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Idiopathic focal epilepsies: the “lost tribe” Volume 18, issue 3, September 2016

TEST YOURSELF

(1) What are the epilepsies in the idiopathic focal epilepsy group and what is the evidence that they are related or form part of a spectrum or continuum? What other clinical overlaps exist?

 

(2) Name two advanced electrophysiological techniques discussed here. What has been their contribution to understanding IFEs?

 

(3) What neurodevelopmental complications and comorbidities occur in Rolandic epilepsy?

 

 

 

 

 

 

 

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Answers

(1) The epilepsies commonly considered as part of the IFE group, also known as the epilepsy-aphasia spectrum, include Rolandic epilepsy (BECTS), Panayiotopoulos syndrome (PS), Landau-Kleffner syndrome, and continuous spikes in slow-wave sleep (CSWS). There are three main lines of evidence that support a group identity: clinical, EEG, and genetic. Clinical observations show that individual patients may exhibit overlap symptoms, e.g. RE/PS, or evolution from one to another, e.g. RE to CSWS. The different epilepsies share EEG characteristics including potentiation of sharp wave discharges during sleep, centrotemporal sharp waves seen in different syndromes, and the shared potential evolution to CSWS. Recent genetic findings of mutations in the post-synaptic glutamate receptor subunit reveal GRIN2A mutations varying in frequency between 5% in typical RE and 30% in more severe phenotypes in the epilepsy-aphasia spectrum.


(2) High-frequency oscillations and magnetoencephalography. HFOs may be a marker for neuropsychological regression in CSWS and may also reflect an active state of epileptogenicity. MEG has helped to localise the generators of centro-temporal spikes to the precentral gyrus.


(3) Complications result from seizure activity and include transient impairments in speech, language or oromotor function, including drooling, speech slowdown, and perioral myoclonia. Evolution to CSWS with accompanying cognitive and behavioural disturbance may also be considered a complication. A comorbid condition occurs in association with the primary condition more often than would be expected by chance on the basis of disease frequencies. Mild language, attention, and literacy impairments are well-known comorbidities in RE.

 

 

 

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