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1.
Muscle Nerve ; 63(5): 765-769, 2021 05.
Artigo em Inglês | MEDLINE | ID: mdl-33583064

RESUMO

INTRODUCTION: Spinal muscular atrophy (SMA) most prominently affects proximal limb and bulbar muscles. Despite older case descriptions, ocular motor neuron palsies or other oculomotor abnormalities are not considered part of the phenotype. METHODS: We investigated oculomotor function by testing saccadic eye movements of 15 patients with SMA. Their performance was compared with that of age-matched healthy controls. Horizontal rightward and leftward saccades were recorded by means of video-oculography, whereas subjects looked at light-emitting diode targets placed at ±5°, ±10°, and ±15° eccentricities. RESULTS: No differences in saccade amplitude gains, peak velocities, peak velocity-to-amplitude ratios, or durations were observed between controls and patients. More specifically, for 5° target eccentricities, patients had a mean saccadic peak velocity of 153°/s, whereas for 10° and 15° these values were 268°/s and 298°/s, respectively. The corresponding mean peak velocities of the control group were 151°/s, 264°/s, and 291°/s. DISCUSSION: Our results indicate that patients with SMA perform fast and accurate horizontal saccades without evidence of extraocular muscle weakness. These quantitative oculomotor data corroborate clinical experience that neuro-ophthalmic symptoms in SMA are not common and, if present, should prompt suspicion for an alternative neuromuscular disorder.


Assuntos
Movimentos Oculares/fisiologia , Debilidade Muscular/fisiopatologia , Atrofia Muscular Espinal/fisiopatologia , Músculos Oculomotores/fisiopatologia , Adulto , Idoso , Medições dos Movimentos Oculares , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Movimentos Sacádicos/fisiologia , Adulto Jovem
2.
BMJ Case Rep ; 14(2)2021 Feb 04.
Artigo em Inglês | MEDLINE | ID: mdl-33541995

RESUMO

Presentation of severe pain syndromes prior to onset of motor weakness is an uncommon but documented finding in patients with Guillain-Barré syndrome (GBS). Sciatica in GBS is a difficult diagnosis when patients present with acute radiculopathy caused by herniated disc or spondylolysis. A middle-aged woman was admitted for severe low back pain, symptomatic hyponatraemia, vomiting and constipation. On further investigation, she was diagnosed with radiculopathy, and appropriate treatment was initiated. Brief symptomatic improvement was followed by new-onset weakness in lower limbs, which progressed to involve upper limbs and right extraocular muscles. With progressive, ascending, new-onset motor and sensory deficits and laboratory evidence of demyelination by Nerve Conduction Study, a diagnosis of variant GBS was made. She was treated with intravenous immunoglobulin 2 g/kg over 5 days. The presentation of severe low back pain that was masking an existing aetiology and possible dysautonomia and the unilateral right extraocular muscles instead of bilateral make our case unique and rare.


Assuntos
Diagnóstico Diferencial , Imunoglobulinas Intravenosas/uso terapêutico , Síndrome de Miller Fisher/diagnóstico , Síndrome de Miller Fisher/tratamento farmacológico , Músculos Oculomotores/fisiopatologia , Radiculopatia/diagnóstico , Dor nas Costas/etiologia , Feminino , Síndrome de Guillain-Barré/diagnóstico , Hospitais , Humanos , Hiponatremia/etiologia , Extremidade Inferior/fisiopatologia , Pessoa de Meia-Idade , Síndrome de Miller Fisher/complicações
3.
Eur J Endocrinol ; 184(2): 277-287, 2021 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-33539318

RESUMO

Background: Tripterygium glycosides (TG) has been used to treat a spectrum of inflammatory and autoimmune diseases. Our preliminary studies have shown that TG is effective in the treatment of active Graves' ophthalmopathy (GO). Objective: We aimed to compare the efficacy and tolerability of TG with intravenous methylprednisolone (iv.MP) in patients with active moderate-to-severe GO. Methods: This study was an observer-masked, single-centre, block-randomised trial. Patients with active moderate-to-severe GO were randomly assigned to receive iv.MP (500 mg once per week for 6 weeks followed by 250 mg per week for 6 weeks) or with TG (20 mg tablet three times per day for 24 weeks). The primary endpoints were the overall response rate and the patients' quality of life at 12 and 24 weeks. Results: In this study, 161 patients were enrolled and randomised from 2015 to 2019. A total of 79 were randomly assigned to receive iv.MP and 82 to receive TG. A greater overall response rate was found in the TG group compared with the iv.MP group at week 24 (90.2% vs 68.4%, P = 0.000). Similarly, the patients' quality of life of the TG group showed a significantly higher response than the iv.MP group at week 24 (89.02% vs 72.15%, P = 0.001). The TG therapy showed a better CAS response than the iv.MP (91.5% vs 70.9% improved, P < 0.05), and up to 91.2% of patients were inactive. Also, the TG group showed a significantly higher improved rate of diplopia, proptosis, visual acuity, soft tissue involved and the decrease of eye muscle motility than the iv.MP group at week 24. Significantly more patients in the iv.MP group than the TG group experienced adverse events. Conclusion: Compared with iv.MP treatment, TG therapy is more effective and safer for patients with active moderate to severe GO.


Assuntos
Glicosídeos/uso terapêutico , Oftalmopatia de Graves/tratamento farmacológico , Extratos Vegetais/uso terapêutico , Tripterygium , Administração Intravenosa , Adulto , Antitireóideos/uso terapêutico , Diplopia/fisiopatologia , Exoftalmia/fisiopatologia , Dor Ocular/fisiopatologia , Feminino , Glucocorticoides/uso terapêutico , Doença de Graves/tratamento farmacológico , Oftalmopatia de Graves/fisiopatologia , Humanos , Hipotireoidismo/tratamento farmacológico , Masculino , Metilprednisolona/uso terapêutico , Pessoa de Meia-Idade , Músculos Oculomotores/fisiopatologia , Índice de Gravidade de Doença , Método Simples-Cego , Tiroxina/uso terapêutico , Resultado do Tratamento , Acuidade Visual/fisiologia
5.
PLoS One ; 15(10): e0240019, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-33002097

RESUMO

Since the inferior rectus muscle (IRM) is a secondary adductor, it is expected to commonly observe esotropia in thyroid-associated inferior rectus myopathy, but this can be improved after the IRM recession. However, variable outcomes regarding the changes in horizontal strabismus after IRM recession ± IRM nasal transposition have been encountered in patients with thyroid eye disease (TED). We, therefore, examined the changes in 62 patients with TED in this retrospective, observational, comparative study. The patients were classified into 3 groups based on the results of postoperative changes in horizontal strabismus: Groups A (reduced esotropia), B (unchanged esotropia), and C (increased esotropia). Consequently, Groups A, B, and C included 23 (38.7%), 11 (17.7%), and 27 (43.5%) patients, respectively. In the multivariate linear regression analysis, the angle of preoperative esotropia (P < 0.001) and the amount of IRM nasal transposition (P = 0.049) were significant predictors of postoperative changes in horizontal strabismus. The results of our study will be helpful to ophthalmologists for formulating an effective preoperative surgical plan.


Assuntos
Oftalmopatia de Graves/complicações , Músculos Oculomotores/fisiopatologia , Estrabismo/complicações , Estrabismo/fisiopatologia , Feminino , Humanos , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Músculos Oculomotores/diagnóstico por imagem , Estudos Retrospectivos , Estrabismo/diagnóstico por imagem , Estrabismo/cirurgia , Resultado do Tratamento
6.
Am J Ophthalmol ; 217: 68-73, 2020 09.
Artigo em Inglês | MEDLINE | ID: mdl-32360860

RESUMO

PURPOSE: Patients with idiopathic nystagmus syndrome often develop an abnormal head position. A horizontal face turn can be treated with the augmented Kestenbaum procedure, while patients with a chin up or chin down position can be treated with surgery on the vertical recti and/or oblique muscles. Although rare, some patients may have a head tilt with no face turn. We report 5 patients who underwent horizontal transposition of the vertical rectus muscles to correct a head tilt. DESIGN: Retrospective case series. METHODS: Five cases from 2 different tertiary referral eye centers and 3 different surgeons were reviewed and analyzed. Surgery for the patients consisted of either a 7-mm or full tendon-width transposition of the vertical rectus muscles of each eye to induce cyclotorsion in the direction of the head tilt. The presenting clinical histories, measurements, and surgical outcomes were reviewed. The primary outcome measure was correction of head tilt. RESULTS: Of 5 patients, 2 had previous horizontal face turns that were corrected with an augmented Kestenbaum procedure and later developed a head tilt, while 3 patients presented primarily with a head tilt. Age at surgery ranged from 5 to 8 years. Initial tilts were measured between 20-45°. Surgery was performed bilaterally except in 1 patient with history of morning glory disc anomaly and therefore transposition surgery was only performed on fixating eye for that patient. Postoperatively, 3 of 5 patients had near complete (0-5°) resolution of their tilt. One patient had a persistent 5-degree head tilt and a small chin up that was nullified with vertical prisms in spectacles. CONCLUSION: Transposition of the vertical rectus to induce cyclotorsion in the direction of the head tilt improves abnormal head titling in patients who have idiopathic nystagmus syndrome. This procedure was safely performed in patients with previous augmented Kestenbaum procedures with no incidence of anterior segment ischemia in our cases.


Assuntos
Movimentos da Cabeça/fisiologia , Cabeça/fisiopatologia , Nistagmo Patológico/cirurgia , Músculos Oculomotores/cirurgia , Procedimentos Cirúrgicos Oftalmológicos/métodos , Postura/fisiologia , Acuidade Visual , Criança , Pré-Escolar , Feminino , Fixação Ocular , Humanos , Masculino , Nistagmo Patológico/fisiopatologia , Músculos Oculomotores/fisiopatologia , Período Pós-Operatório , Estudos Retrospectivos , Síndrome , Resultado do Tratamento
7.
BMC Complement Med Ther ; 20(1): 82, 2020 Mar 13.
Artigo em Inglês | MEDLINE | ID: mdl-32164649

RESUMO

BACKGROUND: Daily "eye exercises," massaging of periocular acupuncture pressure points, have been part of China's national vision care policy in schools for some 50 years. However, the effect of eye exercises on myopia progression and eyeglasses wear has not been definitively investigated. This study evaluates the effectiveness of eye exercises on visual acuity and the propensity of rural children to wear eyeglasses. METHODS: Cohort study in 252 randomly-selected rural schools with baseline in September 2012 and follow up surveys 9 and 21 months later. Outcomes were assessed using propensity-score matching (PSM), multivariate linear regression and logistic regression to adjust for differences between children performing and not performing eye exercises. RESULTS: Among 19,934 children randomly selected for screening, 2374 myopic (spherical equivalent refractive error ≤ - 0.5 diopters in either eye) children (11.9%, mean age 10.5 [Standard Error 1.08] years, 48.5% boys) had VA in either eye ≤6/12 without eyeglasses correctable to > 6/12 with eyeglasses. Among these who completed the 21-month follow up, 1217 (58.2%) children reported practicing eye exercises on school days and 874 (41.8%) did not. After propensity-score matching, 1652 (79%) children were matched: 826 (50%) in the Eye Exercises group and 826 (50%) in the No Exercise group. Performing eye exercises was not associated with change in LogMAR uncorrected visual acuity and wear of eyeglasses, using either logistic regression or PSM at 9 or 21 months. CONCLUSIONS: We found no evidence for an effect of eye exercises on change in vision or eyeglasses wear. TRIAL REGISTRATION: The original trial (Registration site: http://isrctn.org. Registration number: ISRCTN03252665) was retrospectively registered 25/09/2012.


Assuntos
Óculos , Massagem/métodos , Músculos Oculomotores/fisiopatologia , Acuidade Visual , Acupressão , Pontos de Acupuntura , Criança , China , Estudos de Coortes , Feminino , Humanos , Masculino , Inquéritos e Questionários
8.
Zhonghua Yan Ke Za Zhi ; 56(3): 171-175, 2020 Mar 11.
Artigo em Chinês | MEDLINE | ID: mdl-32187945

RESUMO

Horizontal strabismus, the most common type of strabismus, can be complicated by the disorder of inferior oblique muscle or superior oblique muscle. It may cause problems such as compensatory head posture, vertical diplopia, torsional diplopia, and abnormal binocular fusion. In horizontal strabismus cases, the disorder of oblique muscles should be recognized, and appropriate surgical methods should be chosen, so that the best postoperative ortho and binocular vision could be obtained. (Chin J Ophthalmol, 2020, 56: 171-175).


Assuntos
Doenças Musculares/fisiopatologia , Músculos Oculomotores/fisiopatologia , Estrabismo/fisiopatologia , Diplopia , Humanos , Procedimentos Cirúrgicos Oftalmológicos , Estudos Retrospectivos , Visão Binocular
9.
Phys Ther Sport ; 42: 68-74, 2020 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-31935640

RESUMO

OBJECTIVE: To examine differences between male and female adolescents on measures of balance, vestibular and oculomotor function within 3 weeks of concussion among a group of pediatric patients presenting to a sports medicine clinic. DESIGN: Medical record review. SETTING: Sports medicine clinic. PARTICIPANTS: 197 female (median age = 15.2 years) and 381 male (median age = 14.6 years) pediatric patients seen for a concussion, evaluated 9.3 ± 5.2 (mean ± SD) days post-concussion. MAIN OUTCOME MEASURES: Patients completed Balance Error Scoring System (BESS), tandem gait, gaze stability, and near point of convergence (NPC) tests. RESULTS: A higher proportion of female patients exhibited abnormal NPC (22% vs. 14%; p = 0.017), gaze stability (53% vs. 43%; p = 0.028), and tandem gait tests (20% vs. 13%; p = 0.026) compared to male patients. Multivariable analysis indicated an independent association between female sex and increased odds of abnormal NPC (adjusted odds ratio [aOR] = 1.79, 95% CI = 1.07-3.00), and tandem gait tests (aOR = 1.96, 95% CI = 1.12-3.41) following concussion. CONCLUSIONS: Our results indicate that within the first three weeks of a concussion, female pediatric patients demonstrated increased odds of exhibiting abnormal near point of convergence, and tandem gait test performance compared to male patients.


Assuntos
Traumatismos em Atletas/fisiopatologia , Concussão Encefálica/fisiopatologia , Movimentos Oculares/fisiologia , Músculos Oculomotores/fisiopatologia , Equilíbrio Postural/fisiologia , Doenças Vestibulares/fisiopatologia , Vestíbulo do Labirinto/fisiopatologia , Adolescente , Traumatismos em Atletas/complicações , Concussão Encefálica/complicações , Feminino , Marcha/fisiologia , Humanos , Masculino , Doenças Vestibulares/etiologia , Testes de Função Vestibular
10.
BMC Ophthalmol ; 20(1): 32, 2020 Jan 21.
Artigo em Inglês | MEDLINE | ID: mdl-31964376

RESUMO

BACKGROUND: Superior oblique weakening is a common method to treat A-pattern strabismus. This study aims to evaluate the surgical results of the bilateral superior oblique posterior tenectomy procedure to treat A-pattern strabismus patients who had bilateral superior oblique overaction without objective ocular intorsion. METHODS: The records of 18 consecutive patients who underwent surgery of superior oblique posterior tenectomy close to its insertion with superior oblique overaction (SOOA)-associated A-pattern strabismus between September 1, 2015 and August 31, 2018 were retrospectively reviewed. Ocular alignment, objective torsion, A-pattern and ocular motility were assessed. Ocular alignment was measured in the primary position, 25° upgaze, and 25° downgaze using the prism bar cover test, and torsion was measured using fundus photographs. RESULTS: A total of 18 patients (mean age: 15 years; 6 female, 12 male) underwent bilateral superior oblique posterior tenectomy and simultaneous horizontal rectus muscle surgery were included. The mean preoperative A-pattern deviation was 15 PD and the mean postoperative A-pattern deviation was 2.25 PD with a mean reduction of 12.75 PD. The mean preoperative superior oblique overaction was 2.28 and the mean postoperative superior oblique overaction was 0.43 with a mean reduction of 1.85. There was no significant correlation between the ocular torsional, vertical alignment change and the superior oblique posterior tenectomy procedure. CONCLUSIONS: Superior oblique posterior tenectomy surgery selectively improved the A-pattern and superior oblique overaction but not affect the primary position vertical deviation, as well as the ocular torsion. It is an effective procedure to treat the mild to moderate superior oblique overaction associated A pattern strabismus without ocular intorsion.


Assuntos
Exotropia/cirurgia , Músculos Oculomotores/cirurgia , Tendões/cirurgia , Anormalidade Torcional/cirurgia , Adolescente , Adulto , Criança , Pré-Escolar , Exotropia/fisiopatologia , Movimentos Oculares/fisiologia , Feminino , Seguimentos , Humanos , Masculino , Músculos Oculomotores/fisiopatologia , Procedimentos Cirúrgicos Oftalmológicos , Refração Ocular/fisiologia , Estudos Retrospectivos , Tendões/fisiopatologia , Resultado do Tratamento , Acuidade Visual/fisiologia
11.
Int Immunopharmacol ; 80: 106130, 2020 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-31978800

RESUMO

BACKGROUND: The pathogenesis of myasthenia gravis (MG) depends upon T and B cells, as well as complement and cytokines. The activation of functional subpopulations of T and B cells is the basis of the immune response. However, the activation levels of T and B cell subsets remain unclear in the pathogenesis of MG. Here, we evaluated the proportions of T and B cell subsets and related cytokines in ocular MG (oMG) patients and generalized MG (gMG) patients, and analyzed the potential roles of T and B cell subsets in the pathogenesis of oMG. METHODS: In total, 16 patients with oMG, 31 patients with gMG, and 20 healthy controls (HCs) were included in this study. Peripheral blood mononuclear cells (PBMCs) were separated from venous blood via density centrifugation. The percentages of CD3+CD4+ T cells, CD3+CD8+ T cells, CD4+CD25+CD127- regulatory T cells (Tregs), CD19+CD27+CD38- memory B cells, CD19+CD24hiCD27+ regulatory B cells (Bregs), CD19+CD38+CD138+ plasma cells, and CD19+CD40+ B cells in PBMCs were detected by flow cytometry. The levels of interleukin (IL)-2, IL-4, IL-6, IL-10, IL-17, and interferon (IFN)-γ in serum were detected by enzyme linked immunosorbent assay (ELISA). Differences in T and B cell subsets and related cytokines were compared among the three groups of participants. RESULTS: The proportions of CD19+CD27+CD38- memory B cells were significantly higher in the oMG and gMG groups than in the HC group (P = 0.004; P < 0.001), whereas the proportion of CD19+CD27+CD38- memory B cells was significantly lower in the oMG group than in the gMG group (P < 0.001). Moreover, the proportion of CD19+CD24hiCD27+ Bregs was significantly higher in the oMG group than in the gMG and HC groups (P = 0.001). The proportion of CD4+CD25+CD127- Tregs was significantly lower in the gMG group than in the oMG and HC groups (P < 0.001). Finally, the level of serum IL-10 was significantly higher in the oMG group than in the gMG and HC groups (P < 0.05). Compared with the HC group, serum IL-2 levels were significantly increased in the oMG and gMG groups (P = 0.016; P = 0.002). DISCUSSION: The reduced ratios of Tregs and Bregs may contribute to the progression of oMG to gMG, and the increased proportion of memory B cells may be closely related to the progression of oMG. IL-2 and IL-10 are important in the development of oMG.


Assuntos
Linfócitos B Reguladores/imunologia , Miastenia Gravis/sangue , Músculos Oculomotores/fisiopatologia , Linfócitos T Reguladores/imunologia , Adulto , Idoso , Linfócitos B Reguladores/metabolismo , Progressão da Doença , Feminino , Voluntários Saudáveis , Humanos , Interleucina-10/metabolismo , Interleucina-2/metabolismo , Contagem de Linfócitos , Masculino , Pessoa de Meia-Idade , Miastenia Gravis/imunologia , Miastenia Gravis/fisiopatologia , Linfócitos T Reguladores/metabolismo , Adulto Jovem
12.
Am J Ophthalmol ; 213: 97-108, 2020 05.
Artigo em Inglês | MEDLINE | ID: mdl-31770515

RESUMO

PURPOSE: To determine the effect of horizontal rectus muscle surgery on distance-near incomitance. DESIGN: Prospective, comparative, interventional case series. METHODS: Prospective evaluation of patients >7 years old who had medial or lateral rectus muscle surgery at the University of Arkansas Medical Center or Arkansas Children's Hospital between December 2009 and January 2012. Prism and alternate cover testing was performed at distance (6 m) and near (0.3 m) fixation after >1 hour of monocular occlusion at preoperative and postoperative examinations within 1 week, and closest to 1 year after surgery. The change in distance-near incomitance was calculated. Patients with extraocular muscle fibrosis or paralysis were excluded. RESULTS: Forty-five patients met inclusion criteria. Twenty-five patients had medial rectus muscle surgery, and 20 patients had lateral rectus muscle surgery. Postoperative examinations showed a change in distance-near incomitance ≤10 prism diopters (PD) in 42 of 44 patients evaluated within 1 week after surgery and in all 28 patients evaluated 6-24 months after surgery. Horizontal rectus muscle surgery did not induce a clinically significant change in distance-near incomitance (±2 PD equivalence, TOST confidence interval, -1.8 +1.6 PD, P value = 0.014). Contrary to traditional teaching, medial rectus muscle surgery was not more likely to induce a greater effect at near fixation (P = 0.80) and lateral rectus muscle surgery was not more likely to induce a greater effect at distance fixation (P > 0.99). CONCLUSION: Horizontal rectus muscle surgery does not induce a clinically significant effect on distance-near incomitance. Contrary to traditional teaching, medial rectus muscle surgery does not induce a greater effect on ocular alignment at near fixation and lateral rectus muscle surgery does not induce a greater effect on ocular alignment at distance fixation. It is not necessary to consider distance-near incomitance when choosing between medial rectus and lateral rectus muscle surgery.


Assuntos
Percepção de Distância/fisiologia , Fixação Ocular/fisiologia , Músculos Oculomotores/cirurgia , Procedimentos Cirúrgicos Oftalmológicos , Estrabismo/cirurgia , Visão Binocular/fisiologia , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Criança , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Músculos Oculomotores/fisiopatologia , Estudos Prospectivos , Estrabismo/fisiopatologia , Resultado do Tratamento , Adulto Jovem
13.
Indian J Ophthalmol ; 68(1): 170-173, 2020 01.
Artigo em Inglês | MEDLINE | ID: mdl-31856501

RESUMO

Purpose: To report the surgical outcomes in six patients of Helveston syndrome using a "four oblique" procedure. The popular methods for surgical management include superior rectus recessions alone or combined with superior oblique tenectomy. However, large angle exotropia correction would entail a higher risk of anterior segment ischemia when the superior rectus needs to be operated along with the horizontal recti. Hence, we evaluated the long-term results of this uncommon procedure. Methods: This was a retrospective review of six patients diagnosed to have manifest dissociated vertical deviation (DVD) with A pattern exotropia with bilateral superior oblique over action. All patients underwent horizontal muscle recessions/resections for exotropia along with bilateral posterior tenectomy of the superior oblique with inferior oblique anterior transpositioning. Results: The median age was 10 years (Range 5-26 years). The mean postoperative follow-up was 26 ± 14.02 months (Range 12-48 months). The mean reduction in exotropia was from 36.5 ± 21.06 PD (Range 15-65 PD) to 6.1 ± 3.06 PD (Range 3-10 PD). The procedure corrected the A pattern from a mean 23 ± 7 PD (Range 15-35 PD) to 7.6 ± 3.2 PD (Range 3-10 PD). The average DVD in the right eye reduced from 14 ± 4.3 PD (Range 8-20 PD) to 5.3 ± 1.2 PD and in the left eye from 14.33 ± 3.6 PD (Range 10-18 PD) to 4.1 ± 1.1 PD. The DVD asymmetry reduced from 6.33 ± 3.4 PD to 1.5 ± 1.3 PD. Conclusion: "Four oblique" procedure with horizontal muscle surgery seems to be an effective method for significantly correcting the A pattern as well as reducing the DVD with good long-term outcome in our case series.


Assuntos
Exotropia/cirurgia , Músculos Oculomotores/cirurgia , Procedimentos Cirúrgicos Oftalmológicos , Estrabismo/cirurgia , Doenças do Nervo Troclear/cirurgia , Adolescente , Adulto , Criança , Pré-Escolar , Exotropia/fisiopatologia , Movimentos Oculares , Feminino , Humanos , Masculino , Músculos Oculomotores/fisiopatologia , Poliglactina 910 , Refração Ocular/fisiologia , Estudos Retrospectivos , Estrabismo/fisiopatologia , Técnicas de Sutura , Suturas , Tendões/cirurgia , Doenças do Nervo Troclear/fisiopatologia , Acuidade Visual/fisiologia
14.
Graefes Arch Clin Exp Ophthalmol ; 258(2): 445-450, 2020 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-31741045

RESUMO

PURPOSE: To describe characteristics of recurrent intermittent exotropia after bilateral lateral rectus (BLR) recession, and identify factors associated with poor outcome after unilateral medial rectus (MR) resection for recurrent intermittent exotropia. METHODS: We retrospectively reviewed 124 patients who have undergone unilateral MR resection for recurrent intermittent exotropia after BLR recession. Patients were followed for at least 2 years after MR resection. Clinical characteristics and risk factors associated with poor outcome after unilateral MR resection were evaluated. Successful outcome was defined as distant deviation within the range of 4 prism diopters (PD) esotropia and 10 PD exotropia at last visit after MR resection. RESULTS: Among 124 patients, 50 patients (41.1%) were male, and the mean age at the time of MR resection was 9.5 ± 3.1 years. The average follow-up period after MR resection was 43.8 ± 23.7 months. Forty-seven patients (37.9%) were classified to have poor outcome at last visit, and 29 patients (23.4%) underwent third operation. None of the patients was overcorrected after MR resection. Multiple logistic regression analyses showed that distant deviation at post-operative 3 months and male gender were associated with poor outcome (OR 1.49; 95% CI 1.27-1.73; P < 0.001, and OR 5.19; 95% CI 1.42-18.98; P = 0.013, respectively). CONCLUSION: Ocular deviation at 3 months after unilateral MR resection for recurrent intermittent exotropia may play a valuable role in anticipating poor outcome. Patients whose exotropia exceeded 9 PD at distance at 3 months' follow-up tended to recur while those whose exotropia remained below 9 PD at distance showed a stable disease course.


Assuntos
Exotropia/cirurgia , Movimentos Oculares/fisiologia , Músculos Oculomotores/cirurgia , Procedimentos Cirúrgicos Oftalmológicos/efeitos adversos , Complicações Pós-Operatórias , Refração Ocular/fisiologia , Criança , Pré-Escolar , Exotropia/fisiopatologia , Feminino , Seguimentos , Humanos , Masculino , Músculos Oculomotores/fisiopatologia , Prognóstico , Estudos Retrospectivos
15.
J Neurol ; 267(3): 739-745, 2020 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-31734908

RESUMO

BACKGROUND AND PURPOSE: Single-fibre electromyography (SF-EMG) is considered as the most sensitive test for the diagnosis of MG. However, previous studies had limitations, such as a retrospective design, non-consecutive sampling, incorporation bias or were performed in small or mixed populations. Our aims were to determine the diagnostic sensitivity and specificity of SF-EMG of the orbicularis oculi in OMG and the utility of this test in relation to patients' clinical presentation. MATERIALS AND METHODS: We studied 232 consecutive patients referred to the SF-EMG laboratory for a suspected OMG. Stimulated SF-EMG was performed on the orbicularis oculi muscle. RESULTS: OMG was diagnosed in 165 cases and other disorders (OD) in 67. SF-EMG showed a sensitivity of 0.79 (95% CI 0.73-0.85) and a specificity of 0.80 (95% CI 0.71-0.90). False negative results were associated with mild symptoms and with isolated diplopia. Comparison of the diagnostic yield among patients with different clinical presentations showed a similar diagnostic accuracy of SF-EMG in patients with ptosis and in patients with ptosis and diplopia, significantly higher than in patients with isolated diplopia (P < 0.0001). Twenty-two patients with OMG presenting with isolated ptosis or diplopia, who initially tested negative, were re-tested in relation to a worsening of their symptoms showing a positivisation in 91% of cases. CONCLUSIONS: SF-EMG on the orbicularis oculi muscle is very sensitive in patients with ptosis. In contrast, in patients with isolated diplopia SF-EMG does not exclude OMG. Therefore, the interpretation of the results of the test should take into account the patients' clinical presentation.


Assuntos
Eletromiografia/métodos , Miastenia Gravis/diagnóstico , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Músculos Oculomotores/fisiopatologia , Sensibilidade e Especificidade , Adulto Jovem
16.
Muscle Nerve ; 61(2): 226-230, 2020 02.
Artigo em Inglês | MEDLINE | ID: mdl-31650555

RESUMO

INTRODUCTION: Juvenile myasthenia gravis (JMG), a pediatric autoimmune neuromuscular junction disorder, includes generalized (GMG), and ocular (OMG) variants. We sought to determine whether differences existed between OMG and GMG children regarding demographics or treatment response. METHODS: We performed retrospective analysis of 60 children with JMG seen between 1990 and 2018. Osserman scores were used to define OMG and GMG. The myasthenia scale of Millichap and Dodge was used to assess treatment responses. RESULTS: There were no differences between GMG and OMG regarding time interval from disease onset to prednisone initiation (P = .42), or treatment response according to Millichap and Dodge (P = .12). Compared with GMG, OMG children showed younger age of disease onset and better outcomes after treatment. No OMG patients progressed to generalized disease during the follow-up period. DISCUSSION: Compared with GMG, OMG patients had earlier disease onset and improved outcomes after treatment.


Assuntos
Miastenia Gravis/tratamento farmacológico , Adolescente , Idade de Início , Anti-Inflamatórios/uso terapêutico , Criança , Pré-Escolar , Progressão da Doença , Feminino , Humanos , Lactente , Masculino , Miastenia Gravis/fisiopatologia , Músculos Oculomotores/fisiopatologia , Prednisona/uso terapêutico , Estudos Retrospectivos , Resultado do Tratamento
17.
J Neurosci ; 40(1): 143-158, 2020 01 02.
Artigo em Inglês | MEDLINE | ID: mdl-31685652

RESUMO

Down syndrome cell adhesion molecules (dscam and dscaml1) are essential regulators of neural circuit assembly, but their roles in vertebrate neural circuit function are still mostly unexplored. We investigated the functional consequences of dscaml1 deficiency in the larval zebrafish (sexually undifferentiated) oculomotor system, where behavior, circuit function, and neuronal activity can be precisely quantified. Genetic perturbation of dscaml1 resulted in deficits in retinal patterning and light adaptation, consistent with its known roles in mammals. Oculomotor analyses revealed specific deficits related to the dscaml1 mutation, including severe fatigue during gaze stabilization, reduced saccade amplitude and velocity in the light, greater disconjugacy, and impaired fixation. Two-photon calcium imaging of abducens neurons in control and dscaml1 mutant animals confirmed deficits in saccade-command signals (indicative of an impairment in the saccadic premotor pathway), whereas abducens activation by the pretectum-vestibular pathway was not affected. Together, we show that loss of dscaml1 resulted in impairments in specific oculomotor circuits, providing a new animal model to investigate the development of oculomotor premotor pathways and their associated human ocular disorders.SIGNIFICANCE STATEMENT Dscaml1 is a neural developmental gene with unknown behavioral significance. Using the zebrafish model, this study shows that dscaml1 mutants have a host of oculomotor (eye movement) deficits. Notably, the oculomotor phenotypes in dscaml1 mutants are reminiscent of human ocular motor apraxia, a neurodevelopmental disorder characterized by reduced saccade amplitude and gaze stabilization deficits. Population-level recording of neuronal activity further revealed potential subcircuit-specific requirements for dscaml1 during oculomotor behavior. These findings underscore the importance of dscaml1 in the development of visuomotor function and characterize a new model to investigate potential circuit deficits underlying human oculomotor disorders.


Assuntos
Movimentos Oculares/fisiologia , Adaptação Ocular/genética , Adaptação Ocular/fisiologia , Células Amácrinas/fisiologia , Animais , Animais Geneticamente Modificados , Sinalização do Cálcio , Moléculas de Adesão Celular/fisiologia , Movimentos Oculares/genética , Fixação Ocular/genética , Fixação Ocular/fisiologia , Larva , Locomoção , Fadiga Muscular , Mutação , Músculos Oculomotores/crescimento & desenvolvimento , Músculos Oculomotores/fisiopatologia , Retina/crescimento & desenvolvimento , Retina/ultraestrutura , Movimentos Sacádicos/genética , Movimentos Sacádicos/fisiologia , Peixe-Zebra/crescimento & desenvolvimento , Proteínas de Peixe-Zebra/fisiologia
18.
Am J Ophthalmol ; 209: 55-61, 2020 01.
Artigo em Inglês | MEDLINE | ID: mdl-31526795

RESUMO

PURPOSE: Sagging eye syndrome (SES), horizontal and/or vertical strabismus caused by orbital connective tissue degeneration, was first defined 10 years ago. This study investigated SES and other causes of acquired binocular diplopia in adults presenting to a single institution since the description of SES. DESIGN: Retrospective observational case series. METHODS: Medical records were reviewed of all new patients over the age of 40 who presented to the Stein Eye Institute with binocular diplopia between January 2015 and December 2018. Clinical causes of diplopia were tabulated in patients grouped by age and sex. In patients with SES, we tabulated binocular alignment, types of treatment, and surgical outcomes. RESULTS: There were 945 patients of mean age 66.5 years, of whom 514 (54.4%) were female. The most common cause of diplopia was SES (31.4%). The 297 patients with SES were older at 71.2 years (P < 0.0001) and more predominantly female at 59.9% than other patients (52.0%; P = 0.023). The relative proportion of SES patients among all diplopic patients increased with age from 4.7% under age 50 years to 60.9% over the age of 90. Age-related distance esotropia was present in 35% and cyclovertical strabismus in 65% of cases of SES. Strabismus surgery was performed in 50% of cases of SES. Mean esotropia at distance decreased from 6.9 ± 0.7Δ preoperatively to 0.3 ± 0.3Δ postoperatively. Preoperative hypertropia decreased from 3.0 ± 0.3Δ to 0.7 ± 0.2Δ postoperatively. Surgery resolved diplopia in all cases. CONCLUSIONS: It is important to recognize that SES is a very common cause of adult binocular diplopia.


Assuntos
Doenças do Tecido Conjuntivo/epidemiologia , Diplopia/epidemiologia , Doenças Orbitárias/epidemiologia , Estrabismo/epidemiologia , Visão Binocular/fisiologia , Adulto , Idoso , Idoso de 80 Anos ou mais , Envelhecimento/fisiologia , Doenças do Tecido Conjuntivo/fisiopatologia , Doenças do Tecido Conjuntivo/cirurgia , Diplopia/fisiopatologia , Diplopia/cirurgia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Músculos Oculomotores/fisiopatologia , Músculos Oculomotores/cirurgia , Procedimentos Cirúrgicos Oftalmológicos , Doenças Orbitárias/fisiopatologia , Doenças Orbitárias/cirurgia , Prevalência , Estudos Retrospectivos , Estrabismo/fisiopatologia , Estrabismo/cirurgia , Síndrome
19.
Eur J Ophthalmol ; 30(1): 132-138, 2020 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-30360648

RESUMO

PURPOSE: To determine the possible predictors of the motor outcome after corrective surgery for acquired comitant esotropia. METHODS: The study included 40 patients with acquired comitant esotropia. After evaluation of the visual, sensory, and motor status; cycloplegic refraction; fundus examination; and spectacle prescription, all patients underwent corrective strabismus surgery. A successful motor outcome was defined as 0 to 10 prism diopters of horizontal tropia. Statistical analysis of the results was done using the chi-square tests. RESULTS: At the sixth postoperative month, 90% achieved a successful motor outcome, while 2.5% had a consecutive exotropia >10 prism diopters, and 7.5% had a residual esotropia >10 prism diopters. A statistically significant relationship existed between the motor outcome and the following: preoperative results of the Worth four-dot test at 6 m (p = .011) and 0.33 m (p = .009), preoperative distant (p = .016) and near (p = .017) angles of esotropia with glasses, postoperative near angles of deviation at 1 week (p = .005), 1 month (p < .001), 6 weeks (p < .001), 3 months (p = .001), and 6 months (p < .001) following surgery, postoperative distant angles of deviation at 6 weeks (p < .001) and 3 months (p = .03) following surgery, 6 week (p = .01) and 6 month (p = .036) postoperative results of the Worth four-dot test at 0.33 m, and the 6-month postoperative sensory outcome (p = .006). CONCLUSION: Preoperative and postoperative sensory and motor functions are predictors of the 6-month postoperative motor outcome.


Assuntos
Esotropia/cirurgia , Movimentos Oculares/fisiologia , Músculos Oculomotores/cirurgia , Procedimentos Cirúrgicos Oftalmológicos/métodos , Acuidade Visual , Adolescente , Criança , Pré-Escolar , Esotropia/fisiopatologia , Feminino , Humanos , Lactente , Masculino , Músculos Oculomotores/fisiopatologia , Período Pós-Operatório , Estudos Prospectivos , Resultado do Tratamento
20.
Orbit ; 39(1): 5-12, 2020 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-31056988

RESUMO

Purpose: To describe the demographic and clinical characteristics of patients with thyroid eye disease (TED) who present with predominate superior rectus/levator complex involvement.Methods: A multi-institutional retrospective review was performed to identify patients with TED who presented with superior isolated or predominate rectus/levator involvement. Baseline and subsequent visits were reviewed to characterize the clinical course.Results: Nineteen patients were identified. All patients had imaging demonstrating an enlarged levator/superior rectus complex. At presentation, the mean clinical activity score (CAS) was 2.1 (range: 0-5). Nineteen (100%) patients had proptosis on the affected side. Lid abnormalities, including upper/lower eyelid retraction and ptosis were higher on affected side compared to the unaffected side. Eleven (58%) patients had vertical misalignment. Mean thyroid stimulating immunoglobulin (TSI) was 3.7 (range: 1-7.1). Mean follow-up time was 18 months (range: 0-60 months). At last follow-up, the mean CAS was 1.3 (range 0-5). Ten (53%) patients had proptosis. Eleven (58%) patients had vertical misalignment. Repeat imaging in eight patients showed interval enlargement of other extraocular muscles.Conclusions: The presentation of TED with superior rectus/levator complex enlargement may be under-appreciated. Orbital imaging, as well as laboratory evaluation, may help support a diagnosis of TED. In the setting of abnormal TSI and/or thyrotropin receptor antibody, presence of upper eyelid retraction, and an otherwise unremarkable laboratory and systemic evaluation, a presumptive diagnosis of TED may be made, and the patient can be followed closely, as he/she is likely to develop involvement of other extraocular muscles, consistent with a more typical presentation of TED.


Assuntos
Blefaroptose/cirurgia , Oftalmopatia de Graves/diagnóstico , Monitorização Fisiológica , Músculos Oculomotores/fisiopatologia , Adolescente , Adulto , Idoso , Biópsia por Agulha , Blefaroptose/etiologia , Blefaroptose/fisiopatologia , Estudos de Coortes , Feminino , Seguimentos , Oftalmopatia de Graves/patologia , Oftalmopatia de Graves/terapia , Hospitais Universitários , Humanos , Imuno-Histoquímica , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Medição de Risco , Índice de Gravidade de Doença , Testes de Função Tireóidea , Fatores de Tempo , Tomografia Computadorizada por Raios X/métodos , Adulto Jovem
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