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

Although thyrotoxicosis and orbital complications of acute ethmoid or frontal sinusitis are among the most common causes of unilateral exophthalmos, inflammatory pseudotumor is frequently accompanied by progressive acute unilateral proptosis. Because the associated chemosis, scleral erythema, and ophthalmoplegia constitute a spectrum of clinical findings present in numerous inflammatory orbital disorders and systemic diseases, the diagnosis of inflammatory pseudotumor is one of exclusion, often requiring orbital biopsy. Four patients without evidence of sinusitis, endocrinopathy, collagen vascular disease, or Wegener's granulomatosis are described. The diagnosis of orbital pseudotumor was disclosed by computed axial tomography, thus avoiding orbitotomy. The finding of scleral and choroidal thickening with enhancement following intravenous contrast injection represents a select group of patients with orbital pseudotumor and differentiates them from patients with endocrine exophthalmopathy or neoplasms. This noninvasive technique is extremely valuable because early diagnosis is critical for successful treatment. All four patients responded dramatically to high-dose corticosteroid therapy. In the absence of significant clinical response, however, Wegener's granulomatosis, lymphoma, and rhabdomyosarcoma, especially in younger patients, must be carefully excluded. Orbital exploration or decompression or both are used when proptosis, headache, or orbital pain does not resolve promptly, visual acuity deteriorates, or the diagnosis remains unknown.
 ...
PMID:Computerized axial tomography in inflammatory pseudotumor of the orbit. 682 19

Two patients with recurrent painful ophthalmoplegia due to granulomatous involvement of the intracavernous sinus (Tolosa-Hunt syndrome) had circulating anti-neutrophil cytoplasmic antibodies with a granular cytoplasmic immunofluorescence pattern during a flare-up of the disease. The presence of these antibodies suggests that Tolosa-Hunt syndrome might be regarded as a localized form of Wegener's granulomatosis.
 ...
PMID:Is Tolosa-Hunt syndrome a limited form of Wegener's granulomatosis? Report of two cases with anti-neutrophil cytoplasmic antibodies. 833 43

Neurological involvement in Wegener's granulomatosis was studied by reviewing the charts of 324 consecutive patients in whom the diagnosis was made at the Mayo Clinic. One hundred nine patients (33.6%) had neurological involvement. Peripheral neuropathy occurred in 53; cranial neuropathy, in 21; external ophthalmoplegia, in 16; cerebrovascular events, in 13; seizures, in 10; cerebritis, in 5; and miscellaneous involvement, in 25. The mean age and sex ratio were similar in the patients with and those without neurological involvement. Among the patients with peripheral neuropathy, 42 had mononeuropathy multiplex; 6, distal symmetrical polyneuropathy; and 5, unclassified peripheral neuropathy. Multiple mononeuropathy was a major presenting symptom in 8 patients. A significantly higher percentage of patients with peripheral neuropathy, compared to those without peripheral neuropathy, had kidney involvement (p < 0.001). The second, sixth, and seventh cranial nerves were most frequently affected. Multiple cranial nerves were affected in 8 patients. Unusual neurological manifestations in the miscellaneous group were spastic paraparesis, temporal arteritis, Horner's syndrome, and papilledema.
 ...
PMID:Neurological involvement in Wegener's granulomatosis: an analysis of 324 consecutive patients at the Mayo Clinic. 838 87

Wegener's granulomatosis can involve the orbit and sometimes the optic nerve. This usually occurs as a result of contiguous spread from affected sinuses or extraocular muscles, or from a vasculitic posterior optic neuropathy. However, we present an unusual case of isolated orbital apex infiltrative disease in a patient with known Wegener's granulomatosis. This initially caused painless optic neuropathy and progressed to painful ophthalmoplegia and blindness. Optic nerve biopsy, performed to exclude methotrexate-induced lymphoma, confirmed optic nerve infiltration. The condition was refractory to high-dose pulsed methylprednisolone but the patient gained symptomatic relief from rituximab. Wegener's granulomatosis should be considered in cases of isolated posterior optic neuropathy, and close attention should be placed on imaging the orbital apex.
 ...
PMID:Primary Wegener's granulomatosis of the orbital apex with initial optic nerve infiltration. 2128 Oct 75

A 73-year-old man developed double vision and a progressive loss of visual acuity of the left eye over one week. Examination showed disturbances of the left II, III, IV, and VI cranial nerves, that is, an orbital apex syndrome. A brain MRI showed abnormal T2-high signals in the right maxillary sinus and the left mastoid cells without abnormalities in the left orbital apex and the surroundings. Laboratory examination showed an elevated erythrocyte sedimentation rate and a positive perinuclear anti-neutrophil cytoplasmic antibody (MPO-ANCA). After two courses of methyl-prednisolone pulse treatment, his external ophthalmoplegia fully recovered and he regained his left eye's sight. MPO-ANCA was negative and MRI abnormalities were disappeared after treatment. Two years later, the patient developed upper respiratory symptoms associated with an elevation of MPO-ANCA titer, and rapidly progressive renal failure. Renal biopsy specimen showed fibrinoid necrosis with periarteriolar neutrophil infiltration, which suggested that the patient suffered from ANCA-associated vasculitis probably of Wegener's granulomatosis or microscopic polyarteritis. ANCA-associated vasculitis may present with a focal neurological syndrome such as the orbital apex syndrome without a lesion detectable with MRI.
 ...
PMID:[Orbital apex syndrome without MRI lesion caused by ANCA-associated vasculitis]. 2458 92