Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0029089 (ophthalmoplegia)
 3,338 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

To our knowledge, the juvenile form of spongy degeneration of the CNS (SD-CNS); van Bogaert-Bertrand disease) has been described previously only three times. We report the case of 21 1/4-year-old Japanese woman who was first seen at the age of 11 with growth retardation, ptosis, and ophthalmoplegia. Her progressive neurodegenerative disease included retinitis pigmentosa, blindness, partial deafness, cerebellar dysfunction, hyporeflexia, and muscle wasting. Simultaneous endocrine defects were diabetes mellitus and probable hyperaldosteronism. Heart block developed later. She died of bronchopneumonia. Autopsy showed CNS stigmas typical of spongy degeneration. Additional findings included peripheral nerve demyelination, neurogenic muscle atrophy, pituitary and pancreatic atrophy, right adrenal agenesis, and a left adrenal coritcal lipid-cell adenoma. To our knowledge, our patient was the oldest survivor, the first patient of Japanese ancestry, and had a unique concurrence of certain oculoendocrine defects.
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PMID:Spongy degeneration of the CNS: an instance of the rare juvenile form. 50 59

A 70-year-old woman with a long history of nasal and sinus polyps developed bilateral proptosis and left total external ophthalmoplegia. She was known to have hypercalcemia, which was later discovered to be caused by a parathyroid adenoma. X-ray studies, including computerized tomography, revealed increased radiodensity in the ethmoid sinuses (due to dystrophic calcification from the hypercalcemia), partial absence of the orbital walls from earlier surgical procedures, and bilateral rounded, retrobulbar tumors. At surgery, glistening, yellow, transparent, and encapsulated-lobulated masses were removed from the left orbit; they appeared to have prolapsed through a postsurgical dehiscence in the superomedial orbital wall. Light and electron microscopy confirmed that the lesional tissue represented polyps covered by respiratory epithelium; the yellow color was the consequence of secondary lipidization of the stromal fibroblasts.
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PMID:Sino-orbital polyposis. 51 88

A 59 year-old female with hypercereatinekinasemia associated with pituitary apoplexy was presented. The patient showed headache, nausea, vomiting and pyrexia. On admission, slight nuchal rigidity and photophobia were observed. However all the cranial nerves were intact; neither ophthalmoplegia nor visual defect were observed. Cerebrospinal fluid analysis revealed an elevated protein concentration of 164 mg/dl. There were 157 cells/mm2 (30% neutrophils). Skull X-P disclosed the ballooning of the sella turcica. CT scan, endocrinological examination and angiography lead us to the diagnosis of pituitary apoplexy. By the sphenoidal approach necrotic tissue with a little chromophobe adenoma were removed. No haematoma was detected. The isozyme pattern of serum CK showed 100 percent MM type. Serum CK concentration reached as high as 2502 IU/l on the fifth day from the onset of the symptom and then normalized in 12 days. Though the cause of the hypercreatinekinasemia uncertain, the similar pattern of hypercreatinekinasemia is known in the acute stage of cerebrovascular accident, and it is more often observed in thalamic hemorrhage. We assumed that the hypercreatinekinasemia in our case was caused by hypothalamic irritation, which lead hyperpermeability of sarcolemma and leakage of the enzymes of muscle origin.
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PMID:[Pituitary apoplexy with hypercreatinekinasemia]. 235 Sep 34

Acute enlargement of pituitary adenomas due to haemorrhage or ischaemic necrosis in the tumour was described as "pituitary apoplexy" by Brougham et al. in 1950. Since then, more than 200 cases have been reported, but--especially in the German literature--the syndrome has caught only little attention. Therefore, in a series of 12 own patients, typical findings and clinical characteristics are demonstrated and the literature is discussed. 9 patients had a haemorrhage into the tumour, 3 an acute ischaemic necrosis. The guiding symptom was the acute onset with ophthalmoplegia (11 of 12 patients). Only in one case the adenoma was known before the apoplexy. Other symptoms were headache, blurred vision, drowsiness and, in severe cases, hemiparesis, coma, and hypothalamic disorder. Most important is the acute endocrinological substitution with hydrocortisone; this may be life-saving. Neuroophthalmological recovery depends on early operation: cases of oculomotor palsy require an operation within the first 2 weeks after the acute event. An emergency operation is required only by an acute amaurosis. In general there will be enough time for careful clinical endocrinological and radiological investigations.
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PMID:[Acute hemorrhage and ischemic necroses in hypophyseal tumors: hypophyseal apoplexy]. 259 99

Hypophysis apoplexy is a clinical syndrome characterized by the sudden development of headache, visual disturbance, associated with nausea, vomiting, signs of meningeal irritation and ophthalmoplegia. The symptoms are caused by the hemorrhage of a hypophyseal adenoma. This leads to the swelling of the tumor and compression of the perisellar structures. The authors processed the clinical and pathological characteristics of 28 cases occurring among the patient material of the National Institute of Neurosurgery of the past 10 years. According to the experiences the most important factor of a successful treatment of hypophyseal apoplexy is an early diagnosis and quick admittance to an institute of neurosurgery of satisfactory conditions. Thereafter a steroid hormone therapy of large doses and decompression operation performed by transnasal-transsphenoidal approach leads in the majority of cases to recovery.
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PMID:[Surgical treatment of pituitary apoplexy]. 264 84

An unusually therapy-resistant form of Nelson's syndrome developed in a 23-year-old woman two years after treatment of a pituitary-dependent Cushing's syndrome by bilateral adrenalectomy (1977). Removal of a corticotrophic adenoma by a first transsphenoidal pituitary operation (1979) brought only temporary relief. Two years later, regrowth and apoplexy of the pituitary adenoma led to oculomotor paralysis and had to be treated by a second transsphenoidal operation (1981). A second relapse became manifest two years later. Treatment with bromocriptine relieved the headaches but did not diminish the tumor size. A third pituitary operation (1984) became necessary when neurological signs revealed penetration of the cavernous sinus. The tumor was only partly removed by transsphenoidal surgery. Postoperative deterioration of the patient required emergency craniotomy to check a subarachnoidal hemorrhage and for removal of the remaining tumor. The patient died three days later of pneumococcal meningitis which had developed after the third pituitary operation.
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PMID:[A fatal course of Nelson syndrome]. 323 90

Acromegaly is caused by GH-secreting pituitary adenomas and, in rare cases, by ectopic production of GRH with resultant hypersecretion of GH. Important systemic manifestations include acral enlargement, swelling, disfigurement, glucose intolerance and diabetes, hypertension, nerve entrapment, arthropathy, and cardiac disease. Tumor-related major manifestations are visual impairment, oculomotor paralysis, and hypopituitarism. Morbidity is substantial, and mortality is increased. Diagnosis should be made as early as possible by measuring plasma GH after an oral glucose load and plasma somatomedin C levels. Assessment of a pituitary lesion is best made by CT scanning in the coronal plane. Therapy is mandatory and consists of surgical removal of the pituitary adenoma (usually by the transsphenoidal route) or of the ectopic source of GRH (carcinoids or islet cell tumors). Adjunctive radiation and/or drug therapy is often necessary if complete surgical ablation of the adenoma is not possible. Radiation therapy can be administered as conventional supervoltage x-ray treatment or in the form of heavy particle beams. Drugs effective in partially lowering GH levels are bromocriptine and (not yet released) somatostatin analogues. Long-term follow-up of treated patients is important to guard against recurrence, progression, or development of hypopituitarism.
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PMID:Acromegaly. 331 99

Three cases of acute necrosis of a hypophyseal adenoma are reported with a review of the medical literature. The clinical presentation as an acute parasellar compression syndrome was the result of aseptic meningitis in 2 cases and of cavernous sinus thrombosis in the other; the usual symptoms of headaches, oculomotor paralysis and impairment of consciousness were observed. The diagnosis was suspected on clinical grounds, by the radiological changes of the pituitary fossa and confirmed by computerised axial tomography. All three patients had a favourable outcome. Surgery was only required in one case with threatening visual complications. Dissociated anterior pituitary deficiency persisted in all cases; two patients also had diabetes insipidus. In one case, the pituitary necrosis stabilised an acromegaly for a two year period.
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PMID:[Acute parasellar compression syndrome disclosing hypophyseal adenoma. 3 cases]. 383 9

Two patients, one with ataxia, internuclear ophthalmoplegia, muscle weakness, atrophy, fasciculations, and bilateral Babinski's signs, the other with dysarthria, dysphagia, muscle weakness, atrophy, fasciculations, and hyperreflexia, had elevated serum calcium and parathyroid hormone levels, establishing the diagnosis of primary hyperparathyroidism (HPT). Removal of a parathyroid adenoma in one patient and three hyperplastic parathyroid glands in the other resulted in remission of the hyperparathyroidism but left both patients with residual neurological damage. Postmortem examination of the second patient showed typical features of amyotrophic lateral sclerosis. The findings in these patients show that hyperparathyroidism may be associated with signs of severe central nervous system disease and that patients with unexplained neurological signs or symptoms should be checked for hyperparathyroidism.
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PMID:Severe neurological disease associated with hyperparathyroidism. 673 92

A 20-year-old asymptomatic woman suddenly had complete right ophthalmoplegia associated with right ptosis and impairment of the sensory and motor functions of the right papillary cranial nerve. These symptoms were found to be caused by a chromophobe adenoma of the pituitary gland. Complete resolution of the patient's symptoms followed surgical aspiration of the tumor with subsequent radiation therapy.
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PMID:Sudden complete ophthalmoplegia associated with pituitary adenoma. 686 7


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