Hyperekplexia

Hyperekplexia is a rare disorder (or group of disorders) that may include dramatic neonatal onset (68) with nonepileptic convulsive syncopes that may prove fatal. Insofar as effective treatment is possible by repeatedly flexing the baby (69), diagnostic awareness should be high. An early major paper on this topic (70) described a dominantly inherited disorder in which there were stiff hypertonic neonates with later pathologic startles. Some confusion has been engendered by the title of this...

Paroxysmal Dyskinesias

Various complex classifications have been proposed for this group of disorders see Fahn (108) for a summary and a history of terminology . The most clinically relevant and simplest is used here. Most of the literature describes familial cases, which are easier to diagnose (especially once one or more affected family members are known) and are possibly more interesting to report than sporadic cases. However, it is our clinical impression that most people with paroxysmal dyskinesias are sporadic,...

Familial Rectal Pain Syndrome

Although the curiously named familial rectal pain syndrome is without doubt very rare, we have had clinical contact with three families and made or seen ictal video-recordings of three children and one adult, supporting the suggestion that this unpleasant disorder is also underdiagnosed (75). Familial rectal pain syndrome is dominantly inherited, but apparently sporadic cases occur. The presenting feature is dramatic neonatal seizures. Schubert and Cracco (76) thought these might be epileptic...

Pitfalls In Diagnosis Of Epilepsy

When epilepsy presents in a classical fashion, with recurrent complex partial or tonic-clonic seizures, accompanied by interictal epileptiform EEG patterns, TABLE I-4 Pitfalls in Diagnosis of Epilepsy Obtaining an inadequate history Overemphasizing the rare and obscure Leading the patient to an inaccurate history Mixed seizures and psychogenic seizures Over-reading the EEG Overinterpretation of a therapeutic trial Incorrect attribution of causation the diagnosis is easy. Unfortunately, the...

Treatment Epileptic Apnea Infant

Apnea, or cessation of breathing for greater than 15 seconds, is usually not due to an epileptic seizure, particularly if apnea is the sole manifestation, and the patient has not been already treated with anticonvulsant medication. Apnea may be secondary to centrally mediated hypoventilation or an obstructive etiology. Apnea occurs commonly in the premature child, especially during active sleep. This apnea of prematurity is likely to be secondary to brainstem immaturity and is typically...

Periodic Limb Movements Of Sleep

In addition to the nonepileptic conditions previously discussed, two sleep related conditions can occasionally be confused with convulsive epileptic seizures. These are periodic limb movements of sleep (PLMS) and REM sleep behavior disorder (RBD) (see also Chapter 16). PLMS is characterized by recurrent muscle contractions, primarily of the lower extremities, during sleep (50-53). Simultaneous movements of the upper extremities may occur. The movements tend to occur in clusters, each movement...

Tonic Reflex Seizures in Early Infancy

A recent publication 146 has highlighted the occurrence of a new form of nonepileptic seizure in the first 3 months of life. Episodes of sudden stiffening occurred in wakefulness in otherwise normal children and were almost exclusively precipitated by holding the infant upright in a vertical posture. The stiffening lasted for a few seconds and was accompanied by apnea and cyanosis, often followed by crying. Whether this is completely different from the awake apnea of gastroesophageal reflux 61...

Md

Eeg Metabolic Encephalopathy

___ he diagnosis of paroxysmal events in I the elderly presents particular difficulties, especially when contrasted with the problem presented by younger patients. In the first instance, the history is often difficult to obtain, vague, or not available at all. A second problem, common in the elderly, is the coexistence of multiple medical and or neurological problems that complicate the analysis of intercurrent paroxysmal symptoms. A third factor involves the confounding results of diagnostic...

Compulsive Valsalva

Children with aberrant development, including those with autistic disorders, may have atonic or more dramatic syncopal seizures compulsively self-induced by something akin to a Valsalva or Weber maneuver 3,54 . Such episodes may be very severe and, indeed, may have a fatal outcome 55 . The child seems able to obstruct the cerebral circulation completely, so that an anoxic seizure results. Perhaps, because of the cerebral abnormality already present, this is one situation in which...

Eeg Seizure Video Patient

Bilateral Seizures

One patient with a clinical picture of mania was treated with haloperidol and lithium until an EEG indicated a diagnosis of photoconvulsive epilepsy. Subsequent treatment with carbamazepine led to long-term remission of her symptoms 116 . A 33-year-old woman presented with disinhibited speech, hyperactiv-ity, generalized amnesia, auditory hallucinations God was talking to her , visual hallucinations, grandiosity, and complete insomnia 1 week after a skiing accident in which she had lost...

Gastroesophageal Reflux

Much has been written about gastroesophageal reflux in infants, but cinematographic or videorecording or full polygraphic registration of a reflux-associated episode that might be described as a seizure has not been reported, though a true reflux episode associated with an epileptic seizure has been described 60 . Nonetheless, there is a persuasively recognizable condition, the awake apnea syndrome 61 . Having been fed within the previous hour, often following an imposed change of posture, the...

Mesial Temporal Lobe Seizures

Anatomy Temporal Lobe Mri

Mesial temporal lobe seizures are by far the most common seizures originating within the temporal structures and are the single most common seizure type seen in the adult population. They represent the typical complex partial seizure. Mesial temporal lobe seizure are often preceded by an aura, which not uncommonly also occurs in isolation 54,55 . The most common aura are epigastric sensations or abdominal auras, which the patient often describes as a rising sensation, nausea, butterflies in the...

Oculomotor Apraxia

Oculomotor apraxia is a condition in which the child has impaired saccadic eye movements. The child appears to have fixed eye positions although the visual system and eye movement ability are normal. Therefore, in order to view an object, the child will turn his head suddenly so as to move the direction of gaze. These peculiar head thrusts may be confused for epileptic seizures. The idiopathic congenital form of this apraxia is called Cogan's oculomotor apraxia. However, oculomotor apraxia can...

Neonatal Seizures

Eeg Neonatal Seizure

Recognition of neonatal epileptic seizures is problematic. Initially, the diagnosis and delineation of neona Nonepileptic Spells in Neonates and Infants tal epileptic seizures was based on a clinical description of a paroxysmal event. However, subsequent studies of neonatal seizure evaluation with long-term electroencephalography EEG disclosed that some of these clinical seizures do not have a consistent elec-trographic ictal scalp correlate. This is particularly the case for subtle seizures...

Nonepileptic Head Drops

Nonepileptic head drops are characterized by there being no difference in the speed of the initial flexion of the neck and the subsequent extension 141 . These authors found that repetitive head-nods, which they described as bobs in which the velocity of recovery matched that of descent and in which the episodes were repeated bobbing , were a consistent feature of nonepileptic head-drops. Defining epileptic nods as those accompanied by epileptic scalp EEG discharges, they found that...

NonREM Partial Arousal Disorders Arousal Parasomnias Night Terrors

Brief nocturnal arousals are normal in children. They occur typically in stage 4 non-REM sleep, 1 to 2 hours after sleep onset. They vary from normal events such as mumbling, chewing, sitting up, and staring to arousals that can be thought of as abnormal because of the disruption they cause the family. These include calm and agitated sleepwalking, and a spectrum from confusional arousals to night terrors or pavor nocturnus. The child may exhibit automatic behavior, but the events are not truly...

Affective Seizures

Any emotion can be expressed during the course of an epileptic seizure 2.2.2.1 . Irritability, anxiety, and anger can occur from amygdalar seizures or stimulation 46,48 . Fear is commonly a component of hippocam-pal seizures 90 . It is often without content and thus takes on the perception of unreality 91 . An affectless expression of fear can occur with cingulate seizures, as can a feeling of happiness 64,71 . Auras may involve the sudden onset of overwhelming fear without dependence on the...

Dyscognitive Seizures

Time distortion, depersonalization, and disorientation are types of cognitive seizures or dyscognitive seizures 2.3 that have all been ascribed to the parahippocam-pal or fusiform gyri 70 . Forced thoughts of a repetitive nature, with or without pressured speech, can be seen in some frontal lobe seizures 71 . In this section, we deal with those seizures affecting attention and language. Seizures involving mood or affect affective 2.2.2.1 , memory mnemonic 2.2.2.2 , or distorted perceptions...

Eeg In Evaluation Of Ambiguous Disorders Various Practical Points

The following pragmatic thoughts may assist in the evaluation of ambiguous disorders An incomplete or inexact description of spells by patients or observers may erroneously mark these spells as factitious. Generalized seizures with incomplete impairment of awareness may superimpose usually unconnected symptoms and signs 104 . One or two EEGs or even inpatient video monitoring may be necessary to unravel the significance of such disparate data. Metabolic, toxic, or vascular mechanisms may...

Monisha Goyal MD Paul Zarkowski MD Barbara E Swartz MD PhD

He word seizure typically invokes a mental picture of a confusional or tonic-clonic event. However, a myriad of epileptic seizure phenotypes are not easily identified and often misdiagnosed as nonepileptic events. These epileptic seizure manifestations involve the motor system having bizarre postures or stereotypies , the autonomic system, the somatic and special senses, and the cognitive and psychiatric domains. The stereotyped nature of these events and a high index of suspicion can help lead...

Focal Clonic Seizures

Focal clonic seizures consist of the rhythmic jerking of unilateral muscle groups without impairment of consciousness. Clonic motor seizures originate in the primary Mesial anatomy of the brain with correlation to certain seizure types. motor cortex Brodmann area 4 contralateral to the motor activity. The muscle groups that are involved strictly follow the cortical motor representation homunculus, Figure 2.4 . As face and hand have the biggest cortical representation, they are the most commonly...

Benign Nonepileptic Infantile Spasms Benign Myoclonus of Early Infancy and Shuddering

We conclude this section on Miscellaneous Neurologic Events with a disorder that is most important in the differential diagnosis of epileptic infantile spasms. We were tempted to have placed it in Psychological Disorders next to Gratification Including Infantile Masturbation and Stereotypies, but as we shall see there are hints that it may sometimes be a marker of deviant nervous system development, albeit to a very mild degree. In the first description 147 and in the most recent publications...

Cyanotic Syncope Breath Holding Spells

Breath holding spells are common events in infants and young children age 6 months to 6 years. The attacks are precipitated by minor injury, frustration, or anger. The events are heralded by crying followed by apnea that typically occurs in expiration. The child then develops significant cyanosis. If the attacks are prolonged, the child loses consciousness and becomes limp. If significant hypoxia occurs, the child may develop tonic posturing or even a few extremity jerks that may be mistaken...

Seizures That Dont Resemble Seizures In Infants

Epileptic seizures in the preverbal infant remain difficult to recognize because of the lack of a verbal description and the lack of reliable identifiers to assess consciousness. Therefore, correctly classifying focal seizures as simple or complex remains a nearly impossible task. Infantile seizures that prove particularly challenging to identify include astatic or atonic seizures that encompass sudden loss of posture, which is typically a brief head drop. Hypokinetic seizures 1.2.6 often arise...

Hyperkinetic Seizures

Video-EEG has familiarized most neurologists with the fencing or M2e postures of supplementary motor The figure demonstrates a young girl who loses motor control of her right hand during seizures reproduced from video . The EEG of the event indicated at vertical line did not show a clear ictal build-up but demonstrated epileptiform potentials at Cz-Pz arrow during the event. On this and subsequent EEG figures, the calibration bar indicates 50 V, the green vertical line corresponds to the time...

Evaluation Of The Spells Patient Examination

The key to spell diagnosis usually is in the history. The physical examination is of relatively limited value in the diagnosis of epilepsy, unless the examiner is able to examine a patient during a spell. Astute examiners rarely may observe clues to a syndrome associated with epilepsy, such as multiple caf au lait spots or adenoma sebaceum, suggestive of tuberous sclerosis 99 , or papilledema, indicative of increased intracranial pressure. Physical findings are more useful in diagnosing certain...

Rem Behavior Disorder

The second sleep disorder that may mimic convulsive epileptic seizures is REM behavior disorder RBD 58,59 . RBD is characterized by recurrent episodes of vigorous motor activity occurring during REM sleep, movements that often awaken the patient or spouse. The primary presenting complaints are interrupted sleep or injuries during sleep. RBD is more common in older adults, usually beginning after the age of 50. It is two to five times more common in men than in women. It is possible that rare...

Shuddering Attacks

Shuddering attacks may begin in infancy, as early as 4 months, or in childhood. The events consist of a rapid tremor of the head, shoulder, and trunk suggestive of the shuddering episodes from a chill. The duration of the events is brief, lasting only seconds, but the events may occur multiple times a day. The spells are often associated with eating and may represent a pattern of stimulation overflow in a young child. The electro-graphic pattern during these spells is normal. No other...

Discussion

The terms psychogenic seizure, pseudoseizure, and nonepileptic seizure refer to episodes that behaviorally resemble an epileptic seizure, but are secondary to a psychogenic and not an epileptic mechanism. In this chapter, a convulsive psychogenic event refers to a psy-chogenic event characterized by vigorous motor activity that may superficially resemble a convulsive epileptic seizure see also Chapter 19 . A number of observable features may assist in differentiating convulsive psychogenic...

Contributors

Montreal Neurological Institute and Hospital Professor of Neurology and Pediatrics Montreal Neurological Institute and Hospital Associate Professor of Clinical Neurology Columbia University College of Physicians and Surgeons Director, Clinical Anticonvulsant Drug Trials Assistant Professor Department of Neurology Emory University School of Medicine Atlanta, GA Department of Clinical Neurological Sciences The University of Western Ontario London Health Sciences Centre London, Ontario Canada...

Paroxysmal Torticollis

Torticollis is an abnormal sustained posture of the head and neck in which the head tilts to one side and the face rotates to the opposite side. In paroxysmal torticollis, the events begin and end suddenly. The attacks can be brief or prolonged. The child is alert and responsive during an attack although the patient may appear uncomfortable and irritable. The EEG is normal during the event. The etiology of the attacks is unknown, although both a focal dystonia and labyrinth dysfunction have...

Paul B Pritchard III MD

Rolactin PRL is a peptide secreted II by acidophilic pituitary lactomorph I cells under the dual control of a dopaminergic prolactin inhibiting factor PIF and serotonergic prolactin releasing factor PRF . Thyrotropin releasing factor TRF promotes the release of PRL and thryrotopin TSH , whereas estrogen releases PRL via direct stimulation of the pituitary 1 . Normal adults exhibit little change in serum PRL during waking hours, except for brief elevations after naps 2 . Multiple nocturnal PRL...

Changes In Serum Prolactin With Brain Stimulation

The first recognition of episodic hyperprolactinemia induced by seizures were the observations of investigators 11 who were seeking endocrine profiles to predict which patients would benefit from a full course of electrocon-vulsive therapy ECT . Although endocrine changes did not predict the likelihood of successful ECT in women with depression, reproducible elevations in serum PRL followed ECT. ECT produces similar endocrine changes in schizophrenics 12,13 . Subsequent to the ECT reports,...

Paroxysmal Dyskinesia

In 1981, Lugaresi and Cirignota described a group of patients who had brief nocturnal episodes characterized by tonic or dystonic spasms and violent movements 36 . All had normal interictal and ictal EEGs, and all responded favorably to carbamazepine. The authors proposed three possible mechanisms for these events i pavor nocturnus ii epileptic seizure or iii paroxysmal dystonia. Similar cases, with many variations, have been described by others 37-40 , usually under the moniker, paroxysmal...

Vasovagal Syncope

Vasovagal syncope is the most familiar and predominant form of neurally mediated syncope. If classical reflex anoxic seizures with reflex asystolic syncope represent a fairly pure vagal attack, vasovagal syncope involves a vasodepressor component with variable vagal accompaniment. Episodes may begin in infancy, sometimes with reflex anoxic seizures, and thereafter are seen at all ages, becoming most dramatic perhaps in old age 21 . Tables in medical textbooks or works of epileptol-ogy tend to...

Benign Neonatal Sleep Myoclonus BNSM

The major importance of benign neonatal sleep myoclonus 92 is that it may be misdiagnosed as epilepsy and even treated with such heavy doses of antiepileptic medication that the neonate ends up in the intensive care unit on a ventilator 1 . Recognition is easy for someone who has seen a videotape of the condition in which the baby has repetitive, usually rhythmic but possibly arrhythmic, jerks of one or more limbs only during sleep. In some instances, there is a report of the occasional jerk in...