Tag Archives: epilepsy

Juvenile Myoclonic Epilepsy

Common form of childhood epilepsy. cf juvenile absence epilepsy.

One or more of:

  1. Myoclonic jerks on waking and first hour of day, esp if tired
  2. Absences (typical) in about half
  3. Tonic-clonic, on waking and first hour of day, esp if tired
  • Precipitated by alcohol, arousal!
  • Mid teens, hence alcohol/arousal…
  • 3-6 Hz spike and wave seen on EEG.
  • Often photosensitive (40%), unlike Juvenile absence epilepsy.
  • Very sensitive to valproate. Else lamotrigine, levetiracetam.
  • Usually life long, despite the name.

Juvenile absence epilepsy

Quite different from childhood absence epilepsy! Less nice.

  • Rare
  • Onset 9-12 years but occasionally younger.
  • Non-remitting, despite the name
  • Longer absences eg 45 seconds, more frequent
  • Automatisms (eg eye flickering, lip smacking), may be able to continue some automatic activities during absence
  • Occasional tonic clonic seizures, myoclonic jerks despite the name! But much less commonly seen than in juvenile myoclonic epilepsy (JME).
  • Not photosensitive cf JME.
  • Treat with valproate, ethosuximide (unless tonic clonic seizures), or lamotrigine.

Valproate – MHRA warning

Updated December 2023

High risk of serious developmental disorders if exposed in womb (up to 30-40% risk, including 5x higher risk of autism) and congenital malformations (10%), including:

  • Spina bifida
  • face/skull malformations, including cleft lip/palate
  • Limb, heart, kidney, genital abnormalities
  • Deafness

In boys, pre-clinical data on transgenerational risks, and animal studies suggesting infertility.

Oral Valproate must not be started in new patients (male or female) younger than 55 years, unless two specialists independently consider and document that there is no other effective or tolerated treatment, or there are compelling reasons that the reproductive risks do not apply.”

Patient guide and checklist available.

Encephalopathy with Status Epilepticus during Sleep (ESES)

Poorly understood complication of some epilepsies.

Clinically, global regression esp cognitive and behavioural, associated with paroxysmal epileptic activity during sleep, which may of course not be recognized.  The classic example is Benign Rolandic Epilepsy, where continuous spike waves develop druing slow wave sleep (CSWS).  Also seen in Landau Kleffner syndrome.

The problem can be unmasked by anti-epileptic medication, especially carbamazepine.  Appears to be associated with brain pathologies eg polygyria, migrational disorders plus some chromosomal problems eg 8p-.

Diagnosis should be considered when unexpected cognitive impairment (eg memory, temperospatial skills, language) or behaviour changes (eg hyperactivity, aggression, disorientation).  Motor impairments eg ataxia and dystonia have been described.


Dramatic increase in EEG abnormalities of any kind during sleep.


Traditional plus newer AEDs have been used, but evidence does not point to any one being superior to any other.

Steroids appear to be effective – unclear whether ACTH or hydrocortisone better.


Poor prognosis (ie long term neuropsychiatric problems) appear to be associated with longer duration of ESES, plus frontal neuropsychological deficits and frontal EEG anomalies.

[Epilepsy Research and Treatment 2012 http://dx.doi.org/10.1155/2012/642725]


Historically, people with epilepsy were “considered to have unique powers, even hailed as geniuses, regarded as having a sacred disease and leading sacred lives”.  But then demonisation, persecution, social rejection. “Epileptic personality” described by psychiatry in 20th century. [Sacred lives, Ian Bone]
Self control is central to our self image and the manner in which we and society believe we should behave. Epilepsy jeopardises this.  Patients often conceal. Guilt, loss of confidence and low mood common after seizures. 

Definition: At least 2 unprovoked (or reflex) seizures, occurring more than 24hrs apart; else one unprovoked (or reflex) seizure and probability of further seizures similar to that seen in those who have had 2 unprovoked seizures (ie at least 60%); or recognized epilepsy syndrome.  Also part of the definition is that epilepsy is considered “resolved” if age dependent syndrome and past applicable age, or else those who have been seizure free for 10 years and off medication for 5 years. (ILAE 2014)

Note that “seizure” does not have any real medical meaning!  Transient signs/symptoms due to excessive or synchronous neuronal activity in brain (ILAE 2017) – but implies you can tell whether caused by abnormal brain activity, which can be hard!

First assessment:


Should not be started after first tonic-clonic generalized seizure. Try not to start before EEG done as may mask features.

Refer to tertiary if –

  • child fails to respond to two AEDs appropriate to the epilepsy in adequate dosages over a period of 6 months (SIGN), or 3 over 12 months (NICE)
  • children less than 2 years with epilepsy as defined

SPEN network has pathways for first seizure, new diagnosis, continuing seizures.

See also Living with Epilepsy