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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

Myasthenia gravis is a chronic disease characterized by a fluctuating weakness of voluntary muscles, with a preference for the muscles innervated by the cranial nerves. Ocular symptoms (ptosis, diplopia) were present at onset in 65% of 432 own patients and in 10% of these patients the disease remained confined to the extrinsic eye muscles. A complete remission occurred in 30% of the purely ocular cases within 10 years of onset. The diagnosis depends upon the pattern of weakness, the spontaneous or provoked fluctuation of the symptoms and the favourable response to anticholinesterases. The presence of antibodies against acetylcholine receptor protein is the most recent tool to confirm the diagnosis, but they are absent in 10-20% of the patients with generalized MG and in 20-50% of the purely ocular cases. As the reaction to anticholinesterases in ocular MG is sometimes equivocal or even absent auxillary investigations (electromyography, tonography, nystagmography, curaretest) may be necessary. Oral anticholinesterases (Pyridostigmin, Prostigmin, Ambenomium) usually have a moderate effect on the ptosis and a poor effect on the diplopia so that other measures (ptosishooks, covering one eye) are necessary. In selected patients alternate-day Prednisone is the therapy of choice.
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PMID:The ocular signs and symptoms of myasthenia gravis. 706 4

Myasthenia gravis (MG) is a rare complication of allogeneic bone marrow transplantation (BMT). We present the 11th case in the medical literature, a 23-year-old female 100 months post-allogeneic bone marrow transplantation for acute myelogenous leukemia (AML). After discontinuation of immunosuppression for chronic graft-versus-host disease (GVHD) involving skin, gastrointestinal tract and lacrimal glands, the patient developed severe, progressive dysphagia initially attributed to esophageal candidiasis. With the development of muscle weakness, ptosis, and dysphonia the diagnosis of generalized myasthenia gravis was suspected, and confirmed by elevated anti-acetylcholine receptor antibody titer and a positive edrophonium challenge. Prednisone and pyridostigmine produced improvement, and thymectomy was performed without pathologic evidence of thymoma. Recurrent post-operative respiratory distress required transient mechanical ventilation. Twenty-seven months after diagnosis, the patient requires maintenance prednisone to control symptoms of myasthenia gravis. The clinical features of all reported cases of MG post-allogeneic BMT are reviewed, and universal features include an association with decreasing immunosuppression, the presence of other manifestations of chronic GVHD, anti-acetylcholine receptor antibodies, and the absence of an associated thymoma. HLA Cw1, Cw7 and DR2 were identified at frequencies significantly above that expected from HLA antigen prevalance studies, and may be markers for increased risk of developing MG post-allogeneic BMT. No statistically significant associations with HLA A2, B7, B35 or donor-recipient sex mismatch were present. Reinstitution of immunosuppression and standard therapies for myasthenia gravis were effective in the majority of cases. The role of thymectomy in this population remains unclear.
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PMID:Myasthenia gravis in association with allogeneic bone marrow transplantation: clinical observations, therapeutic implications and review of literature. 915 70

A 82-year-old female was admitted to hospital because of deteriorated general condition, severe diffuse headache and complete left-sided ptosis. A computed tomography scan of the head revealed no subarachnoid haemorrhage. Based on the hypothesis that the symptoms resulted from an infarction in the brain stem, the previous medication with Aspirin was continued. After repeated vomitus hypotensive dehydration developed and was adequately treated. Because of confusion, elevated white blood counts and signs of meningism, a spinal puncture was performed. Only the serology for Borrelia-IgG was positive, therefore the patient received Rocephin. During treatment only the ptosis persisted, therefore the substitution with sodium and the medication with Prednisone were stopped. Afterwards the symptoms reappeared and the laboratory results showed insufficiency of the pituitary. A magnetic resonance scan showed a microadenoma of the pituitary with local bleeding. Nine months after pituitary apoplexy, with hormonal substitution only a divergent strabism on the left side persisted. Clinical findings, course and therapy of pituitary apoplexy are discussed.
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PMID:[Headache, general malaise and left-side ptosis]. 978 50

A woman with Takayasu arteritis is reported who presented with constitutional symptoms and persistent thrombocytosis documented since 3 years before the diagnosis. Disease-specific symptoms such as arm claudication, transient loss of vision, and self-remitting eye ptosis present at the time were apparently missed, because she is a non-English-speaking Hispanic woman whose history was obtained through an interpreter. Extensive workup done at the time failed to reach a definite etiology. A computed tomography scan of the chest done because of midthoracic back pain and an elevated erythrocyte sedimentation rate showed circumferential wall thickening and mild surrounding edema throughout the thoracic and abdominal aorta and both carotid arteries consistent with Takayasu arteritis. Prednisone at a dosage of 1 mg/kg twice a day decreased the platelet count within 45 days of its initiation. Takayasu arteritis should be considered in the differential diagnosis of unexplained thrombocytosis, particularly in young women.
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PMID:Reactive thrombocytosis: an early manifestation of Takayasu arteritis. 1635 75

Patients diagnosed with ocular myasthenia gravis (MG) and mitral valve disease represent a significant perioperative management problem, especially for the anaesthesiologist, due to complex inter-actions between the disease, drugs to treat the disease, and anaesthetic agents, such as neuromuscu-lar blocking agents (NMBAs). This paper describes the successful management of a 31-year-old female with mitral valve stenosis and ocular MG who was diagnosed with MG 4 years prior to the indication for cardiac surgery. Preoperatively, the patient was under treatment with Pyridostigmine and Prednisone. Mitral valve replacement and full thymectomy were performed, under general anaesthesia, using Fentanyl, Sevoflurane and low doses of non-depolarising NMBAs. The postoperative course was uneventful, the patient was extubated at 6 hours postoperatively, in-tensive care unit stay was 48 hours, and the patient was discharged after 6 days without any compli-cations. After 3 months, at the follow-up examination, the patient's ocular symptoms (eyelid ptosis) disappeared.
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PMID:Mitral Valve Replacement with Thymectomy in a Patient with Ocular Myasthenia Gravis: Case Report. 3159 8

Giant cell arteritis (GCA) or temporal arteritis is a granulomatous vasculitis that affects medium-to-large vessels seen primarily in older Caucasian populations. Here, we describe a 67-year-old male who presented with atypical symptoms of worsening headaches associated with left-sided pupil-sparing, isolated third nerve palsy, blurry vision, diplopia and myalgias in bilateral extremities. He was immediately started on intravenous Methylprednisolone for suspected GCA. Subsequent biopsy of the temporal arteries showed panarteritis without giant cells and disruption of the internal elastic lamina. His symptoms improved in a day following treatment and he was discharged on a Prednisone taper. At the time of writing this case, there are only two cases in the literature of ptosis as a presenting symptom in GCA, thus highlighting the importance of recognizing rare red flag symptoms such as ptosis and diplopia. More study is needed in the prognostic significance of these unusual clinical features.
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PMID:An interesting case of temporal arteritis that manifested as ptosis and diplopia. 3326 83