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 LearningDisabled Persons LandauKleffner 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...