Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0013362 (dysarthria)
 3,768 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The term sudden hearing loss refers to hearing losses of sensorineural origin that evolve over a short period of time and are often of unknown origin. Common causes include damage to the cochleovestibular nerve by viral infection, autoimmune disease, vascular insult, and labyrinthine membrane rupture. A 70-year-old man had a history of recent diplopia, dysarthria, syncopal episodes, dysequilibrium, and the sudden onset of deafness in his right ear. Angiography demonstrated severe ulcerative stenosis of the right internal carotid origin and an anomalous vessel (probable remnant of hypoglossal artery) originating from the distal right internal carotid artery that perfused the entire distal vertebral and basilar artery circulation. Arch angiograms confirmed the absence of a proximal right vertebral artery and revealed a small left vertebral artery that ended in the cervical region without reaching the posterior fossa. Standard right carotid endarterectomy with patch angioplasty resolved all neurologic symptoms except for persistent unilateral deafness at 9 months' follow-up. Presumed embolization through this anomalous vessel to the internal auditory artery and subsequent cochlear and vestibular branches represents the first reported case of sudden hearing loss as a result of anterior circulatory ulcerative disease.
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PMID:Unilateral sudden hearing loss as a result of anomalous carotid anatomy. 239 91

Adult-onset myasthenia gravis is an acquired autoimmune disorder of neuromuscular transmission in which acetylcholine receptor antibodies attack the postsynaptic membrane of the neuromuscular junction. Although the cause of this disease is unknown, the role of immune responses in its pathogenesis is well established. Circulating acetylcholine receptor antibodies are present in 80% to 90% of patients with the generalized form of myasthenia gravis. Most patients have ptosis, diplopia, dysarthria and dysphagia. The weakness and fatigue worsen on exertion and improve with rest. Respiratory muscle and limb weakness are rare at the onset of the disease. For the past two decades, there has been considerable progress in understanding the diagnosis and management of myasthenia gravis. The diagnosis is based on clinical presentation, neurologic examination, and confirmation by means of electrophysiologic testing and immunologic studies. Myasthenia gravis mimics many neuromuscular diseases and even illnesses such as depression and chronic fatigue syndrome. One should always exclude drug-induced myasthenia gravis for all patients. With the introduction of new modalities of treatment, particularly immunosuppressive or immunomodulating drugs, plasma exchange and thymectomy, the morbidity and mortality of myasthenia gravis have decreased dramatically to the point that myasthenia gravis should not be considered as serious a disease as it once was. Although the several therapeutic options are usually effective and have meant independence in daily life to many patients with myasthenia gravis, well-designed, controlled, prospective studies are still lacking.
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PMID:Myasthenia gravis. 911 87

Myasthenia gravis is an autoimmune disease resulting from the production of antibodies against the ACh receptors of the neuromuscular synapse. The thymus gland is involved in the autosensitization process, and the disease frequently is associated with thymic morphologic abnormalities. There is a consensus that all adults with generalized MG should have a thymectomy. This recommendation has been propagated by the safety of the procedure and excellent outcome. Removal of as much thymic tissue as possible (anterior mediastinal exenteration) by transsternal approach is the logical goal of thymectomy in the treatment of MG. Transcervical approach and VATS, however, are less invasive and have been used in patients who have MG unaccompanied by thymoma. Optimization of the condition of the myasthenic patients can markedly decrease the risk of surgery and improve the outcome. Two techniques have been recommended for general anesthesia in the myasthenic patient. Because of the unpredictable response to succinylcholine and the marked sensitivity to nondepolarizing muscle relaxants, some anesthesiologists avoid muscle relaxants and depend on deep inhalational anesthesia, such as halothane, isoflurane, or sevoflurane, for tracheal intubation and maintenance of anesthesia. Others, however, use a balanced technique of anesthesia that includes the use of carefully titrated muscle relaxants. The most important preoperative factor predicting the need for postoperative mechanical ventilation is the severity of bulbar involvement (Ossermann group 3 and 4), usually indicated by significant dysphagia and dysarthria associated with borderline respiratory dysfunction. Thymectomy benefits nearly 96% of patients: 46% develop complete remission, 50% are asymptomatic or improve on therapy, and 4% remain the same. The time from diagnosis to surgery is shorter than 8 months, and mild or moderate myasthenic symptoms are the main prognostic factors that predict the best outcome after thymectomy.
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PMID:Anesthesia and critical care of thymectomy for myasthenia gravis. 1141 60

A patient with Schmidt's syndrome and atypical symptoms of Addison's disease is presented. Autoimmune thyroiditis was diagnosed at the age of 12 years and then at 15 years; a diagnosis of migraine was made following 10 days of headache, visual field defects and dysarthria. One week later the patient was admitted to hospital in critical condition. Addison's disease was diagnosed and replacement therapy was introduced which brought about a rapid clinical improvement. Positive adrenal autoantibodies confirmed the diagnosis. We suggest that patients with organ-specific autoimmune disease undergo annual screening for adrenal activity, and in the event of abnormal results, adrenal autoantibody evaluation should be carried out, to avoid a life-threatening crisis caused by Addison's disease, which is often difficult to recognize due to atypical clinical presentation, as in the patient reported here.
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PMID:Unusual presentation of Addison's disease in Schmidt's syndrome. 1288 Jan 29

A 78 year-old woman was admitted to our hospital because of subacutely progressive dysarthria, dysphagia, proximally dominant muscle weakness and erythema in the neck and back. She was diagnosed as having rheumatoid arthritis (RA) at the age of 60 and treated with bucillamine (BUC) for 8 years. Laboratory tests included a rheumatoid factor of 1,472U/ml. Serum creatine kinase level was slightly elevated. The activated T cells in the peripheral blood were markedly increased. Needle EMG demonstrated myogenic changes. The magnetic resonance image of the left upper arm showed diffuse muscle atrophy and inflammatory changes in the triceps muscle. The muscle biopsy revealed perivascular inflammatory cell infiltraton and type II fiber atrophy. A biopsy from the skin showed mild perivascular inflammatory cell infiltraton. According to the results of these findings, she was thought to have dermatomyositis due to BUC. After withdrawal of BUC followed by the administration of prednisolone 1mg/kg, her symptoms improved and activated T cells in the peripheral blood were decreased. In Japan, BUC is widely accepted as an effective drug in the treatment of RA, even though it is known to induce some autoimmune diseases. However, the mechanism of the development of autoimmune disease is unclear. We considered that the long-term use of bucillamine could trigger an autoimmune response such as an increase in activated T cells and the development of dermatomyositis-like clinical features in our patient. In conclusion, when RA patients treated with BUC show a clinical picture compatible with dermatomyositis, its causative relationship has to be considered.
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PMID:[Bucillamine-induced dermatomyositis-like clinical features in a patient with rheumatoid arthritis]. 1571 1

Paraneoplastic cerebellar degeneration (PCD) is an autoimmune disease that can be associated with cancer of the breast, lung, and ovary. The clinical presentation of PCD commonly includes ataxia, visual disturbances, and dysarthria. The speech disturbances associated with PCD have not been well characterized, despite general acceptance that dysarthria is often part of the initial presentation. A retrospective study was conducted of the speech, language, and swallowing concerns of patients with PCD evaluated at the Mayo Clinic in Rochester, MN, between 1990 and 2001. Prospective speech and language assessments were then conducted with 5 patients who had PCD. While ataxic dysarthria was the most common speech diagnosis, a spastic component was recognized frequently enough to suggest that the subacute (days to weeks) emergence and progression of an ataxic or mixed ataxic-spastic dysarthria in the setting of a more diffuse cerebellar ataxia should raise suspicions about PCD and justify further investigation of a possible immune-related etiology.
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PMID:Speech and language findings associated with paraneoplastic cerebellar degeneration. 1622 71

Thrombotic thrombocytopenic purpura (TTP) is an autoimmune disorder characterised by thrombocytopenia, haemolytic anemia, fluctuating neurological deficits, fever, and renal impairment. This case report is about a young man who presented with acute onset right sided paralysis, dysarthria, and central facial paralysis, suggestive of cerebrovascular accident, but eventually diagnosed as TTP. In addition, the clinical presentation of TTP is discussed and some teaching points for the emergency physicians are emphasised.
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PMID:Thrombotic thrombocytopenic purpura mimicking acute ischemic stroke. 1692 Oct 72

Myasthenia Gravis is an autoimmune disorder that generally presents with ocular symptoms, specially diplopia and ptosis. Dysphagia may be a manifestation of the generalised type of the disease, but rarely is its presenting feature. The authors describe a case of Myasthenia Gravis in an old patient complaining of dysphagia and dysarthria. Because Myasthenia Gravis is a potentially serious but treatable disease, we emphasize the need to consider it in the differential diagnosis of dysphagia, namely in the elderly. In fact, the disorder is probably underdiagnosed in this population group, because clinicians tend to accept other more frequent diagnosis to explain these symptoms.
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PMID:[Dysphagia and dysarthria: unusual presentation of Myasthenia Gravis]. 1828 45

Glycogen storage disease type Ib (GSD Ib, OMIM 232220) is an inborn disorder of glucose metabolism, caused by mutations in the G6PT gene, encoding a glucose 6-phosphate transporter (G6PT). GSD Ib is mainly associated with fasting hypoglycaemia and hepatomegaly. Most GSD Ib patients also show neutropenia and neutrophil dysfunction and therefore are at risk of developing severe infections and inflammatory bowel disease (IBD). An increased risk for autoimmune disorders, such as thyroid autoimmunity and Crohn-like disease, has also been demonstrated, but no systematic study on the prevalence of autoimmune disorders in GSD Ib patients has ever been performed. We describe a 25-year-old patient affected by GSD Ib who developed 'seronegative' myasthenia gravis (MG), presenting with bilateral eyelid ptosis, diplopia, dysarthria, severe dysphagia, dyspnoea and fatigue. The repetitive stimulation of peripheral nerves test showed signs of exhaustion of neuromuscular transmission, particularly evident in the cranial area. Even in the absence of identifiable anti-acetylcholine receptor antibodies, seronegative MG is considered an autoimmune disorder and may be related to the disturbed immune function observed in GSD Ib patients.
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PMID:Myasthenia gravis in a patient affected by glycogen storage disease type Ib: a further manifestation of an increased risk for autoimmune disorders? 1843 26

Myasthenia gravis is a rare, chronic, autoimmune disorder characterized by postsynaptic dysfunction at the neuromuscular junction. The disease affects more females than males. We describe the case of a 17-year-old female adolescent with recurrent episodes of dysarthria and dysphagia and a history of aspiration pneumonia. A bedside edrophonium (Tensilon) test in our emergency department confirmed the diagnosis of myasthenia gravis.
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PMID:Myasthenia gravis in an adolescent patient presenting to the pediatric emergency department. 2045 93


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