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)

Three patients showed dramatic psychic akinesia after recovery from toxic encephalopathy. They had no or only mild motor disorders. The spontaneous psychic akinesia was reversible when the patient was stimulated, as if there was a loss of self psychic activation. Intellectual capacities were normal. Two patients had stereotyped behaviours resembling compulsions. In all patients CT cans showed bilateral lesions in the basal ganglia, mainly within the globus pallidus.
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PMID:Pure psychic akinesia with bilateral lesions of basal ganglia. 672 63

We investigated the existence, clinical symptoms, and brain pathology of cerebrovascular parkinsonism. In this study, clinicopathological criteria of cerebrovascular parkinsonism was defined as follows: 1) at least 2 or more symptoms from the following 4 symptoms: tremor, rigidity, akinesia or bradykinesia, and short stepped gait or freezing, 2) no depigmentation, no Lewy body at the substantia nigra, no other degenerative disease, and 3) existence of cerebrovascular lesions. Among consecutive 4,000 autopsy series in the elderly, clinicopathologically confirmed cerebrovascular parkinsonism was found in 24 patients with mean age of 80 years. Cerebrovascular parkinsonism was characterized by the short-stepped gait as initial symptoms, absence of the resting tremor, lead-pipe rigidity, the symmetry of findings, and negative response to levodopa. Pseudobulbar palsy was observed in 54%, pyramidal findings in 63% of the cases. Most cases had multiple vascular lesions of the basal ganglia, but the distribution of lesions was not different from that in the cases of progressive subcortical vascular encephalopathy of Binswanger type without parkinsonism. Diffuse pallor and the loss of oligodendrocytes in the frontal white matter observed in the cerebrovascular parkinsonism suggested that the symptoms of parkinsonism resulted from the white matter damages in the frontal lobe.
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PMID:[Cerebrovascular parkinsonism--clinicopathologic study]. 875 29

The natural history of the rare association "multicystic encephalopathy-arthrogryposis" was traced in a fetus carefully followed after artificial insemination. The fetus exhibited normal viability and brain morphology up to the 32nd week. At 36 weeks, active movements diminished and at 37 weeks, hydramnios and signs of fetal distress led to cesarean section. The infant presented with severe arthrogryposis of the limbs and spine, but not with the other elements of a long-lasting akinesia. US showed multicystic encephalopathy. Both the clinical and the neuropathological findings established that multicystic encephalopathy was neither the cause nor the sequential consequence of the fetal akinesia, but the result of a recent diffuse, acute malacic process that also involved the anterior horn cells. Acute fetal distress, responsible for major ischemic damage to CNS but compatible with fetal survival, remains an obscure condition which allows for the development of severe arthrogryposis in a few weeks.
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PMID:Arthrogryposis and multicystic encephalopathy after acute fetal distress in the end stage of gestation. 1131 3

We report the case of a 32-year-old man presenting symptoms of parkinsonism. Neurological examination revealed parkinsonism symptoms such as akinesia and postural instability, dementia and frontal lobe signs. He was diagnosed as having human immunodeficiency virus (HIV) encephalopathy. Brain MRI, 99mTc ECD-SPECT and 1H-MR spectroscopy demonstrated symmetrical cerebral white matter lesions, predominantly in the bilateral frontal lobes. Frontal lobe dysfunction could be responsible for his parkinsonism associated with HIV encephalopathy. His neurological symptoms improved transiently after the initiation of HAART but fluctuated when antiretroviral drugs were changed because of their side effects. Although HAART effectively decreased plasma HIV-RNA load and increased peripheral blood CD4 cell count, his parkinsonism and dementia eventually exacerbated. Our results suggest that a combination of antiretroviral drugs affects the therapeutic efficacy against HIV encephalopathy, and that CNS symptoms could be aggravated during HAART, even when plasma HIV-RNA load and CD4 cell count are maintained under favorable conditions.
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PMID:[A patient with HIV encephalopathy presenting with parkinsonism during HAART therapy]. 1685 18

A 60-year-old male was admitted because he had developed tremulous movement in both upper and lower limbs and gait disturbance over the course of 3 months. He had been on continuous ambulatory peritoneal dialysis almost 1 year earlier due to end-stage diabetic nephropathy. A neurological examination revealed a mild disturbance of his consciousness, asterixis in the upper limbs, bilateral extensor plantar responses and parkinsonism, which were characterized by bradykinesia, akinesia, rigidity, and bilaterally tremors at rest. Cranial magnetic resonance imaging (MRI) revealed swollen bilateral basal ganglia legions, which appeared hyperintense on T2-weighted images. The patient was treated for metabolic acidosis and continued hemodialysis three times a week; however, the parkinsonism remained 1 year later. Follow-up MRI revealed decreased swelling of the basal ganglia, and the pattern of diffusion-weighted images and the apparent diffusion coefficient (ADC) map indicated vasogenic and cytotoxic edema in bilateral globus pallidus. The case was diagnosed as encephalopathy due to diabetic uremic syndrome, initially characterized by Wang et al. (2003). Only 17 cases with parkinsonism have been reported. Diabetic uremic syndrome is characterized by acute or subacute onset consciousness disturbance and movement disorders such as parkinsonism, chorea and the other extrapyramidal signs to various degrees related to bilateral lesions of the basal ganglia.
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PMID:[A case of subacute parkinsonism presenting as bilateral basal ganglia legions by MRI in diabetic uremic syndrome]. 2352 2

Here we report a case of a 10-year-old female with unclassified epileptic encephalopathy who showed forced normalization after administration of levetiracetam (LEV). She initially presented with intractable tonic and myoclonic seizures that were observed about 10 times a day along with frequent multifocal sharp and slow wave complexes on electroencephalography (EEG). We were forced to decrease the topiramate dose because of the appearance of nystagmus, and her myoclonic seizures became worse. We added LEV (250 mg/day) and her tonic and myoclonic seizures disappeared one day after initiation of LEV administration. However, she showed hyporesponsiveness and akinesia. The disappearance of paroxysmal discharges on EEG confirmed the diagnosis of forced normalization. Despite continuous administration of LEV, tonic and myoclonic seizures relapsed within a month but her psychotic symptoms resolved simultaneously. To the best of our knowledge, this is the first reported case of forced normalization after LEV administration. It should be noted that LEV may cause forced normalization although it can be started at an adequate dosage.
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PMID:[Epileptic encephalopathy associated with forced normalization after administration of levetiracetam]. 2420 93

Tauopathies are neurodegenerative disorders characterized by the deposition of abnormal tau protein in the brain. The spectrum of tau pathologies expands beyond the traditionally discussed disease forms like Pick disease, progressive supranuclear palsy, corticobasal degeneration, and argyrophilic grain disease. Emerging entities and pathologies include globular glial tauopathies, primary age-related tauopathy, which includes neurofibrillary tangle dementia, chronic traumatic encephalopathy (CTE), and aging-related tau astrogliopathy. Clinical symptoms include frontotemporal dementia, corticobasal syndrome, Richardson syndrome, parkinsonism, pure akinesia with gait freezing and, rarely, motor neuron symptoms or cerebellar ataxia. Some disorders show specific neuroimaging features, while examination of the cerebrospinal fluid awaits markers for in vivo stratification of cases. The possibility of cell-to-cell propagation is a novel aspect of the pathogenesis of tauopathies, which is partly reflected by the hierarchic involvement of anatomic regions. This concept might have relevance for the development of therapies. For cost-effective screening for tau pathologies in neuropathologic practice, examination of the hippocampus, amygdala, and basal ganglia is recommended. Uncommon morphologies or unusually extensive forms of tau pathologies should raise the suspicion of a genetic background. Ongoing multidisciplinary studies are needed to understand the whole spectrum and significance of tau pathologies.
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PMID:Tauopathies. 2898 82