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)

Postpartum cerebral angiopathy mostly occurs in the large or medium-sized cerebral arteries. In this case, we aimed to report a case of postpartum cerebral angiopathy presented as an asymmetrical penetrating arterial territory infarct with severe surrounding vasogenic edema. A 26-year-old woman admitted because of sudden headache after an attack of seizure. On initial computerized tomography (CT), hypodense lesion in the right basal ganglia was observed. The diffusion-weighted image on 5th day revealed focal acute ischemic infarction with surrounding extensive vasogenic edema in right basal ganglia. The CT angiography showed multifocal arterial narrowing of intracranial cerebral arteries that completely resolved on the follow-up study. This case suggested that asymmetrical small penetrating arterial territory infarct can occur as an atypical presentation of postpartum cerebral angiopathy.
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PMID:Atypical presentation of postpartum cerebral angiopathy shown as a small penetrating arterial territory infarct with severe peri-infarct edema. 1880 76

The association of brain malformations and symptomatic epilepsy in the setting of neurofibromatosis type 1 (NF1) is rarely reported. When it occurs, patients can present clinically with infantile spasms, focal seizures, generalized tonic clonic seizures or atypical absences. We report on a 10-year-old (molecularly proven) NF1 girl manifesting a complex epileptic syndrome resembling the Foix-Chavany-Marie spectrum (also known as opercular syndrome) associated with bilateral (opercular and paracentral lobular) polymicrogyria (PMG). Anecdotal cases of unilateral PMG in the setting of NF1 have been described in association with other-than-opercular epileptic syndromes. The typical clinical opercular syndrome consisting in mild mental retardation, epilepsy and pseudobulbar palsy is usually associated to bilateral perisylvian PMG (BPP) CONCLUSION: To the best of our knowledge, the complex epileptic syndrome hereby reported has not been previously recorded in the setting of NF1. In addition, the present girl manifested all the clinical features of an opercular syndrome but had an asymmetrical PMG (not a BPP).
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PMID:Complex epileptic (Foix-Chavany-Marie like) syndrome in a child with neurofibromatosis type 1 (NF1) and bilateral (opercular and paracentral) polymicrogyria. 1914 Nov 42

Polymicrogyria (PMG) is a relatively common malformation of the cortex for which the pathogenesis remains poorly understood. Both acquired and genetic causes are known, and to date more than 70 cases of PMG have been associated with chromosomal abnormalities. Here we report on a 12-year-old girl presenting with asymmetrical PMG predominantly affecting the right occipital lobe. She was the only child of consanguineous parents. At 7 years of age she was referred for mental retardation with speech delay and seizures. Cytogenetic studies of the patient revealed an inverted 9p duplication/deletion and bacterial artificial chromosomes (BACs)-array also showed a 22q11.2 microduplication confirmed by quantitative PCR. This case is of interest in the search for candidate genes and emphasizes the importance of the 22q11 region in PMG. It also highlights the efficiency of BACs-array in detecting complex rearrangements.
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PMID:Polymicrogyria in a child with inv dup del(9p) and 22q11.2 microduplication. 1921 26

Electroconvulsive therapy (ECT) is a mainstay in the treatment of severe, medication-resistant depression. The antidepressant efficacy and cognitive side effects of ECT are influenced by the position of the electrodes on the head and by the degree to which the electrical stimulus exceeds the threshold for seizure induction. However, surprisingly little is known about the effects of other key electrical parameters such as current directionality, polarity, and electrode configuration. Understanding these relationships may inform the optimization of therapeutic interventions to improve their risk/benefit ratio. To elucidate these relationships, we evaluated a novel form of ECT (focal electrically administered seizure therapy, FEAST) that combines unidirectional stimulation, control of polarity, and an asymmetrical electrode configuration, and contrasted it with conventional ECT in a nonhuman primate model. Rhesus monkeys had their seizure thresholds determined on separate days with ECT conditions that crossed the factors of current directionality (unidirectional or bidirectional), electrode configuration (standard bilateral or FEAST (small anterior and large posterior electrode)), and polarity (assignment of anode and cathode in unidirectional stimulation). Ictal expression and post-ictal suppression were quantified through scalp EEG. Findings were replicated and extended in a second experiment with the same subjects. Seizures were induced in each of the 75 trials, including 42 FEAST procedures. Seizure thresholds were lower with unidirectional than with bidirectional stimulation (p<0.0001), and lower in FEAST than in bilateral ECS (p=0.0294). Ictal power was greatest in posterior-anode unidirectional FEAST, and post-ictal suppression was strongest in anterior-anode FEAST (p=0.0008 and p=0.0024, respectively). EEG power was higher in the stimulated hemisphere in posterior-anode FEAST (p=0.0246), consistent with the anode being the site of strongest activation. These findings suggest that current directionality, polarity, and electrode configuration influence the efficiency of seizure induction with ECT. Unidirectional stimulation and novel electrode configurations such as FEAST are two approaches to lowering seizure threshold. Furthermore, the impact of FEAST on ictal and post-ictal expression appeared to be polarity dependent. Future studies may examine whether these differences in seizure threshold and expression have clinical significance for patients receiving ECT.
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PMID:Focal electrically administered seizure therapy: a novel form of ECT illustrates the roles of current directionality, polarity, and electrode configuration in seizure induction. 1922 53

Myoclonic jerks and myoclonic status (MS) are sometimes difficult to distinguish clinically from movement disorders such as hand stereotypies, tremor, and dystonia in Rett syndrome. We describe a rare and complete video-polygraphic study of a girl with Rett syndrome (MECP2 mutation) and MS misdiagnosed as movement disorders and disclosed after video-polygraphic recordings. Corresponding to closely recurring activity of diffuse spike and polyspikes-wave-type paroxysms, rhythmic and, especially, arrhythmic myoclonias, usually asymmetrical and asynchronous, involving mainly right muscle deltoid and rarely followed by an inhibitory phenomenon, appeared. The MS improved and, most importantly, disappeared after the use of levetiracetam, with an evident antimyoclonic efficacy and a marked improvement of daily life for the patient and her caregivers. The difficulty in differentiating some typical nonepileptic behavioral features and movement disorders of patients with Rett syndrome from seizures was overcome using prolonged video-polygraphic recordings in our case.
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PMID:Myoclonic status misdiagnosed as movement disorders in Rett syndrome: a video-polygraphic study. 1960 60

The following aspects are reviewed: Does the aetiology influence the outcome of infantile spasms? Does the treatment influence the outcome? Can the outcome be predicted? Can we improve the prognosis? Favourable factors are the following: cryptogenic aetiology, age at onset > or =4 months, absence of atypical spasms and partial seizures, and absence of asymmetrical EEG abnormalities, short treatment lag, and an early and sustained response to treatment. Not only patients with a cryptogenic aetiology have a favourable outcome. We can already at the first clinical evaluation tell the parents if the prognosis looks favourable. The final goal of the treatment is improved mental outcome. Steroids and vigabatrin are the first-line drugs for infantile spasms in Europe. In a prospective study from the United Kingdom short-term outcome was better with hormonal than with vigabatrin therapy (tuberous sclerosis excluded). However, the numbers of patients who were seizure-free at 3-4 months in different studies have been very similar. Moreover, an early response to treatment seems to be of predictive value for the cognitive outcome in children with cryptogenic spasms. The long-term outcome is known only after hormonal therapy. The side effects of steroids are usually treatable and reversible. In Finland ACTH therapy is given at the minimum effective dose and for the minimum effective time with minimal side effects. The risks of VGB are irreversible visual field defects. As of yet there is no method to examine the visual fields in patients with infantile spasms. Early treatment of infantile spasms seems to be important. Prevention of infantile spasms with some aetiological groups might be possible.
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PMID:Favourable prognostic factors with infantile spasms. 1936 67

Many important central nervous system (CNS) syndromes can develop following microbial infections. The most severe forms of post-infectious encephalitis include acute disseminated encephalomyelitis (ADEM), acute hemorrhagic leukoencephalitis and Bickerstaff's brainstem encephalitis. ADEM is an inflammatory demyelinating disorder of the CNS. It typically follows a minor infection with a 2-30 days latency period and is thought to be immune-mediated. It is clinically characterized by the acute onset of focal neurological signs and encephalopathy. Patients can require intensive care unit admission because of coma, seizures or tetraplegia. Cerebrospinal fluid analysis usually shows lymphocytic pleocytosis but, unlike viral or bacterial encephalitis, no evidence of direct CNS infection is found. There are no biologic markers of the disease and cerebral magnetic resonance imaging is essential to diagnosis, detecting diffuse or multifocal asymmetrical lesions throughout the white matter on T2- and FLAIR-weighted sequences. High-dose intravenous steroids are accepted as first-line therapy and beneficial effects of plasma exchanges and intravenous immunoglobulins have also been reported. Outcome of ADEM is usually favorable but recurrent or multiphasic forms have been described.
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PMID:Post-infectious encephalitis in adults: diagnosis and management. 1936 74

Eosinophilic meningoencephalitis (EME) has been described in various species of animals and in humans. In dogs it has been associated with protozoal infections, cuterebral myiasis and various other aetiologies. Ten cases of idiopathic eosinophilic meningoencephalitis have been reported in dogs and one in a cat where the origin was uncertain or unknown. The dogs were all males, of various breeds but with a predominance of Golden Retrievers and Rottweilers; they generally had a young age of onset. Two cases with no apparent underlying aetiology were diagnosed on post mortem examination. The 18-month-old, male Boerboel presented with sudden onset of cerebellar ataxia, as well as various asymmetrical cranial nerve deficits of 2 weeks' duration and without progression. Haematology revealed a peripheral eosinophilia. Necropsy showed extreme generalised congestion especially of the meninges and blood smear and histological sections of various tissues showed intravascular erythrocyte fragmentation with the formation of microcytes. Histopathology revealed severe diffuse cerebrocortical subarachnoidal meningitis and submeningeal encephalitis, the exudate containing variable numbers of eosinophils together with neutrophils and mononuclear cells. There was also deeper white matter and hippocampal multifocal perivascular mononuclear encephalitis and multifocal periventricular malacia, gliosis and phagocytosis of white matter. The cerebellum, brain stem and spinal c showed only mild multifocal oedema or scattered occasional axon and myelin degeneration respectively, with no inflammation. Immunohistochemical staining of central nervous tissue for Toxoplasma gondii failed to show any antigen in the central nervous tissue. Ultrastructure of a single submeningeal suspected parasitic cyst showed it to be chromatin clumping within a neuron nucleus indicating karyorrhexis. Gram stain provided no evidence of an aetiological agent. The 3-year-old Beagle bitch had a Caesarian section after developing a non-responsive inertia 8 days prior to presentation. This animal's clinical signs included status epilepticus seizures unrelated to hypocalcaemia and warranted induction of a barbiturate coma. She died 4 hours later. Post mortem and histopathological findings in the brain were almost identical to those of the Boerboel and she also showed histological evidence of recent active intravascular haemolysis with microcyte formation. Rabies, distemper and Neospora caninum immunohistochemical stains were negative in the brains of both dogs. Immunohistochemical staining of the cerebral and meningeal exudates of the Beagle for T- and B-lymphocyte (CD3 and CD79a) markers showed a predominance of T-lymphocytes with fewer scattered B lymphocytes. A possible allergic response to amoxicillin/clavulanate is considered, as this appeared to be the only feature common to the recent history of both animals. An overview of EME in humans, dogs and cats is given and the previously published cases of idiopathic EME in dogs and the single published cat case are briefly reviewed.
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PMID:Review of idiopathic eosinophilic meningitis in dogs and cats, with a detailed description of two recent cases in dogs. 1949 20

A female infant suffered from epilepsy since the neonatal period, which evolved into West syndrome at the age of 2 months. Spasms in series and hypsarrhythmia disappeared after treatment with high-dose phenobarbital; however, single spasms persisted with right-sided predominance, and polyspike activity in the left parieto-temporal areas preceded or coincided with these spasms. Magnetic resonance imaging revealed a small calcification in the right occipital area, and positron emission tomography showed hypometabolism over the right hemisphere. Widespread epileptic discharges gradually increased on electroencephalography (EEG) during sleep thereafter. The patient presented with daytime unresponsiveness at 1 year and 6 months, when diffuse, irregular spike and wave activity characterized the waking EEG. Spasms or brief tonic seizures with right-sided predominance were provoked by auditory stimuli during this period, particularly by her mother's voice, with ictal EEG of right posterior predominant fast activity and subsequent desynchronization. The administration of clobazam resulted in the marked improvement of EEG findings and transient disappearance of spasms. Presumably, certain patients with asymmetrical epileptic spasms may be regarded as a unique type of localization-related epilepsy, and can show an unusual course of evolution in comparison to other cases of epilepsy that evolve after West syndrome.
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PMID:Non-convulsive status epilepticus and audiogenic seizures complicating a patient with asymmetrical epileptic spasms. 1973 86

Audiogenic kindling (AK) represents a model of naturally occurring epileptogenesis in which intensification of repeatedly induced audiogenic seizures results from propagation of epileptic activity from the brainstem to forebrain. Previously it has been shown that unilateral cortical spreading depression (SD) is a reliable earliest manifestation of mild AK produced by repetition of minimal audiogenic seizures (running) in Wistar rats. The unilateral triggering SD suggests the existence of asymmetry in the forebrain recruitment during the kindling and the present study examined whether epileptogenesis produced by this mild AK paradigm is a lateralized process. Twenty five running episodes were induced by brief sound stimulation in Wistar rats susceptible to audiogenic seizures. Behavioral and EEG correlates of AK development were assessed. Running behavior elicited by brief sound stimulation had an asymmetrical pattern with profound preference for one direction. Most rats expressing leftward running displayed full kindling development whereas the majority of rats with rightward running were resistant to AK. The EEG marker of AK, a cortical epileptiform discharge, was recorded only in rats with leftward running and the first discharge appeared in the left cortex. Cortical SD was recorded after repeated running seizures in all rats with reproducible audiogenic response irrespective of the running lateralization and propensity to kindling. Until the late kindling stages, SD was triggered unilaterally in the cortex ipsilateral to the running direction. These findings indicate intrinsically determined lateralization of epileptogenic process in the mild AK model and enhanced vulnerability of the left hemisphere to epileptogenesis.
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PMID:Interhemispheric difference in susceptibility to epileptogenesis: evidence from the audiogenic kindling model in Wistar rats. 2030 40


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