Sleep Wake Transition Disorders

Rhythmic movement disorders such as nocturnal head banging (jactatio capitis nocturna), body rocking, and head rolling typically occur in infants and toddlers as they are trying to fall asleep. They can be present in deep sleep and in wakefulness. They are more common in children with ID. They will typically remit by five years of age but may persist into adult life. Management relies on good sleep hygiene and padding the headboard so the rest of the house is not awakened. Rhythmic movement...

Anxiety Disorders

Anxiety, phobias, and fear are common associations with the diagnosis of epilepsy, paroxysmal fear or anxiety being the most commonly reported aura (30 ) in temporal lobe epilepsy (TLE). Because anxiety is a normal emotion and some anticipatory anxiety may be expected in people with a paroxysmal disorder that is not completely controlled, attention is not always given to anxiety disorders in the clinic setting. A disorder requires that impairment of social, occupational, and other areas of...

Clinical Efficacy of VNS Therapy

A consensus of publishedstudies based on a mixed sample of individuals with and without ID has demonstrated the benefits of VNS Therapy.13 16 26 Initially pilot studies showed the short-term benefit of VNS Therapy in refractory epilepsy.1123 27,28 Uthman and colleagues28 reported findings from two single-blind studies (designated EO1 and EO2) that treated 14 patients (no information given about IQ status). The mean reduction in seizure frequency after 14 to 35 months was 46.6 . Five (35.7 )...

Syncope

Syncope is an abrupt and transient loss of consciousness due to a sudden decrease in cerebral perfusion. It is the condition most commonly confused with epilepsy. Table 2.2 contrasts syncope with epilepsy. Also, like epilepsy, syncope is a clinical diagnosis, for which an eye-witnessed account is essential. There are two main subtypes. The main form of neurocardiogenic syncope is vasovagal syncope this is the commonest cause of loss of consciousness. There is usually a clear precipitating...

Fractures

It is well documented that the rate of fractures for people with epilepsy is higher than for the population as a whole.30 While this is partly due to falls occurring during seizures, other mechanisms also exist. In particular, attention has focused on the impact of anticonvulsants on bone mineral density and the development of osteoporosis. In addition, a further impact may be the sedatory effects of anticonvulsants, which may increase the risk of falls.31 Patients with ID and coexisting...

Nonconvulsive Status Epilepticus

This condition can present at any age, and it is of particular interest to note that it may do so de novo in the elderly, in some cases with no previous history of epilepsy.11 The individual presents with loss of skills, of unexplained origin. Again, this has been discussed in detail elsewhere.10,12 It is certainly a situation that requires urgent assessment and treatment. The clinical presentation of this condition is tremendously variable and, in particular, the degree of additional...

Should a Low IQ Be a Contraindication to Epilepsy Surgery

The first major multicenter study to examine this issue analyzed data retrospectively from over 1,000 adults who had undergone temporal lobe resective surgery in eight centers in the United States6 and had full pre- and postoperative neuropsychological assessments. Only 24 patients (2.3 ) had an IQ less than 70, highlighting the tendency for such patients to not receive resective surgical treatment. This study did show a relationship between preoperative IQ and seizure outcome, but the effect...

Breath Holding Attacks

Breath-holding spells have been described for centuries, but controversy as to what they are remains.25,26 The term breath-holding is not at all satisfactory.27 It tends to give offense to parents of affected children. It seems to imply temper tantrums and bad behavior. In some pediatric textbooks breath-holding attacks are to be found in the section on psychiatric or psychological disorders. However, studies have shown that however one defines breath-holding spells, behavioral disorders in...

NonREM Partial Arousal Disorders Arousal Parasomnias Night Terrors

Brief nocturnal arousals are normal in children and adults. They occur typically in stage IV non-REM sleep disorders, one to two hours after sleep onset. They vary from normal events such as mumbling, chewing, sitting up, and staring to arousals which can be thought of as abnormal because of the disruption they cause the family. There is frequently a family history of parasomnias, and although non-REM partial arousals Arsoual parasomnias Night terrors are primarily a problem of childhood they...

Paroxysmal Kinesigenic Dyskinesia PKD

Typically, onset is in early childhood or adolescence with brief episodes of choreoathetosis, dystonia, or a mixed pattern. Attacks tend to become less frequent or remit totally in adult life. Attacks last seconds to five minutes and are precipitated by sudden movements, change in position, or change in movement velocity.59 Getting up from a chair or getting out of a car frequently trigger attacks. Consciousness is retained, and some individuals may have a brief nonspecific warning or aura...

Electroencephalography EEG

Besides a detailed and comprehensive history, an EEG assessment is the most important diagnostic method in the diagnosis of epilepsy. Obtaining an EEG recording in patients with ID is difficult and time consuming in many cases. This is true especially in timid and restless persons. In those cases it is often helpful to customize the patient slowly to the situation. Providing a familiar situation by allowing the patient to hear her or his favorite music or to bring a familiar object would have a...

Specific Components of the Specialist Epilepsy Nurses Role

Epilepsy specialist nurses have an important role in supporting the overall management of epilepsy in persons with ID. The individual management of epilepsy is essential given that epilepsy impacts people in many different ways. The NICE guidelines state that each person should have a comprehensive care plan, including medical and lifestyle issues, that is agreed upon by the individual and the primary, secondary, or tertiary services. The epilepsy specialist nurse will undertake an initial...

The Narcolepsy Cataplexy Syndrome

Narcolepsy is a disorder characterized by excessive daytime sleepiness, cataplexy a loss of tone in response to strong emotion, typically laughter , sleep paralysis, hypnagogic hallucinations, and disturbed nighttime sleep. A third of adults describe onset before 16 years of age, about 16 before 10 years of age, and around 4 less than 5 years.54 Consciousness is maintained during cataplexy even though the eyes may be closed. Typically the loss of tone spreads from the face down the body. The...

Nurse Led Epilepsy Clinics

Although numerous publications have examined the effectiveness of nurse-led epilepsy clinics, they all tend to focus on secondary care.75-80 The majority of the literature exploring nurse-led clinics tends to be found within the popular nursing press and often extends to only a few pages. There are very few studies that have examined the effectiveness of nurse-led epilepsy clinics within primary care and tertiary care. Such papers tend to be highly positive regarding the clinics, but are...

Driving

Drivers with epilepsy have a greater risk of accidents and road death.14 Decisions on driving eligibility in the UK are made by the Driving and Vehicle Licensing Agency DVLA . Group 1 licenses motorcars and motorcycles require seizure freedom for 12 months, or if the seizures are solely sleep-related, then this pattern must have been established for 3 years prior to obtaining a license. Group 2 licenses trucks gt 3.5 tons, passenger vehicles of 9 seats, and vehicles for hire require seizure...

Loss of Skills Related to Nocturnal Seizures

In a consecutive series of 24-hour video EEG recordings of children and teenagers with epilepsy under my care, most of whom had ID, analysis of the videotapes revealed that a surprising number of these individuals were having frequent nocturnal seizures that were unobserved and unsuspected by awake night staff. Two of the boys in this series had over 200 seizures in a single night. Frequent nocturnal seizures are likely to affect daytime behavior, not only because of postictal effects but also...

Sleep Disorders

Sleep disorders commonly accompany epilepsy obstructive sleep apnea appears twice as commonly in people with epilepsy compared to the background population. There are three main categories of sleep disorders. 2. Parasomnias or sleep-intrusive behavior. 3. Sleep disorders associated with medical or psychiatric illness. Parasomnias are commonly misdiagnosed as epilepsy conversely, nocturnal seizures are commonly mistaken for sleep disorders. The main distinguishing clue is the timing of events...

Diagnostic Evaluation Aims and Questions to be Answered

Together with historical data, the diagnostic evaluation must answer several questions. First, it must determine whether a paroxysmal event is of epileptic nature and whether the patient should be diagnosed with epilepsy. Second, if the diagnosis is epilepsy, it must be assigned to an epilepsy syndrome see Table 4.3 . Third, a possible structural cause of the seizures and ID should be looked for, as well as a possible underlying genetic disorder with epilepsy and ID . Classification with a...

Reflex Anoxic Seizures or Reflex Asystolic Syncope RAS

Gastaut used the term reflex anoxic cerebral seizures to describe all the various syncopes, sobbing spasms, and breath-holding spells that followed noxious stimuli in young children.9 Since 1978, reflex anoxic seizure has been used more specifically to describe a particular type of nonepileptic convulsive event, most commonly induced in young children by an unexpected bump to the head.1 0 Although other terminology, such as pallid white breath-holding and pallid infantile syncope, has been...

Ring Chromosome 20 Syndrome

Ring chromosome 20 is a rare disorder characterized by ring-shaped chromosome 20, learning disabilities, seizures that are generally resistant to AED, behavioral problems, and dysmorphic features. Ring 20 r 20 syndrome was first described by Atkins and colleagues,54 and more than 30 cases have been reported so far. The majority of cases describe severe intractable seizures, but some very rare cases are reported without seizures, probably due to phenotype variation. Ring chromosomes are caused...

Vagovagal Syncope

By contrast to vasovagal syncope, convincing vagovagal syncope is rare. The reflex is usually triggered by swallowing or vomiting cardiac standstill results if the asystole is sufficiently prolonged, with a convulsive syncope anoxic seizure . This is probably not a life-threatening disorder, but the symptoms can be troublesome, particularly if the patient also has migraine with associated vomiting. Pacemaker therapy can be considered if events are frequent and interfering with quality of life.

Dysembryoplastic Neuroepithelial Tumor DNT

Dysembryoplastic neuroepithelial tumors are benign glioneuronal tumors frequently associated with refractory epilepsy in children and young adults.i2 They have characteristic findings on imaging with a mixed signal lesion on MRI which is based in the cortex but may involve the white matter, often with overlying skull abnormalities indicating a chronic lesion.i3 They typically have a disorganized arrangement of neuronal and glial elements without cytological atypia and frequent association of...

VNS in Specific Learning Disabled Persons Landau Kleffner Syndrome and Autism

Landau-Kleffner syndrome LKS is a rare childhood neurological disorder characterized by gradual or sudden loss of ability to comprehend or express spoken language. Children develop LKS between three and nine years of age, and approximately 80 of these children experience seizures. Park48 analyzed retrospective data from the VNS Therapy patient outcome registry Cyberonics, Inc Houston, TX, USA . Six patients mean age at implantation 10.3 4.2 years with LKS were identified. Among the LKS...

Paroxysmal Movement Disorders

There is a complex relationship between epilepsy and movement disorders, the boundaries of which are difficult to define.57 They share many symptoms and are frequently confused with each other. Paroxysmal movement disorders are characterized by variable duration of motor symptoms, usually with few if any interictal abnormalities on examination. Some children with intermediate exertion-related dystonia have subtle dystonia or signs of developmental dys-praxia, even on good days. The major...