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Paroxysmal Non-Epileptic Events 0021-7557/02/78-Supl.1/S73 Jornal de Pediatria - Vol. 78, Supl.1 , 2002 S73 Jornal de Pediatria Copyright © 2002 by Sociedade Brasileira de Pediatria REVIEW ARTICLE Paroxysmal non-epileptic events Márcio A. Sotero de Menezes* 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. J Pediatr (Rio J) 2002; 78 (Supl.1): S73-S88: epilepsy, convulsions, seizures, paroxystic non-epileptic events, syncope, movement disorder, tetany, hyperekplexia, vertigo. Introduction About 10-30% of the children referred to neurology- least in part epileptic seizures.4 These events show epilepsy clinics have an incorrect diagnosis.1-3 According resemblance with one or many of the aspects of epileptic to Bye and Nunan up to 1/3 of the patients with suspected seizures such as loss of consciousness, behavioral arrest, seizures evaluated with video-EEG have in actuality non- autonomic changes and repetitive motor or psychological epileptic events. A large variety of events may mimic at behavior. This review aims at discussing only some of the most common paroxysmal non-epileptic seen in pediatric neurology practice. The tables are organized alphabetically but not all the diseases mentioned included in the tables are * Associate Professor, Department of Neurology and Pediatrics, Washington discussed. Table 1 lists alphabetically the most common University. Co-Director of the Pediatric Epilepsy Program, Children’s types of paroxysmal non-epileptic events, which are seen in Hospital and Regional Medical Center. Seattle, Washington, USA. pediatric neurology practice. S73 S74 Jornal de Pediatria - Vol. 78, Supl.1, 2002 Paroxysmal non-epileptic events - Sotero de Menezes MA Table 1 - Paroxysmal non-epileptic events Paroxysmal movement disorders Benign neonatal myoclonus Benign myoclonus of early infancy Benign paroxysmal tonic upgaze Benign paroxysmal torticollis Benign paroxysmal vertigo Dystonia with diurnal variation (a.k.a. Segawa syndrome) Hyperekplexia Paroxysmal ataxia (with or w/o response to acetazolamide) Paroxysmal non-kinesiongenic dystonia-chorea Paroxysmal kinesiongenic dystonia-chorea Shuddering attacks Stereotypies Others paroxysmal activity Apnea (especially infants) Breath-holding spells Cyclic vomiting Jitteriness of infants and neonates (physiological, hypoglycemic, hypocalcemic) Masturbation Psychogenic seizures (pseudoseizures) Sandifer syndrome SIDS - sudden infant death syndrome Spasmus nutans Syncope Transient cerebral hypoperfusion-hypoxemia causing hypotonia), followed by complete flattening of the EEG paroxysmal signs and symptoms tracing during the tonic phase. As the hypoxia gets corrected Among the most common seizure imitators are breath- the slow waves re-appear and at the same time that a few holding spells and syncope. We start this review with these jerks of the limbs are seen before resumption of normal 7,8 two entities because they are very common in the general EEG activity. population but also due to their similar physiopathology. The basis of the symptoms of both syncope and breath- holding spells is a decrease in perfusion and delivery of Syncope oxygen delivery to the brain leading to energy metabolism Syncope is a commonly confused with epileptic seizures. failure. Authoritative sources list a few critical values which Syncope accounts for about 1% of pediatric emergency in an adult individuals would lead to CNS dysfunction: room visits.9,10 One study the prevalence of syncope among bradycardia of less than 40 beats per minute, tachycardia of adults was 3% for men and 3.5% for women.11 One third to more than 150 beats per minute, asystole of more than four half of the cases of syncope show when followed up for 5 seconds, systolic pressure of less than 50 mmHg or venous years.12 02 pressure of less than 20 mmHg.5,6 This cortical hypoxia- ischemia leads to a sequence of event including: loss of During a syncope there is a transient cerebral consciousness and of postural tone, increase in tone hypoperfusion, followed by spontaneous recovery which is (decorticate/opisthotonus) followed by a few beats of clonus. associated loss of consciousness and postural tone.7,13-15 This sequence is probably due to liberation of corticoreticular An interruption of cerebral blood flow lasting 8 to 10 inhibition. Cortical hypoxia produce slowing of frequency seconds causes loss of consciousness.13 There are many of the brain electrical activity (loss of consciousness- causes and triggers of syncope (see Table 2). Paroxysmal non-epileptic events - Sotero de Menezes MA Jornal de Pediatria - Vol. 78, Supl.1 , 2002 S75 Table 2 - Causes-precipitators of syncope Cardio-inhibitory syncope of infants (a.k.a. breath holding spells) Cardiovascular Atrio-ventricular conduction abnormalities (AV heart-block) Cardiac arrhythmias (long QT syndrome) Congenital Heart Disease Rheumatic Heart Disease Congestive Heart failure Drug-induced syncope Hyperventilation syndrome-related syncope Neurocardiogenic Situational-valsalva-parasympathetic activation syncope: cough, defecation, deglutition, diving, hair grooming, micturition, sneezing, trumpet-playing, weight-lifting Suffocation syncope Psychogenic syncope The diagnosis of syncope rests mainly on clinical postural tone, which in the prolonged events is followed by grounds.16 The syncope is often precipitated by assuming tonic posturing and a brief clonic activity. Less frequently the upright posture (orthostasis), prolonged standing, heat, incontinence may be seen. After the syncope a spontaneous fatigue, hunger, Valsalva maneuver, physical or emotional recovery without persistent neurologic deficits is the rule stress including venipuncture, pain, and fear, are also but nausea, pallor, diaphoresis and fatigue may be seen. The commonly identified.17,18 Several activities which produce recovery takes place in less than an hour.15 Even though a valsalva maneuver and/or parasympathetic activation can most cases syncope in children are benign and self-limited cause syncope including cough, defecation, deglutition, falls may lead to physical injuries. diving, hair grooming, micturition, sneezing, trumpet- Even though syncope is usually can be distinguished playing, weight-lifting.6,19,20 Other stimuli include getting 8,21 22 from epilepsy both by the clinically and EEG picture in into or out of a bath, or stretching. Less commonly some cases both diseases may co-exist. syncope may result from primary cardiac events (see primary cardiac syncope below). Syncope is often preceded by a sensation of lightheadedness, blurred vision, things going far away Syncopes of cardiac origin (sometimes described as tunnel vision), pallor, nausea, Syncopes of cardiac origin should be considered apart epigastric discomfort or diaphoresis.13,15 In most cases since they require a different focus in their work-up. Syncopes the loss of tone is characterized by a progressive and slow of cardiac are less frequent than reflex vasovagal or slumping to the ground. Less frequently the loss of posture cardioinhibitory attacks.6 The conditions that cause these may be abrupt with a sudden fall which may be associated syncopes are associated with sudden death, which is the with tongue lacerations. The latter the cuts are typically in reason why it is important to recognize them. Both congenital the tip of the tongue and tongue biting with lateral laceration and acquired (rheumatic) aortic stenosis that can produce occurs only exceptionally in syncopes.8,23 Urinary syncopes on effort and the same is true for cardiomyopathies incontinence is not uncommon reflecting cortical inhibition and disturbances of cardiac conduction. Among the and does not indicate an epileptic mechanism. conduction abnormalities associated with syncope are the The loss of consciousness during a syncope usually lasts long Q-T syndromes, Wolff-Parkinson-White syndrome, from a few seconds to a few minutes. During the period of and congenital atrioventricular block.24 Among the cases impairment of consciousness the patient has decreased showing a long Q-T on the EKG there are those with the S76 Jornal de Pediatria - Vol. 78, Supl.1, 2002 Paroxysmal non-epileptic events - Sotero de Menezes MA Jervell-Lange-Nielsen syndrome features an associated Breath-holding spells (Cardio-inhibitory or Pallid neurosensory deafness and is recessively inherited and the syncope and Cyanotic breath holding spells) Ward-Romano syndrome, which is dominantly transmitted.
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