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1 Walsh & Hoyt: Myasthenia GravisMG is a disease characterized clinically by muscle weakness and fatigability, caused by a reduction in the number of available ACh receptors at NMJs. Receptor depletion is mediated by one or more antibodies directed against ACh receptors or other NMJ postsynaptic membrane constituents, resulting in ...Ocular Motor System; Myasthenia GravisNeuro-ophthalmology Virtual Education Library: NOVEL http://NOVEL.utah.edu
2 Walsh & Hoyt: Pediatric Myasthenia GravisAutoimmune MG can occur at any age. One clinical challenge in ""juvenile-onset"" myasthenia is to distinguish antibody-mediated disease from gene mutations affecting NMJ ion channels. Juvenile-onset autoimmune MG differs little from adult-onset disease, except that it may improve with age. It respon...Ocular Motor System; Pediatric Myasthenia GravisNeuro-ophthalmology Virtual Education Library: NOVEL http://NOVEL.utah.edu
3 Walsh & Hoyt: Eaton-Lambert Syndrome: A Paraneoplastic Disorder of the Neuromuscular JunctionA disorder resembling myasthenia gravis, occurring in patients with bronchogenic carcinoma, was first described by Anderson et al. in 1953. During the next decade, Eaton, Lambert, Rooke, and their associates at the Mayo Clinic studied the clinical and physiologic characteristics of this disorder in ...Neoplasms; Paraneoplastic Syndrome; Eaton-Lambert Syndrome; Paraneoplastic Disorder, Neuromuscular JunctionNeuro-ophthalmology Virtual Education Library: NOVEL http://NOVEL.utah.edu
4 Walsh & Hoyt: Disorders Associated with Myasthenia GravisPatients with MG have a higher prevalence of other autoimmune disorders than otherwise normal persons, about 14% in a Danish cohort and 15% in a Dutch patient series. Disorders of the thyroid gland, including subclinical disease indicated by serum antibody studies, are more common in a myasthenic po...Ocular Motor System; Disorders Associated with Myasthenia GravisNeuro-ophthalmology Virtual Education Library: NOVEL http://NOVEL.utah.edu
5 Walsh & Hoyt: Diagnosis of Mitochondrial Myopathy in Patients with OphthalmoplegiaIt is important to distinguish the mitochondrial myopathies from autosomal-dominant oculopharyngeal muscular dystrophy and myasthenia gravis, because these disorders have distinct therapeutic and genetic implications. Muscle biopsy is the single most useful diagnostic tool for evaluating patients wi...Ocular Motor System; Extraocular Muscles; Myopathies; Muscular Dystrophy; Ion Channel Disorders; Mitochondrial Myopathies; Encephalomyopathy; Ophthalmoplegia; Mitochondrial MyopathyNeuro-ophthalmology Virtual Education Library: NOVEL http://NOVEL.utah.edu
6 Walsh & Hoyt: Evaluation and Management of Abducens Nerve PalsyThere is a tendency to assume that all patients with an esotropia and unilateral or bilateral abduction weakness have an abducens nerve paresis. The physician should first consider the possibility that the condition is myopathic (e.g., thyroid eye disease)or neuromuscular (e.g., myasthenia gravis). ...Ocular Motor System; Ocular Motility Disorders; Abducens Nerve Palsy, Evaluation; Abducens Nerve Palsy, ManagementNeuro-ophthalmology Virtual Education Library: NOVEL http://NOVEL.utah.edu
7 Walsh & Hoyt: Evaluation and Management of Trochlear Nerve PalsyThe three-step test is used to diagnose a trochlear nerve palsy. It is used to isolate the involved paretic muscle in concomitant or incomitant vertical deviations. It is useful only when the paresis involves a single cycloverted muscle. The three-step test is extremely useful in patients with troch...Ocular Motor System; Ocular Motility Disorders; Trochlear Nerve Palsy, Evaluation; Trochlear Nerve Palsy, ManagementNeuro-ophthalmology Virtual Education Library: NOVEL http://NOVEL.utah.edu
8 Walsh & Hoyt: Mitochondrial Disorders Associated with External OphthalmoplegiaCPEO is the most frequent manifestation of mitochondrial myopathies. Harding et al. reviewed the records of 60 patients with mitochondrial myopathy examined at the National Hospital, Queen Square, and found that 54 of the patients (90%) had ocular or oculomotor abnormalities and that CPEO was the pr...Ocular Motor System; Extraocular Muscles; Myopathies; Muscular Dystrophy; Ion Channel Disorders; Mitochondrial Myopathies; Encephalomyopathy; Mitochondrial Disorders; External OphthalmoplegiaNeuro-ophthalmology Virtual Education Library: NOVEL http://NOVEL.utah.edu
9 Walsh & Hoyt: DiagnosisMFS should be considered in any patient who develops the rapidly progressive triad of ophthalmoparesis (with or without pupillary and eyelid involvement), ataxia, and areflexia, and also in patients who develop only a part of the syndrome such as an isolated unilateral or bilateral ophthalmoparesis,...Demyelinating Diseases; Axonal Disorders; Miller Fisher Syndrome; Ophthalmoplegia; Ataxia; Areflexia; Fisher's Syndrome; DiagnosisNeuro-ophthalmology Virtual Education Library: NOVEL http://NOVEL.utah.edu
10 Walsh & Hoyt: Differential Diagnosis of Acquired Trochlear Nerve Palsy: Skew DeviationAlthough trochlear nerve paresis is the most common cause of acquired vertical diplopia, other causes include ocular myopathies (e.g., dysthyroid eye disease), disorders of the neuromuscular junction (e.g., myasthenia gravis), and skew deviation. The ocular myopathies and disorders of the neuromuscu...Ocular Motor System; Ocular Motility Disorders; Acquired Trochlear Nerve Palsy; Skew DeviationNeuro-ophthalmology Virtual Education Library: NOVEL http://NOVEL.utah.edu
11 Walsh & Hoyt: EpidemiologyPatients with CIDP range in age from 2 to 70 years with somewhat more males than females being affected, at least in some series. Well-defined antecedent events are less common in CIDP than in AIDP, but CIDP nevertheless develops after acute upper respiratory tract and other presumed viral infection...Demyelinating Diseases; Axonal Disorders; Chronic Inflammatory Demyelinating Polyneuropathy; EpidemiologyNeuro-ophthalmology Virtual Education Library: NOVEL http://NOVEL.utah.edu
12 Walsh & Hoyt: Graves' DiseasePerhaps the most common systemic disorder associated with diplopia, ophthalmoparesis, and infiltration of extraocular muscles is Graves disease. Graves orbitopathy often occurs in the context of mild hyperthyroid myopathy, producing mild proximal limb muscle weakness. Serum CK activity is normal in ...Ocular Motor System; Extraocular Muscles; Myopathies; Muscular Dystrophy; Ion Channel Disorders; Mitochondrial Myopathies; Encephalomyopathy; Graves' Disease; Thyroid OrbitopathyNeuro-ophthalmology Virtual Education Library: NOVEL http://NOVEL.utah.edu
13 Walsh & Hoyt: Neuro-Ophthalmologic ComplicationsNumerous visual symptoms may occur in patients on cyclosporine, including visual hallucinations, visual field defects, visual agnosia, Balints syndrome, cortical blindness, Antons syndrome, and optic neuropathy. In a series of 582 patients undergoing allogeneic BMT, 4 patients developed transient un...Neoplasms; Neoplasms, Therapy; Chemotherapy, Adverse Effects; Radiotherapy, Adverse Effects; Bone Marrow Transplantation, Adverse Effects; Neuro-Ophthalmologic Complications; Complications of CancersNeuro-ophthalmology Virtual Education Library: NOVEL http://NOVEL.utah.edu
14 Walsh & Hoyt: Normal Neuromuscular TransmissionTo understand the pathophysiology of the defects in myasthenia gravis (MG) and other disorders that damage the neuromuscular junction (NMJ), it is necessary to have some knowledge of the basic events of neuromuscular transmission. Acetylcholine (ACh), the natural transmitter, is synthesized chiefly ...Ocular Motor System; Normal Neuromuscular TransmissionNeuro-ophthalmology Virtual Education Library: NOVEL http://NOVEL.utah.edu
15 Walsh & Hoyt: Disturbances in Disorders of the Neuromuscular JunctionMyasthenia gravis (MG) is an autoimmune disorder characterized by pathogenic antibodies against acetylcholine receptors at motor terminals. Clinically apparent weakness of the intraocular muscles is rare. Nonetheless, abnormalities of pupillary function and accommodation are occasionally reported in...Ocular Motor System; Accommodation, Ocular; Tears; Neuromuscular Junction Disorders; DisturbancesNeuro-ophthalmology Virtual Education Library: NOVEL http://NOVEL.utah.edu
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