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Query: UMLS:C0085584 (encephalopathy)
18,178 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Following aneurysmal subarachnoid hemorrhage (SAH) many patients complain of and exhibit disturbances of a cognitive nature. The etiology behind this encephalopathy is less well understood. Disturbed CSF-hydrodynamics and/or spasm in the lentriculostriate and thalamoperforating vessels might be responsible for such disturbances. This study comprised 56 patients subjected to early aneurysm operation, of whom 21 received intravenous nimodipine during the critical phase for SAH-induced vasoconstriction. There was no difference in cognitive disturbances measured with a specifically conceived battery of psychometric test instruments 2 years or more after SAH and surgery.
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PMID:Does intravenous nimodipine treatment influence cognition in patients subjected to early aneurysm operation? 335 11

Morphological study of the choroid plexuses of two patients with mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) revealed an enormously increased number of mitochondria with structural abnormalities in almost all of the choroidal epithelial cells. The majority of the cells also showed loss of microvilli and collapsed or attenuated apical cytoplasmic processes with increased electron density and lysosome-like dense bodies. The blood vessels of the choroid plexus showed the features of mitochondrial angiopathy previously described in the pial arteries of the same patients. These findings are interpreted as the morphological expression of a primary biochemical defect of the mitochondrial function in the choroid plexus, and as the probable explanation for increased CSF lactate and pyruvate levels in this disease.
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PMID:Involvement of choroid plexus in mitochondrial encephalomyopathy (MELAS). 343 8

The clinical diagnosis of granulomatous angiitis can be extremely difficult because of the diversity of presentation. A clinical presentation of progressive multifocal encephalopathy is most common but signs suggesting isolated mass lesions or dementia may be seen. Laboratory studies are helpful, particularly when CSF examination reveals elevated protein or a mononuclear cell response. Cerebral angiogram may demonstrate vasculitis but tissue biopsy is extremely important in firmly establishing a diagnosis. Leptomeningeal biopsy should be performed before initiation of appropriate immunosuppressive therapy and before major irreversible loss of neurologic function occurs. Corticosteroid therapy is the initial treatment of choice, although recent anecdotal reports suggest additional benefits of immunosuppressive drugs such as cytoxan in refractory cases. Whether immunosuppressive drugs should be added routinely cannot be ascertained at present. However, in view of the likelihood of an extended period of treatment, and perhaps relapses when treatment is discontinued, the need to establish an unequivocal diagnosis is of utmost importance when treating with potentially hazardous drugs.
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PMID:Therapy for granulomatous angiitis. 351 24

Recent reports have shown that despite an apparently satisfactory recovery from previous subarachnoid hemorrhage (SAH), many patients still have minor sequelae when specifically looked for. The cause of this so-called post-SAH-encephalopathy is uncertain. This prospective study comprises 54 patients who underwent aneurysmal surgery after SAH between September 1978 and March 1985. One patient died, and 6 patients were non-biased drop-outs. CSF hydrodynamics, determined by infusion test and isotope cisternography, were evaluated on the remaining 47 patients in the recovery stage. Five patients (11%) were found to have typical clinical, CSF dynamic and radiological manifestations of normal pressure hydrocephalus (NPH), and all were shunted with good results. Twelve (26%) had abnormal results consistent with disturbed CSF-hydrodynamics, although there were no clinical or radiological findings supporting the diagnosis of shunt-demanding NPH. Disturbed CSF-hydrodynamics as one of the possible etiological factors of post-SAH-encephalopathy is discussed.
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PMID:CSF hydrodynamics after subarachnoid hemorrhage. 361 9

An 8-year-old boy presented with large diffuse myelinoclastic bifrontal and right parietal peripherally enhancing white matter lesions with high intracranial pressure. Intravenous treatment with ACTH and high dose cyclophosphamide (cytoxin) resulted in shrinkage of the lesion, loss of peripheral enhancement and clear clinical improvement. After nine months following the onset of the first episode, an isolated left frontal enhancing lesion appeared along with a new right hemiparesis and speech difficulty. The same therapy again resulted in prompt definitive improvement of the CT scan and reversal of the clinical course. This case was extensively investigated and characterized as the 1912 variety of Schilder's disease, to be distinguished from post-infectious encephalopathy and other forms of white matter disease such as adrenoleukodystrophy. No elevation of CSF IgG, oligoclonal bands, or characteristic abnormality of T-cell subset distribution were identified. However, the pathological picture most closely resembled that of multiple sclerosis. A striking feature of this child's clinical history was the relatively rapid clinical improvement with immunosuppression.
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PMID:Schilder's disease: additional aspects and a therapeutic option. 368 53

A 2-year-old girl with 5,10-methylenetetrahydrofolate reductase deficiency developed subacute combined degeneration of the cord and a leuco-encephalopathy which was confirmed at necropsy. Total folate concentrations in serum, red cells and CSF were markedly reduced whereas vitamin B12 concentrations were normal. In addition the patient had Parkinsonism and reduced concentrations of homovanillic acid, 5-hydroxyindoleacetic acid and total biopterins in cerebrospinal fluid. Folic acid administration was accompanied by fits and acute deterioration in the movement disorder. At necropsy the basal ganglia showed no detectable abnormality.
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PMID:Subacute combined degeneration of the cord, dementia and parkinsonism due to an inborn error of folate metabolism. 375 52

We measured levels of gamma-aminobutyric acid (GABA) in the CSF and in the autopsied brain of patients with dialysis encephalopathy. GABA concentrations were low in the CSF of three of five living patients. Mean GABA content was reduced by 30 to 50% in five brain regions (frontal, occipital, and cerebellar cortex, caudate nucleus, and medial dorsal thalamus) in five fatal cases. GABA content was normal in brain regions where GABA is characteristically reduced in Huntington's disease. Choline acetyltransferase activity was diminished (by 25 to 35%) in cerebral cortex of the dialysis encephalopathy patients.
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PMID:Brain gamma-aminobutyric acid deficiency in dialysis encephalopathy. 396 5

Two patients had clinical findings of encephalopathy that progressed in 4 to 5 months. One patient had headache, fatigue, lethargy, hemiparesis, and a seizure. The second patient had only forgetfulness, confusion, and lethargy without focal signs. Herpes simplex virus was grown from brain biopsy in the first patient and from CSF in the second patient. These cases suggest that herpes simplex virus caused the encephalitis and that it should be considered in the differential diagnosis of chronic encephalopathy.
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PMID:Chronic encephalitis possibly due to herpes simplex virus: two cases. 403 28

We report on a newborn with peracute glycine encephalopathy. The child exhibited poor feeding, incipient respiratory failure and increasing muscular hypotonia from the first few days of life onwards and was admitted to hospital at six weeks due to regression of the symptoms. Following respiratory arrest the child had to be placed on controlled ventilation and died at the age of four months in spite of therapeutic measures. Previous papers on this rare disease have described elevated CSF glycine levels, EEG patterns, CT scan and acoustic and visual evoked potentials. We have supplemented these for the first time by somatosensory evoked potentials. The following is an account of the clinical course and the therapy given.
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PMID:[Glycine encephalopathy: a non-ketotic disorder of glycine catabolism]. 403 19

A study of 170 patients with juvenile rheumatoid arthritis and a review of the literature indicate that this disease can significantly affect the central nervous system. Signs of CNS dysfunction were observed in 13 children. During the acute toxic stages the EEG is abnormal in many cases. Other manifestations of toxic encephalopathy such as irritability, drowsiness, stupor, convulsions and marked meningismus may be evident in severe cases. Meningitis is often suspected but ruled out by the finding of normal CSF. Steroids can rapidly improve the condition of these children. If ;unexplained' seizures occur during the chronic stage, the diagnosis of cerebral vasculitis should be entertained.
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PMID:Cerebral complications in juvenile rheumatoid arthritis. 466 94


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