Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0011168 (dysphagia)
 15,644 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 74-year-old man presented sudden onset hoarseness and dysphagia. Two months before this event, he had developed arthralgia of the shoulders, elbows, hands and foot and pleuritis which had been alleviated by a treatment with prednisolone. On admission, the patient could not phonate nor swallow at all. His soft palate was elevated at the right side. The uvula moved left when the patient tried to speak. Laryngoscopic examination revealed the paralysis of right vocal cord. The erythrocyte sedimentation rate (79mm/1h), C-reactive protein (5.3mg/dl), rheumatoid factor (310 IU/ml) and Clq-binding immune complex (4.5 micrograms/ml) were elevated. Hepatitis C virus antibody titer was more than 10.8 IU/l. Anti-nuclear antibody was 1:20 (normal < 1:20) and anti-neutrophil cytoplasmic antibody (p-ANCA) was positive. Blood study also revealed the evidences of hemolytic anemia and hypoproteinemia. Hepatitis B virus markers, cryoglobulin, anti-ds DNA, anti-Sm, anti-RNP, anti-SS-A, anti-SS-B antibodies were negative. Magnetic resonance imaging of the brainstem was normal. A sural nerve biopsy revealed patchy demyelination of the fascicles. The teasing of nerve fibers showed segmental demyelination. Chest X-ray showed the interstitial pneumonia and pleuritis in the right lower lung. Otological examination revealed the bilateral secretory otitis media. A treatment with high dose prednisolone, ciclosporin and cyclophosphamide was partially effective. However we could not continue these medication because of the induction of liver damage. The patient died of multi-organ failure around a year after the emergence of aphonia and dysphagia. The autopsy specimen of the right vagus nerve showed the similar patchy damage of nerve fibers as was observed in the biopsied sural nerve. The present case was diagnosed as systemic rheumatoid vasculitis. The syndrome of aphonia and dysphagia due to paralysis of the unilateral soft palate and vocal cord is called Avellis syndrome. This syndrome has been reported mainly in relation with the infarction of lateral medulla. The present case shows that Avellis syndrome can be produced by mononeuritis of the vagus nerve.
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PMID:[Avellis syndrome in systemic rheumatoid vasculitis]. 882 3

We reported two cases of silicosis exhibiting MPO-ANCA associated disorder. Case 1 was a 69 year-old man with silicosis and chronic interstitial pneumonia. He was admitted because of fever, dry cough, left chest pain, dyspnea and body weight loss. He was diagnosed as acute exacerbation of interstitial pneumonia, pericarditis and gastrointestinal bleeding. Case 2 was a 67 year-old man with silicosis. He repeated attack of fever, hoarseness, dysphagia and headache. The cell counts of cerebrospinal fluid increased and the thickness of cerebellar tent and left dura mater was observed in the brain MRI. Therefore, he was diagnosed as pachymeningitis and neuropathy of cranial nerves. Both cases were complicated by silicosis and the laboratory findings showed high serum levels of P-ANCA, ANA and rheumatoid factor and inflammatory responses, indicating they were suspected vasculitis. The two cases were treated by steroid and immunosuppressive therapy and had good clinical response. Silicosis may affect multiple organ involvement associated with P-ANCA.
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PMID:[Two cases of silicosis exhibiting MPO-ANCA associated disorder]. 1069 8

A 65-year-old man who was occupationally exposed to asbestos for 40 years was admitted to our hospital with fever and cough. Chest CT revealed paraseptal emphysema, subpleural fibrosis in both lungs, and pleural plaques. On bronchoalveolar lavage fluid contained elevated levels of lymphocytes and neutrophils (15% and 17%, respectively), and asbestos bodies were also found. Because serum MPO-ANCA titer was elevated to 188 EU, we suspected ANCA-associated disease with interstitial pneumonia. Prednisolone was begun at 30 mg/day and his lung opacities partially disappeared. Six weeks later, he complained of headache, dysphagia and hoarseness, and was admitted to the neurology department of the hospital. Under a diagnosis of either hypertrophic pachymeningitis or neuritis due to angiitis of the lower cranial nerves, steroid pulse therapy was performed. Asbestos exposure may have been a contributing factor for ANCA generation in this case. Furthermore, the fact that cranial nerves palsy occurred in spite of steroid therapy may also be important.
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PMID:[Patient with MPO-ANCA-associated disease with interstitial pneumonia and lower cranial nerves palsy who was previously exposed to asbestos]. 2114 Oct 70

We report a 76-year-old male with ANCA-associated hypertrophic pachymeningitis, who presented with crescentic glomerulonephritis. At the initial visit, he had episodic frontal headache and multiple cranial nerve palsy, including double vision, right deafness, hoarseness, and dysphagia. Because proteinuria and hematuria were detected on urinalysis, we performed a kidney biopsy, leading to the diagnosis of crescentic glomerulonephritis. The presence of vascular inflammation in the kidney biopsy led us to consider that this patient may show progression to the systemic type of MPO-ANCA-positive hypertrophic pachymeningitis. This proved useful for prognostic and treatment determination. Based on the results of laboratory tests, imaging studies, and biopsies of the dura mater and kidney, the patient was diagnosed with ANCA-associated hypertrophic pachymeningitis.
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PMID:[A case of MPO-ANCA positive hypertrophic pachymeningitis associated with vascular inflammation in the kidney biopsy]. 2645 70

A 66-year-old woman presented with dysesthesia over the right side of her face, hypoglossal nerve dysfunction, dysphagia, and dysgeusia of the right side. A MRI scan of the brain revealed cerebral dural thickening on the right side of the skull base, and histopathological examination revealed granulomatous inflammation of the dura. Based on paranasal sinusitis, bronchodilatation, laboratory tests showing weakly positive MPO-ANCA, intact renal function, and the patient's favorable response to steroids, we diagnosed the patient with limited granulomatosis with polyangiitis (GPA). Reportedly, autoimmune disease might occur in patients with exacerbation of monoclonal gammopathy of undetermined significance, which was observed in this case. This suggests the utility of immunoelectrophoresis.
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PMID:[MPO-ANCA-associated hypertrophic pachymeningitis with monoclonal gammopathy of undetermined significance: a case report]. 3253 62