological effects include a burning sensation, erythema, and hyperalgesia. Ophthalmic effects involve blepharospasm, conjunctivitis, peri-orbital oedema, and corneal pathology. Following inhalation, a stinging or burning sensation can be felt in the nose with sore throat, chest tightness, or dyspnoea. The major pathophysiology is neurogenic inflammation caused by capsaicinoid in the pepper spray. There is no antidote for oleoresin capsicum. Treatment consists of thorough decontamination, symptom-directed supportive measures, and early detection and treatment of systemic toxicity. Decontamination should be carefully carried out to avoid contamination of the surrounding skin and clothing. Pepper (oleoresin capsicum) spray is an effective riot-control agent and does not cause life-threatening clinical effects in the majority of exposed individuals. Early decontamination minimises the irritant effects.

Effect of an intravitreal cyclosporine implant on experimental uveitis in horses.

PubMed

Gilger, B C; Malok, E; Stewart, T; Horohov, D; Ashton, P; Smith, T; Jaffe, G J; Allen, J B

2000-10-31

The purpose of this study was to determine the effects of an intravitreal device releasing cyclosporine A (CsA) on recurrent inflammatory episodes in experimental uveitis. Nine normal horses were immunized peripherally with H37RA-mTB antigen twice, and then received 25 microg of H37RA-mTB antigen intravitreally in the right eye and an equal volume of balanced salt solution intravitreally in the left eye. Two weeks later, the animals randomly received either a CsA or a polymer implant (without CsA) in both eyes 1 week following implantation of the devices, 25 microg of H37RA-mTB antigen was reinjected into the right eye of each animal. Clinical signs of ophthalmic inflammation were graded following injections and implantation. The animals from each group were euthanized at 3, 14, and 28 days following the second injection. Aqueous and vitreous humor protein concentrations were measured. The presence, number, and type (CD4, 5 and 8) of infiltrating inflammatory cells and amount of tissue destruction were determined. Total RNA was isolated and quantitative reverse transcriptase-polymerase chain reaction was performed for equine specific interleukin (IL) 2 and 4, interferon-gamma (IFN gamma) and beta-actin. In addition, aqueous and vitreous humor and peripheral blood were collected at the termination of the experiments and analyzed for CsA concentration by HPLC. Within 4h of the first intravitreal H37RA-mTB antigen injection, each animal developed epiphora, blepharospasm, mild corneal edema, aqueous flare, myosis, and vitreous opacity. The severity of signs peaked 48 to 72 h after injection and subsequently decreased back to normal within 14 days. Following the second injection, clinical signs in the eyes with the CsA device were less severe and significantly shorter in duration than signs with the polymer only implant eyes. Aqueous and vitreous humor protein levels, infiltrating cell numbers, total number of T-lymphocytes, and levels of IL-2 and IFN gamma-mRNA were significantly less in eyes with the CsA implant compared to eyes with the polymer only. CsA implants did not completely eliminate the development of a second ('recurrent') experimental inflammatory episode in these horses. However, the duration and severity of inflammation, cellular infiltration, tissue destruction, and pro-inflammatory cytokines RNA transcript levels were significantly less in those eyes implanted with the CsA device.

Technique, postoperative complications, and visual outcomes of phacoemulsification cataract surgery in 21 penguins (27 eyes): 2011-2015.

PubMed

Church, Melanie L; Priehs, Daniel R; Denis, Heidi; Croft, Lara; DiRocco, Stacy; Davis, Michelle

2018-02-06

To describe surgical technique, postoperative complications, and visual outcome in penguins after phacoemulsification lens extraction surgery. Twenty-one penguins (27 eyes) that had phacoemulsification from 2011 to 2015 at Animal Eye Associates. Species included are as follows: 14 southern Rockhopper (18 eyes, 66.6%), 4 Gentoo (4 eyes, 19%), 2 King (3 eyes, 9.5%), and 1 Chinstrap penguin (2 eyes, 4.8%). Eleven of the penguins were females, and 10 were males with average age at the time of surgery being 27.5 years (range of 22-31 years). This is a retrospective study of phacoemulsification cataract surgery patients from 2011 to 2015. Visual outcome was evaluated by veterinary ophthalmologists at postoperative recheck examinations and subjectively by penguin keepers using individual bird surveys and paired t tests for statistical analysis. All eyes were functionally visual after surgery and at the time of last follow-up. Based on keeper surveys, 81% (17/21) of penguins showed immediate improvements in overall quality of life and 90% (19/21) of penguins exhibited improvement in mobility and behavior within their exhibit following cataract removal. Of the 14 penguins that received 1:5 intracameral atracurium during surgery, 10 (71.4%) had moderate mydriasis, 1 (7.1%) had minimal mydriasis, and 3 (21.4%) showed no effect to the pupil. Seventy percent of the cases had phacoemulsification times less than 60 seconds/eye; the mean time was 72 seconds. Sixteen eyes (59.3%) underwent anterior capsulotomy only, planned anterior and posterior capsulotomies were performed in 3 eyes (11.1%), and the entire lens capsule was removed due to capsular fibrosis and wrinkling in 8 eyes (29.6%). The most common short-term postoperative complication was temporary mild blepharospasm and/or epiphora, reported in 8 eyes (29.6%) from 7 penguins (33.3%). Long-term complications, 2-6 years postoperatively, included posterior synechiation resulting in dyscoria (10 of 24 eyes, 41.7%) and capsular fibrosis in all penguins with residual lens capsule (19/19, 100%). The prognosis for vision and behavioral improvement after phacoemulsification surgery in penguins is good, with low incidence of vision-threatening complications. © 2018 American College of Veterinary Ophthalmologists.

Ophthalmic Manifestations of Congenital Zika Syndrome in Colombia and Venezuela.

PubMed

Yepez, Juan B; Murati, Felipe A; Pettito, Michele; Peñaranda, Carlos F; de Yepez, Jazmin; Maestre, Gladys; Arevalo, J Fernando

2017-05-01

The ocular manifestations and sequelae of Zika virus infection are not well known. Recently, the World Health Organization changed the declaration of Zika as a public health emergency and designated the viral outbreak and related microcephaly clusters as a long-term program of work. This change indicates the urgent need to evaluate and document ophthalmic manifestations in patients for timely management of this disease. In addition, confirmation whether the public health problem in Brazil extends to other regions in South America is needed. To report the ocular manifestations of congenital Zika syndrome with microcephaly in Colombia and Venezuela. This prospective case series included 43 patients from 2 ophthalmic centers in Colombia and Venezuela who underwent evaluation from October 1, 2015, through June 30, 2016, and were clinically diagnosed with congenital Zika syndrome. Twenty patients were Hispanic; 13, African; 8, white; and 2, Native American. Ophthalmic and systemic evaluations and serologic testing were performed on all infants. Patients underwent external ocular examination and dilated ophthalmoscopy. Serologic testing ruled out toxoplasmosis, rubella, cytomegalovirus, syphilis, and human immunodeficiency virus. Ophthalmic manifestations of congenital Zika syndrome. Of the 43 patients included in this series (28 female and 15 male), the mean (SD) age at examination was 2.1 (1.5) months. The mothers of all the children had no ophthalmic findings and did not report ocular symptoms during pregnancy. All patients had bilateral ophthalmic manifestations. Optic nerve findings included hypoplasia with the double-ring sign, pallor, and increased cup-disc ratio in 5 patients (11.6%). Macular abnormalities included mild to severe pigment mottling in 27 patients (63%) and lacunar maculopathy in 3 (6.9%). Chorioretinal scarring was present in 3 patients (7%). Eleven patients (26%) had a combination of lesions in the posterior pole. Five patients (12%) were diagnosed with congenital glaucoma, characterized by the clinical triad of epiphora, photophobia, and blepharospasm; increased intraocular pressure; corneal clouding at birth; and buphthalmos. These data reveal that 12% (95% CI, 5%-24%) of cases of congenital Zika with microcephaly had anterior segment abnormalities and 88% (95% CI, 76%-94%) had important macular and optic nerve abnormalities. The visual sequelae of these ophthalmic manifestations remain unknown. Congenital Zika syndrome in the current study had severe ocular abnormalities, and all patients had bilateral involvement. Ocular findings were focal macular pigment mottling, chorioretinal atrophy with a predilection for the macular area, congenital glaucoma and optical nerve hypoplasia, and optic disc abnormalities. Ophthalmic examination is recommended in patients with congenital Zika syndrome.

Aristotle's illusion reveals interdigit functional somatosensory alterations in focal hand dystonia.

PubMed

Tinazzi, Michele; Marotta, Angela; Fasano, Alfonso; Bove, Francesco; Bentivoglio, Anna Rita; Squintani, Giovanna; Pozzer, Lara; Fiorio, Mirta

2013-03-01

In focal hand dystonia, the cortical somatosensory representation of the fingers is abnormal, with overlapping receptive fields and reduced interdigit separation. These abnormalities are associated with deficits in sensory perception, as previously demonstrated by applying tactile stimuli to one finger at a time. What is still unknown is whether the sensory deficits can be observed when tactile perception involves more than one finger. To address this issue, we applied 'Aristotle's illusion' to 15 patients with focal hand dystonia, 15 patients with dystonia not affecting the hand (blepharospasm and cervical dystonia) and 15 healthy control subjects. In this illusion, one object touching the contact point of two crossed fingertips is perceived as two objects by a blindfolded subject. The same object placed between two parallel fingertips is correctly perceived as one. The illusory doubling sensation is because of the fact that the contact point between the crossed fingers consists of non-adjacent and functionally unrelated skin regions, which usually send sensory signals to separate spots in the somatosensory cortex. In our study, participants were touched by one sphere between the second-third digits, the second-fourth digits and the fourth-fifth digits of both hands, either in crossed or in parallel position, and had to refer whether they felt one or two stimuli. The percentage of 'two stimuli' responses was an index of the illusory doubling. Both healthy control subjects and dystonic patients presented Aristotle's illusion when the fingers were crossed. However, patients with focal hand dystonia presented a significant reduction of the illusion when the sphere was placed between the crossed fourth and fifth digits of the affected hand. This reduction correlated with the severity of motor disease at the fingers. Similar findings were not observed in non-hand dystonia and control groups. The reduction of Aristotle's illusion in non-affected fingers and its preservation in affected fingers suggests dissociation between the abnormal processing of sensory signals and the motor impairment. Based on previous evidence showing that the sensory signals coming from the fourth digit determine lower activation in the somatosensory cortex than those coming from the fifth digit, we suggest that in the crossed position, the tactile information conveyed by the fifth digit prevailed over the fourth digit, thus resulting in the perception of one stimulus. The reduction of the illusory doubling perception, therefore, may represent the functional correlate of the different level of activation between the fourth and the fifth digit in the somatosensory cortex.

Acute intensive care unit management of mustard gas victims: the Turkish experience.

PubMed

Kilic, Ertugrul; Ortatatli, Mesut; Sezigen, Sermet; Eyison, Rusen Koray; Kenar, Levent

2018-05-07

Sulphur mustard (SM) is an highly toxic and vesicant chemical weapon that was used in various military conflicts several times in the history. The severity of ocular, dermal, and pulmonary symptoms that may appear following a characteristic asymptomatic period are depending on the SM concentration and exposure duration. The aim of this study is to present the clinical features and share the intensive care unit (ICU) experiences for the medical management of mustard gas victims. Thirteen Free Syrian Army soldiers near Al-Bab region of North Syria were reportedly exposed to oily blackish smoke with garlic smell due to the explosion of a trapped bomb without causing any blast or thermal effect on 26th November 2016. None of them wore any chemical protective suits or gas masks during explosion. Since they observed skin lesions including bullous formation next day, they were admitted to the Turkish Field Hospital at the Turkish - Syrian border and then evacuated to the State Hospital of Gaziantep Province, Turkey for further management. Eight victims who were very close to point of explosion suffered burning eyes, sore throat, dry cough and dyspnoea after the chemical attack. On admission to hospital, all cases had conjunctivitis, hoarseness and bullae on various body areas. Blepharospasm and opacity were found in 8 patients and 5 of them had corneal erosions and periorbital oedema. Temporary loss of vision in 4 cases lasted for 24 h. Multiple fluid-filled blisters were observed especially on the scalp, neck, arms and hands, where direct skin exposure to the agent occurred. A definitive clinical care and infection prophylaxis measures along with the burn treatment and bronchodilators for respiratory effects were applied in ICU. Two patients received granulocyte-colony-stimulating factor due to the SM-mediated bone marrow suppression on the 16th day of exposure and one of them died because of necrotic bronchial pseudomembrane obstruction resulting in cardiopulmonary arrest. SM was first used during the First World War and it is still considered one of the major chemical weapons recently used by non-state actors in Syria and Iraq. In case of SM exposure, medical treatment of SM-induced lesions is symptomatic because no antidote or causal therapy does exist even though SM is very well known for over 100 years. However, clinical management in intensive care medicine of SM victims have improved since the 1980s, this study which is one of the largest recent SM-exposed case series since that time is important for the contribution to the clinical experience.

Local injections of corticotropin releasing factor reduce doxorubicin-induced acute inflammation in the eyelid.

PubMed

McLoon, L K; Wirtschafter, J

1997-04-01

Doxorubicin chemomyectomy is an effective alternative treatment option for patients with blepharospasm and hemifacial spasm. One side effect of the use of doxorubicin in localized injections is the development of acute inflammation and skin injury at the injection site. Corticotropin releasing factor (CRF) was reported to reduce inflammation after acute inflammatory injuries due to other causes and at other sites. This study was performed to assess the potential of CRF to prevent the development of skin injury and eyelid soreness after local doxorubicin injection. Rabbits received lower eyelid injections of either 75 or 150 micrograms CRF followed by injection of either 0.5, 1, or 2 mg doxorubicin or doxorubicin alone. Eyelids were assessed for changes in acute inflammation by immunohistochemical localization of macrophages and monocytes using anti-CD11, an antibody specific for these cell types. Short-term alterations in vascular permeability were assessed using an Evans blue assay. Additional eyelids were followed daily for changes in the skin over the injection site to determine day of onset of skin injury and the total duration of skin injury. After 1 month, the eyelids were processed histologically for morphometric analysis of muscle fiber loss. Monkey eyelids also were examined for the effect of CRF and doxorubicin injections. Doxorubicin alone produced an acute inflammatory reaction in the treated eyelids, with a large influx of macrophages and monocytes throughout the connective tissue at 1 and 2 days. Corticotropin releasing factor pretreatment significantly reduced this influx of inflammatory cells into the connective tissue. Doxorubicin produced a large increase in vascular permeability in the treated eyelids, with resultant edema. Corticotropin releasing factor did not alter this change in vascular permeability, indicating that CRF appears to have a specific effect on migration of inflammatory cells rather than just a generalized effect on vascular permeability. Corticotropin releasing factor and doxorubicin cotreatments delayed the onset of skin injury and decreased the total duration of injury to the skin compared to doxorubicin alone. The effectiveness of doxorubicin chemomyectomy was maintained; muscle loss was significant at all doses of CRF combined with doxorubicin. Corticotropin releasing factor dramatically decreased the acute inflammatory reaction that results in the eyelid from local doxorubicin injections. Not only did CRF reduce the acute influx of monocytes and macrophages, but it protected the skin overlying the injection site, substantially reducing the extent of skin injury. The efficacy of doxorubicin-induced muscle toxicity was maintained. A treatment protocol that combines myotoxicity with antiinflammatory activity in the treated eyelids may lead to a more effective patient treatment by increasing patient acceptance. The potential should be explored that CRF may be of clinical use in limiting tissue injury when administered immediately after extravasation during cancer chemotherapy.

Ophthalmic Manifestations of Congenital Zika Syndrome in Colombia and Venezuela

PubMed Central

Yepez, Juan B.; Murati, Felipe A.; Pettito, Michele; Peñaranda, Carlos F.; de Yepez, Jazmin; Maestre, Gladys

2017-01-01

Importance The ocular manifestations and sequelae of Zika virus infection are not well known. Recently, the World Health Organization changed the declaration of Zika as a public health emergency and designated the viral outbreak and related microcephaly clusters as a long-term program of work. This change indicates the urgent need to evaluate and document ophthalmic manifestations in patients for timely management of this disease. In addition, confirmation whether the public health problem in Brazil extends to other regions in South America is needed. Objective To report the ocular manifestations of congenital Zika syndrome with microcephaly in Colombia and Venezuela. Design, Setting, and Participants This prospective case series included 43 patients from 2 ophthalmic centers in Colombia and Venezuela who underwent evaluation from October 1, 2015, through June 30, 2016, and were clinically diagnosed with congenital Zika syndrome. Twenty patients were Hispanic; 13, African; 8, white; and 2, Native American. Interventions Ophthalmic and systemic evaluations and serologic testing were performed on all infants. Patients underwent external ocular examination and dilated ophthalmoscopy. Serologic testing ruled out toxoplasmosis, rubella, cytomegalovirus, syphilis, and human immunodeficiency virus. Main Outcomes and Measures Ophthalmic manifestations of congenital Zika syndrome. Results Of the 43 patients included in this series (28 female and 15 male), the mean (SD) age at examination was 2.1 (1.5) months. The mothers of all the children had no ophthalmic findings and did not report ocular symptoms during pregnancy. All patients had bilateral ophthalmic manifestations. Optic nerve findings included hypoplasia with the double-ring sign, pallor, and increased cup-disc ratio in 5 patients (11.6%). Macular abnormalities included mild to severe pigment mottling in 27 patients (63%) and lacunar maculopathy in 3 (6.9%). Chorioretinal scarring was present in 3 patients (7%). Eleven patients (26%) had a combination of lesions in the posterior pole. Five patients (12%) were diagnosed with congenital glaucoma, characterized by the clinical triad of epiphora, photophobia, and blepharospasm; increased intraocular pressure; corneal clouding at birth; and buphthalmos. These data reveal that 12% (95% CI, 5%-24%) of cases of congenital Zika with microcephaly had anterior segment abnormalities and 88% (95% CI, 76%-94%) had important macular and optic nerve abnormalities. The visual sequelae of these ophthalmic manifestations remain unknown. Conclusions and Relevance Congenital Zika syndrome in the current study had severe ocular abnormalities, and all patients had bilateral involvement. Ocular findings were focal macular pigment mottling, chorioretinal atrophy with a predilection for the macular area, congenital glaucoma and optical nerve hypoplasia, and optic disc abnormalities. Ophthalmic examination is recommended in patients with congenital Zika syndrome. PMID:28418539

Clostridium botulinum: a bug with beauty and weapon.

PubMed

Shukla, H D; Sharma, S K

2005-01-01

Clostridium botulinum, a Gram-positive, anaerobic spore-forming bacteria, is distinguished by its significant clinical applications as well as its potential to be used as bioterror agent. Growing cells secrete botulinum neurotoxin (BoNT), the most poisonous of all known poisons. While BoNT is the causative agent of deadly neuroparalytic botulism, it also serves as a remarkably effective treatment for involuntary muscle disorders such as blepharospasm, strabismus, hemifacial spasm, certain types of spasticity in children, and other ailments. BoNT is also used in cosmetology for the treatment of glabellar lines, and is well-known as the active component of the anti-aging medications Botox and Dysport. In addition, recent reports show that botulinum neurotoxin can be used as a tool for pharmaceutical drug delivery. However, BoNT remains the deadliest of all toxins, and is viewed by biodefense researchers as a possible agent of bioterrorism (BT). Among seven serotypes, C. botulinum type A is responsible for the highest mortality rate in botulism, and thus has the greatest potential to act as biological weapon. Genome sequencing of C. botulinum type A Hall strain (ATCC 3502) is now complete, and has shown the genome size to be 3.89 Mb with a G+C content of approximately 28.2%. The bacterium harbors a 16.3 kb plasmid with a 26.8% G+C content--slightly lower than that of the chromosome. Most of the virulence factors in C. botulinum are chromosomally encoded; bioinformatic analysis of the genome sequence has shown that the plasmid does not harbor toxin genes or genes for related virulence factors. Interestingly, the plasmid does harbor genes essential to replication, including dnaE, which encodes the alpha subunit of DNA polymerase III which has close similarity with its counterpart in C. perfringens strain 13. The plasmid also contains similar genes to those that encode the ABC-type multidrug transport ATPase, and permease. The presence of ABC-type multidrug transport ATPase, and permease suggests putative involvement of efflux pumps in bacteriocin production, modification, and export in C. botulinum. The C. botulinum plasmid additionally harbors genes for LambdaBa04 prophage and site-specific recombinase that are similar to those found in the Ames strain of Bacillus anthracis; these genes and their products may play a role in genomic rearrangement. Completion of genome sequencing for C. botulinum will provide an opportunity to design genomic and proteomic-based systems for detecting different serotypes of C. botulinum strains in the environment. The completed sequence may also facilitate identification of potential virulence factors and drug targets, as well as help characterize neurotoxin-complexing proteins, their polycistronic expression, and phylogenetic relationships between different serotypes.

Medical management of the traumatic consequences of civil unrest incidents: causation, clinical approaches, needs and advanced planning criteria.

PubMed

Ballantyne, Bryan

2006-01-01

In the context of this review, civil unrest is defined as disharmony, expressive dissatisfaction and/or disagreement between members of a community, which leads to a situation of competitive aggression that may find expression as disruption of organisation, conflicts, damage to property and injuries. Such a breakdown of harmonious relationships, which may result in property damage and human injuries that may be threatening to life, varies in magnitude from participation of a very few individuals up to the involvement of large crowds of people, which may evolve into a full-scale riot. It is the latter situation often involving demonstrators, opposing groups and law enforcement personnel that can result in multiple casualties and present a very significant challenge to the resources of local healthcare institutions. The causation of civil unrest incidents is multifactorial and has generic, specific and potentiating elements. With the current national and international societal, political and discriminatory problems, it is likely that civil unrest incidents on both small and large scales will continue to occur at a high and possibly increasing rate on a worldwide basis, and for these not infrequent incidents, the medical community should be in a state of informed preparation. The circumstances of civil unrest incidents are very variable with respect to causation, overall magnitude, frequency, timing, geographical location, numbers of persons involved, demographics of participants, influence of extremists, confrontation with opposing groups and control measures used by law enforcement agencies. Methods used by police and security forces for the control of civil unrest incidents, if advanced negotiations with organisers and verbal warnings have failed, fall basically into two categories: physical and chemical measures. Physical methods include restraint holds, truncheons, batons, mounted horses, projectiles (such as bean bags, plastic and rubber bullets), water cannons, tasers and (rarely) live ammunition. All of these physical measures are associated with pain and immobilisation, and there is a high potential for soft tissue and bone injuries. Some of the more severe physical methods, including plastic and rubber bullets, may cause lethal injuries. The basis for using chemicals in civil unrest incidents is that they cause distraction, transient harassment and incapacitation, temporary impairment of the conduct of coordinated tasks and cause a desire to vacate the area of unrest. Although screening smokes and malodors have sometimes been employed, the major group of chemicals used are peripheral chemosensory irritants (PCSIs), which reversibly interact with sensory nerve receptors in exposed skin and mucosal surfaces, resulting in the production of local uncomfortable sensations and associated reflexes. Major effects are on the eye, respiratory tract and (to a lesser degree) skin. Thus, the induced transient pain and discomfort in the eye, respiratory tract and skin, together with associated lacrimation, blepharospasm, rhinorrhoea, sialorrhoea, cough and breathing difficulties, produce temporary incapacitation and interference with the conduct of coordinated tasks, and form the basis for harassment of malefactors. Currently used peripheral chemosensory irritants are 1-chloroacetophenone, 2-chlorobenzylidene malononitrile, dibenz(b.f)-1,4-oxazepine, oleoresin capsicum and pelargonic acid vanillylamide. Depending on operational circumstances, irritants may be dispersed as a smoke, powder cloud, aerosol, vapour, or in solution; the mode of generation and dispersion of irritant can influence hazard. Brief acute exposure to chemosensory irritants produces effects that generally resolve within an hour, leaving no long-term sequelae. However, sustained exposure to high concentrations may produce tissue injury, notably to the eye, respiratory tract and skin. With solutions of sensory irritants, other formulation constituents may enhance PCSI toxicity or introduce additional local and/or systemic toxicity. By the very circumstances of civil unrest incidents, injuries are inevitable, particularly when emotions are heightened and police and security forces have to resort to various chemical and/or physical means of control. Trauma may include slight to severe physical and/or chemical injuries, psychological problems and occasional deaths. Hospitals should be prepared for a wide range of casualties, and the fact that those seeking help will constitute a heterogeneous group, including wide age range, male, female, and individuals with pre-existing ill health. A major civil unrest incident necessitates that the local receiving hospital should be prepared and equipped for decontamination and triage processes. It is necessary to reassure patients who have been exposed to sensory irritants that the signs and symptoms are rapidly reversible, and do not result in long-term sequelae. With respect to chemical exposures, detailed evaluation should be given to possible ocular, cutaneous, respiratory and gastrointestinal effects. Also, exposure to chemosensory irritants results in transient increases in blood pressure, bradycardia and increased intraocular pressure. This indicates that those with cardiovascular diseases and glaucoma may be at increased risk for the development of complications. This article details the pharmacological, toxicological and clinical effects of chemicals used in civil disturbance control and discusses the management of contaminated individuals. Additionally, the potential for adverse effects from delivery systems and other physical restraint procedures is summarised. Due to the emergency and specialised circumstances and conditions of a civil unrest incident, there is a clear need for advanced planning by healthcare institutions in the event that such an incident occurs in their catchment area. This should include ensuring a good information base, preparations for medical and support staff readiness, and availability of required equipment and medications. Ideally, planning, administration and coordination should be undertaken at both local (regional) and central (governmental) centres. Regional centres should have responsibilities for education, training, ensuring facilities and staffing are appropriate, and that adequate equipment and medicines are available. There should be cooperative interactions and communications with local police and other emergency services. Centrally directed functions should include ensuring adequacy of the information base, coordinating activities and agreeing approaches between the regional centres, and periodic audits of regional centres with respect to the staffing, facility, equipment and training needs. Also, there is a need for most countries to introduce detailed guidelines and formal (regulatory) schemes for the assessment of the safety-in-use of chemicals and the delivery systems that are to be used against heterogeneous human populations for the control of civil unrest incidents. Such regulatory approval schemes should also cover advisory functions for safe use and any required restrictions.