- Benign childhood epilepsy with centrotemporal spikes (Rolandic seizures)
- Gastaut-type idiopathic childhood occipital epilepsy
- Panayiotopoulos syndrome
Benign childhood focal seizures are the most common and probably the most fascinating and rewarding topic in pediatric epileptology. They affect 25% of children with non-febrile seizures. Seizures are infrequent, usually nocturnal, and remit within 1 to 3 years from onset. Brief or prolonged seizures, even status epilepticus, may be the only clinical event of the patient’s lifetime. Ictal autonomic manifestations are frequent and may occasionally appear in isolation as in pure autonomic seizures. Febrile seizures are common. Neurological examination and intellect are normal, but some children may experience mild and reversible cognitive problems during the active stage of the disorder. Brain imaging is normal.
Severe EEG abnormalities of high-amplitude focal spikes are disproportionate to the frequency of seizures. A normal EEG is rare and should provoke a sleep EEG study. Similar EEG features resolving with age are frequently found in normal school-age children (2% to 4%) and children having an EEG for reasons other than seizures.
Recognized syndromes with benign childhood focal seizures are:
- Benign childhood epilepsy with centro-temporal spikes (Rolandic seizures/epilepsy) (widely known by pediatricians)
- Gastaut type-idiopathic childhood occipital epilepsy (a pure form of idiopathic occipital epilepsy that occurs in older children)
- Panayiotopoulos syndrome (despite high prevalence and often dramatic autonomic seizures, this syndrome has only recently been recognized but again misdiagnosis as non-epileptic disorder is common)
The clinical and EEG characteristics of one syndrome may evolve into another or a child may simultaneously develop features of another form of benign childhood focal seizures. All these conditions may be linked together in a broad age-related and age-limited benign childhood seizure susceptibility syndrome, which may also constitute a biological continuum with febrile seizures and benign infantile and benign neonatal seizures.
The epileptic syndromes and their significance
A major advance in recent epileptology is the recognition of epileptic syndromes that allows an accurate diagnosis and management of seizure disorders.[1-3]
Medical diagnosis is the identification of a disease by investigation of its symptoms and history, which provides a solid basis for the treatment and prognosis of the individual patient. An accurate diagnosis is the golden rule in medicine, and epilepsies should not be an exception to this. Like in any other disease, the recognition of non-fortuitous clustering of symptoms and signs in epilepsies requires the study of detailed clinical and laboratory data.[1-3] However, often in current practice, the diagnosis is limited to either epilepsy or seizures, which is unsatisfactory because this cannot provide guidance on important items such as severity of the disease, prognosis, short- and long-term therapeutic decisions, and genetics (research and counselling), which are all factors that crucially affect personal, family, and social life; education; and career choices of patients. Defining the type of epilepsy should now be considered mandatory as it offers the best guide to both management and prognosis. Most epileptic syndromes and diseases are well defined and easy to diagnose. The benefits of syndromic diagnosis over seizure/symptom diagnosis or an inclusive diagnosis such as epilepsy far outweigh any morbidity from incorrect categorization that may arise in difficult cases.[4]
Important clinical features of a syndrome include the type of seizures, their localization, frequency, sequence of events, circadian distribution, precipitating factors, age at onset, mode of inheritance, physical or mental symptoms and signs, prognosis, and response to treatment.
Epilepsies or epilepsy?
The clinical and practical significance of the syndromic diagnosis of epilepsies is well illustrated by 3 common epileptic disorders. Benign childhood focal epilepsies, juvenile myoclonic epilepsy (JME), and hippocampal epilepsy have nothing in common other than the fact that they may all be complicated by generalized tonic clonic seizures (GTCS), which are primarily GTCS in JME and secondarily GTCS in benign childhood focal epilepsies and hippocampal epilepsy.
Furthermore, the short-and long-term treatment strategies are entirely different for each disorder: benign childhood focal epilepsies may or may not require medication for a few years, appropriate anti-epileptic drug (AED) treatment is lifelong in JME while neurosurgery may be life-saving for patients with hippocampal epilepsy. What may be a life-saving drug such as carbamazepine for hippocampal epilepsy may be ill-advised for JME.
It should not be difficult to distinguish an intelligent child with benign focal seizures or childhood absence epilepsy from a child with Kozhevnikov-Rasmussen, Lennox-Gastaut, Down, or Sturge-Weber syndrome or a child with severe post-traumatic cerebral damage, brain anoxia, or catastrophic progressive myoclonic epilepsy. Describing all these children as simply having epilepsy just because they have seizures offers no more benefit than a diagnosis of febrile illness irrespective of cause, which may be a mild viral illness, a life-threatening acute bacterial meningitis, or a malignancy. Inappropriate generalizations with regard to terminology, diagnosis, and treatment are the single most important factor of mismanagement in epilepsies.[4]
C. P. Panayiotopoulos, MD, PhD, FRCP
Reviewed and revised June 2008 by Steven C. Schachter, MD
Back to topPrevalence
~15% of children with non-febrile seizures aged 1 to 15 years.
Incidence
10 to 20/100,000 children aged 0 to 15 years.
Age at onset
1 to 14 years, 3/4 start between 7 and 10 years; peak at 8 to 9 years.
Sex
1.5 male preponderance.
Neurological and mental state
Normal.
Genetics
Linkage to chromosome 15q14 in one study. Siblings or parents with the same seizures are rare. Febrile seizures are common (10% to 20%).
Clinical manifestations
Infrequent focal seizures of unilateral facial sensory-motor symptoms, oro-pharyngo-laryngeal manifestations, speech arrest, and hypersalivation.
Hemifacial seizures (1/3 of patients) consist of clonic contractions of the lower lip, sometimes spreading to the arm. Inability to speak and hypersalivation are common concurrent symptoms.
Oro-pharyngo-laryngeal manifestations are unilateral numbness and dysesthesia inside the mouth, cheek, teeth, and tongue alone or usually with motor phenomena producing strange sounds such as death rattle, gargling, guttural sounds, and their combinations.
Consciousness is retained in 58%.
Duration is ~1 to 2 min but lasts longer if seizures progress to convulsions (1/3).
Reversible linguistic and learning abnormalities, usually mild, may be common.
Timing
75% in sleep.
Inter-ictal EEG
Normal background with centrotemporal spikes (CTS), which are abundant, usually in clusters, and markedly accentuated during sleep. In 10% to 20%, these are also evoked by somatosensory stimuli. Rarely, CTS occur only during sleep, the spikes may be very small or the EEG may be normal.
CTS occur in 2% to 3% of normal school-age children and in a variety of organic brain diseases. They are age-dependent and disappear before age 16.
Frequency, location, and persistence of CTS do not determine clinical manifestations, severity, and frequency of seizures or prognosis.
Ictal EEG
Slow waves and spikes, with onset from the Rolandic regions.
Prognosis
Excellent; <2% develop epilepsy. Seizures are infrequent (10% to 20% singular) and remit <16 years of age. 10% to 20% may have frequent seizures.
Less than 1% progress to severe syndromes of linguistic, behavioral, and neuropsychological deficits.
Differential diagnosis
Other epilepsies.
Management options*
Continuous AED therapy may not be needed. Most clinicians use carbamazepine for recurrent seizures though this AED may exceptionally cause marked aggravations. Clobazam#, levetiracetam and sulthiame# are other possible alternatives.
*Expert opinion, please check FDA-approved indications and prescribing information
#Not approved by the FDA
See also: http://professionals.epilepsy.com/page/syndromes_benign.html
This page was adapted from:
The educational kit on epilepsies
The epileptic syndromes
By C. P. Panayiotopoulos
Originally published by MEDICINAE
21 Cave Street, Oxford OX4 1BA
First published 2006 and reprinted in 2007
Reviewed and revised June 2008 by Steven C. Schachter, MD
Prevalence
~2% to 7% of benign childhood focal seizures.
Age at onset
3 to 15 years; mean ~8 years.
Sex
Males = females.
Neurological and mental state
Normal.
Genetics
Probably genetically determined.
Clinical manifestations
Frequent visual seizures of mainly elementary visual hallucinations, blindness, or both. Ictal elementary visual hallucinations consist of small multicolored circular patterns that often appear in the periphery of a visual field. They develop fast in sec and last for several sec to 1 to 3 min. These may progress and co-exist with other occipital symptoms (sensory illusions of ocular movements and ocular pain, tonic deviation of the eyes, repetitive eye closures). Complex visual hallucinations, visual illusions, and other symptoms from anterior spreading may terminate with hemiconvulsions or generalized convulsions. Ictal blindness usually appears suddenly and usually lasts for 3 to 5 min.
Ictal headache, mainly orbital, occurs in 1/10 of patients.
Consciousness is usually intact. It becomes impaired with seizure progression.
Spreading to temporal lobe involvement is exceptional and may indicate a symptomatic cause.
Post-ictal headache, sometimes indistinguishable from migraine headache, occurs in half of patients.
Timing
Predominatly in alert stages.
Diagnostic procedures
All tests other than EEG are normal. High-resolution MRI is mandatory because symptomatic occipital epilepsies may have similar clinico-EEG manifestations.
Inter-ictal EEG
Occipital paroxysms, often with fixation off sensitivity. Others may have only random occipital spikes, some may have occipital spikes in sleep EEG alone, and a few may consistently have normal EEG. Whether occipital photosensitivity is part of this syndrome or not is debated.
Ictal EEG
Occipital discharge of fast rhythms, fast spikes, or both.
Prognosis
Relatively good. Half of patients will remit within 2 to 4 years from onset. The others will continue having visual seizures and infrequent secondarily generalized tonic-clonic seizures, particularly if not appropriately treated with carbamazepine.
Differential diagnosis
Migraine, symptomatic occipital epilepsy, and coeliac disease with visual seizures.
Management options*
Drug treatment, mainly with carbamazepine, is probably mandatory.
*Expert opinion, please check FDA-approved indications and prescribing information
See also: http://professionals.epilepsy.com/page/syndromes_occipital.html
This page was adapted from:
The educational kit on epilepsies
The epileptic syndromes
By C. P. Panayiotopoulos
Originally published by MEDICINAE
21 Cave Street, Oxford OX4 1BA
First published 2006 and reprinted in 2007
Reviewed and revised June 2008 by Steven C. Schachter, MD
Prevalence
13% of children with non-febrile seizures aged 3 to 6 years, 6% in the age group 1 to 15 years.
Age at onset
3 to 6 years in 74% of cases; range 1 to 14 years.
Sex
Males = females.
Neurological and mental state
Normal.
Genetic and other factors
Febrile seizures in ~17%. Occurrence in other siblings is exceptional. High incidence of abnormal birth deliveries.
Clinical manifestations
The seizures mainly manifest with autonomic symptoms. Emesis often culminating to vomiting is the most common (70% to 80%) but is not always present or apparent. Others include pallor, incontinence of urine and feces, mydriasis or miosis, cardio-respiratory irregularities, hypersalivation, thermoregulatory changes, and cephalic sensations. Ictal syncope (unresponsive and flaccid) is common before or without convulsions.
Behavioral ictal features are irritability or quietness, patient looks or feels unwell.
Consciousness is usually intact at onset but severely disturbed in the seizure progression.
Other ictal features include eye deviation (60% to 80%); half progress to hemiconvulsions or generalized convulsions. Visual hallucinations are rare (10%) and do not appear at the onset.
Duration is long, typically 5 to 10 min; nearly half have autonomic status epilepticus (>30 min).
Timing
2/3 of the seizures occur in sleep.
Inter-ictal EEG
Multifocal spikes are the more common; occipital spikes +/- extra-occipital spikes predominate, extra-occipital spikes (1/3) or brief generalized discharges may occur, 1/10 have normal EEG.
Ictal EEG
Focal slow waves intermixed with spikes. May start posteriorly or anteriorly.
Prognosis
Excellent, remission within 1 to 2 years, 1/3 of patients have a single seizure, only ~10% have >10 seizures; 1/5 (21%) may develop other seizure types, mainly Rolandic (13%), which are usually infrequent and remit before the age of 16 years. Risk for later epilepsy is the same as in normal population. However, exceptionally autonomic seizures may be life-threatening causing cardio-respiratory arrest.
Differential diagnosis
Encephalitis, atypical migraine, syncope, gastroenteritis, unspecified epilepsy, febrile convulsions, sleep disorder, motion sickness.
Management options*
Continuous AED therapy is not recommended for children with a single seizure or brief seizures. For recurrent seizures, most clinicians use carbamazepine. Clobazam#, levetiracetam and sulthiame# are other possible alternatives. Lengthy seizures are a medical emergency; rectal diazepam is prescribed for home administration.
*Expert opinion, please check FDA-approved indications and prescribing information
#Not approved by the FDA
See also: http://professionals.epilepsy.com/page/syndromes_occipital.html
This page was adapted from:
The educational kit on epilepsies
The epileptic syndromes
By C. P. Panayiotopoulos
Originally published by MEDICINAE
21 Cave Street, Oxford OX4 1BA
First published 2006 and reprinted in 2007
Reviewed and revised June 2008 by Steven C. Schachter, MD
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