Epilepsy syndromes represent clinical entities in which :
Q-age
Q-pattern of clinical events
Q-EEG features
Q-natural history
Q-prognosis
*are distinctive
1-
Benign childhood epilepsy with centrotemporal spikes :
begin between 5-10 years
typically occur only during sleep , or on awakening in more than half of patients
also known as Benign rolandic epilepsy
it is among most common epilepsy syndromes
affected children usually have focal motor seizures involving the face , arm (abnormal movement or sensation around the face and mouth , drooling , rhythmic guttural sound )
speech and swallowing may be affected
seizures sometimes secondarily generalize
interictal EEG demonstrate independent bilateral centrotemporal sharp waves but is otherwise normal
with classic history and EEG , and normal neurological examination , the diagnosis can be made with confidence and neuroimaging not required
prognosis : seizures usually respond promptly to anticonvulsant therapy , intellectual outcome is normal , and epilepsy resolves after puberty , comorbid learning difficulties and attention deficit /hyperactivity disorder (ADHD) are common
#centrotemporal spikes , focal seizure , ADHD common , hx,exam,eeg required , imaging not required
2-
childhood absence epilepsy :
another common syndrome , begin in the early school years , and usually resolve by late childhood or adolescence
if absence seizure does not remit , 44% will go on to develop juvenile myoclonic epilepsy
ethosuximide is the first-choice therapy
A subset of patients also have generalized tonic-clonic seizures , for these children , valproic acid is the first choice as it can prevent both absence and convulsive seizures
comorbid learning disabilities and ADHD are common , ther eis no contraindication to treating these children's ADHD with stimulant medication
3-
juvenile myoclonic epilepsy :
(of janz)is the most common generalized epilepsy among adolescents and young adults
onset typically in early adolescence with myoclonic jerks (exacerbated in the morning , often causing the patient to drop objects), generalized tonic-clonic seizures, and absence seizures
seizures usually resolve promptly with anticonvulsant medication , classically valproic acid , but several medications have been shown to be efficacious , but therapy must be maintained for life
4-
infantile spasm :
are brief contractions of the neck , trunk, arm muscles , followed by a phase of sustained muscle contraction lasting less than 2 seconds
spasms occur most frequently when the child is awakening form or going to sleep
each jerk is followed by a brief period of relaxation with repeated spasms in clusters of variable duration , many clusters occur each day
5-
west syndrome :
triad of infantile spasm , developmental regression , dramatically abnormal EEG pattern (hypsarrhythmia)
hypsarrhythmia consists of chaotic high-voltage slow waves ,spikes and polyspikes
the peak age at onset is 3-8 months , when flexion of the thighs and crying are prominent
the syndrome may be mistaken for colic or gastroesophageal reflux
the underlying etiology of the spasms dictates the prognosis , more than 200 different etiologies have been identified , including :
tuberous sclerosis , malformations of cortical development (lissencephaly),
genetic syndrome (trisomey 21), acquired brain injury (stroke , perinatal hypoxic-ischemic encephalopathy ),and metabolic disorders(phenylketonuria)
infants for whom , an etiology is determined , are classified as having symptomatic infantile spasms and are at very high risk for long-term neurodevelopmental difficulties .
the etiology is not determined for a small subset of children , these patients with cryptogenic spasms have somewhat better long-term prognosis
-but remain at high risk of adverse outcome .
first line treatment options for infantile spasms includes :
1-adrencorticotropic hormone
2-high dose oral corticosteroid
3-vigabatrin
for infants with underlying tuberous sclerosis , vigabatrin is considered the treatment of choice ,
for other patients , treatment determinations are made on a case-by-case basis
6-
Lennox-Gastaut syndrome :
is a severe epilepsy syndrome
with variable age of onset , most children present before age 5 years
frequent , multiple seizure types , including atonic , focal , atypical absence , and generalized tonic , clonic , or tonic-clonic varieties , characterize the disorder , many children have underlying brain injury or malformations , the seizures usually respond poorly to treatment , and most patients have significant intellectual disability
7-
Benign neonatal convulsions :
are an Autosomal dominant genetic disorder , linked to abnormal neuronal potassium channels , otherwise well newborns present with focal seizures toward the end of the first week of life , leading to the colloquial term fifth-day-fits ,
response to treatment is generally excellent , and the long term outcome is typically favorable
8-
status epilepticus :
is neurologic emergency and is defined as
ongoing seizure activity or repetitive seizures without return of consciousness for greater than 30 minutes , status epilepticus carries an approximately 14% risk of new born neurologic deficits , most secondary to the underlying pathology , similarly , the mortality rate of status epilepticus (4% to 5%), is related to the underlying etiology
etiology include :
-new onset epilepsy of any type
-drug intoxication
-drug withdrawal (especially missed anticonvulsant doses among children with preexisting epilepsy )
-hypoglycemia ,hypoxia , electrolyte imbalance
-acute head trauma ,intracranial hemorrhage ,ischemic stroke
-infection
-metabolic disorders .
the first priority of the treatment is to ensure an adequate airway , breathing , circulation vital signs should be obtained and oxygen administered if needed , if respiration are inadequate , positive pressure ventilation may be required , intravenous (IV) access should be obtained , in patients with no history of seizures , laboratory evaluation should be undertaken , diazepam distributes rapidly to the brain but has short duration of action , if iv access is not available , liquid preparations may be administered per rectum , if the seizure does not resolve after two doses of benzodiazepine , a second-line agent must be administered , either IV phenytoin or fosphenytoin is effective , but cardiac monitoring is required to evaluate for arrhythmia , if the seizure persist , a loading dose of phenobarbital or valproic acid is appropriate
Laboratory and Diagnostic evaluation
EEG is the most useful neuro-diagnostic test for distinguishing seizures from nonepileptic paroxysmal disorders and for classifying seizures as having focal or generalized onset , the EEG must be interpreted in the context of the clinical history , because some normal children have focal or epileptiform EEG patterns
-children with seizures may have normal interictal EEG ,
when the diagnosis is unclear , more sophisticated EEG with prolonged recordings and simultaneous video monitoring of the patient in an attempt to capture a typical event may be necessary
MRI is superior to CT in showing most brain pathology , but in the emergency department setting , CT can be performed rapidly and often shows acute intracranial hemorrhage more clearly than MRI , MRI is unnecessary in patients with the primary generalized epilepsies , such as typical absence and juvenile myoclonic epilepsy
even when the clinical examination and EEG do not suggest focal features , identification of some lesions , such as focal cortical dysplasia , hamartoma , and mesial temporal sclerosis , can assist in consideration of surgical treatment of pharmacoresistat epilepsy
