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      Distúrbios paroxísticos não-epilépticos Translated title: Paroxysmal non-epileptic events

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          Objetivo: este artigo tem como objetivo discutir um dos principais problemas com os quais um pediatra geral tem que lidar no campo da neurologia infantil, que são os distúrbios paroxísticos não-epilépticos. Estes episódios também são uma causa freqüente de consultas aos neuropediatras e de internação em unidades de monitorização por vídeo-eletrencefalograma. Fontes dos dados: revisão da literatura sobre o assunto na Medline. Vários compêndios de neurologia pediátrica também foram usados, por conterem informações importantes sobre o assunto. Síntese dos dados: muitas das entidades discutidas neste artigo são freqüentes na população pediátrica, como, por exemplo, a síncope, as crises de perda do choro, e os movimentos patológicos associados ao refluxo gastroesofágico. Outras entidades são mais raras, como as distonias paroxísticas e a distonia com flutuação diurna. Conclusões: o conhecimento básico das várias síndromes associadas com distúrbios paroxísticos não-epilépticos é extremamente importante para o pediatra geral, porque pode evitar exames desnecessários e o diagnóstico errôneo de epilepsia, expondo as crianças às medicações que não vão melhorar o quadro clínico, e que podem causar efeitos colaterais.

          Translated abstract

          Objective: this publication aims at reviewing one of the most important problems faced by the pediatrician in the field of child neurology. The paroxistic non-epileptic events are also a frequent reason for pediatric neurology consultations and admission for diagnostic video-eletroencephalogram monitoring. Methods: literature review on the subject was perform on Medline, data was also collected from the main Pediatric Neurology Textbooks, which were found to be important and unique source of information on the subject. Results: many of entities discussed in this paper are very common in the pediatric population like syncope, breath-holding spells and the movement disorders associated with gastroesophageal reflux. Other syndromes are less frequent such as the pararoxymal dystonias and the Segawa Syndrome (dystonia with diurnal variation). Conclusions: the basic knowledge of these syndromes is very important since it may avoid unnecessary procedures and the wrongful diagnosis of epilepsy. Patients who are mistakenly diagnosed as epileptics are exposed to anti-convulsant medications, which are probably not going to be effective and may expose them to the risk of side effects.

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          Most cited references 125

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          Mutations in the alpha 1 subunit of the inhibitory glycine receptor cause the dominant neurologic disorder, hyperekplexia.

          Hereditary hyperekplexia, or familial startle disease (STHE), is an autosomal dominant neurologic disorder characterized by marked muscle rigidity of central nervous system origin and an exaggerated startle response to unexpected acoustic or tactile stimuli. Linkage analyses in several large families provided evidence for locus homogeneity and showed the disease gene was linked to DNA markers on the long arm of chromosome 5. Here we describe the identification of point mutations in the gene encoding the alpha 1 subunit of the glycine receptor (GLRA1) in STHE patients from four different families. All mutations occur in the same base pair of exon 6 and result in the substitution of an uncharged amino acid (leucine or glutamine) for Arg271 in the mature protein.
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            Evaluation and outcome of patients with syncope.

             Shikha Kapoor (1990)
            We studied 433 patients with syncope to derive insights into the diagnostic evaluation and outcome of patients with this common problem. This study shows that the etiology of syncope was not found in approximately 41% of patients. When a cause of syncope was determined, it was most frequently established on the basis of initial history, physical examination and an electrocardiogram (EKG). Furthermore, many of the other entities (e.g., aortic stenosis, subclavian steal) were suggested by findings on the history and physical examinations that required directed diagnostic testing. Initial EKG was abnormal in 50% of patients but led to a cause of syncope infrequently (less than 7%). Prolonged electrocardiographic monitoring, which has assumed a central role in the evaluation of syncope, led to a specific cause in only 22% of patients. Other tests were less often helpful in assigning a cause of syncope. At 5 years, the mortality of 50.5% in patients with a cardiac cause of syncope was significantly higher than the 30% mortality in patients with a noncardiac cause or 24.1% in patients with an unknown cause. At 5 years, a mortality of 50.5% in patients with a cardiac cause of syncope was noted. There were 54 actual deaths in this group as compared to 10.7 expected deaths based on 1980-86 mortality data from Allegheny County, PA (standardized mortality ratio = 5.02). At 5 years, a 33.1% incidence of sudden death was noted in patients with cardiac cause of syncope, as compared with 4.9% in patients with a noncardiac cause and 8.5% in patients with an unknown cause. Mortality and sudden death remained significant for the first 3 years after which the survival curves were parallel. A cardiac cause of syncope was an independent predictor of sudden death and mortality. Recurrences were common but were not associated with an increased risk of mortality or sudden death. Major vascular events were also more frequent in patients with cardiac causes of syncope. The results of this study will be helpful in designing future studies to evaluate the usefulness of newer diagnostic techniques. Furthermore, short- and long-term outcome data will be useful in planning intervention strategies in these patients.
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              Syncope: a videometric analysis of 56 episodes of transient cerebral hypoxia.

              To investigate the clinical features of transient cerebral hypoxia, syncope was induced in 56 of 59 healthy volunteers through a sequence of hyperventilation, orthostasis, and Valsalva maneuver. All events were monitored on video by two cameras. Complete syncope with falling and loss of consciousness was observed in 42 subjects, lasting 12.1 +/- 4.4 seconds. Myoclonic activity occurred in 38 of these 42 episodes (90%). The predominant movement pattern consisted of multifocal arrhythmic jerks both in proximal and distal muscles. Superposition of generalized myoclonus was common. Additional movements such as head turns, oral automatisms, and righting movements occurred in 79%. Eyes remained open throughout syncope in most subjects and initial upward deviation was common. Sixty percent reported visual and auditory hallucinations. Thirteen subjects had incomplete syncope with falls but partially preserved consciousness. These episodes were shorter and usually not accompanied by myoclonus and hallucinations. Transient amnesia and unresponsiveness without falling occurred in 1 subject.

                Author and article information

                Jornal de Pediatria
                J. Pediatr. (Rio J.)
                Sociedade Brasileira de Pediatria (Porto Alegre, RS, Brazil )
                August 2002
                : 78
                : suppl 1
                : S73-S88
                orgnameChildren's Hospital
                orgnameUniversidade de Washington orgdiv1Departamentos de Neurologia e de Pediatria
                Washington orgnameRegional Medical Center EUA
                S0021-75572002000700011 S0021-7557(02)07800011

                This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License.

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