Mandatory | Alerts | Exclusionary | |
---|---|---|---|
Seizures | Myoclonus of orofacial muscles, triggered by reading/language tasks | Myoclonic seizures predominantly affect upper limbs | All other seizure types, except generalized tonic–clonic seizures |
EEG | Slow background (outside the postictal period) | ||
Age at onset | >20 years | ||
Development at onset | Normal | ||
Neurological exam | Normal | ||
Imaging | Normal | ||
Are MRI or ictal EEG required for diagnosis? An MRI is required for diagnosis to exclude a structural cause An ictal EEG is not required, observation of orofacial myoclonus during reading/language task (either directly, or on video) is highly recommended |
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Syndrome without laboratory confirmation: in resource-limited regions, this syndrome can be diagnosed in a patient who meets all mandatory criteria and have no exclusionary seizure types |
NOTE Alert criteria are absent in the vast majority of patients with the syndrome, but rarely can be seen. Their presence should result in caution in diagnosing the syndrome and consideration of other conditions