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)

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

We described a 12-year-old girl with systemic lupus erythematosus (SLE) associated with myasthenia gravis (MG). She had absence seizures from 6 years old. She admitted to our hospital at 12 years of age because of absence seizures and dyspnea. The diagnosis of SLE was made on the basis of convulsion, arthritis, pleurisy, and positive antinuclear factor and was started therapy with prednisolone. The clinical course was complicated by the appearance of dysphagia and hoarseness. On the basis of positive Tensilon test and a high level of serum anti-acetylcholine receptor antibody, we made a diagnosis of the systemic type of MG. Her condition was improved by methylprednisolone pulse therapy and gamma-globulin therapy after plasmapheresis. The association of early-onset SLE with MG is rare.
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PMID:[A childhood case of systemic lupus erythematosus associated with myasthenia gravis]. 929 15

A 20-year-old man with aplastic anemia developed myasthenia gravis (MG) 7 months after bone marrow transplantation (BMT) from an HLA one locus-mismatched sister. Proximal muscle weakness (predominant in the lower limbs) and dysphagia occurred without any other sign of graft-versus-host disease (GVHD), 1 month after cessation of immunosuppression with cyclosporine. The diagnosis of MG was based on clinical symptoms and on neurophysiologic investigations showing a significant increase of the Jitter in single-fiber electromyography and a significant decremental response during repetitive stimulation at slow rates, but antibodies against the acetylcholine receptor (AchRab) were negative. All clinical and neurophysiological signs normalized within 1 month of treatment with low-dose prednisolone and pyridostigmine, and the patient is perfectly well 1 year after cessation of all therapy. All cases of BMT-associated MG previously published are reviewed in comparison with ours. The originality of this new observation is that this case is the only one not associated with chronic GVHD and negative for AchRab. Alternatively, MG may have been the sole manifestation of chronic GVHD in this patient.
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PMID:Myasthenia gravis without chronic GVHD after allogeneic bone marrow transplantation. 970 30

A 61-year-old woman in excellent health and taking no medications presented with progressive dyspnea on exertion. She did not exhibit diplopia, dysphagia, dysarthria or muscle weakness. Her condition deteriorated, and respiratory distress developed. The patient was intubated and placed on mechanical ventilation. A computed tomogram of her chest revealed a moderately enlarged thymus gland unsuspected on a plain x-ray film. Myasthenia gravis was diagnosed on the basis of a high acetylcholine receptor antibody titer. Treatment started with prednisolone, anticholinesterase agent, and plasma exchange. The patient underwent a thymectomy 3 weeks after her diagnosis. Her symptoms were brought under control by anticholinesterase agent and prednisolone. This case illustrates the need to consider myasthenia gravis as well as other motor-neuron disorders when evaluating individuals presenting acute respiratory failure of unknown origin.
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PMID:[Myasthenia gravis presenting as isolated respiratory failure]. 989 33

We report the case of a 53-year-old female patient, who developed bilateral blepharoptosis, limb weakness, dysphagia, and dyspnea several days after human natural interferon-beta (IFN-beta) treatment for chronic active hepatitis C. A positive edrophonium test, an elevated anti-acetylcholine receptor antibody titer, and decrements in the amplitude of muscle action potentials evoked by repetitive stimulation confirmed the diagnosis of myasthenia gravis (MG). Since she had been suffering from drooping of her right eyelid, fluctuating diplopia and easy fatiguability of limbs before receiving IFN-beta, her symptoms of MG were considered to be exacerbated by IFN-beta. It is recommended that IFN-beta should be used with particular care in patients with known MG or its compatible symptoms.
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PMID:Exacerbation of myasthenia gravis in a patient after interferon-beta treatment for chronic active hepatitis C. 1045 Aug 5

A 68-year-old man was hospitalized on March 4, 1998 for disturbances in consciousness. In 1995, he had received proximal subtotal gastrectomy and reconstructive surgery of the jejunal interposition for gastric cancer. Thereafter he had been taking enough food without the habit of taking liquor. In October 1997, his short term memory was becoming gradually worse. On February 12, 1998, he suffered from numbness in the feet, and then dysphagia, unsteady gait, and diplopia developed gradually. On February 26, brain MRI showed no abnormalities. On March 3, he had a fever of 38.5 degrees C and his consciousness became unclear. Neurological examination revealed semi-coma, total ophthalmoplegia, and absence of doll's eye movement. Deep tendon reflexes were absent. The serum thiamine level was 9 ng/ml (normal range: 20-50). Brain MRI demonstrated symmetrical high intensity lesions in the periaqueductal area of the midbrain, dorsomedial nuclei of bilateral thalami, and vestibular nuclei. About 30 seconds after intravenous infusion of thiamine, his consciousness improved dramatically, but returned to semi-coma after about two minutes. Wernicke-Korsakoff syndrome usually occurs acutely. In the present case, however, the disease showed slow onset, chronic progression, and then rapid worsening after fever. Reconstructive surgery of the jejunal interposition might have caused the slow onset of Wernicke-Korsakoff syndrome, and fever might have facilitated the rapid progression of the disease. An immediate high concentration of thiamine modifies the kinetics of acetylcholine receptor ion channels, thereby maintaining wakefulness, and the level of consciousness may change dramatically.
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PMID:[A case of Wernicke-Korsakoff syndrome with dramatic improvement in consciousness immediately after intravenous infusion of thiamine]. 1068 93

Tacrolimus, a potent immunosuppressive agent, has recently become available for the treatment of myasthenia gravis (MG). However, few reports have evaluated the usefulness of tacrolimus in the elderly and in intractable MG classified as Osserman's grade IIb or higher. In this study, we examined the effects of tacrolimus in two patients with postthymectomy Osserman's grade III and IIb MG. The effects of tacrolimus were evaluated using the modified quantitative MG score (QMG score) and anti-acetylcholine receptor antibody (AChRAb) titer. Patient 1 was a 39-year-old woman with Osserman grade III MG. Her MG score improved after tacrolimus administration (3 mg/day), allowing gradual decreases in the doses of prednisolone and pyridostigmine because of improvement in MG symptoms. The AChRAb titer also decreased from 100.0 to 25.7 nmol/l during tacrolimus treatment. The concentrations of tacrolimus in whole blood ranged from 5.5 to 8.2 ng/ml during treatment. Patient 2 was a 68-year-old man diagnosed with Osserman grade IIb MG. After the initiation of tacrolimus treatment, dysphagia improved dramatically, and he was able to swallow food without a nasal feeding tube. The AChRAb titer decreased from 42.4 to 23.4 nmol/l during the treatment period. The concentration of tacrolimus in whole blood was 7.0 ng/ml 6 days after the initiation of treatment. These findings suggest that tacrolimus is effective in intractable and elderly MG patients.
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PMID:[Successful treatment by tacrolimus in two patients with Osserman's grade III intractable myasthenia gravis and elderly IIb]. 1506 88

An 83-year-old woman was admitted to our hospital complaining of respiratory distress. She developed blepharoptosis and dysphagia two months previously. On admission, blood pressure was 150/84 mmHg. There were bilateral blepharoptosis and bulbar palsy. Tendon reflexes were brisk, but pathological reflexes were absent. Repetitive stimulation test demonstrated decrement. Tensilon test was positive. Thus, a diagnosis of myasthenic crisis was made. Vital capacity was 0.71, which necessitated intubation and artificial ventilation. During the first course of immunoadsorption plasmapheresis, blood pressure fell to 80/50 mmHg. ECG showed deeply inverted T waves in leads V2 through V6. Echocardiographic examination demonstrated extensive akinesis around the apex. CK was slightly increased. A diagnosis of "Takotsubo"-shaped cardiomyopathy was made. To maintain blood pressure, dopamine was continuously injected. Left ventricular function returned to normal within a week. No thymoma was seen on the chest CT scan. She was treated with pyridostigmine and tacrolimus. Ten weeks after admission, she was weaned off the ventilator and she had no dysphagia anymore. Anti-acetylcholine receptor antibody titers had decreased. In Japan, approximately 17% of patients with myasthenia gravis are in the seventh decade or older. Approximately 10% of the patients of this age group suffer from severe generalized form. Careful ECG monitoring is necessary while treating elderly patients with myasthenic crisis, because they are at potential risk of developing "Takotsubo"-shaped cardiomyopathy.
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PMID:[A case of transient left ventricular ballooning ("Takotsubo"-shaped cardiomyopathy) developed during plasmapheresis for treatment of myasthenic crisis]. 1523 76

There have been reported cases of children with histories of pauciarticular juvenile chronic arthritis (JCA) later developing myasthenia gravis (MG) as young adults. This is intriguing because it had been considered rare to diagnose a second autoimmune disease in a patient with pauciarticular JCA, unlike in those with adult-onset rheumatoid arthritis. We report a case of MG in a 20-year-old woman with a history of pauciarticular JCA. She presented with bilateral ptosis, weakness, and a history of dysphagia. The diagnosis was confirmed with positive serum acetylcholine receptor antibodies (2000 nm/L) and electromyography showing a decremental response to repetitive muscle stimulation. The patient's inflammatory arthritis was quiescent at diagnosis. The patient underwent a surgical thymectomy and was treated with pyridostigmine, intravenous immunoglobulin, and corticosteroids with a fluctuating clinical course. Previous cases have been reported of MG associated with this subtype of JCA, suggesting a connection in autoimmune pathology. The earlier recognition and management of MG in a patient with pauciarticular JCA presenting with weakness may improve the prognosis of this disease.
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PMID:Myasthenia gravis in a patient with pauciarticular juvenile chronic arthritis. 1704 84

A 54-year-old woman was admitted to our hospital because of diplopia, dysphagia, dropped head, and muscle weakness with easy fatigability. A neurological examination showed bilateral ptosis, ocular motility disorder, dysphagia, and weakness of the neck extensor muscles. Edrophonium and repetitive nerve stimulation tests of the thenar muscles showed positive results. The serum titer of anti-acetylcholine receptor antibody was negative. A thymoma was not detected in her chest CT. Finally, she was diagnosed with anti-MuSK antibody-positive myasthenia gravis based on the high serum titer of anti-MuSK antibody (239 nmol/l). Her symptoms improved after administration of prednisolone. However, the symptoms were aggravated when the prednisolone dosage was reduced, and the titer of anti-MuSK antibody rose at the same time. We evaluated the possible association between changes in the severity of her clinical symptoms and the titer of the antibody during prednisolone therapy. It was revealed that the titer of the antibody was correlated to the severity of clinical symptoms expressed by a QMG (Quantitative Myasthenia Gravis) score. These findings indicate that monitoring the titer of anti-MuSK antibody can be useful for assessing disease activity as well as decision making during treatment.
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PMID:[Titer of anti-muscle-specific receptor tyrosine kinase (MuSK) antibody correlated with symptomatic improvement in response to corticosteroid therapy in a patient with anti-MuSK antibody-positive myasthenia gravis: a case report]. 1763 10


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