Ocular myasthenia gravis(OMG)is an autoimmune disease caused by neuromuscular junction transmission disorders and manifested mainly as fluctuating blepharoptosis and diplopia,with the extraocular muscles as the main involveed sites.While the pathogenesis of OMG remains unclear,some antibodies,complements,and cytokines may be the contributing factors.The diagnosis and treatment of OMG have been defined in recent years.This article reviews the pathogenesis,diagnosis,and treatment of OMG.
Antibodies ; Complement System Proteins ; Cytokines ; Humans ; Myasthenia Gravis ; diagnosis ; pathology ; therapy ; Oculomotor Muscles ; pathology

We report two cases of systemic lupus erythematosus (SLE) in myasthenia gravis (MG) patients who had undergone thymectomy. SLE developed in the patients 3 months or 13 yr after thymectomy, and polyarthritis was the main clinical manifestation of SLE. Both patients fulfilled at least four of the revised criteria for the classification of SLE. In this report, we describe two postthymectomy lupus patients and perform a comparative review of previous cases.
Adult ; Female ; Human ; Lupus Erythematosus, Systemic/*diagnosis/etiology ; Myasthenia Gravis/*diagnosis/therapy ; Thymus Gland/*surgery ; Time Factors

OBJECTIVEThough myasthenia gravis (MG) is a typical autoimmune disorder, there was some controversy on the treatment of the childhood-onset MG. By observing the efficacy of different therapies, the authors analyzed the affecting factors of prognosis in childhood-onset MG.

METHODThe retrospective data of 155 patients with childhood-onset MG (age of MG onset was less than 15 years) were collected from Department of Neurology, Beijing Tongren Hospital (January 2000 - February 2010). The patients were non-randomly divided according to their treatment into 3 groups (glucocorticoid, thymectomy and glucocorticoid combined with thymectomy groups). Postintervention status meeting the criteria of Myasthenia Gravis Foundation of America (MGFA) "complete stable remission, CSR", "pharmacologic remission, PR", "minimal manifestations, MM", or "Improved, I" was regarded as desirable response, which was used as primary indicator of observation. The authors assessed the efficacy of three therapies and analyzed the influencing factors of prognosis by using Chi-square test and Logistic regression.

RESULTAt 3 months of treatment, glucocorticoid group showed the highest effective rate. At the end of 1 year or 2 years of treatment, glucocorticoid combined with thymectomy group showed the highest effective rate respectively. The generalization rate of MG at 2 years, 10 years and 20 years in childhood-onset ocular MG patients were 4.3%, 10.7%, and 41.5%, respectively. Of patients with generalization of MG, 48.1% occurred within 2 years, 92.6% within 20 years. Univariate analysis showed that in childhood-onset ocular MG patients, variables such as age at onset (> 10 years), LG-MG and with chronic fatigue were significantly associated with general MG conversion. Whereas multivariate analysis showed that patients with age at onset (> 10 years) and chronic muscle fatigue were apt to convert to generalized MG.

CONCLUSIONGlucocorticoid appeared to have an effect that leads to early remission of symptoms in childhood-onset MG patients and glucocorticoid combined with thymectomy appeared to have better long-term effect. For those childhood-onset ocular MG patients with longer course of disease, older age of onset, chronic fatigue, or LG-MG, physicians should try to prevent the generalization of MG by immunosuppressive therapies.

Adolescent ; Child ; Child, Preschool ; Female ; Glucocorticoids ; therapeutic use ; Humans ; Male ; Myasthenia Gravis ; diagnosis ; therapy ; Prognosis ; Retrospective Studies ; Thymectomy ; Young Adult

OBJECTIVETo study the diagnosis and treatment of thymoma and to assess its prognostic factors.

METHODSThe clinical data of 116 patients with thymoma were collected. A retrospective analysis was performed, by comparing the survival rate calculated by the Kaplan-Meier method with the rate of recurrence or metastasis.

RESULTSThe standard posteroanterior and lateral chest radiographs were reliable means of detection of most thymomas. Myasthenia gravis was the most commonly paraneoplastic disease (25.0%, 29/116). The extensive radical resection was beneficial for reducing the rate of recurrence of stage I or stage II thymomas (chi(2) = 4.941, P = 0.0219). The survival time could be prolonged by postoperative radiotherapy and chemotherapy. There was a strong correlation between the clinical stage and the histological classification (according to MH classification), through which the invasive behavior of thymoma could be predicted (chi(2) = 19.76, P = 0.007, RR = 1.47). The 3- 5- and 10-year survival rates were 81.2%, 67.9%, and 40.5%, respectively. Statistical analysis showed a significant negative correlation between the stage and the survival rate (chi(2) = 29.73, P = 0.0000, RR = 0.15).

CONCLUSIONThe prognosis of thymoma depends mainly on the histological classification, clinical stage and multimodality treatment rather than on the paraneoplastic diseases.

Adolescent ; Adult ; Aged ; Child ; Child, Preschool ; Female ; Humans ; Male ; Middle Aged ; Myasthenia Gravis ; etiology ; Prognosis ; Retrospective Studies ; Thymoma ; diagnosis ; mortality ; therapy ; Thymus Neoplasms ; diagnosis ; mortality ; therapy

PURPOSE: In order to make an accurate diagnosis, we investigated clinical manifestations and ocular findings in children with ocular myasthenia gravis that can be easily misdiagnosed. METHODS: The medical records of 7 boys and 18 girls under the age of 15 years with ocular myasthenia gravis were reviewed retrospectively. The mean follow-up period was 37 months. RESULTS: The mean age at onset was 37 months. Ptosis was found in 22 patients(88%), strabismus in 21 patients(84%) and amblyopia in 5 patients(20%). Exotropia combined with vertical heterotropia was the most frequent type of strabismus. Ocular motility abnormality was found in 17 patients(68%). The limitation of supraduction or infraduction was most frequently observed. The manifestations of strabismus and ocular motility abnormality were variable and frequently changed during follow-up period. Prednisolone was used more often than pyridostigmine as a maintenance therapy. Ptosis was more responsive to drug therapy than strabismus. CONCLUSIONS: The main ocular findings in ocular myasthenic children were ptosis and exotropia that was not commonly found in Caucasian children. Vertical heterotropia as well as ptosis were commonly accompanied with the horizontal heterotropia. The type and angle of strabismus as well as ocular motility in duction/version were variable during follow-up period
Amblyopia ; Child* ; Diagnosis ; Drug Therapy ; Exotropia ; Female ; Follow-Up Studies ; Humans ; Medical Records ; Myasthenia Gravis* ; Prednisolone ; Pyridostigmine Bromide ; Retrospective Studies ; Strabismus

Hyperthyroidism may be associated or present with a variety of neuromuscular disorders, including thyrotoxic myopathy, exophthalmic ophthalmoplegia, periodic paralysis and myasthenia gravis. In contrast to muscle, peripheral nerve involvement in hyperthyroidism is exceedingly rare, and has received little attention. Paraplegia-like weakness during severe hyperthyroidism was first described by Charcot in 1889, and called Basedow's paraplegia' by Joffory in 1894. However, there has been no reported case in Korea. A 38-year-old woman was admitted for evaluation of progressive weakness and a gait disturbance. Her endocrinological results were compatible with hyperthyroidism. The polyneuropathy was also confirmed with sequential electrophysiological studies of the nerves and muscles. Drug therapy for hyperthyroidism resulted in resolution of the clinical neurological symptoms and progressive improvement of electrophysiological findings. Hyperthyroidisms are common medical disorders, which are often accompanied by diverse types of neurological and neuromuscular dysfunctions. All of these neurological manifestations are important, as they can serve as important clues to the diagnosis of a thyroid disorder. Furthermore, they are often related to the patient's presenting complaint. Therefore, the physician must be alert to the diverse manifestations of thyroid dysfunction, as they can lead to the diagnosis of potentially serious but treatable disorders. Herein is reported a case of myopathy and neuropathy associated with hyperthyroidism (Basedow's paraplegia), with a review of the literature
Adult ; Diagnosis ; Drug Therapy ; Female ; Gait ; Humans ; Hyperthyroidism ; Korea ; Muscles ; Muscular Diseases ; Myasthenia Gravis ; Neurologic Manifestations ; Ophthalmoplegia ; Paralysis ; Paraplegia* ; Peripheral Nerves ; Polyneuropathies ; Thyroid Gland

Hyperthyroidism may be associated or present with a variety of neuromuscular disorders, including thyrotoxic myopathy, exophthalmic ophthalmoplegia, periodic paralysis and myasthenia gravis. In contrast to muscle, peripheral nerve involvement in hyperthyroidism is exceedingly rare, and has received little attention. Paraplegia-like weakness during severe hyperthyroidism was first described by Charcot in 1889, and called Basedow's paraplegia' by Joffory in 1894. However, there has been no reported case in Korea. A 38-year-old woman was admitted for evaluation of progressive weakness and a gait disturbance. Her endocrinological results were compatible with hyperthyroidism. The polyneuropathy was also confirmed with sequential electrophysiological studies of the nerves and muscles. Drug therapy for hyperthyroidism resulted in resolution of the clinical neurological symptoms and progressive improvement of electrophysiological findings. Hyperthyroidisms are common medical disorders, which are often accompanied by diverse types of neurological and neuromuscular dysfunctions. All of these neurological manifestations are important, as they can serve as important clues to the diagnosis of a thyroid disorder. Furthermore, they are often related to the patient's presenting complaint. Therefore, the physician must be alert to the diverse manifestations of thyroid dysfunction, as they can lead to the diagnosis of potentially serious but treatable disorders. Herein is reported a case of myopathy and neuropathy associated with hyperthyroidism (Basedow's paraplegia), with a review of the literature
Adult ; Diagnosis ; Drug Therapy ; Female ; Gait ; Humans ; Hyperthyroidism ; Korea ; Muscles ; Muscular Diseases ; Myasthenia Gravis ; Neurologic Manifestations ; Ophthalmoplegia ; Paralysis ; Paraplegia* ; Peripheral Nerves ; Polyneuropathies ; Thyroid Gland

OBJECTIVESTo study the diagnosis and treatment of thymoma and to assess prognosis factors.

METHODSThe clinical data on 116 patients with thymoma were collected. A retrospective analysis was performed by comparison of their survival rates computed by the actuarial method and rate of recurrence and metastasis.

RESULTSChest radiograph was used chiefly for the preoperative diagnosis of thymoma; myasthenia gravis (MG) (25.0%, 29/116) was the most common paraneoplastic disease. An extensive and radical resection was carried out to reduce the recurrence rate of thymoma with stage I and stage II (chi(2) = 4.941 P = 0.0219). The survival time was prolonged by postoperative radiotherapy and chemotherapy. A strong correlation was noted between the clinical stage and histologic subtype of M-H classification, by which the invasive behavior of thymoma was predicted (r = 0.385, P = 0.007). The 3-, 5-, and 10-year survival rates were 81.2%, 67.9% and 40.5%, respectively. Statistical analysis showed a significant negative correlation between stage and survival rate (r = -0.897, P = 0.0000).

CONCLUSIONThe prognosis of thymoma depends mainly on the histologic subtype, clinical stage and multimodality treatment rather than paraneoplastic diseases.

Adolescent ; Adult ; Aged ; Child ; Child, Preschool ; Female ; Humans ; Male ; Middle Aged ; Myasthenia Gravis ; etiology ; Prognosis ; Radiography ; Retrospective Studies ; Thymoma ; complications ; diagnosis ; diagnostic imaging ; therapy

PURPOSE: To compare the clinical manifestations between patients with ocular myasthenia gravis and those with generalized myasthenia gravis (MG). METHODS: The medical records of 71 patients diagnosed with MG between January 1995 and December 2007 were reviewed. Demographics, sensitivities of diagnostic methods, the presence of systemic autoimmune diseases, ophthalmic complications caused by MG, and treatments were evaluated and compared. RESULTS: Fourteen patients (20%) were diagnosed with ocular MG and 57 patients (80%) with generalized MG. Sensitivities of anti-acetylcholine receptor antibody and repetitive nerve stimulation tests were significantly higher in the generalized MG group (84%, 89%) compared to those in the ocular MG group (50%, 54%) (p = 0.011, p = 0.008). The sensitivity of the neostigmine test was the highest in both groups (98% of generalized MG, 79% of ocular MG), and the difference between the two groups was borderline significant (p = 0.058). The most common symptoms were ptosis and diplopia, and both groups presented with pain, blurred vision, and tearing. Systemic autoimmune disease was more prominent in the generalized MG group (21%) than in the ocular MG group (14%), and steroid therapy was used more frequently in the generalized MG group (82%) than in the ocular MG group (57%). Ophthalmic complications associated with long-term steroid treatment were more profound in the generalized MG (30%) compared to those of the ocular MG (21%). CONCLUSIONS: The generalized MG group was associated with higher sensitivities to diagnostic tests, more systemic steroid use, higher ophthalmic complications caused by systemic autoimmune disease, and long-term steroid treatment compared to those of the ocular MG group.
Adolescent ; Adult ; Aged ; Aged, 80 and over ; Autoimmune Diseases/complications ; Child ; Drug Administration Schedule ; Eye Diseases/chemically induced/immunology ; Female ; Humans ; Male ; Middle Aged ; Myasthenia Gravis/complications/*diagnosis/*drug therapy ; Steroids/*administration & dosage/adverse effects ; Young Adult

A 63-year-old male experienced sudden diplopia after 9 weeks of administration of pegylated interferon (IFN) alpha-2b and ribavirin for chronic hepatitis C (CHC). Ophthalmologic examinations showed ptosis on the right upper lid and restricted right eye movement without any other neurological signs. A brain imaging study and repetitive nerve stimulation test indicated no abnormality. The acetylcholine receptor antibody titer and response to acetylcholinesterase inhibitors were negative, and the results of thyroid function tests were normal. The patient's ophthalmological symptoms improved rapidly 3 weeks after discontinuation of pegylated IFN alpha-2b and ribavirin. The ocular myasthenia associated with combination therapy of pegylated IFN alpha-2b and ribavirin for CHC is very rarely reported; therefore, we present this case with a review of the various eye complications of IFN therapy.
Antiviral Agents/*adverse effects/therapeutic use ; Drug Therapy, Combination ; Hepatitis C, Chronic/complications/*drug therapy ; Humans ; Interferon Alfa-2b/*adverse effects/therapeutic use ; Male ; Middle Aged ; Myasthenia Gravis/*chemically induced/*diagnosis ; Polyethylene Glycols/*adverse effects/therapeutic use ; Ribavirin/*adverse effects/therapeutic use