Figure 235

Occipital paroxysms with fixation off sensitivity of an 11-year-old boy with idiopathic childhood occipital epilepsy. Occipital paroxysms occur immediately after and as long as fixation and central vision are eliminated by any means (eyes closed, darkness, plus 10 spherical lenses, Ganzfeld stimulation). Under these conditions, even in the presence of light, eye opening does not inhibit the occipital paroxysms. Conversely, occipital paroxysms are totally inhibited by fixation and central vision. Symbols of eyes open without glasses indicate conditions in which fixation is possible. Symbols of eyes with glasses indicate conditions in which central vision and fixation are eliminated. At age 18 years, he is entirely normal and is not receiving medication.

These syndromes may mimic symptomatic occipital lobe epilepsies, and neuroimaging, preferably MRI, is indicated. No other investigations, except EEG, are routinely required.

Treatment. Given the frequency of seizures in idiopathic childhood occipital epilepsy, including the likelihood of occasional GTCS, regular AED treatment is considered necessary in most if not all subjects. There are no controlled studies comparing alternatives, although carba-mazepine appears to be most often used in subjects who are not photosensitive. It is appropriate to attempt withdrawal after two seizure-free years, although there is a significant risk of relapse.

Some subjects with idiopathic photosensitive occipital lobe epilepsy who are only mildly photosensitive and who do not have spontaneous seizures can remain seizure free by avoiding precipitants. Others will require AED treatment. Broad spectrum agents, such as sodium valproate and levetiracetam, active against focal and generalized seizures and photosensi-tivity, would appear to be reasonable choices. However, it appears that carbamazepine, not usually considered a useful drug for photosensitivity, may sometimes be effective.

Course and Prognosis. The prognosis for both idio-pathic childhood occipital epilepsy and idiopathic photosensitive occipital lobe epilepsy is variable. A majority of the former, perhaps 50% to 60%, have remission of seizures within 2-4 years of them starting. However, in a significant minority seizures will continue into adulthood. In those with idiopathic photosensitive occipital lobe epilepsy who are only mildly photosensitive and can control their exposure to relevant provoking factors, freedom from seizures may be easy. For others, particularly those who are highly photosensitive, the likelihood of seizures continuing into adult life is high.

Atypical Evolutions of the Benign Focal Epilepsies of Childhood

Less than 1% of children with rolandic epilepsy have so-called atypical evolutions (24, 32, 83). These include the development of severe linguistic, cognitive, or behavioral problems. If such problems develop in a child with rolandic epilepsy, a sleep EEG should be obtained, because continuous spike-and-wave during slow-wave sleep (CSWS) may be present. The Landau-Kleffner syndrome is sometimes said to develop from rolandic epilepsy. CSWS may also be seen in children with opercular status characterized by continuous positive or negative myoclonias around the mouth or elsewhere in the face and pseudobulbar problems. Atypical focal epilepsy of childhood in which other seizure types, including tonic and atypical absence seizures, occur may also develop in children with otherwise typical rolandic epilepsy.

There are also case reports of atypical evolutions in Panayiotopoulos syndrome, including the development of absences and drop attacks (32, 84, 85) and in idiopathic childhood occipital epilepsy with cognitive deterioration and CSWS (86).

Carbamazepine is sometimes implicated in precipitating such atypical evolutions (87, 88).

Other Described Benign Focal Epilepsies of Childhood

The syndromes discussed previously are the only benign focal epilepsies of childhood currently recognized by the ILAE. However, others have been proposed and are more or less well characterized. They include the following.

Benign Childhood Seizures with Affective Symptoms (89). This is reported to have its onset between 2 and 9 years of age and is characterized by multiple, usually short, daytime and nighttime seizures in which the predominant symptom appears to be fear or terror, accompanied by autonomic disturbances (pallor, sweating, abdominal pain, and salivation), arrest of speech, and mild impairment of consciousness with automatisms. Interictal EEG shows sharp and slow wave complexes similar to those in rolandic epilepsy but located in the frontotemporal and parietotemporal electrodes. Remission in 1 to 2 years from onset is expected. This is likely to be an intermediate phenotype between Panayiotopoulos syndrome and rolandic epilepsy.

Benign Childhood Epilepsy with Parietal Spikes and Frequent Giant Somatosensory Evoked Potentials (28, 90). This putative disorder is mainly defined by its interictal EEG features reflected in its name. These features are, however, said to often be associated with a phenotype characterized by mainly daytime versive seizures, which are infrequent and have an excellent prognosis.

Benign Childhood Focal Seizures Associated with Frontal or Midline Spikes (5). Again this putative disorder is mainly defined by its interictal EEG features. These EEG features can be seen in children with febrile seizures, rolandic epilepsy, Panayiotopoulos syndrome, and idio-pathic childhood occipital epilepsy.

Benign Focal Epilepsy in Infants with Central and Vertex Spikes and Waves During Sleep (91, 92). Benign focal epilepsy in infants with central and vertex spikes and waves during sleep has been recently described as a new benign syndrome. In terms of age of onset, it is on the borderline between benign infantile seizures and Panayio-topoulos syndrome. Age at onset is in the first 2 years of life with both sexes equally affected. Infants are normal and all tests other than EEG are normal. Seizures consist mainly of staring, motor arrest, facial cyanosis, loss of consciousness, and stiffening of the arms. Clonic convulsions and automatisms are rare. Duration is from 1 to 5 minutes. Seizures are mainly diurnal (but may also occur during sleep) and may occur in clusters, but are generally infrequent (1-3 per year). Interictal EEG abnormalities are seen only in non-REM sleep and consist of small, mostly singular, spikes and waves local/zed at the vertex and central electrodes.

There is a strong family history of epilepsy with benign epilepsies prevailing. The prognosis is excellent with remission of seizures, normal development, and normalization of the EEG before the age of 4 years.

Benign Focal Seizures of Adolescence (5, 93). This syndrome of the second decade, and predominantly occurring in males, features a single seizure or a single cluster of seizures over a period of up to 36 hours. The seizures are mainly diurnal, with consciousness initially preserved. The main manifestations are focal clonic jerking, usually without a Jacksonian march, and somatosen-sory symptoms. Secondary GTCS occur in about 50% of cases. EEG and brain neuroimaging are normal. The prognosis is excellent and treatment is not required.

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