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A healthy 9-year-old girl is brought to the emergency room after her parents witnessed her having an unusual event shortly after going to bed. They reported hearing gurgling noises coming from her room. When they found her, she was sitting up in bed and unable to speak. She had jerking of her right lower face with excessive drooling. She appeared awake during the spell. It stopped spontaneously after 2 minutes. She has had a cold but otherwise has been well recently.PHYSICAL EXAMINATION
General Examination: Normal. Neurologic Examination: Mental Status: Alert and cooperative. Language: Fluent without dysarthria. Cranial Nerves: II through XII intact. Motor: She has normal bulk and tone with 5/5 strength throughout. Coordination: There is no dysmetria on finger-to-nose testing. Sensory: No deficits are noted. Gait: She has a normal heel, toe, flat, and tandem gait. Reflexes: 2+ throughout with bilateral plantar flexor responsesDISCUSSIONThe patient is a healthy 9-year-old girl who presents after a simple partial seizure involving right facial jerking, drooling, and speech arrest. Her neurologic exam is normal.LocalizationRight facial twitching as well as speech arrest localize to the left centrotemporal, rolandic region.Differential Diagnosis
The patient in this case presents with the classic features of a focal seizure in the setting of BECTS, but the diagnosis of BECTS can be made only when both the clinical history and the EEG findings are consistent with this syndrome. Other localization-related epilepsies caused by underlying structural lesions (trauma, cerebrovascular disease, tumor, neuronal migration abnormalities, mesial temporal sclerosis) should also be considered.Benign childhood epilepsy with centrotemporal spikes (BECTS) or benign rolandic epilepsy is the most common form of idiopathic epilepsy in childhood. The peak ages of onset are between 5 and 10 years. However, onset may occur in children as young as 3 years or as old as 13 years. Affected children are typically developmentally and intellectually normal. There is a strong genetic predisposition to epilepsy in BECTS. A gene on chromosome 15q14 has been implicated in some families.The seizures in BECTS are brief and infrequent. Approximately 10% of children will experience only one seizure. In the majority of children (70%), seizures will occur only two to six times. Twenty percent of patients will experience more frequent seizures. In approximately 70% of children, seizures occur only at night. The seizures in BECTS have a characteristic semiology, involving hemifacial clonic movements, speech arrest, dysarthria, and excessive drooling. Preceding paresthesias around the mouth, gums, cheek, or lips may occur. During the seizure, there may be involvement of the ipsilateral arm or leg, as well as secondary generalization. The seizures usually last 1 to 2 minutes.The EEG background in BECTS is normal in the awake and sleep states. Epileptiform paroxysms are described as focal, diphasic spike-and-slow-wave activity of medium to high voltage located over the rolandic or centrotemporal regions. Epileptiform discharges may occur either unilaterally or independently and bilaterally. A horizontal dipole with maximum spike negativity over the central and temporal regions with positivity in the frontal regions is also a classic EEG finding. Characteristically in this disorder, spike-and-wave activity increases in frequency and amplitude during drowsiness and nonrapid eye movement (REM) sleep. Centrotemporal spikes may occur only during sleep in approximately 30% of children.Spontaneous resolution of seizures by adulthood (18 years of age) occurs in almost all patients with BECTS and, thus, it is given the designation of a benign epilepsy. Some academic and behavioral problems have been reported in children with BECTS, but the overall prognosis for intellectual and neurologic outcome is excellent
Dr. Jayaprakash
Superintendent, ICH.
Institute of Child Health.
Gov. Medical College Kottayam.
Kerala, India.