Your browser doesn't support javascript.
Mostrar: 20 | 50 | 100
Resultados 1 - 20 de 1.074
Filtrar
1.
BMJ Case Rep ; 12(9)2019 Sep 12.
Artigo em Inglês | MEDLINE | ID: mdl-31519722

RESUMO

Monocular elevation deficit can result from either inferior rectus restriction, superior rectus palsy or from supranuclear causes. We report a case of monocular elevation deficit after scleral perforation repair which was managed by surgery on contra lateral eye. This improved elevation of the affected eye with no diplopia in the postoperative period.


Assuntos
Diplopia/etiologia , Transtornos da Motilidade Ocular/cirurgia , Oftalmoplegia/etiologia , Esclera/lesões , Adulto , Diagnóstico Diferencial , Diplopia/diagnóstico , Diplopia/prevenção & controle , Movimentos Oculares/fisiologia , Humanos , Masculino , Transtornos da Motilidade Ocular/fisiopatologia , Músculos Oculomotores/patologia , Músculos Oculomotores/fisiopatologia , Músculos Oculomotores/cirurgia , Oftalmoplegia/fisiopatologia , Período Pós-Operatório , Esclera/patologia , Esclera/cirurgia , Perfuração Espontânea/cirurgia , Resultado do Tratamento
2.
World Neurosurg ; 132: 33-40, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31470146

RESUMO

BACKGROUND: Pituitary apoplexy (PA) can manifest with visual and endocrine defects. The literature lacks strong support for either surgical or conservative management with respect to symptomatic improvement of these deficits. This meta-analysis compared visual and endocrine outcomes in conservative and surgical treatment of PA. METHODS: A systematic literature search was performed in PubMed, Cochrane, and Ovid MEDLINE for articles published between 1988 and 2018. Recovery outcomes were binarized, such that complete and partial improvements were combined as "improvement." Primary outcome variables evaluated via a binary random-effects model were improvements in endocrine dysfunction, visual field and acuity deficits, and ophthalmoplegia or ocular nerve palsy. RESULTS: Of 483 published articles, 14 studies comprising 457 cases (259 surgical treatments and 198 conservative treatments) were included. On initial examination, 58% of patients had endocrine dysfunction, 37% had visual acuity or field deficit, and 47% had ophthalmoplegia or ocular nerve palsy. Evaluation of outcomes for surgically and conservatively treated patients yielded odds ratios of 0.609 (95% confidence interval [CI], 0.199-1.859; P = 0.383), 0.763 (95% CI, 0.307-2.374; P = 0.763), 1.167 (95% CI, 0.433-3.146; P = 0.760), and 0.801 (95% CI, 0.305-2.105; P = 0.653) for improvements in endocrine dysfunction, visual acuity dysfunction, visual field dysfunction, and ophthalmoplegia or ocular nerve palsy. CONCLUSIONS: Both surgical intervention and conservative management of PA can lead to visual and endocrine recovery, although the management decision may heavily rely on severity of initial deficits. Treatment of PA can be multifaceted and tailored to the individual case and clinical judgment. Further investigation into appropriate intervention based on longitudinal outcome data is warranted.


Assuntos
Tratamento Conservador , Hipopituitarismo/fisiopatologia , Procedimentos Neurocirúrgicos , Oftalmoplegia/fisiopatologia , Apoplexia Hipofisária/terapia , Recuperação de Função Fisiológica , Transtornos da Visão/fisiopatologia , Humanos , Hipopituitarismo/etiologia , Oftalmoplegia/etiologia , Apoplexia Hipofisária/complicações , Apoplexia Hipofisária/fisiopatologia , Transtornos da Visão/etiologia , Acuidade Visual , Campos Visuais
3.
J Hum Genet ; 64(11): 1117-1125, 2019 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-31451716

RESUMO

Whole exome sequencing (WES) is an effective tool for the genetic diagnosis of mitochondrial disorders due to various nuclear genetic defects. In this study, three patients affected by extremely rare mitochondrial disorders caused by nuclear genetic defects are described. The medical records of each patient were reviewed to obtain clinical symptoms, results of biochemical and imaging studies, and muscle biopsies. WES and massive parallel sequencing of whole mtDNA were performed for each patient. The oxygen consumption rate (OCR) and complex activity I and IV was measured. Patients 1 and 2 had exhibited global developmental delay and seizure since early infancy. Blood lactate, the lactate-to-pyruvate ratio, and urinary excretion of Krebs cycle intermediates were markedly elevated. Patient 1 also was noted for ophthalmoplegia. Patient 2 had left ventricular hypertrophy and ataxia. Patient 3 developed dysarthria, gait disturbance, and right-side weakness at age 29. Brain magnetic resonance imaging demonstrated abnormal signal intensity involving the bilateral thalami, midbrain, or pons. Based on WES, patient 1 had p.Glu415Gly and p.Arg484Trp variants in MTO1. In patient 2, p.Gln111ThrfsTer5 and RNA mis-splicing were identified in TSFM. Patient 3 carried p.Met151Thr and p.Met246Lys variants in AARS2. Skin fibroblasts of three patients exhibited decreased OCRs and complex 1 activity, and mitochondrial DNA was normal. These results demonstrate the utility of WES for identifying the genetic cause of extremely rare mitochondrial disorders, which has implications for genetic counseling.


Assuntos
Alanina-tRNA Ligase/genética , Doenças Mitocondriais/genética , Proteínas Mitocondriais/genética , Fatores de Alongamento de Peptídeos/genética , Proteínas de Ligação a RNA/genética , Doenças Raras/genética , Adulto , Encéfalo/diagnóstico por imagem , Encéfalo/patologia , Criança , DNA Mitocondrial/genética , Disartria/genética , Disartria/fisiopatologia , Exoma/genética , Sequenciamento de Nucleotídeos em Larga Escala , Humanos , Hipertrofia Ventricular Esquerda/genética , Hipertrofia Ventricular Esquerda/fisiopatologia , Imagem por Ressonância Magnética , Masculino , Mitocôndrias/genética , Doenças Mitocondriais/diagnóstico por imagem , Doenças Mitocondriais/fisiopatologia , Mutação , Oftalmoplegia/genética , Oftalmoplegia/fisiopatologia , Linhagem , Doenças Raras/diagnóstico por imagem , Doenças Raras/fisiopatologia , Sequenciamento Completo do Exoma
4.
Curr Opin Ophthalmol ; 30(5): 314-318, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31313749

RESUMO

PURPOSE OF REVIEW: Congenital fibrosis of the extraocular muscles (CFEOM) is caused by abnormal development of the innervation of extraocular muscles. We update the recent literature regarding the clinical, anatomic, genetic, and molecular characteristics of CFEOM. Surgical considerations are addressed. RECENT FINDINGS: CFEOM is broken down into three main subtypes, CFEOM1, CFEOM2, and CFEOM3. Several recent reports of individuals, as well as family pedigrees, highlight the phenotypic heterogeneity of CFEOM. Intracranial and intraorbital radiologic findings have enhanced our understanding of the disease pathophysiology. Molecular genetics research has increased our understanding of the development of extraocular muscles and their innervation as well as pathophysiology of CFEOM. SUMMARY: Our understanding of the pathophysiology of CFEOM has increased with the recent contributions from neuroimaging, molecular genetics, and pedigree analysis. Surgical management of patients with CFEOM continues to be challenging.


Assuntos
Fibrose , Oftalmoplegia , Blefaroptose/fisiopatologia , Blefaroptose/cirurgia , Oftalmopatias Hereditárias/fisiopatologia , Oftalmopatias Hereditárias/cirurgia , Fibrose/classificação , Fibrose/fisiopatologia , Fibrose/cirurgia , Humanos , Transtornos da Motilidade Ocular/fisiopatologia , Transtornos da Motilidade Ocular/cirurgia , Oftalmoplegia/classificação , Oftalmoplegia/fisiopatologia , Oftalmoplegia/cirurgia , Fenótipo
5.
Muscle Nerve ; 60(1): 80-87, 2019 07.
Artigo em Inglês | MEDLINE | ID: mdl-31004442

RESUMO

INTRODUCTION: The objective of this study was to obtain a 6-month natural history of motor function performance in individuals with RYR1- related myopathy (RYR1-RM) by using the Motor Function Measure-32 (MFM-32) and graded functional tests (GFT) while facilitating preparation for interventional trials. METHODS: In total, 34 participants completed the MFM-32 and GFTs at baseline and 6-month visits. RESULTS: Motor deficits according to MFM-32 were primarily observed in the standing and transfers domain (D1; mean 71%). Among the GFTs, participants required the most time to ascend/descend stairs (>7.5 s). Functional movement, determined by GFT grades, was strongly correlated with MFM-32 (D1; r ≥ 0.770, P < 0.001). Motor Function Measure-32 and GFT scores did not reflect any change in performance between baseline and 6-month visits. DISCUSSION: The MFM-32 and GFTs detected motor impairment in RYR1-RM, which remained stable over 6 months. Thus, these measures may be suitable for assessing change in motor function in response to therapeutic intervention. Muscle Nerve 60: 80-87, 2019.


Assuntos
Movimento/fisiologia , Miopatias Congênitas Estruturais/fisiopatologia , Canal de Liberação de Cálcio do Receptor de Rianodina/genética , Adolescente , Adulto , Criança , Progressão da Doença , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Miopatias Congênitas Estruturais/genética , Miopatia da Parte Central/genética , Miopatia da Parte Central/fisiopatologia , Oftalmoplegia/genética , Oftalmoplegia/fisiopatologia , Canal de Liberação de Cálcio do Receptor de Rianodina/deficiência , Adulto Jovem
6.
J Stroke Cerebrovasc Dis ; 28(4): e5-e6, 2019 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-30638944

RESUMO

A 54-year-old male with a history of left posterior parietal ischemic stroke, epilepsy, tobacco and marijuana smoking, and alcohol abuse, presented with acute left visual loss and diplopia. On examination, he had reduced left visual acuity and a left oculomotor nerve palsy. CT angiogram from aortic arch to circle of Willis identified extensive thrombus occluding the left common and internal carotid arteries, extending to the left ophthalmic artery. This case demonstrates acute visual loss from ophthalmic artery occlusion, and left oculomotor nerve palsy from occlusion of the inferolateral trunk of the internal carotid artery (cavernous sinus portion).


Assuntos
Artéria Carótida Interna , Estenose das Carótidas/complicações , Doenças do Nervo Oculomotor/etiologia , Artéria Oftálmica , Oftalmoplegia/etiologia , Trombose/complicações , Transtornos da Visão/etiologia , Artéria Carótida Interna/diagnóstico por imagem , Artéria Carótida Interna/fisiopatologia , Estenose das Carótidas/diagnóstico por imagem , Estenose das Carótidas/fisiopatologia , Angiografia Cerebral/métodos , Angiografia por Tomografia Computadorizada , Humanos , Masculino , Pessoa de Meia-Idade , Doenças do Nervo Oculomotor/diagnóstico , Doenças do Nervo Oculomotor/fisiopatologia , Artéria Oftálmica/diagnóstico por imagem , Artéria Oftálmica/fisiopatologia , Oftalmoplegia/diagnóstico , Oftalmoplegia/fisiopatologia , Trombose/diagnóstico por imagem , Trombose/fisiopatologia , Transtornos da Visão/diagnóstico , Transtornos da Visão/fisiopatologia , Acuidade Visual
7.
Hum Mol Genet ; 28(11): 1872-1884, 2019 06 01.
Artigo em Inglês | MEDLINE | ID: mdl-30689883

RESUMO

Here we characterized a mouse model knocked-in for a frameshift mutation in RYR1 exon 36 (p.Gln1970fsX16) that is isogenic to that identified in one parent of a severely affected patient with recessively inherited multiminicore disease. This individual carrying the RYR1 frameshifting mutation complained of mild muscle weakness and fatigability. Analysis of the RyR1 protein content in a muscle biopsy from this individual showed a content of only 20% of that present in a control individual. The biochemical and physiological characteristics of skeletal muscles from RyR1Q1970fsX16 heterozygous mice recapitulates that of the heterozygous parent. RyR1 protein content in the muscles of mutant mice reached 38% and 58% of that present in total muscle homogenates of fast and slow muscles from wild-type (WT) littermates. The decrease of RyR1 protein content in total homogenates is not accompanied by a decrease of Cav1.1 content, whereby the Cav1.1/RyR1 stoichiometry ratio in skeletal muscles from RyR1Q1970fsX16 heterozygous mice is lower compared to that from WT mice. Electron microscopy (EM) revealed a 36% reduction in the number/area of calcium release units accompanied by a 2.5-fold increase of dyads (triads that have lost one junctional sarcoplasmic reticulum element); both results suggest a reduction of the RyR1 arrays. Compared to WT, muscle strength and depolarization-induced calcium transients in RyR1Q1970fsX16 heterozygous mice muscles were decreased by 20% and 15%, respectively. The RyR1Q1970fsX16 mouse model provides mechanistic insight concerning the phenotype of the parent carrying the RYR1 ex36 mutation and suggests that in skeletal muscle fibres there is a functional reserve of RyR1.


Assuntos
Canais de Cálcio Tipo L/genética , Debilidade Muscular/genética , Miopatias Congênitas Estruturais/genética , Oftalmoplegia/genética , Canal de Liberação de Cálcio do Receptor de Rianodina/deficiência , Adulto , Alelos , Animais , Modelos Animais de Doenças , Mutação da Fase de Leitura/genética , Heterozigoto , Humanos , Camundongos , Microscopia Eletrônica , Fibras Musculares Esqueléticas/metabolismo , Fibras Musculares Esqueléticas/patologia , Fibras Musculares Esqueléticas/ultraestrutura , Debilidade Muscular/patologia , Miopatias Congênitas Estruturais/fisiopatologia , Oftalmoplegia/fisiopatologia , Canal de Liberação de Cálcio do Receptor de Rianodina/genética , Canal de Liberação de Cálcio do Receptor de Rianodina/ultraestrutura
9.
J Neurol ; 266(2): 476-479, 2019 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-30556099

RESUMO

To define the prevalence and characteristics of single ocular motor nerve palsy (OMNP) associated with positive serum anti-GQ1b antibody. We performed a prospective multicenter study that recruited 82 patients with single OMNP without identifiable causes from the history and neuroimaging in six neurology clinics of university hospitals. We measured serum anti-GQ1b antibody in all participants. Twelve patients with multiple OMNP and 30 with identifiable causes served as the controls. Overall, the prevalence of anti-GQ1b antibody syndrome was 10% (8/82) in patients with single OMNP and 6% (5/78) in those with single OMNP in isolation. None of the 14 patients with OMNP with identifiable causes showed positive serum anti-GQ1b antibody. The prevalence of anti-GQ1b antibody syndrome was much higher in patients with multiple OMNP than in those with single OMNP (50% vs. 10%, p < 0.01). Patients with single OMNP and positive anti-GQ1b antibody are younger (42 ± 16 vs. 58 ± 15, p < 0.05) and had a significantly higher frequency of preceding infection (75 vs. 19%, p < 0.05) and other neurological signs (38 vs. 1%, p < 0.05) than those with negative antibody. Eight patients with single OMNP and positive serum anti-GQ1b antibody involved the abducens (n = 6), trochlear (n = 1), or oculomotor nerve (n = 1). Single OMNP accompanying other neurological signs and multiple OMNP are more likely to be associated with anti-GQ1b antibody. Anti-GQ1b antibody syndrome should be considered even in patients with single OMNP, especially when antecedent infection was associated in younger patients.


Assuntos
Autoanticorpos/sangue , Gangliosídeos/imunologia , Oftalmoplegia , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Oftalmoplegia/sangue , Oftalmoplegia/epidemiologia , Oftalmoplegia/fisiopatologia , Prevalência , Adulto Jovem
11.
Ophthalmic Plast Reconstr Surg ; 34(6): e184-e186, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-30320722

RESUMO

Hyaluronic acid injection can lead to skin necrosis, visual loss, and other complications. The blindness with ophthalmoplegia and ptosis is a rare, but terrible and devastating complication. The disfigured appearance usually has significant impact on patient's social life. There is no standard treatment. A patient with hyaluronic acid induced blindness, ophthalmoplegia, and ptosis is reported. Six days after the onset, peribulbar/retrobulbar injections of high dosage hyaluronidase were performed. The orbital edema and ptosis immediately improved. The ophthalmoplegia and ptosis improved significantly within a month and resolved completely within 3 months, although the right vision remained blind. Peribulbar/retrobulbar injection of hyaluronidase is a potential rescue therapy for the hyaluronic acid induced ophthalmoplegia and ptosis.


Assuntos
Blefaroptose/induzido quimicamente , Cegueira/induzido quimicamente , Ácido Hialurônico/efeitos adversos , Oftalmoplegia/induzido quimicamente , Adulto , Blefaroptose/diagnóstico , Cegueira/diagnóstico , Cegueira/fisiopatologia , Técnicas Cosméticas/efeitos adversos , Movimentos Oculares , Sobrancelhas , Feminino , Seguimentos , Humanos , Ácido Hialurônico/administração & dosagem , Injeções , Oftalmoplegia/diagnóstico , Oftalmoplegia/fisiopatologia , Viscossuplementos/administração & dosagem , Viscossuplementos/efeitos adversos , Acuidade Visual
13.
J Binocul Vis Ocul Motil ; 68(1): 4-6, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-30196777

RESUMO

Ophthalmoplegia can be caused by multiple etiologies. There are restrictive, paretic, neurologic, and myasthenic conditions that can lead to reduced mobility of the eyes that at first glance may be difficult to discern from one another. While the examiner may be tempted to order various lab tests and scans to reach a diagnosis, the assessment of ophthalmoplegia by clinical examination alone can often provide enough information to determine the etiology, or at least rule out causes that can spare the patient and facility from unnecessary time-consuming and costly tests. This article will discuss the definition of ophthalmoplegia as well as various techniques that can be performed in the clinical setting that may help distinguish its underlying causes.


Assuntos
Técnicas de Diagnóstico Oftalmológico , Movimentos Oculares/fisiologia , Músculos Oculomotores/fisiopatologia , Oftalmoplegia/diagnóstico , Diagnóstico Diferencial , Humanos , Oftalmoplegia/fisiopatologia
14.
J Binocul Vis Ocul Motil ; 68(1): 10-19, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-30196781

RESUMO

Ophthalmoplegia or ophthalmoparesis are the terms given to paralysis or paresis of one or more of the extraocular muscles in one or both eyes. It may be representative of serious neurological or systemic disease. Other mechanisms may cause limitation of eye movement, including restrictive or myasthenic conditions, or combinations of several etiologies. Evaluating and differentiating the mechanisms causing ophthalmoplegia is important in determining the correct diagnosis and selecting the appropriate management in these complex cases.


Assuntos
Movimentos Oculares/fisiologia , Oftalmopatia de Graves/complicações , Miastenia Gravis/complicações , Miosite/complicações , Músculos Oculomotores/inervação , Oftalmoplegia , Adulto , Idoso , Idoso de 80 Anos ou mais , Criança , Diagnóstico Diferencial , Feminino , Humanos , Masculino , Músculos Oculomotores/fisiopatologia , Oftalmoplegia/diagnóstico , Oftalmoplegia/etiologia , Oftalmoplegia/fisiopatologia
15.
J Binocul Vis Ocul Motil ; 68(1): 28-30, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-30196783

RESUMO

Surgical management-depending upon the severity of the ophthalmoplegia-ranges from restorative to palliative. In paresis with reasonable residual muscle function and ductions, the goal of the surgery is not only to restore single vision in primary position but also to provide a relatively normal field of single binocular vision. With complete paralysis of a single muscle or more than one muscle served by a single cranial nerve (third), in addition to conventional recess or resect surgery, the transposition of still-functioning muscles is often needed to obtain a durable result and restore at least some field of single binocular vision. In complete ophthalmoplegia, the benefits of the surgery are limited; but even in these cases, surgery can often reduce the need for an awkward head posture and improve appearance.


Assuntos
Movimentos Oculares/fisiologia , Músculos Oculomotores/cirurgia , Procedimentos Cirúrgicos Oftalmológicos/métodos , Oftalmoplegia/cirurgia , Procedimentos Cirúrgicos Reconstrutivos/métodos , Humanos , Músculos Oculomotores/inervação , Oftalmoplegia/fisiopatologia
16.
Pediatr Neurol ; 87: 36-41, 2018 10.
Artigo em Inglês | MEDLINE | ID: mdl-30197221

RESUMO

BACKGROUND: Isolated ocular myasthenia gravis (MG) is sparingly common in children relative to adults, ranging from 71% to 93% of all children with MG. PURPOSE: We aimed to characterize the ocular manifestations and outcomes in children with isolated ocular MG. METHODS: Medical records of consecutive 62 subjects less than 15 years of age with ocular MG, were retrospectively reviewed. Demographic data, presenting ocular features, types and variabilities of duction limitation, MG confirmatory tests, types of and responses to treatment, and generalized MG conversion were reviewed. RESULTS: Mean age at onset and follow-up time were 49 months (range, one to 173 months) and 95 months (range, six to 226 months), respectively. Female-to-male ratio was 1.5:1. Initially, ptosis was found in 60 subjects (96.8%), while duction limitation was observed in 28 subjects (45.2%). Total ophthalmoparesis was the most common type of duction limitation. Variability of duction limitation was found in 68% of subjects during the follow-up. Pyridostigmine alone was the most common medication used (48.4%); ptosis was more responsive to therapy than duction limitation. Conversion to generalized MG occurred in 19.4% of subjects, with a mean interval to conversion of nine months after symptom onset. Most conversions (91.7%) occurred in the first two years. CONCLUSIONS: Ptosis was more responsive to treatment than duction limitation. Thus other treatment modalities, as well as strabismic amblyopia screening, should be considered in children with prolonged duction limitation that is refractory to medication. In contrast with adults, a much lower proportion of children converted to generalized MG. This may explain the higher prevalence of isolated ocular MG among the juvenile population.


Assuntos
Blefaroptose , Inibidores da Colinesterase/farmacologia , Miastenia Gravis , Oftalmoplegia , Brometo de Piridostigmina/farmacologia , Adolescente , Blefaroptose/tratamento farmacológico , Blefaroptose/etiologia , Blefaroptose/fisiopatologia , Criança , Pré-Escolar , Progressão da Doença , Feminino , Seguimentos , Humanos , Masculino , Miastenia Gravis/complicações , Miastenia Gravis/tratamento farmacológico , Miastenia Gravis/fisiopatologia , Oftalmoplegia/tratamento farmacológico , Oftalmoplegia/etiologia , Oftalmoplegia/fisiopatologia , Estudos Retrospectivos
18.
Curr Eye Res ; 43(12): 1471-1476, 2018 12.
Artigo em Inglês | MEDLINE | ID: mdl-30081646

RESUMO

Purpose/Aims: To compare the clinical and radiological findings of the two rare entities of congenital orbital fibrosis and congenital fibrosis of the extraocular muscles (CFEOM). Materials Methods: Clinical characteristics and magnetic resonance imaging (MRI) findings of three congenital orbital fibrosis patients were analyzed and compared to 13 CFEOM patients. Results: In all three patients with congenital orbital fibrosis, unilateral EOM limitation and enophthalmos were found with a retrobulbar infiltrating mass and normal oculomotor and abducens nerves on MRI. Conversely, most of the patients with CFEOM showed bilateral involvement with hypoplasia of oculomotor nerves and/or abducens nerves and hypotrophic EOM on MRI. Conclusion: All the patients with congenital orbital fibrosis had normal oculomotor and abducens nerves with a retrobulbar mass infiltrating between EOMs, suggesting that congenital orbital fibrosis is a unique disease entity distinguished from CFEOM.


Assuntos
Fibrose/diagnóstico , Imagem por Ressonância Magnética/métodos , Músculos Oculomotores/diagnóstico por imagem , Nervo Oculomotor/diagnóstico por imagem , Oftalmoplegia/diagnóstico , Estrabismo/etiologia , Adulto , Criança , Movimentos Oculares/fisiologia , Feminino , Fibrose/complicações , Fibrose/fisiopatologia , Seguimentos , Humanos , Lactente , Masculino , Músculos Oculomotores/fisiopatologia , Oftalmoplegia/complicações , Oftalmoplegia/fisiopatologia , Estudos Retrospectivos , Estrabismo/diagnóstico , Estrabismo/fisiopatologia
19.
Muscle Nerve ; 58(4): 542-549, 2018 10.
Artigo em Inglês | MEDLINE | ID: mdl-29790193

RESUMO

INTRODUCTION: Although immunotherapies such as prednisone are effective in treating myasthenic muscle weakness, their effect on resolution of myasthenic-induced persistent ophthalmoparesis is unknown. METHODS: We observed patients with myasthenia gravis during their first year of immunotherapy, documenting ophthalmoplegia scores and drug doses. RESULTS: Seventy-six of 87 cases had persistent ophthalmoparesis. With immunotherapy, the median time to resolution of ophthalmoparesis was 7 months, and 37% of cases resolved within 3 months. Patients starting therapy within 12 months of symptom onset were twice as likely to have resolution in the first year (P = 0.028). Resolution of ophthalmoparesis within 3 months, compared with later resolution, was associated with higher initial prednisone doses (mean 0.5 vs. 0.3 mg/kg/day; P = 0.014). However, 25% of the higher dose group also received intravenous immunoglobulin/plasma exchange; after their exclusion, the finding was not significant. DISCUSSION: One-third of cases with myasthenic ophthalmoparesis resolved within 3 months of immunotherapy, particularly in response to more aggressive immunotherapy. Muscle Nerve 58: 542-549, 2018.


Assuntos
Imunoglobulinas Intravenosas/uso terapêutico , Imunossupressores/uso terapêutico , Miastenia Gravis/terapia , Oftalmoplegia/terapia , Recuperação de Função Fisiológica , Adulto , Azatioprina/uso terapêutico , Feminino , Glucocorticoides/uso terapêutico , Humanos , Fatores Imunológicos/uso terapêutico , Masculino , Metotrexato/uso terapêutico , Pessoa de Meia-Idade , Miastenia Gravis/complicações , Miastenia Gravis/fisiopatologia , Oftalmoplegia/etiologia , Oftalmoplegia/fisiopatologia , Troca Plasmática , Prednisona/uso terapêutico , Modelos de Riscos Proporcionais , Fatores de Tempo , Resultado do Tratamento , Adulto Jovem
SELEÇÃO DE REFERÊNCIAS
DETALHE DA PESQUISA