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To study the ability of anticholinesterase drugs to reverse the potentially fatal paralytic effects of cobra venom, we conducted a placebo-controlled, double-blind crossover trial of intravenous edrophonium (Tensilon) in 10 adults with neurotoxic envenoming caused by bites of the Philippine cobra (Naja naja philippinensis). There was significantly more improvement in ptosis and endurance of upward gaze after edrophonium than after placebo. Five minutes after injection, the mean difference (+/- SD) in the percentage of the iris that was uncovered was 39 +/- 5.47 (70 vs. 31 percent; P less than 0.01), and the mean difference in the number of seconds of upward gaze was 33.1 +/- 9.29 (39.7 vs. 6.6 seconds; P less than 0.01). The expiratory and inspiratory pressures, forced vital capacity, and ability to cough, speak, and swallow also improved after edrophonium. In both the patients who were studied electromyographically, pretreatment and postplacebo responses were typical of myasthenia gravis and became normal after edrophonium. We conclude that anticholinesterases are beneficial in the management of neurotoxic envenoming by Asian cobras (Naja naja), and we recommended a test of edrophonium in any patient with signs of neurotoxic envenoming after snakebite.
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PMID:Positive response to edrophonium in patients with neurotoxic envenoming by cobras (Naja naja philippinensis). A placebo-controlled study. 353 83

The response of eye movements to edrophonium is easily missed by clinical observation alone. Binocular horizontal ten degree saccades were recorded by infrared oculography, whilst the vision of one eye was occluded, before and after fatigue repeated intravenous injection of dilute edrophonium, and fatigue induced during anticholinesterase inhibition by intravenous edrophonium, in 26 patients with diplopia or ptosis of uncertain aetiology. The most reliable criterion of a positive response was an increase in the amplitude of the saccades of the fixating eye by 10% or more after each of several injections of dilute edrophonium. The response was positive in 13 patients and was difficult to observe clinically when the responses of the saccades of the eyes moving conjugately were unequal and when the patient presented with ptosis and no diplopia. Edrophonium infrared oculography proved to be a sensitive test for weakness due to the neuromuscular junction defect of myasthenia gravis affecting extraocular muscles.
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PMID:Edrophonium test in myasthenia: quantitative oculography. 358 98

Ocular myasthenia is a special form of general myasthenia gravis characterized by unilateral or bilateral ptosis and eye muscle pareses of distinct variability, depending on the time of day and the state of fatigue of the patient. Most important for diagnosis is the Tensilon test, which can, however, produce negative results. In such cases a combination of the Tensilon test with electromyography is indispensable. In ocular myasthenia there is not always an increase in the antibody titer against acetylcholine receptors in the blood. The treatment of ocular myasthenia is based on the application of cholinesterase inhibitors. The drug of choice is Mestinon; however, the reaction of the eye muscles to this drug is often unsatisfactory. Local application of cholinesterase inhibitors in the form of Eserine, Prostigmin etc. is an additional important therapy. Also in ocular myasthenia the modern treatment with Cortisone (alternate-day therapy with 100 mg Prednisone every second day) has proved very useful. Another possible method of interfering with the immunological systems of myasthenia is immunosuppression with Azathioprin or Cyclophosphamide. The pathognomonic significance of the thymus in the autoimmune process of myasthenia gravis is demonstrated by the good results obtained by thymectomy, which can also be performed successfully in ocular myasthenia, not only in young patients in whom the condition is severe, but also in older patients in whom it is chronic. Often, the therapeutic measures mentioned have to be tested one after another or in combination in order to achieve an optimal therapeutic effect.
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PMID:[Ocular myasthenia]. 399 98

We reported a case of 53-year-old male who was admitted to our hospital for ptosis and difficulty in chewing. Because of positive for Tensilon test and high dose anti-acetylcoline receptor antibody, we established diagnosis for myasthenia gravis. Additionally he was pointed out a cystic lesion at anterior superior mediastinum on CT and MRI. As a result we diagnosed him as thymic cyst with myastenia gravis. The extended thymectomy was performed. Histological examination revealed that the mass was a multilocular thymic cyst which is reported by Suster, who suggested that the multilocular thymic cyst arises from processes of reactions to an acquired inflammatory change. There was no report for cases of multilocular thymic cyst with myastenia gravis. MTC-like changes are sometimes presented in association with thymic Hodgkin's disease or thymic seminoma. Malignant transformation of thymic cyst were also reported. So careful examination and evaluation for cystic lesion within thymus are required.
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PMID:[A case of multilocular thymic cyst with myasthenia gravis]. 761 46

We describe 2 patients presenting with isolated unilateral ptosis without other signs of cranial or peripheral nerve involvement or sympathetic denervation. Both patients (one case of progressive systemic sclerosis and one of rheumatoid arthritis) were currently taking D-penicillamine. In these cases, the ptosis was reversed a few minutes after a Tensilon test, hallmark of myasthenia gravis. Antibodies to acetylcholine receptors were present. Myasthenia gravis should be suspected with ptosis without other cranial nerve involvement or miosis, even if the ptosis is unilateral. Thus, unilateral ptosis can be the first manifestation of a toxic side reaction to D-penicillamine.
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PMID:Unilateral ptosis as an initial manifestation of D-penicillamine induced myasthenia gravis. 816 22

This article reviews the diagnostic testing used in the evaluation of several common neuro-ophthalmologic entities including optic nerve disease, pseudotumor cerebri, anisocoria, ptosis, and ocular motor palsies. Emphasis is placed on these bedside tests that help to establish the diagnosis of these common clinical problems. The utility of the cocaine and Tensilon (edrophonium chloride) tests as well as the role of neuroimaging in these conditions are reviewed.
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PMID:Diagnostic tests in neuro-ophthalmology. 867 44

Myasthenia gravis was diagnosed on the basis of a positive Tensilon test in a 4-year-old male child with alopecia totalis. His scalp hair and eyebrows had began to disappear at the age of 10 months. No other physical abnormalities such as motor paralysis, were seen except for left ptosis and alopecia totalis. His serum titer of anti-acetylcholine receptor antibody was elevated. No immune system abnormalities (C3, C4, CH50, C-reactive protein, antinuclear antibody or lymphocyte function disorders) were detected. Although alopecia areata and alopecia totalis are sometimes present in adults with autoimmune diseases and myasthenia gravis, this association is rare in children. The present case represents the youngest patient with myasthenia gravis associated with alopecia totalis.
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PMID:Myasthenia gravis with alopecia totalis. 958 14

A 34-year-old woman presented with bilateral ophthalmoplegia, ptosis, and mild gait ataxia. Tensilon test, magnetic resonance imaging, and cerebrospinal fluid analysis were normal. She initially denied any alcohol intake but later admitted to significant alcohol and multisubstance abuse. The patient improved dramatically after treatment with thiamine. Wernicke's encephalopathy is discussed, highlighting that it may be present with normal mentation. It should be considered in the differential diagnosis of bilateral ophthalmoplegia even in the absence of altered mental status.
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PMID:Acute bilateral ophthalmoplegia in a young woman. 1090 82

Seventy three patients with myasthenia gravis were studied over 9 years period (1987-1995) in departments of neuromedicine, respiratory care unit, cardiothoracic unit of Institute of Postgraduate Medical Education and Research and Bangur Institute of Neurology, Calcutta, with reference to their clinical presentations, laboratory findings and various modes of treatment. Commonest age of presentation was 5th decade in men and 3rd decade in women. Fifty five percent of patients belong to type 2A myasthenia gravis (Osserman classification). Presentation was insidious (67.2%) and course was slowly progressive (65.7%) in majority of cases. Fatigability and ptosis were commonest clinical presentation and diurnal variation was noticed in 60% of cases. Edrophonium test was positive in 90.4% of cases and repetitive nerve stimulation showed 93.5% positivity in 30 cases. 27 patients (36.9%) underwent thymectomy and out of these, 89% of patients showed hyperplastic change and thymoma in 11% of cases. Mortality rate including both operated and nonoperated patients was recorded to be 9.6%. We observed earlier onset of myasthenia in male, higher incidence of oculo-bulbar involvement and lower incidence of respiratory problem and thymoma.
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PMID:Clinical profile of myasthenia gravis. 1122 17

In a 67-year-old woman with ptosis, double vision, dysphagia, ambiguous Tensilon tests, normal acetylcholine-receptor antibodies, normal thymus, and repeatedly abnormal responses to low-frequency repetitive stimulation, ocular myasthenia was suspected. Pyridostigmin was ineffective, but corticosteroids improved the abnormalities. Despite this therapy, lower-limb weakness developed. Reevaluation disclosed abnormal increase of serum lactate during slight exercise, myogenic electromyography, ragged-red fibers, reduced oxidative enzyme staining and abnormally shaped and structured mitochondria on muscle biopsy, and a respiratory chain complex-I defect on biochemical investigation of the muscle homogenate. Respiratory chain disorder due to complex-I defect with abnormal decremental response to low-frequency repetitive stimulation was diagnosed. It is concluded that respiratory chain disorders due to a complex-I defect may mimic ocular myasthenia clinically, electrophysiologically, and even therapeutically.
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PMID:Respiratory chain complex-I defect mimicking myasthenia. 1189 8


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