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JUVENILE MYOCLONIC EPILEPSY (JME)

  Mandatory Alerts Exclusionary
Seizures Myoclonic seizures Generalised tonic-clonic status epilepticus
Consistent focal features at seizure onset
Myoclonic absence, atonic, tonic, atypical absence or focal seizures
Myoclonic seizures predominantly/exclusively from sleep
Myoclonic seizures exclusively with reading
Cortical tremor with myoclonus
EEG 3-5.5Hz generalized spike- or poly-spike wave   Myoclonic event captured on EEG without spike- or poly-spike wave discharge
Focal slowing or consistently focal epileptiform abnormality
Generalised slow (<2.5Hz) spike-wave (measured at the beginning of a spike-wave discharge)
Diffuse background slowing
Age at onset   8-9 or 25-40 years <8 or >40 years
Development at onset   Mild intellectual impairment Moderate or greater intellectual impairment
Neurological exam   Abnormal exam  
Imaging   Abnormal, potentially relevant  
Course of illness     Progressive cognitive decline
Progressive myoclonic seizures with impaired fine motor function
Are MRI or ictal EEG required for diagnosis?
An MRI is not required for diagnosis
An ictal EEG is not required
Syndrome without laboratory confirmation: in resource-limited regions, this syndrome can be diagnosed in patients without alerts who meet all other mandatory and exclusionary criteria

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

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