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Mandatory seizures

  • Reflex myoclonic seizures in response to reading. These are low-amplitude myoclonic jerks, mainly affecting the masticatory, oral, and perioral muscles (jaw, lip, tongue). They can cause a clicking sensation, stuttering, or altered speech. The reading time to seizure onset varies from patient to patient and in individual patients. In an individual patient, the reading trigger may be specific e.g. when only when reading silently but not when reading aloud, only when reading a specific language, or topic (e.g. reading music).
May have
  • If the patient continues reading after myoclonus appears, the myoclonus can increase in severity, spread to trunk and limb muscles, and have associated focal impaired awareness, or evolve to a tonic-clonic seizure.
  • Orofacial myoclonic jerks may be precipitated not only by reading, but also by other language-related tasks (language-induced seizures), e.g. when talking (including specifically e.g. argumentative), when writing, or when making complex decisions. Hand myoclonic jerks are seen in those with writing precipitation of seizures.
  • A minority of patients with this syndrome have been described to have co-occurring ocular and visual ictal manifestations (e.g., blinking, difficulty with ocular fixation, nystagmus, complex visual hallucinations)
  • Spontaneous myoclonus can rarely occur
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