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)

We present a case of malignant neuroleptic syndrome in a 55 years old male diagnosed 3 years ago of alcoholic paranoid psychosis who was chronically treated with haloperidol, clothiapine, and phenobarbital. Twenty one days after neuroleptic drug withdrawal the patient was admitted to the recovery room because of hyperthermia (40.2 degrees C), left basal pneumonia, acute respiratory insufficiency, extrapyramidal rigidity, mutism, dysarthria, deep coma, hypotension, and tachycardia. Two days after he presented massive rhabdomyolysis, atrial flutter with hemodynamic deterioration which reverted to sinus rhythm and acute anterolateral and inferior myocardial infarction documented by enzyme rise and electrocardiographic alterations. Rhabdomyolysis and myocardial infarction were the precipitating factors of the renal insufficiency. A malignant neuroleptic syndrome was suspected and intravenous treatment with dantrolene sodium 1.5 mg/kg every 24 hours was initiated. Bromocriptine was not administered. The patient died 14 days after in the course of a sepsis and cardiogenic shock.
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PMID:[Malignant neuroleptic syndrome associated with myocardial infarction, acute renal insufficiency and rhabdomyolysis]. 168 57

A patient development deteriorating mental status, quadriparesis, and severe pseudobulbar palsy with the inability to speak or swallow following orthotopic liver transplantation (OLT). Subsequently, abnormalities were found in the pons on MRI that were consistent with central pontine myelinolysis (CPM). Marked recovery occurred following transfer to the rehabilitation medicine service. Seven months following development of CPM, a mild dysarthria has persisted, but full ambulation has returned. Although no significant fluctuations in serum sodium were seen perioperatively, multiple risk factors associated with the development of CPM were present, including end-stage liver disease, a history of alcohol abuse, malnutrition, hypoxia, and use of cyclosporin medication postoperatively. This case demonstrates that the development of CPM may occur following OLT despite meticulous attention to serum sodium concentrations. We conclude that CPM is multifactorial in nature. There can be a great variation in its clinical course.
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PMID:Central pontine myelinolysis after liver transplantation: a case report. 757 24

Topiramate is a new antiepileptic drug, which is not yet marketed in Germany. The anticonvulsive activity is probably mediated by sodium-channel blockade combined with gabaergic and weak antiglutamatergic properties. We investigated 23 patients with partial seizures in this open prospective study. The efficacy was analysed under stable concurrent antiepileptic drugs. Two of the patients became seizure free. Ten patients had a reduction of seizure frequency of at least 50%. The responder rate was 57%. The maximum daily dosages were 400 to 850 mg. Side-effects without relationship to dose were nervousness and aggression. In two patients an psychotic episode occurred. Dose-related side-effects were ataxia, dizziness, somnolence and dysarthria. This study is underlining that topiramate is efficacious in the treatment of partial onset seizures but also associated with a narrow therapeutic width.
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PMID:[Topiramate--an effective new anticonvulsant. An open prospective study]. 944 Dec 57

Continuous spikes and waves during slow sleep (CSWS) is a syndrome with a serious prognosis due to the frequent association with neuropsychological dysfunction. It is mandatory to consider this syndrome if an epileptic child suffers from behavioral changes, dysarthria or learning difficulties. We report on two patients with CSWS syndrome with focal abnormalities on the nondominant hemisphere and a proportion of generalized spike-waves discharges in more than 85% of their NREM sleep on the EEG. Both had a good response to the treatment with sodium valproate and ethosuximide at high doses. Both suffered a relapse of their clinical and EEG semiology after withdrawal of their treatment. After restarting treatment they became clinically normal with a normal sleep EEG recording. We propose the association of sodium valproate and ethosuximide for CSWS; this treatment should be maintained until adolescence.
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PMID:[The efficacy of the valproic acid-ethosuximide combination in the continuous slow point wave syndrome during sleep]. 947 Nov 63

A case of rhabdomyolysis after a possible viral infection and the use of a cold medication is reported. A 41-year-old man who presented with dysarthria, dysphagia, progressive weakness of his muscles and a high grade fever was admitted. He suffered from massive rhabdomyolysis, acute renal failure, and bronchopneumonia. Hemodialysis, antibiotics, and hydration therapy were effective in the treatment of his illness. Although the cause of the rhabdomyolysis was not completely clear, he was subsequently shown to be susceptible to malignant hyperthermia (MH) based on the results of a caffeine-halothane contracture test. When a mild recurrence occurred during a follow-up muscle biopsy, intravenous dantrolene sodium was administered and he improved immediately. This case suggests that MH should be considered in patients with rhabdomyolysis when the cause is unclear. The caffeine-halothane contracture test may also be helpful in the diagnosis.
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PMID:Rhabdomyolysis after infection and taking a cold medicine in a patient who was susceptible to malignant hyperthermia. 955 May 99

A 65-year-old man who had muscle weakness and dysarthria was admitted for investigation of motor neuron disease. He had lost 12 kg of weight in 6 months. Neurological findings disclosed upper and lower motor neuron disturbances with normal sensory nerve function, and needle electromyography showed a neurogenic pattern. Laboratory findings on admission demonstrated dilutional hyponatraemia due to an excessive secretion of antidiuretic hormone (ADH). Based on these findings, the patient was diagnosed as having the syndrome of inappropriate secretion of antidiuretic hormone (SIADH) associated with amyotrophic lateral sclerosis (ALS). During the night of first hospital day, the patient complained of severe dyspnoea, and mechanical ventilation was commenced. Following the mechanical ventilation, plasma ADH levels and serum sodium concentration were normalized. We propose that respiratory failure secondary to the atrophy of respiratory muscle might be responsible for the development of SIADH.
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PMID:Syndrome of inappropriate secretion of antidiuretic hormone associated with amyotrophic lateral sclerosis in respiratory failure. 1038 39

We report an autopsy-confirmed case of amyotrophic lateral sclerosis (ALS) accompanied by syndrome of inappropriate secretion of antidiuretic hormone (SIADH). A 67-year-old man was admitted to our hospital with muscle weakness, dysarthria and dysphagia. During hospitalization, respiratory insufficiency was ingravescent, and hyponatremia, hypo-osmolarity, elevated osmotic pressure of urine, and elevated urinary sodium excretion were noted. Based on these findings we diagnosed ALS with SIADH, and treatment with infusion of concentrated NaCl was started. However, the patient died of respiratory failure on day 50. We assumed that severely restrictive ventilatory impairment was the cause of SIADH in this case.
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PMID:An autopsy case of amyotrophic lateral sclerosis accompanied by syndrome of inappropriate secretion of antidiuretic hormone. 1205 91

Seizures are an uncommon but serious complication of hyponatremia which can lead to permanent brain damage and even death. It is recommended that patients with hyponatremic-induced seizures be treated with 3% hypertonic saline, however, a rapid rate of correction may result in central pontine myelinolysis (CPM), a severe neurological disorder characterized by mutism, dysarthria, spastic quadriparesis, and pseudobulbar palsy. The patient in this case developed a hyponatremia-induced generalized tonic-clonic seizure which was aborted by rapid therapy with diazepam, followed by hypertonic saline and phenytoin. Subsequent replacement of hypertonic saline with normal saline and salt tabs in combination with phenytoin allowed gradual correction of serum sodium without any subsequent seizures or neurological complications.
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PMID:Therapy with hypertonic saline in combination with anti-convulsants for hyponatremia-induced seizure: a case report and review of the literature. 1263 26

Familial hemiplegic migraine (FHM) is an autosomal dominant subtype of migraine with hemiparesis during the aura. In over 50% of cases the causative gene is CACNA1A (FHM1), which in some cases produces a phenotype with cerebellar signs, including ataxia and nystagmus. Recently, mutations in ATP1A2 on chromosome 1q23 encoding a Na+/K+ -ATPase subunit were identified in four families (FHM2). We now describe an FHM2 pedigree with a fifth ATP1A2 mutation coding for a G301R substitution. The phenotype was particularly severe and included hemiplegic migraine, seizure, prolonged coma, elevated temperature, sensory deficit, and transient or permanent cerebellar signs, such as ataxia, nystagmus, and dysarthria. A mild crossed cerebellar diaschisis during an attack further supported the clinical evidence of a cerebellar deficit. This is the first report suggesting cerebellar involvement in FHM2. A possible role for CACNA1A in producing the phenotype in this family was excluded by linkage studies to the FHM1 locus. The study of this family suggests that the absence of cerebellar signs may not be a reliable indicator to clinically differentiate FHM2 from FHM1.
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PMID:A G301R Na+/K+ -ATPase mutation causes familial hemiplegic migraine type 2 with cerebellar signs. 1545 25

Central pontine myelinolysis (CPM) is a demyelinating disease of the pons often associated with the demyelination of extrapontine areas of the central nervous system. Although the etiology and pathogenesis are unclear, CPM is usually associated with hyponatremia or its rapid correction, and chronic alcoholism is also a common underlying condition. We observed a 43-year-old man with diabetes mellitus who developed central pontine and extrapontine myelinolysis with no apparent evidence of hyponatremia, serum hyperosmolality or associated rapid correction, or history of alcohol abuse. On admission, the patient was lethargic with dysarthria, dysphagia, and mild tetraparesis and his face and lower extremities were severely edematous. Laboratory examination showed normoglycemia and normonatremia, although hypokalemia, elevated HbA1c, and nephrotic syndrome were also present. Magnetic resonance imaging (MRI) revealed abnormal signal intensity in the pons, the deep layers of the cerebral cortex, and the adjacent white matter consistent with central pontine and extrapontine myelinolysis. Generalized edema was reduced by the use of diuretics and extracorporeal ultrafiltration without significant changes of serum sodium or osmolality. His consciousness level and paresis gradually improved within a few weeks. Our patient is a rare case of CPM associated with diabetes without apparent evidence of sodium or glucose imbalances.
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PMID:Central pontine and extrapontine myelinolysis associated with type 2 diabetic patient with hypokalemia. 1581 68


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