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11,717 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

This paper reports the findings in a 32-year-old man who presented in adult life with a myasthenic syndrome characterized by dysphagia, dysphonia, fluctuating ptosis, episodic diplopia and a variable weakness of the limbs and trunk. Electromyography showed a small decremental response, increased neuromuscular jitter and blocking of some components. Histochemical and electron microscopic studies revealed changes in end-plate morphology and prominent tubular aggregations within the muscle fibres. Radiochemical investigations using di-iodinated 125I-alpha-bungarotoxin demonstrated a reduction in the number of AChR and alterations in receptor affinity which suggested an abnormality of the AChR macromolecule.
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PMID:Alterations in the number and affinity of junctional acetylcholine receptors in a myopathy with tubular aggregates. A newly recognized receptor defect. 626 5

Two cases of type A botulism in elderly patients are presented, followed by an overview of the disease. Typical features demonstrated by these patients included home food preservation, multiple cases, and early GI tract symptoms followed by diplopia, ptosis, generalized weakness, and respiratory impairment while sensation and deep tendon reflexes remained intact. Fatal outcomes are more likely in the elderly. The CDC should be contacted early in cases of suspected botulism.
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PMID:Botulism in the elderly. 638 54

Clinical, pathological, and immunological analysis of 20 patients with ocular adnexal lymphoid disease has demonstrated several parameters which are useful for distinguishing malignant from benign lesions. Patients in the fourth or fifth decade of life presenting with an acute history of pain, oedema, epiphora, double vision, and ptosis, with a mass localised in the lacrimal gland area, are more likely to have a pseudolymphoma or a chronic inflammatory lesion than a true non-Hodgkin lymphoma (NHL). It is not possible to obtain a definite diagnosis without surgical intervention, because only three out of nine patients with orbital NHL had evidence of a monoclonal B cell population in peripheral blood on admission to the Orbital Centre. Furthermore it was confirmed that the identification of the various orbital lymphoid infiltrates becomes more distinct when immunological techniques are added to the clinical and histopathological methods of investigation. Multidisciplinary cooperation leads to further improvement of diagnosis and treatment of ocular adnexal lymphoproliferative disease.
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PMID:Lymphoid proliferations in the orbit: malignant or benign? 639 35

The clinical features and genetic background of 18 patients with rheumatoid arthritis were investigated following the development of penicillamine-induced myasthenia (PIM). The initial myasthenia symptoms in all patients consisted of variable diplopia and/or ptosis with progression to a more generalized involvement in 7 of them. No clinical, humoral, or genetic factor was determined which would allow identification of individuals developing generalized as opposed to ocular myasthenia. Withdrawal of penicillamine was associated over 4-60 weeks with a slow resolution of symptoms, facilitated in 12 patients by the use of anticholinesterase agents. In 2 patients a persistent partial unilateral ptosis remains after 15 and 25 months, while in a further patient diplopia is present 42 months after resolution of the other myasthenic symptoms. The patients with PIM when compared with a healthy 'control' population had a significant increase in HLA Dr1 (p corr less than 0.005) and an absence of HLA Dr 3. A genetic susceptibility to the development of PIM, distinct from that observed in myasthenia gravis of spontaneous onset, is suggested by this abnormal distribution of HLA Dr antigens.
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PMID:Penicillamine-induced myasthenia in rheumatoid arthritis: its clinical and genetic features. 660 18

A 17-year-old woman presented with orbital pain, diplopia, ptosis, and upper lid swelling due to an orbital pseudotumor involving the superior rectus-levator complex. Although she had no prior symptoms of chronic inflammatory bowel disease, diagnostic evaluation revealed Crohn's colitis. Diagnostic evaluation of patients with "idiopathic" orbital pseudotumor should include testing to rule out chronic inflammatory bowel disease.
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PMID:Orbital pseudotumor in Crohn's colitis. 671 71

A 49-year-old woman with typical skin lesions of Degos' disease was found to have an afferent pupillary defect, and altitudinal field loss. This loss was probably secondary to vascular occlusion in the optic nerve of a patient with a congenital anomalous retinal vascular pattern. The patient has had no other systemic involvement after a follow-up of 4 years. Three other patients seen in the past had eye involvement and involvement of the central nervous system and died between 1 and 16 years of the diagnosis. Multiple ocular manifestations of Degos' disease have been reported affecting the eyelids, bulbar conjunctivae, retina, and choroid in varying degrees. Diplopia, visual field defects, ophthalmoplegia, ptosis, papilledema, and optic atrophy are ocular changes secondary to involvement of the central nervous system.
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PMID:Ophthalmic changes of Degos' disease (malignant atrophic papulosis). 671 15

A 32-year-old woman with 5 years of oligomenorrhea and 2 years of amenorrhea became pregnant after ovulation induced by clomiphene citrate, human menopausal gonadotropin, and human chorionic gonadotropin. Although there were no neurologic or ophthalmologic symptoms before pregnancy, ptosis and diplopia developed progressively from 14 weeks' gestation. Physical, radiologic, and laboratory examinations revealed hyperprolactinemia with pituitary macroadenoma. In an attempt to delay surgery, 7.5 mg bromocriptine was administered daily. The serum prolactin level was lowered and clinical symptoms improved with bromocriptine treatment. The pregnancy continued uneventfully and normal twins were born at 39 weeks' gestation. Two weeks after delivery, a transsphenoidal hypophysectomy was performed.
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PMID:Effective bromocriptine treatment of a pituitary macroadenoma during pregnancy. 682 41

Seven cases of chronic progressive external ophthalmoplegia (CPEO) have been studied. They all present palpebral ptosis, slowly progressive ophthalmoparesis without diplopia, descending myopathy and hypoacusia. Additional symptoms were small stature in 5 cases, vestibulo-cerebellar dysfunction in 4 cases, cardiac conductive defects in 6 cases, pigmentary degeneration of the retina in 2 cases, endocrine abnormalities in 2 cases. Muscle biopsy displays in all patients numerous ragged red fibers with typical mitochondrial changes, glycogen accumulation and abnormal amounts of lipid droplets. Metabolic studies reveal in all cases abnormal levels of pyruvic and lactic acid both in basal condition and after an oral glucose load. All the patients have been treated with pyridoxine-alpha-ketoglutarate (PAK). This substance is known to reduce pyruvic and lactic acid concentration in normal subjects after muscular exercise. Two months later a reduction of blood pyruvic and lactic acid both in normal condition and after oral glucose load was observed. The AA. discuss the possible physiological mechanism which can explain their findings.
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PMID:Ophthalmoplegia plus: neuropathological and metabolic studies with a therapeutic trial in seven cases. 693 62

Thirty cases of orbital floor blow-out fractures proven by tomography were retrospectively reviewed to determine the accuracy of the plain films. Using the maximum diameter from the tomograms, the area and volume of the fractures were calculated and correlated with the presence of diplopia and enophthalmos at the time of presentation and at subsequent follow-up. Nine of the 30 cases underwent surgical repair. Orbital floor fractures were recognized in 29 of 30 cases using only the 28 degrees Caldwell and Waters views. Routine tomography is unnecessary and should be reserved as a preoperative evaluation in patients with enophthalmos to establish the presence of a significant associated medial wall prolapse.
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PMID:Another look at blow-out fractures of the orbit. 697 48

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


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