Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0085584 (encephalopathy)
 18,178 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

During the 1994-1995 outbreak of dengue fever in New Caledonia (1079 cases), two cases of encephalopathy were seen. Both patients suffered transient clinical (drowsiness, confusion) and electroencephalographic disturbances from which they fully recovered. Although this condition has been described in dengue infection a number of different pathological mechanisms are probably implicated.
 ...
PMID:Brain involvement in Dengue fever. 1114 85

A 55 year old female receiving gemcitabine for stage IV non-small cell carcinoma of the lung developed the clinical-radiologic syndrome of posterior reversible encephalopathy syndrome (PRES). She had clinical manifestations of headaches, increasing somnolence and tonic-clonic seizures. The fluid-attentuated inversion recovery (FLAIR) MR imaging sequence conspicuously showed bihemispheric, symmetrical cortical and subcortical white matter hyperintensities that preponderantly involved the parietal and occipital lobes. Diffusion-weighted imaging (DWI) sequence reflected the preponderant existence of vasogenic edema in the involved areas. MR spectroscopy showed no significant N-acetyl aspartate (NAA) depletion or lactate elevation prospectively, indicating the absence of significant neuronal loss and reversibility of the brain parenchymal changes. The clinical and radiologic manifestations essentially resolved completely with discontinuation of the drug.
 ...
PMID:Gemcitabine-associated posterior reversible encephalopathy syndrome: MR imaging and MR spectroscopy findings. 1129 55

Hyperammonemia associated with inherited disorders of amino acid and organic acid metabolism is usually manifested by irritability, somnolence, vomiting, seizures, and coma. Although the majority of these patients present in the newborn period, they may also present in childhood, adolescence, and adulthood with failure to thrive, persistent vomiting, developmental delay, or behavioral changes. Persistent hyperammonemia, if not treated rapidly, may cause irreversible neuronal damage. After the diagnosis of hyperammonemia is established in an acutely ill patient, certain diagnostic tests should be performed to differentiate between urea cycle defects and other causes of hyperammonemic encephalopathy. In a patient with a presumed inherited metabolic disorder, the aim of therapy should be to normalize blood ammonia levels. Recent experience has provided treatment guidelines that include minimizing endogenous ammonia production and protein catabolism, restricting nitrogen intake, administering substrates of the urea cycle, administering compounds that facilitate the removal of ammonia through alternative pathways, and, in severe cases, dialysis therapy. Initiation of dialysis in the encephalopathic patient with hyperammonemia is indicated if the ammonia blood level is greater than three to four times the upper limit of normal. Hemodialysis is the most effective treatment for rapidly reducing blood ammonia levels. Continuous hemofiltration and peritoneal dialysis are also effective modalities for reducing blood ammonia levels. An improved understanding of the metabolism of ammonia and neurological consequences of hyperammonemia will assist the nephrologist in providing optimal care for this high-risk patient population.
 ...
PMID:Hyperammonemia in urea cycle disorders: role of the nephrologist. 1132 92

We report here a nine-year-old girl with multiple sclerosis having consciousness disturbance at admission. Neurological examination revealed drowsiness, unstable emotion, decreased visual acuity, disturbance of convergence, and clumsy coordination movements. Her cerebrospinal fluid IgG and myelin basic protein were increased. Electroencephalogram showed intermittent, high voltage slow waves predominant in the frontal lobes. Magnetic resonance imaging (MRI) found multiple demyelinating plaques in the brainstem, thalamus, periventricular white matter. The brainstem reticular formation was involved. Since she had had bilateral acute optic neuritis and papillitis two years before the admission, the diagnosis of multiple sclerosis was made. Methylprednisolone pulse therapy improved her neurological symptoms and MRI findings. Multiple sclerosis in children, unlike that in adults, may present with symptoms mimicking an encephalopathy. Our case suggested that consciousness disturbance in childhood multiple sclerosis results from lesions in the brainstem activating reticular formation including the thalamus.
 ...
PMID:[Multiple sclerosis with consciousness disturbance: a case report]. 1139 71

MELAS is characterized by mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes, but cardiac involvement also frequently occurs. An 80-year-old female patient had been suffering from insulin-dependent diabetes mellitus and neurosensory hearing loss. At the age of 79 she suffered metabolic acidosis with persistent drowsiness and was subsequently found to have severe cardiac dysfunction. Muscle biopsy disclosed the presence of abnormal mitochondria, and the MELAS gene mutation (A3243G of the tRNA(Leu(UUR))) was demonstrated. It is noteworthy that this mitochondrial disease patient has survived until a great age, which shows the wide clinical spectrum of MELAS, especially in the age of onset.
 ...
PMID:An 80-year-old mitochondrial disease patient with A3243G tRNA(Leu(UUR)) gene presenting cardiac dysfunction as the main symptom. 1139 11

Acute necrotizing encephalopathy is a relatively new disease. The characteristic clinical findings are of febrile illness followed by rapid deterioration in mental status and seizures. The hallmark of the disease is multifocal bilateral symmetric lesions affecting the thalamus, hypothalamus, brainstem tegmentum, cerebral white matter, and cerebellum. The etiology is unknown, but immune-mediated mechanism was suggested. We present a 12-year-old previously healthy girl who developed increased sleepiness progressing to stupor and coma. Magnetic resonance imaging (MRI) of the brain showed the characteristic findings previously described in acute necrotizing encephalopathy. Her mental status improved dramatically with steroid treatment, and the MRI findings resolved completely within 6 months. Following the acute illness, she developed a complex neuropsychiatric disorder consistent with basal ganglia syndrome.
 ...
PMID:Acute necrotizing encephalopathy presenting as a basal ganglia syndrome. 1141 19

Recurrent episodes of confusion are most commonly due to transient brain dysfunction related to vascular, epileptic or metabolic abnormalities. We report here a 54-year-old patient who, in the months following an extensive surgery of the small intestine, developed several acute confusional episodes characterized by encephalopathic signs (such as behavioral changes, desorientation and somnolence), ataxia, nystagmus and dysarthria. A number of metabolic changes were demonstrated during the episodes, including metabolic acidosis with increased anion gap and an elevated blood level of D-lactate. Symptoms disappeared after treatment with antibiotics. D-lactate encephalopathy is a rare cause of recurrent confusion which could be suspected based on a characteristic association of clinical signs. Diagnosis is confirmed by appropriate dosages. We discussed the possible mechanisms leading to confusional episodes.
 ...
PMID:[Recurrent encephalopathy after small bowel resection. Implication of D-lactate]. 1145 87

A case of a patient with astrocytoma who showed somnolence and asterixis one month after tumour resection is presented. Although primidone had been prescribed preoperatively for five years and the same dose was maintained after the operation, the serum concentration of the primidone metabolite phenobarbital was elevated and she demonstrated hyperammonemic encephalopathy, which disappeared on withdrawal of the drug. A description of this seldom reported phenomenon during primidone therapy is given, with reference to valproate cases.
 ...
PMID:Primidone-induced hyperammonemic encephalopathy in a patient with cerebral astrocytoma. 1174 25

The nervous system is the major target of the toxic effect of manganese (Mn) and its compounds. Nowadays, neurological diagnostics is directed towards early detection of symptoms and abortive forms, and the cases of serious damage of the nervous system are no longer reported. The aim of the present study was to assess the effects of manganese on the functions of the nervous system in workers exposed to this metal in the ship and electrical industries. The study covered a selected group of 75 male workers (mean age 39.17 yr +/- 9.79; range 20-56 yr), including 62 welders and fitters, as well as 13 workers involved in the battery production. Their employment duration ranged between 1 and 41 yr (mean 17.5 yr +/- 10.81). During the welding process the air Mn concentrations varied from 0.004 to 2.67 mg/m3 (arithmetic mean, 0.399 mg/m3; geometric mean, 0.154 mg/m3; standard deviation, 0.586). Of the 62 workers, 30 worked in the area with exceeding MAC value of 0.3 mg/m3. At the battery production workposts, Mn concentrations fell within 0.086-1.164 mg/m3 (arithmetic mean, 0.338 mg/m3, geometric mean, 0.261 mg/m3; standard deviation, 0.292). The values of current Mn exposure in the study group fell within the range below 0.01 and 2.67 mg/m3 (arithmetic mean, 0.4 mg/m; geometric mean, 0.15 mg/m3). Of the 13 subjects, 6 worked at the Mn air concentration exceeding MAC values. In the exposed group, the values of cumulated exposure index ranged from 0.008 to 35.52 (arithmetic mean, 8.045; geometric mean, 4.615; standard deviation, 6.562). The control group consisted of 62 men non-occupationally exposed to Mn, matched by sex, age and work shift distribution. Clinically, the increased emotional irritability, dysmnesia, concentration difficulties, sleepiness and limb paresthesia predominated among the disorders of the nervous system functions in workers chronically exposed to manganese. Neither in the central nor in the peripheral nervous system, the objective examinations revealed organic lesions that could provide grounds for diagnosing toxic encephalopathy or polyneuropathy. Generalized and paroxysmal changes were the most common recordings in the abnormal electroencephalography. Visual evoked potentials examinations showed abnormalities in the response evoked, which could be a signal of the optic neuron disorders and their significant relationship with cumulated exposure. The results of the study demonstrate that Mn exposure within the range of <0.01-2.67 mg/m3 (arithmetic mean, 0.4 mg/m3; geometric mean, 0.15 mg/m3) induces subclinical effects on the nervous system.
 ...
PMID:Neurological and neurophysiological examinations of workers occupationally exposed to manganese. 1188 16

The portal-systemic venous shunt is uncommon in patients without portal hypertension. We present two cases of portal-systemic encephalopathy due to extrahepatic shunt without liver cirrhosis and portal hypertension. Two women in their seventies were admitted to our hospital because of recurrent episodes of altered sensorium, drowsiness, slurred speech, disorientation, asterexis and high blood ammonia levels. There was no history of abdominal surgery or abdominal trauma. Clinical examination revealed no signs of portal hypertension or stigmata of chronic liver diseases. Brain CT and MRI scanning were unremarkable except for a high intensity signal in the basal ganglia on T1 weighted MRI images. Laboratory tests were almost normal except for the hyperammonemia occurring on several occasions. There was no evidence of liver cirrhosis by imaging. However, color Doppler showed an extra-hepatic shunt in both patients and pulsed Doppler showed decreased velocity and volume of the portal venous flow. These sonographic findings were confirmed during percutaneous transhepatic portography (PTP). Portal pressures measured during PTP were 9 and 11 mmHg. Needle biopsy ruled out idiopathic portal hypertension and liver cirrhosis. The diagnosis was portal systemic encephalopathy due to extra-hepatic portosystemic venous shunting. Both patients were treated by embolization of the shunting vessel with metallic coils.
 ...
PMID:Portal-systemic encephalopathy in two patients without liver cirrhosis and portal hypertension. 1204 66


<< Previous 1 2 3 4 5 6 7 8 9 10 Next >>