Diagnostic approach

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BECTS accounts for 13-23% of all epilepsy in children . The diagnosis of BECTS will be strongly suggested by the clinical semiology of the seizures, with sensorimo-tor seizures without cognitive impairment (simple partial seizures) Typically, the seizures affect facial musculature and may be associated with aphasia or dysarthria, often with drooling The seizures are most likely to occur shortly after sleep onset or before awakening . Diurnal seizures occur in approximately 46% of children . Secondary generalization is not uncommon, and is seen in nearly 50% of patients . Status epilepticus has also been reported, though it is very unusual BECTS is a childhood disorder with the most common age of onset at 7-8 years and within a range of 6 months to 14 years . Postpubertal onset essentially excludes the diagnosis . Many children will experience only a single seizure with three-quarters experiencing five or fewer

A normal developmental history and normal examination support the diagnosis, but abnormal findings should not exclude the diagnosis In addition to seizure semi-ology, the diagnosis of BECTS rests on the presence of typical EEG findings . The interictal EEG will have a normal background Epileptiform activity consisting of high-voltage diphasic or surface-negative spikes with following slow wave is usually present and is usually activated by light sleep. As the syndrome's name indicates, the epileptiform activity is typically midtemporal and/or central . A horizontal dipole may be noted that is negative in the central-temporal region and positive frontally (See Figures 21 . 1 and 21 . 2 .) Atypical spike localization may be seen . Bilaterally independent epileptiform activity is seen in somewhat less than half of the patients Typically, there is marked activation of epileptiform activity in light sleep, and therefore a sleep-deprived EEG (preferably with natural sleep) should be obtained, especially if the waking state EEG is normal Generalized spike-wave discharges may be seen in drowsiness and light sleep, and should not discourage the diagnosis

The presence of CTS in the absence of seizures does not establish the diagnosis of BECTS CTS are seen in up to 3 5% of normal children without a history of seizures and do not in themselves indicate a seizure diathesis CTS may be seen in children with other neurologic disorders such as ADHD as well as in other forms of

Eeg Referential MontageBects Eeg Ref
FIGURE 21.2 The same EEG data as in Figure 21.1 shown on a reference montage to Al. Note the small positivity at Fp2 and F4 consistent with a $ horizontal dipole (open arrow).

epilepsy They occur in a significant proportion of neurologically normal siblings of children with BECTS, supporting an autosomal dominant mode of inheritance for the EEG abnormality but not for the epileptic disorder itself

For the patient with classic features of BECTS, including normal examination, typical seizures, and CTS, neuroimaging is not usually indicated Abnormal medical and developmental history or examination in children with typical features of BECTS do not exclude the diagnosis and do not predict a more ominous course Some children will demonstrate abnormalities on neuropsychological testing, especially relating to language

Children with atypical clinical or EEG features should have imaging performed because treatable CNS abnormalities may be present, including malformations of cortical development Rarely, children with what appears to be typical BECTS at onset may experience a more disabling course, developing other seizure types, becoming medication resistant, and experiencing progressive neurocognitive dysfunction

Video-EEG monitoring is generally not of value in establishing the diagnosis Typically, seizures do not occur with sufficient frequency to be recorded with any confidence Light sleep can almost always be recorded in an appropriate outpatient setting Nocturnal polysomnography may be suggested by the occurrence of events during sleep, but careful history and typical interictal EEG will rule out a primary sleep disorder Although magnetoencephalography (MEG) has been employed for investigation of the pathophysiology of BECTS, MEG has no demonstrated clinical value in this setting

Reports regarding neuropsychological abnormalities in children with BECTS have suggested that neuropsychological testing may be of value in such patients However, in the absence of demonstrated learning and cognitive difficulties, such assessments need not be performed routinely

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