1/37. Congenital myasthenia gravis: clinical and HLA studies in two brothers.Two brothers with congenital myasthenia gravis are described. In both, ptosis and ophthalmoplegia responded poorly to oral anticholinesterase therapy and to thymectomy. The brothers had two different HLA haplotypes and neither had the HLA-A1-B8-DW3 haplotypes which are commonly associated with myathenia gravis in adult-onset cases.- - - - - - - - - - ranking = 1keywords = myasthenia gravis, myasthenia, gravis (Clic here for more details about this article) |
2/37. lambert-eaton myasthenic syndrome with ophthalmoparesis and pseudoblepharospasm.We report a patient initially diagnosed as having ocular myasthenia gravis who showed progressive ophthalmoparesis and pseudoblepharospasm together with positive acetylcholine receptor antibodies. Repeated evaluation with high-frequency repetitive stimulation revealed an incremental response and elevated titers of antibodies against presynaptic calcium channels, confirming lambert-eaton myasthenic syndrome. Systemic evaluation revealed no malignant neoplasm but revealed euthyroid Hashimoto's disease. Immunomodulative therapy including plasma exchange and administration of an immunosuppressent (azathioprine) combined with a potassium-channel blocker (3,4-diaminopyridine) reduced the ocular abnormalities. We conclude that the ocular manifestations in this patient were probably caused by lambert-eaton myasthenic syndrome.- - - - - - - - - - ranking = 0.19692498348581keywords = myasthenia gravis, myasthenia, gravis (Clic here for more details about this article) |
3/37. Recurrent isolated ptosis in presumed ophthalmoplegic migraine of childhood.PURPOSE: To report a rare case of isolated, recurrent unilateral eyelid ptosis as the sole manifestation of ophthalmoplegic migraine in a healthy young girl. DESIGN: Single observational case report with review of the literature. TESTING: Serologic evaluation, electromyographic, nerve conduction and Tensilon testing, magnetic resonance and computerized tomographic imaging, and magnetic resonance angiography were performed. MAIN OUTCOME MEASURES: Four-year descriptive history of recurrent eyelid ptosis. RESULTS: Between 1994 and 1998, seven discrete episodes of right upper eyelid ptosis without ophthalmoplegia or pupil abnormality occurred. On each occasion, spontaneous resolution occurred over a period of 6 to 10 weeks. Extensive clinical and laboratory evaluation, including testing for myasthenia gravis and magnetic resonance cerebral angiography, was unremarkable. CONCLUSIONS: Recurrent isolated ptosis is a rare manifestation of presumed ophthalmoplegic migraine in childhood. As far as we are aware, this has not been previously reported.- - - - - - - - - - ranking = 0.19692498348581keywords = myasthenia gravis, myasthenia, gravis (Clic here for more details about this article) |
4/37. myasthenia gravis and associated autoimmune diseases in children.myasthenia gravis has been associated with other autoimmune disorders. We report two children with myasthenia gravis and another autoimmune disease: an 18-month-old boy with ocular myasthenia gravis and Hashimoto's disease and a 14-year-old girl presenting with autoimmune polymyositis, then generalized myasthenia gravis 2 years later. The rare combinations of myasthenia gravis and Hashimoto's disease or polymyositis in children are discussed, and we also briefly review myasthenia gravis and other associated autoimmune diseases in children.- - - - - - - - - - ranking = 1.0615003302838keywords = myasthenia gravis, myasthenia, gravis (Clic here for more details about this article) |
5/37. cocaine abuse, generalized myasthenia, complete external ophthalmoplegia, and pseudotonic pupil.We present the case of a 29-year-old woman with generalized myasthenia. Myasthenia with complete external ophthalmoplegia was unmasked by cocaine abuse. It was associated with changes of the pupillary motility, including light-near dissociation and positive 0.1% pilocarpine test. Treatment with acetylcholinesterase inhibitors improved the patient's condition rapidly, and led to complete normalization of extraocular movements and pupillary function. To our knowledge, this is the fourth case of cocaine-related myasthenia, and the first case of myasthenia with pseudotonic pupil.- - - - - - - - - - ranking = 0.40987962858535keywords = myasthenia (Clic here for more details about this article) |
6/37. Ocular myasthenia mimicking a one-and-a-half syndrome.A 52-year-old patient developed an eye movement disorder first resembling a left internuclear ophthalmoplegia and subsequently a "one-and-a-half syndrome" as the presenting symptoms of ocular myasthenia gravis. No accompanying myasthenic features were present except for the fluctuation in the amplitude of dissociated nystagmus. This patient shows that an oculomotor disorder considered a typical pontine lesion may instead be caused by myasthenia gravis, even in the absence of other clinical and electrophysiologic features of neuromuscular deficit.- - - - - - - - - - ranking = 0.62806689759182keywords = myasthenia gravis, myasthenia, gravis (Clic here for more details about this article) |
7/37. Pseudomyasthenia resulting from a posterior carotid artery wall aneurysm: a novel presentation: case report.OBJECTIVE AND IMPORTANCE: Painful oculomotor palsy can result from enlargement or rupture of intracranial aneurysms. The IIIrd cranial nerve dysfunction in this setting, whether partial or complete, is usually fixed or progressive and is sometimes reversible with surgery. We report an unusual oculomotor manifestation of a posterior carotid artery wall aneurysm, which mimicked ocular myasthenia gravis. CLINICAL PRESENTATION: A 47-year-old woman developed painless, intermittent, partial IIIrd cranial nerve palsy. She presented with isolated episodic left-sided ptosis, which initially suggested a metabolic or neuromuscular disorder. However, digital subtraction angiography revealed a left posterior carotid artery wall aneurysm, just proximal to the origin of the posterior communicating artery. INTERVENTION: The aneurysm was successfully clipped via a pterional craniotomy. During surgery, the aneurysm was observed to be compressing the oculomotor nerve. The patient's symptoms resolved after the operation. CONCLUSION: The variability of incomplete IIIrd cranial nerve deficits can present a diagnostic challenge, and the approach for patients with isolated IIIrd cranial nerve palsies remains controversial. Although intracranial aneurysms compressing the oculomotor nerve classically produce fixed or progressive IIIrd cranial nerve palsies with pupillary involvement, anatomic variations may result in atypical presentations. With the exception of patients who present with pupil-sparing but otherwise complete IIIrd cranial nerve palsy, clinicians should always consider an intracranial aneurysm when confronted with even subtle dysfunction of the oculomotor nerve.- - - - - - - - - - ranking = 0.43114191410601keywords = myasthenia gravis, myasthenia, gravis (Clic here for more details about this article) |
8/37. Respiratory chain complex-I defect mimicking myasthenia.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.- - - - - - - - - - ranking = 0.3513253959303keywords = myasthenia (Clic here for more details about this article) |
9/37. Progressive external ophthalmoplegia and benign retinal pigmentation.A 17-year-old boy had progressive external ophthalmoplegia, normal visual acuity, and a pigmentary retinopathy. Neurologic evaluation showed an abnormal electroencephalogram with diffuse slow (theta) waves. myasthenia gravis was excluded. fluorescein angiography showed a normal vascular pattern with diffuse hyper- and hypopigmentation. Results of retinal testing, including color vision, visual fields, electroretinography, and dark adaptometry, were within normal limits except for a slightly subnormal electroretinogram with normal implicit times. Progressive external ophthalmoplegia may be associated with either progressive or non-progressive pigmentary retinopathies, and adequate evaluation of retinal function in these cases must be obtained.- - - - - - - - - - ranking = 0.01537508257095keywords = gravis (Clic here for more details about this article) |
10/37. hyperthyroidism, ophthalmoplegia and unilateral mydriasis.Clinical description of a 33 years-old woman, with hyperthyroidism, admitted to the emergency unit with external bilateral ophthalmoplegia and left mydriasis, unreactive to light. The external ophthalmoplegia is proven to be due to myasthenia. The left mydriatic pupil demonstrates features typical for Adie's tonic pupil. The association of a tonic pupil with an auto-immune disease is infrequent. The axiom that internal ophthalmoplegia in a patient with external ophthalmoplegia excludes myasthenia gravis should be reevaluated.- - - - - - - - - - ranking = 0.25547921614086keywords = myasthenia gravis, myasthenia, gravis (Clic here for more details about this article) |
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