Seizures



Seizures


Katherine S. Taub

Nicholas S. Abend



INTRODUCTION

A seizure is an episode characterized by altered behavior, consciousness, movement, sensation, or autonomic function due to hypersynchronous excessive firing of a network of neurons. The differential diagnosis of seizures is listed in Table 68-1. Generalized seizures rapidly engage bilaterally distributed networks. Focal seizures begin in one portion of the brain with symptoms related to that region’s function. Prior classification systems separated focal seizures into simple partial seizures (remain restricted to a region without affected alertness), complex partial seizures (spread to involve adjacent regions such that consciousness is affected), and secondarily generalized seizures (spread widely to involve the entire cortex). In the most recent International League Against Epilepsy (ILAE) classification, the terms simple and complex are replaced with the more descriptive phrases “without impairment of consciousness or awareness” and “with impairment of consciousness or awareness,” respectively, and can also be designated as “evolving to a bilateral, convulsive seizure.”

Seizures may occur in an individual with an underlying tendency to have recurrent seizures (epilepsy) or may be secondary to an ongoing process that primarily or secondarily affects the brain (acute symptomatic seizures). The initial evaluation of a patient with a seizure must focus on identifying and treating any underlying condition that may be causing seizures. Prolonged or multiple recurrent seizures without return to baseline mental status are called status epilepticus. Types of seizures are listed in Table 68-2.

Epilepsy is defined by recurrent unprovoked seizures. In more detail, the ILAE defines epilepsy as a disorder of the brain characterized by an enduring predisposition to generate epileptic seizures and by the neurobiologic, cognitive, psychological, and social consequences of this condition. Epilepsy may be classified based on the underlying etiology as genetic, structural-metabolic, and unknown. Genetic refers to epilepsy caused by a known or presumed genetic defect. The genetic mutation may be known, such as a mutation in a sodium ion channel (e.g., SCN1A mutation) or may be suspected due to a family history of seizures. Structuralmetabolic includes epilepsy related to an underlying structural or metabolic etiology. Structural defects include cortical malformations (e.g., dysplasias, tubers) or acquired defects such as strokes or trauma. Unknown indicates that the etiology of the epilepsy is not known and may be related to an undiagnosed genetic defect or an unrecognized disorder. In some patients, the clinical history, age, seizure type, and electroencephalographic (EEG) findings can be grouped into an epilepsy

syndrome that can guide management and suggest prognosis. Common pediatric epilepsy syndromes are listed in Table 68-3 and described in the following.








TABLE 68-1 Nonseizure Etiologies of Transient Neurological Events























Neurological




  • Stroke/transient ischemic attack



  • Migraine headache



  • Movement disorders



  • Reflex movements


Cardiac




  • Vasovagal syncope (which may be followed by a seizure in syncopal convulsion)



  • Arrhythmia



  • Hypotension


Endocrine/metabolic




  • Hypoglycemia



  • Hyponatremia Sleep disorders



  • Hypnic jerks



  • Benign sleep myoclonus



  • Parasomnias



  • Narcolepsy/cataplexy


Psychological




  • Nonepileptic psychogenic seizures (pseudoseizures)—conversion disorder


Others




  • Breath holding



  • Masturbation



  • Gastroesophageal reflux



  • Behavior



  • Apparent life-threatening events









TABLE 68-2 Classification of Epileptic Seizures by ILAE 2010 Terminology





























Generalized


Tonic-clonic


Absence




  • Typical absence



  • Atypical absence



  • Absence with special features (myoclonic absence, eyelid myoclonia)


Myoclonic




  • Myoclonic



  • Myoclonic atonic



  • Myoclonic tonic


Clonic


Tonic


Atonic


Focal seizures




  • With impairment of awareness or consciousness



  • Without impairment of awareness or consciousness



  • Evolving to bilateral convulsive seizure


Unknown




  • Epileptic spasms (including infantile spasms)




DIFFERENTIAL DIAGNOSIS LIST


Neurological Etiologies


Neurovascular



  • Ischemic stroke


  • Sinovenous thrombosis


  • Intracerebral hemorrhage


  • Subarachnoid hemorrhage


  • Arteriovenous malformation


  • Hyperperfusion syndrome


Tumor



  • Primary


  • Metastatic


Infection



  • Meningitis


  • Encephalitis


  • Abscess


  • Cat-scratch disease


  • Tick-borne disorders


Inflammatory



  • Acute disseminated encephalomyelitis


  • Vasculitis


  • Lupus cerebritis


  • Rasmussen


Trauma



  • Contusion


  • Epidural hematoma


  • Subdural hematoma


  • Subarachnoid hemorrhage


  • Depressed skull fracture

Neurosurgery


Inherited



  • Neurodegenerative disorders


Congenital and Intrauterine



  • Brain malformations


  • Neonatal hypoxic ischemic injury


  • Congenital infections

Primary Epilepsy


Systemic Etiologies

Hypoxia-ischemia


Drug Toxicity



  • Amphetamines


  • Cocaine


  • Phencyclidine


  • Local anesthetics


  • Antipsychotics


  • Antidepressants


  • Immunosuppressants


  • Antibiotics


Drug Withdrawal



  • Benzodiazepines


  • Barbiturates


  • Opiods


  • Alcohol

Febrile Infection (not involving the central nervous system [CNS])


Metabolic Abnormalities



  • Hypoglycemia


  • Hyponatremia


  • Hypophosphatemia


  • Renal dysfunction


  • Hepatic dysfunction


Metabolic Disorders



  • Urea cycle disorders


  • Aminoacidopathies


  • Organic acidurias


  • Mitochondrial encephalopathies


  • Storage diseases


  • Vitamin B6 (pyridoxine) deficiency


  • Folinic acid-responsive seizures


  • Nonketotic hyperglycinemia










TABLE 68-3 International League Against Epilepsy Classification of Electroclinical Syndromes and Other Epilepsies































Neonatal period




  • Benign familial neonatal epilepsy (BFNE)



  • Early myoclonic encephalopathy (EME)



  • Ohtahara syndrome


Infancy




  • Epilepsy of infancy with migrating focal seizures



  • West syndrome



  • Myoclonic epilepsy of infancy (MEI)



  • Benign infantile epilepsy



  • Benign familial infantile epilepsy



  • Dravet syndrome (severe myoclonic epilepsy of infancy)



  • Myoclonic encephalopathy in nonprogressive disorders


Childhood




  • Febrile seizures plus (FS+)



  • Panayiotopoulos syndrome (early onset benign childhood occipital epilepsy)



  • Epilepsy with myoclonic atonic seizures



  • Benign epilepsy with centrotemporal spikes (BECTS, Rolandic epilepsy)



  • Autosomal dominant nocturnal frontal lobe epilepsy (ADFNLE)



  • Late onset childhood occipital epilepsy (Gastaut syndrome)



  • Epilepsy with myoclonic absences



  • Lennox-Gastaut syndrome



  • Landau-Kleffner syndrome (LKS)



  • Epileptic encephalopathy with continuous



  • spike-and-waves during sleep (CSWS)



  • Childhood absence epilepsy (CAE)


Adolescence-adult




  • Juvenile absence epilepsy (JAE)



  • Juvenile myoclonic epilepsy (JME)



  • Epilepsy with generalized tonic-clonic seizures alone



  • Progressive myoclonic epilepsies (PME)




    • Lafora body disease



    • Unverricht-Lundborg syndrome (Baltic myoclonus)



    • Neuronal ceroid lipofuscinosis



    • Myoclonic epilepsy with ragged red fibers (MERRF)



    • Sialidoses



  • Autosomal dominant epilepsy with auditory features (ADEAF)



  • Reflex epilepsies




    • Photosensitive occipital lobe epilepsy



    • Primary reading epilepsy



    • Startle epilepsy


Distinctive constellations




  • Mesial temporal lobe epilepsy with hippocampal sclerosis



  • Rasmussen syndrome



  • Gelastic seizures with hypothalamic hamartoma



  • Hemiconvulsion-hemiplegia-epilepsy


Epilepsies attributed to and organized by structural-metabolic causes




  • Malformations of cortical development (e.g., hemimegalencephaly, heterotopias)



  • Neurocutaneous syndromes



  • Tumor



  • Infection



  • Trauma



  • Angioma



  • Perinatal insults



  • Stroke


Conditions with epileptic seizures that are traditionally not diagnosed as a form of epilepsy




  • Benign neonatal seizures (BNS)



  • FS



DIFFERENTIAL DIAGNOSIS DISCUSSION


Benign Epilepsy with Centro-Temporal Spikes (Rolandic Epilepsy)

Onset is typically between 2 and 13 years, with most between 5 and 10 years. It most often presents with predominantly nocturnal seizures that include salivation, clonic mouth movements, and gurgling sounds. Daytime seizures are less common and generally consist of speech arrest and face/arm clonic movements with preserved consciousness. These children have normal cognitive function and a normal neurological examination. EEG demonstrates sharp waves in one or both central-temporal regions. Antiepileptic drug treatment is considered optional, and it is usually easy to control seizures if treatment is initiated. The syndrome is often outgrown by adolescence.

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Sep 14, 2016 | Posted by in PEDIATRICS | Comments Off on Seizures

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