Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UNIPROT:P50583 (asymmetrical)
 12,197 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Thirty-eight cases of subacute sclerosing panencephalitis (SSPE) were reviewed. Deterioration in school performance, personality changes, and seizures were common early symptoms. Initial examination frequently showed myoclonus, spasticity, and extrapyramidal dysfunction, and in two-thirds of patients these findings were asymmetrical or focal. Retinitis or papilledema was present on initial examination in 50% of the patients. At last follow-up 24 children had died, with a mean survival of 42 months. Most patients reached a state of severe neurological impairment within 13 months. Subsequent evidence of improvement was noted in 10 children and was sustained in 4. Fifteen patients received antiviral treatment. Ten treated patients died from 5 to 133 months (mean, 58) from onset of their illness, while 15 untreated patients survived a mean of 33 months. Duration of survival appeared to be affected most by treatment with amantadine. Three patients treated with the drug were alive 97 to 139 months after onset of SSPE, and 5 died with a mean survival of 78 months. Five of 6 individuals treated with rifampin died after a mean survival of 27 months. Prolonged remissions occurred only in patients treated with amantadine. Although the number of treated individuals was small, our data suggest that amantadine may affect the natural course of SSPE.
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PMID:Review of 38 cases of subacute sclerosing panencephalitis: effect of amantadine on the natural course of the disease. 743 85

The clinical and pathologic features of five young Maltese dogs with a necrotizing meningoencephalitis were studied and compared with published reports of the necrotizing meningoencephalitis of Pug dogs. The ages of the Maltese dogs ranged from 9 months to 4 years. Four dogs were male, and one was female. The dogs had a history of seizures with or without other neurologic signs for 3 days to 20 weeks prior to death. Cerebrospinal fluid examination in three dogs revealed a pleocytosis and elevated levels of protein. At necropsy, the cerebrum was asymmetrically swollen in four dogs, with a loss of distinction between the gray and white matter and mild to moderate asymmetrical dilation of the lateral ventricles. Histologically, there was extensive necrosis and nonsuppurative inflammation of the cerebral gray and white matter, overlying meninges, and adjacent thalamus and hippocampus. The 4-year-old dog had the longest duration of clinical signs and had little inflammation but extensive atrophy of affected areas, with astrocytosis. The clinical course and pathologic changes in these Maltese dogs are indistinguishable from those in reported cases of necrotizing meningoencephalitis of Pug dogs, indicating that this lesion is probably not unique to Pug dogs.
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PMID:Necrotizing meningoencephalitis of Maltese dogs. 760 89

Wheatgerm agglutinin-horseradish peroxidase (WGA-HRP) histochemistry was combined with post-embedding immunogold cytochemistry in order to establish whether the subthalamic nucleus (STN) gives origin to glutamate (Glu)-enriched nerve terminals in substantia nigra, pars reticulata (SNr). Two adult cats served as normal controls and in two other animals crystalline WGA-HRP had been implanted bilaterally in STN. In all four animals ultrathin sections from SN were subjected to an immunogold procedure using antiserum raised against either Glu or gamma-aminobutyric acid (GABA). In some experiments the sections were subjected to consecutive incubations with both GABA and Glu antisera. These two antisera label two morphologically distinct types of boutons in SNr. The GABA antiserum labels boutons with pleomorphic vesicles, and they establish symmetrical synaptic contacts, mainly with dendritic shafts and spines, and occasionally with cell bodies. The Glu antiserum labels boutons with vesicles which are smaller and more uniform with regard to size and shape than those seen in the GABA-labelled boutons. The Glu-labelled boutons are engaged in asymmetrical synaptic contacts mainly with dendritic shafts and more rarely with cell bodies. The number of GABA-labelled boutons in SNr greatly exceeds the number of Glu-labelled ones. In the experimental material a considerable number of boutons in SNr are labelled with WGA-HRP reaction product. Several of these boutons are enriched in Glu-like immunoreactivity (Glu-LI), but not in GABA-LI. It is concluded that the subthalamonigral projection in the cat is likely to use Glu as a transmitter. The findings are briefly discussed with respect to the role played by STN in movement disorders and the involvement of excitatory amino acids in SN for the propagation of epileptic seizures and development of neurotoxicity.
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PMID:Terminals of subthalamonigral fibres are enriched with glutamate-like immunoreactivity: an electron microscopic, immunogold analysis in the cat. 767 8

We describe a woman with right hemifacial atrophy, a high palate, partial left motor seizures, and mild atrophy of the left arm. CT scan showed asymmetrical lateral ventricles and MRI (magnetic resonance imaging) showed atrophy of the right cerebral hemisphere and agenesis of the head of the right caudate nucleus. To our knowledge, this is the first report of Parry-Romberg syndrome associated with structural abnormalities of the basal nuclei documented by MRI. We suggest that a neurovascular aetiology can explain the spectrum of segmental defects associated with hemifacial atrophy.
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PMID:Progressive hemifacial atrophy with agenesis of the head of the caudate nucleus. 789 83

The clinical features of three affected members of a British pedigree with familial Alzheimer's disease are presented. This pedigree is one of six included in an earlier study which demonstrated linkage to chromosome 14. The individuals were investigated clinically and neuropsychologically, using both PET and MRI over a 4-year period. Further information from three deceased individuals was obtained, including histopathological confirmation of Alzheimer's disease in one case which came to autopsy. The mean age at onset for this family was 43 years. Neurological examination revealed myoclonic jerks in all cases, and one patient was documented to have seizures. Strikingly similar neuropsychological profiles were observed, characterized by an initial memory deficit with early dyscalculia and an impairment in speech production with relative absence of anomia. All individuals showed mild degrees of cerebral atrophy and two individuals had periventricular white matter lesions. PET scanning using [18F]fluorodeoxyglucose showed parieto-temporal hypometabolism in all cases and the two severely affected patients with speech production changes had additional left-sided frontal hypometabolism involving Broca's area. The least affected case initially had a more asymmetrical reduction in metabolism in the left inferior temporal and supramarginal gyri; a follow-up scan showed that this deficit had become bilateral and more severe. These clinical and neuroimaging features have not been previously reported in chromosome 14 linked pedigrees; the phenotypic variability between families suggests allelic heterogeneity at the chromosome 14 locus.
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PMID:Chromosome 14 linked familial Alzheimer's disease. A clinico-pathological study of a single pedigree. 789 4

To maximize tibial coverage during total knee arthroplasty, a study was performed to evaluate the morphology of the proximal tibia at the resection level and assess tibial coverage with respect to existing tibial implants. Unmagnified radiographs of 42 tibial resection specimens were produced and digitized with existing tibial implants from the asymmetrical Genesis and the symmetrical Insall-Burstein II and Press Fit Condylar total knee arthroplasty designs. The average total profile coverage ranged from 80.62% to 84.73%, whereas the average posteromedial coverage ranged from 76.05% to 82.09%. The shape of the tibia at the resection level was asymmetrical, and the overall tibial coverage was never greater than 85%. It appears that the actual shape of the tibial tray and the number of accommodating sizes provide the best ability of a total knee arthroplasty system to maximize tibial coverage, and not simply an asymmetrical or a symmetrical design.
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PMID:Resection specimen analysis of tibial coverage in total knee arthroplasty. 799 56

A case of early epileptic encephalopathy (EIEE) with suppression-bursts or Ohtahara's syndrome, associated with focal cortical dysplasia is reported. Infantile spasms and brief tonic unilateral seizures began on the fifth day of life. Interictal EEG demonstrated an asymmetrical "suppression-burst" pattern with no wake or sleep cycling. Seizures were refractory to all antiepileptic drug (AED) and steroid therapy. Magnetic resonance imaging (MRI) showed right frontotemporal cortical thickening. After three weeks of an ineffective medical treatment a preoperative evaluation with single photon emission computed tomography (SPECT) and electrocorticography (ECoG) was performed to characterize epileptogenic focus. Surgical resection of the precentral area was performed at age 1 month. Neuropathologic examinations confirmed diagnosis of focal cortical dysplasia by identifying cytoarchitectural disarray and ectopic neurons located deep in subcortical white matter. During follow-up, 1-year postoperative the child had a single febrile seizure. Neurologic examination showed minor developmental delay and slight left-sided weakness.
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PMID:Surgical treatment of an early epileptic encephalopathy with suppression-bursts and focal cortical dysplasia. 800 6

Spectral analysis of interictal background EEG activity recorded through foramen ovale (FO) electrodes during monitoring (mean 5.8 days per patient) was studied in 10 medically refractory complex partial seizure (CPS) patients who were candidates for epilepsy surgery. Data of the spectral analysis was plotted as compressed spectral array (CSA) with spectral edge frequency (SEF) markers. For each patient, time-varying fluctuations of the SEF markers were compared visually and by a computer-assisted method between two symmetrical FO electrode EEG channels recording from both mesiobasotemporal lobes (MTL). The amount of asymmetrical variations of the SEF markers ("rigidity" phenomenon) was first determined visually and then quantified by the computer-assisted method. These findings were correlated with the results of other clinical tests, including FO electrode-recorded seizure onset (FO ict), positron emission tomography with [18F]fluorodeoxyglucose (FDG-PET), and magnetic resonance imaging (MRI) to investigate whether the rigidity phenomenon could lateralize the primary epileptogenic zone. The rigid side had 80, 70, and 60% coincidence rates with the pathologic side indicated by FDG-PET, FO ict, and MRI, respectively, in a single test. We conclude that the rigidity phenomenon of FO electrode-recorded interictal background EEG activity is a valuable sign for lateralization of the primary epileptogenic zone in MTL epilepsy. The relative invariance of SEF may be caused by interictal deafferentation of epileptic neurons.
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PMID:Regional "rigidity" of background EEG activity in the epileptogenic zone. 802 94

A patient with Lennox-Gastaut syndrome developed an unusual feature of periodic spasms (PS), intermixed with tonic seizures. At age 14, she began to have unilateral seizures on awaking, followed by series of spasms Each spasm consisted of rotation of the head to the left and global muscle contraction of the whole body lasting for 0.5-1 second. Ictal EEGs revealed diffuse high voltage slow waves (HVSW), sometimes superimposed upon by diffuse low voltage fast activity, which was related to the increase of muscle activity following the spasms. When the duration of the fast activity on EEG became longer than 2 seconds, the peculiar seizures consisting of combination of asymmetrical spasms and tonic seizures occurred. It is speculated that the fast activity superimposed on HVSW seen in PS represents a transition from PS to tonic seizures.
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PMID:[Lennox-Gastaut syndrome with series of spasms intermixed with tonic seizures]. 818 81

This is a presentation of 2 cases in which the intraictal regional cerebral blood flow distribution was measured with the 99mTc-HMPAO single photon emission computerized tomography technique during an electrically induced seizure. Although the seizure was verified as generalized on electroencephalography, the regional neuronal activity expressed as rCBF unexpectedly was markedly asymmetrical in one of the cases. These findings demonstrated that the 99mTc-HMPAO technique makes it possible to discriminate intraictal variation in cortical and subcortical activation between the hemispheres during electroencephalography-verified generalized seizures.
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PMID:Disparity in regional cerebral blood flow during electrically induced seizure. 821 8


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