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Hemiconvulsion-hemiplegia-epilepsy (HHE) syndrome is an uncommon outcome of prolonged focal status epilepticus in childhood. The prolonged focal motor seizure usually occurs during the course of a febrile illness and is followed by hemiplegia ipsilateral to the side of convulsions. This is accompanied by radiologic evidence of acute cytotoxic edema in the affected hemisphere followed by chronic atrophy. Intractable epilepsy may develop at a time remote from the initial presentation. The clinical features of HHE syndrome were first described more than 5 decades ago but its pathophysiology remains poorly understood and the long-term cognitive outcomes are unclear. Early recognition of the syndrome may help provide patients and families with an accurate prognosis regarding the subsequent development of epilepsy. Neurology ® 2012;79:e1–e4

Although leptomeningeal angioma is the hallmark of Sturge-Weber syndrome (SWS),1 some patients with suspected SWS show no MRI evidence of pial contrast enhancement.2 A girl with a left port wine stain developed right-sided motor seizures. MRI was performed at 18 months of age. Post-gadolinium T1-weighted images …

A 28-year-old woman presented with focal motor seizure involving the right upper extremity, which was well controlled with an antiepileptic agent. MRI demonstrated an arteriovenous malformation. This lesion depicted on MR angiography (figure, A) appeared to involve the sensorimotor cortices on transaxial T2-weighted MR images (figure, B; central sulcus on contralesional hemisphere …

Supplementary motor seizures (SMS) are among the group of frontal lobe seizures that may often be misdiagnosed as pseudoseizures (PS). We designed this study to determine the value of clinical phenomena in distinguishing between the two. In a series of patients with SMS, we identified those with signs and symptoms mimicking PS and compared the clinical phenomena with those of clinically similar PS. We found that SMS are short in duration, stereotypic, tend to occur in sleep, and often present with a tonic contraction of the upper extremities in abduction. This sign was specific for SMS, particularly when occurring at the onset. Conversely, PS are long in duration, nonstereotypic, and occur in the awake state. We conclude that clinical phenomena may be useful in distinguishing PS from SMS, although the final diagnosis must be documented by neurophysiologic means.

The natural course of acid-base changes in arterial blood and lumbar cerebrospinal fluid of patients admitted after major motor seizures was correlated with simultaneously determined lactate levels. In 10 patients with idopathic seizures studied less than 3 hours after the seizure, arterial lactate and cerebrospinal fluid lactate were elevated in association with a mild arterial metabolic acidosis. The elevated cerebrospinal fluid lactate persisted despite a return to normal of the arterial lactate in seven patients studied between 3 and 6 hours after the seizure. All values were normal in five patients studied more than 4 days after a major seizure.

Clinical features were studied in 83 patients with slow spike-wave activity in the EEG. Epileptic seizures, usually intractable, occurred in 82 patients. The majority had the onset of seizures during the first 2 years of life. Minor motor seizures alone or in combination with other types of seizures occurred in 80 percent, and most had more than one type of seizure. The combination of tonic-clonic, minor motor, and absence seizures was the commonest, occurring in 37 percent. Sixty-six patients were definitely retarded and 49 showed definite motor impairment. Etiologic factors responsible for cerebral insult were identified in 53 patients. Serial EEG studies showed a close relationship between the EEG patterns of hypsarrhythmia, independent multifocal spike discharges, and slow spike-wave activity. The eponym "Lennox-Gastaut" syndrome is appropriate for a patient who has slow spike-wave activity in the EEG, exhibits mental retardation, and has intractable seizures of various types. However, the syndrome does not imply a pathologic entity because many diverse processes, both static and progressive, can produce this syndrome.

In a prospective study, the risk of recurrence after a first postneonatal nonfebrile seizure was 61% by age 7 years. The risk of recurrence for nonsymptomatic seizures was considerably higher than for seizures attributed to immediate precipitating factors. Focal motor seizures were more likely than generalized motor seizures to recur. Children who had prior neonatal seizures were at greater risk for nonfebrile recurrence than children with no prior seizure. Family history and neurodevelopmental status were not significantly related to recurrence risk. Almost 90% of recurrences took place within 1 year, and 96% within 2 years.

The clinical and EEG features of 11 patients with seizures arising in the supplementary motor area (SMA) were reviewed. All patients underwent prolonged EEG with simultaneous video recording. Three patients had recordings and electrical stimulation of the SMA using subdural electrode arrays. All patients had preservation of consciousness during the seizure unless it became secondarily generalized. Tonic posturing of the extremities was present in all patients, and in seven it was present bilaterally. Adversive movements were not seen unless the seizure became secondarily generalized. Interictal and/or ictal abnormalities were present at or adjacent to the midline in ten patients. Seizures arising from the supplementary motor region are clinically distinct, and the diagnosis can almost always be verified with prolonged EEG/video recording.

Seizures may occur in acute intermittent porphyria or other hepatic porphyrias. Management is difficult, because barbiturates and hydantoins exacerbate the porphyric state. We studied one patient with major motor seizures and acute intermittent porphyria. The seizure disorder was exacerbated by phenytoin and did not respond to a high-carbohydrate diet or to intravenous hematin. Clonazepam was ineffective in treating the seizures and, in high doses, seemed to exacerbate the porphyria. Both clonazepam and valproate were porphyrinogenic in experimental test systems. Because both drugs may exacerbate the acute hepatic porphyrias, bromide remains the drug of choice to treat these seizures.

Five children, aged 3 to 11 years, treated with carbamazepine for epilepsy, had an acute aberrant reaction characterized by the onset of myoclonic, atypical absence and/or atonic (minor motor) seizures within a few days. When the carbamazepine was discontinued, two of the children returned to their former state very quickly, two had the minor motor seizures resolve in 3 and 6 months, and one had the seizures persist. The child in whom the seizures persisted was later found to have ceroid lipofuscinosis. The other children are doing well on other anticonvulsants.