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)

Experimental infection was produced in white mice, rats, hamsters and rabbits inoculated with a virus isolated from the blood of a patient suffering from encephalopathy. Investigation of the pathomorphology of the experimental infection in white mice with with manifest clinical picture revealed acute meningoencephalomyelitis characterized by marked disorders in the central nervous system. In white rats and rabbits showing no symptoms of disease chronic affection of the CNS was established characterized by signs of encephalomyelopathy pathomorphologically similar to lesions observed in human cases of encephalopathy.
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PMID:[Pathomorphology of experimental infections caused by a virus isolated from a case of encephalomyelopathy]. 116 53

A 30 year old male patient presented with the clinical picture of progressive encephalomyelopathy eleven years after a portacaval shunt for non-cirrhotic portal fibrosis (NCPF). The spinal cord involvement in this patient was restricted to the corticospinal and the spinocerebellar pathways. The clinical picture and neuropathology of encephalopathy and of myelopathy have been described in patients with cirrhosis. Cases of encephalopathy and myelopathy have also been reported following shunt surgery in NCPF. We report the first case of encephalomyelopathy developing eleven years after portacaval shunt in a patient with noncirrhotic portal fibrosis.
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PMID:Encephalomyelopathy following portocaval shunt in noncirrhotic portal fibrosis: a case report. 130 85

In mechanical jaundice there may arise two kinds of CNS abnormalities: light disorders consistent with clinical signs of the underlying disease and neurological complications such as encephalopathy, encephalomyelopathy, polyneuropathies, neurasthenia. Neurological disorders are attributed mainly to initial severe autotoxication as well as hemodynamic affections. Of importance are also other risk factors (premorbid personality traits, advanced age, etc.).
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PMID:[The status of the nervous system in mechanical jaundice]. 281 Dec 23

Patients with mechanical jaundice showed signs of organic lesions of the nervous system: the syndrome of cholestatic encephalopathy (n = 56) and encephalomyelopathy (n = 9). Pathomorphological disorders are presented in the form of toxico-hypoxic changes of the neural cells and neuroglia in combination with organic changes in the vascular wall and functional disturbances of the blood flow and the cerebrospinal fluid dynamics. It is suggested that marked autointoxication and hemodynamic disturbances play the leading role in the pathogenesis of neurological disorders in mechanical jaundice.
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PMID:[Cholestatic toxic-vascular encephalopathy and encephalomyelopathy]. 343 62

It has been shown that the autosomal recessive mutation, gray tremor (gt) was associated in the homozygous state (gt/gt) with a rapidly fatal spongiform encephalopathy. Heterozygotes (+/gt) developed mild asymptomatic spongiform brain lesions as did recipient inbred mice inoculated with gt/gt brain homogenates, some of whom also showed behavioral abnormalities [Sidman, R. L., Kinney, H. C. & Sweet, H. O. (1985) Proc. Natl. Acad. Sci. USA 82, 253-257]. In these studies, inbred NFS/N mice inoculated intracerebrally at birth or as adults with gt/gt or first passage gt brain homogenates developed a progressive disease characterized by tremor, ataxia, and spasticity. The symptoms were milder and more slowly progressive than in the gt/gt homozygote, in the paralytic syndrome that followed neonatal inoculation of NFS/N mice with a wild murine leukemia virus (Cas-Br-M MuLV), or in the rapidly progressive ataxia and terminal bradykinesia that followed scrapie inoculation of NFS/N mice. The noninflammatory spongiform encephalopathy in affected NFS/N mice resembled that observed in gt/gt homozygotes, +/gt heterozygotes, and asymptomatic recipient inbred mice inoculated with gt/gt brain homogenates. Neither infectious MuLV nor MuLV proteins were detected in gt/gt brain homogenates or in affected recipient mouse brains. Scrapie-associated fibrils, readily identifiable in subcellular fractions of brains from scrapie-inoculated NFS/N mice, were not detected in similar brain fractions from NFS/N mice inoculated with gt brain homogenates. These results confirm and extend the suggestion that gt spongiform encephalopathy has both heritable and transmissible properties. Moreover, the transmissible agent of gt disease differs from both Cas-Br-M MuLV and scrapie in its disease-inducing properties in NFS/N mice. The capacity of NFS/N mice to express transmitted gt encephalopathy as clinical disease, to rapidly express Cas-Br-M MuLV spongiform encephalomyelopathy, and to develop mouse-adapted scrapie after a very short incubation time suggest a distinct sensitivity of NFS/N mice to transmissible spongiform encephalopathy.
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PMID:Transmission in NFS/N mice of the heritable spongiform encephalopathy associated with the gray tremor mutation. 347 86

A 29-month-old female Alaskan husky was presented recumbent, tetraparetic and in a state of dementia, with blindness and cranial nerve deficits. The dog's progress was followed for over two months, as the signs resolved to an non-progressive mild hypermetria with slight proprioceptive ataxia, a diminished menace response and inability to prehend food. Magnetic resonance imaging (MRI) revealed bilateral cavitation extending from the thalamus to the medulla, with less pronounced degenerative lesions in the caudate nucleus, putamen and claustrum. Cerebrospinal fluid lactate and pyruvate concentrations were in their normal ranges. Necropsy and histological examination confirmed the MRI findings as well as neuronal degeneration of the cerebellar cortex in the vermis and degenerative changes in the neocortex at the depths of the cerebral sulci. In view of the similarity of lesions to subacute necrotising encephalomyelopathy, known as Leigh's disease in humans, a tentative diagnosis of a mitochondrial encephalopathy was made.
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PMID:Subacute necrotising encephalopathy in an Alaskan husky. 1066 57

Chronic traumatic encephalopathy (CTE) has been linked to participation in contact sports such as boxing and American football. CTE results in a progressive decline of memory and cognition, as well as depression, suicidal behavior, poor impulse control, aggressiveness, parkinsonism, and, eventually, dementia. In some individuals, it is associated with motor neuron disease, referred to as chronic traumatic encephalomyelopathy, which appears clinically similar to amyotrophic lateral sclerosis. Results of neuropathologic research has shown that CTE may be more common in former contact sports athletes than previously believed. It is believed that repetitive brain trauma, with or possibly without symptomatic concussion, is responsible for neurodegenerative changes highlighted by accumulations of hyperphosphorylated tau and TDP-43 proteins. Given the millions of youth, high school, collegiate, and professional athletes participating in contact sports that involve repetitive brain trauma, as well as military personnel exposed to repeated brain trauma from blast and other injuries in the military, CTE represents an important public health issue. Focused and intensive study of the risk factors and in vivo diagnosis of CTE will potentially allow for methods to prevent and treat these diseases. Research also will provide policy makers with the scientific knowledge to make appropriate guidelines regarding the prevention and treatment of brain trauma in all levels of athletic involvement as well as the military theater.
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PMID:Long-term consequences of repetitive brain trauma: chronic traumatic encephalopathy. 2203 90

Wernicke's encephalopathy is a triad of ophthalmoplegia, ataxia and confusion seen in alcoholics with dietary vitamin B1 (thiamine) deficiency. A rare genetic defect of thiamine transporter-2 may lead to similar clinical features, biotin-thiamine responsive basal ganglia disease (BTBGD). A 15-year-old girl developed rapid onset ptosis and ophthalmoplegia evolving into a subacute encephalopathy. Neuroimaging demonstrated symmetrical basal ganglia and mid-brain lesions reminiscent of Leigh's subacute necrotising encephalomyelopathy. Oral biotin and thiamine were started, and symptoms improved dramatically the next day. The therapeutic response suggested SLC19A3, encoding thiamine transporter-2, as a strong candidate gene and Sanger sequencing revealed a novel homozygous c.517A>G;p.Asn173Asp mutation, which segregated with disease within the family. BTBGD is a potentially treatable neurological disorder and should be considered in the differential diagnosis of Leigh syndrome and Wernicke's encephalopathy. Since delayed treatment results in permanent neurological dysfunction or death, prompt diagnosis and early initiation of biotin and thiamine therapy are essential.
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PMID:Treatable Leigh-like encephalopathy presenting in adolescence. 2409 34