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

The conversion of the cellular isoform of the prion protein (PrP(C)) to the abnormal disease-associated isoform (PrP(Sc)) has been simulated in cell-free conversion reactions in which PrP(Sc)-enriched preparations induce the conformational transition of PrP(C) into protease-resistant PrP (PrP-res). We explored the utility of recombinant hamster (Ha)PrP(C) purified from baculovirus-infected insect cells (bacHaPrP(C)) as a replacement for mammalian-derived HaPrP(C) in the conversion reactions. Protease-resistant recombinant HaPrP was generated after incubation of (35)S-bacHaPrP(C) with PrP(Sc)-enriched preparations. Moreover strain-specific PrP-res was also reproduced using insect-cell derived HaPrP(C) and PrP(Sc) from two different strains of hamster-adapted transmissible mink encephalopathy, designated hyper (HY) and drowsy (DY). Two strain-mediated properties were tested: (i) molecular mass of the protease-digested products and (ii) relative resistance to proteinase K (PK) digestion. Similar to in vivo generation of PrP(HY) and PrP(DY), the converted products selectively reproduced both characteristics, with the DY conversion product being smaller in size and less resistant to PK digestion than the HY product. These data demonstrate that non-mammalian sources of recombinant HaPrP can be converted into PK-resistant form and that strain-mediated properties can be transmitted into the newly formed PrP-res.
J Gen Virol 2000 Oct
PMID:Strain-specific propagation of PrP(Sc) properties into baculovirus-expressed hamster PrP(C). 1099 47

BACKGROUND: Short Latency Somatosensory Evoked Potentials (SEPs) may serve to the testing of the somatosensory tract function, which is vulnerable and affected in vascular encephalopathy. The aim of the current study was to search for clinical and neuroimaging correlates of abnormal SEPs in vascular dementia (VD) patients. MATERIALS AND METHODS: The study included 14 VD patients, aged 72.93 PlusMinus; 4.73 years, and 10 controls aged 71.20 PlusMinus; 4.44 years. All subjects underwent a detailed clinical examination, blood and biochemical testing, brain MRI and were assessed with the MMSE. SEPs were recorded after stimulation from upper and lower limbs. The statistical Analysis included 1 and 2-way MANCOVAs and Factor analysis RESULTS: The N13 latency was significantly prolonged, the N19 amplitude was lower, the P27 amplitude was lower and the N11-P27 conduction time was prolonged in severely demented patients in comparison to controls. The N19 latency was prolonged in severely demented patients in comparison to both mildly demented and controls. The same was true for the N13-N19 conduction time, and for the P27 latency. Patients with subcortical lesions had all their latencies prolonged and lower P27 amplitude. DISCUSSION: The results of the current study suggest that there are significant differences between patients suffering from VD and healthy controls in SEPs, but these are detectable only when dementia is severe or there are lesions located in the subcortical regions. The results of the current study locate the abnormal SEPs in the white matter, and are in accord with the literature.
Ann Gen Hosp Psychiatry 2003 Sep 05
PMID:Clinical and neuroimaging correlates of abnormal short-latency Somatosensory Evoked Potentials in elderly vascular dementia patients: A psychophysiological exploratory study. 1449 95

We report that the replication-deficient DY strain of transmissible mink encephalopathy (TME) can delay disease caused by the pathogenic HY TME strain. In this study, competition between the HY and DY TME agents was investigated following superinfection of the sciatic nerve and peritoneal cavity. Initially, DY TME infection was examined in the absence of superinfection and it was found that inoculation into the brain and sciatic nerve resulted in prion disease and PrP(Sc) deposition in brain but not lymphoreticular tissues. Conversely, intraperitoneal inoculation of the DY TME agent did not result in clinical symptoms, DY TME agent replication or PrP(Sc) deposition 400-600 days after infection. These findings indicate that the DY TME agent does not replicate in secondary lymphoid organs and is non-pathogenic when neuroinvasion is dependent on prior infection of the lymphoreticular system. However, intraperitoneal inoculation of the DY TME agent at 60 days, but not at 30 days, prior to intraperitoneal inoculation of the HY TME agent resulted in an extension of the HY TME incubation period. Inoculation of the DY TME agent into the sciatic nerve at 60 days prior to intrasciatic nerve inoculation of the HY TME agent did not delay the incubation period of HY TME. The ability of the DY TME agent to delay HY TME infection following extraneural inoculation, but not neural infection, suggests that HY and DY TME agent competition can occur in a common replication site whose cellular location precedes infection of both the lymphoreticular and peripheral nervous systems.
J Gen Virol 2004 Jan
PMID:Delay in onset of prion disease for the HY strain of transmissible mink encephalopathy as a result of prior peripheral inoculation with the replication-deficient DY strain. 1471 42

Viral encephalopathy and retinopathy is a devastating disease that causes neurological disorders and high mortality in a large number of cultivated marine fish species around the world. It is now established that several viral strains classified in the genus Betanodavirus of the family Nodaviridae are the aetiological agents of this disease. Betanodaviruses can be classified into four genotypes based on the coat protein gene sequence. Here, the coat protein genes of the three major strains isolated from sea bass (Dicentrarchus labrax) in France were found to be different. In addition, 21 novel strains of betanodavirus from several fish species from France, Spain, Tunisia and Tahiti were classified by using phylogenetic analysis of a partial sequence (383 nt) of the coat protein gene. Most of the isolates were grouped in the red-spotted grouper nervous necrosis virus type, which was subdivided into two subtypes, one of them containing only French isolates. Furthermore, an isolate obtained from sea bass during an outbreak at low temperature (15 degrees C) was classified as the barfin flounder nervous necrosis virus type. This is the first reported isolation from sea bass of such a strain, which is known to infect several cold-water marine fish species. In addition, a betanodavirus belonging to the striped jack nervous necrosis virus type was detected in Senagalese sole (Solea senegalensis) farmed in Spain, which is the first indication of the presence of this genotype outside Japan. These findings suggest that the different genotypes can infect a variety of fish species and thus have a low host-fish species specificity.
J Gen Virol 2004 Oct
PMID:Genomic classification of new betanodavirus isolates by phylogenetic analysis of the coat protein gene suggests a low host-fish species specificity. 1544 71

We report a case of Hashimoto's encephalopathy with detailed neuropsychological testing before, during and after steroid treatment, allowing a more precise characterization of the deficits and their response to treatment. It highlights that behavioral and psychotic symptoms remit before cognitive deficits and suggests that the latter may be more appropriate for guiding the duration of steroid treatment.
Gen Hosp Psychiatry
PMID:Hashimoto's encephalopathy: a case report with neuropsychological testing. 1748 46

Carnitine is an essential co-factor in fatty acid metabolism. Carnitine deficiency can impair fatty acid oxidation, rarely leading to hyperammonemia and encephalopathy. We present the case of a 35-year-old woman who developed acute mental status changes, asterixis, and diffuse muscle weakness. Her ammonia level was elevated at 276 microg/dL. Traditional ammonia-reducing therapies were initiated, but proved ineffective. Pharmacologic, microbial, and autoimmune causes for the hyperammonemia were excluded. The patient was severely malnourished and her carnitine level was found to be extremely low. After carnitine supplementation, ammonia levels normalized and the patient's mental status returned to baseline. In the setting of refractory hyperammonemia, this case illustrates how careful investigation may reveal a treatable condition.
J Gen Intern Med 2008 Feb
PMID:Hyperammonemic encephalopathy caused by carnitine deficiency. 1808 Jan 67

Pellagra is associated with low levels of vitamin B3 (niacin) and/or tryptophan and often involves other other B vitamins. Since the time Gasper Casal first described the disease in 1972, it was observed that the patients with pellagra were all poor, subsisted mainly on maize, and rarely ate fresh meat. Subsequent occurrences have been in the form of epidemic outbreaks, consequent to either introduction to maize as a major food or increased consumption of other niacin-deficient diets like Jowar (Sorgum vulgare). The virtual disappearance of pellagra as an endemic health problem in recent years can be attributed to a rise in the standard of living of farmers and diversification of the diet globally. The clinical picture is a combination of multisystem alterations typically involving gastrointestinal, skin and central nervous system abnormalities. The cardinal manifestations have been popularly known as the three D's, which are dementia, dermatitis and diarrhea. Psychiatric manifestations are fairly common but are easily overlooked due to their non specific nature. These are commonly seen as irritability, poor concentration, anxiety, fatigue, restlessness, apathy and depression. The occurence of psychosis in pellagra is an uncommon finding, which is usually seen in advanced stages of pellagroid encephalopathy, commonly found in chronic alcoholics. Delusional parasitosis has been also reported in this condition, although the association is still controversial. We report a case of pellagra manifesting with delusional parasitosis in a man whose delusion resolved rapidly after he started niacin-augmentation therapy. This case may provide clues to the biological underpinnings of delusional parasitosis as well as niacin treatment as treatment option in similar cases.
Gen Hosp Psychiatry
PMID:Rapid resolution of delusional parasitosis in pellagra with niacin augmentation therapy. 1906 87

Most patients contract hypoxic encephalopathy after suffering a cardiac arrest. They usually endure severe neurological sequelae and the temporal profile of the disease progression remains unclear. This case study shows how the effects of hypoxic encephalopathy continue to progress for several years after the initial event. Up to eight years after the hypoxic insult, the patient's intellect steadily deteriorated, and brain atrophy progressed. As the hypoxic insult on the brain is only transient, the neurological disability seems not to be exacerbated for years. However, our case indicates that this disorder may have a long progression.
Int J Gen Med 2008 Nov 30
PMID:A case of hypoxic encephalopathy with delayed exacerbation. 2042 10

Case reports of mothball ingestion have shown that paradichlorobenzene, the organic compound found in mothballs, can induce multiple organ effects, including encephalopathy. Psychiatrists are often involved in these cases due to presumed depression. Diagnosis is frequently delayed and/or inaccurate due to the inability to obtain a full history. A delay in diagnosis may result in an inappropriate treatment plan. We present a case involving a woman who ingested mothballs due to Pica emphasizing the importance and challenges of getting a thorough history. Barriers include cultural differences, shame and obtaining collateral information.
Gen Hosp Psychiatry
PMID:Mothball induced encephalopathy presenting as depression: it's all in the history. 2043 Feb 45

Catatonia is a distinct neuropsychiatric syndrome with prominent motor manifestations. Posterior reversible encephalopathy syndrome (PRES) is a clinicoradiologic syndrome usually precipitated by malignant hypertension. Given the overlapping neuropathology in both syndromes, we present a case of catatonia precipitated by PRES, with full resolution of the former after successful treatment of the latter.
Gen Hosp Psychiatry
PMID:A case of catatonia due to posterior reversible encephalopathy syndrome treated successfully with antihypertensives and adjunctive olanzapine. 2160 35


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