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Early-onset benign occipital seizure susceptibility syndrome
Insights
A variant of childhood epilepsy with occipital paroxysms (CEOP) featuring nonvisual symptoms like head deviation and vomiting may warrant separate classification as early-onset benign occipital seizure syndrome (EBOSS). This distinct syndrome shows excellent prognosis.
Area of Science:
- Neurology
- Epileptology
- Pediatric Neurology
Background:
- Childhood epilepsy with occipital paroxysms (CEOP) typically presents with visual hallucinations and occipital EEG abnormalities.
- A variant of CEOP has been noted, characterized by nonvisual symptoms such as head/eye deviation, vomiting, and status epilepticus.
Purpose of the Study:
- To fully document the electroclinical features of patients with this CEOP variant.
- To determine if the distinct clinical presentation justifies a separate classification from typical CEOP.
Main Methods:
- A multicenter study involving the submission of patient data by investigators.
- Inclusion criteria focused on idiopathic occipital seizures with ictal head/eye deviation and vomiting.
Main Results:
- 113 patients were recruited, with seizures starting in early childhood (mean age 4.6 years).
- Common symptoms included ictal eye deviation (79%), vomiting (70%), and head deviation (35%).
- Partial status epilepticus occurred in 44%, and 74% had occipital interictal EEG abnormalities; prognosis was excellent with a mean active seizure duration of 1 year.
Conclusions:
- Despite shared EEG features with CEOP, distinct clinical symptoms suggest a separate classification is warranted.
- The proposed name for this variant syndrome is early-onset benign occipital seizure syndrome (EBOSS).
Purpose:
Childhood epilepsy with occipital paroxysms (CEOP) is characterised by ictal visual hallucinations and occipital epileptiform activity on interictal EEG. A variant has been described with nonvisual symptoms including tonic head and eye deviation, vomiting, and episodes of partial status epilepticus. We fully documented the electroclinical features of such patients to determine whether classification separate from CEOP is justified.
Methods:
This was a multicentre study with participating investigators submitting details of patients with idiopathic occipital seizures characterised by ictal head or eye deviation and vomiting.
Results:
One hundred thirteen patients were recruited. Seizures began in early childhood (mean, 4.6 years) and occurred infrequently (mean total seizures, 3); 30% of patients had only a single seizure. Two thirds of seizures were nocturnal. Ictal eye deviation occurred in 79%, vomiting in 70%, and head deviation in 35%. Seizures were predominantly complex partial in type. Partial status epilepticus occurred in 44% of patients. Seventy-four percent of patients had occipital interictal EEG epileptiform activity, predominantly right sided, with fixation-off sensitivity. Extraoccipital EEG abnormalities occurred in 35% of patients. Prognosis was excellent: the mean duration of active seizures was 1 year.
Conclusions:
Although the two groups shared identical EEG features, the distinct clinical symptoms probably justify separate classification. Early-onset benign occipital seizure syndrome (EBOSS) is suggested as an appropriate name for the variant group.