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interictal states [1]. Synchronization patterns were also found to depend on epileptic
syndromes with primary generalized absence seizures displaying more long-range
synchrony in frequency bands (3-5 Hz) than generalized tonic motor seizures of
secondary (symptomatic) generalized epilepsy or frontal lobe epilepsy [6]. In ad-
dition, we hypothesize that coupling strength and amount of mutual information
between different brain cortical regions exit in the patients with higher severity of
ULD. In addition, the coupling strength and amount of mutual information in brain
cortical regions are positively correlated with the degree of severity (higher UMRS
scores).
Unverricht-Lundborg disease is one type of progressive myoclonic epilepsy
(PME); a rare epilepsy disorder with complex inheritance. It was first described
by Unverricht in 1891 and Lundborg in 1903 [36, 17].
AEDs are the mainstay treatments of ULD with overall unsatisfactory efficacy.
Due to the progression of the severity of myoclonus, the efficacy of AED treat-
ment is difficult to clinically measure especially in the later stages of the disease.
EEG recordings of ULD subjects usually demonstrate abnormal slow background
rhythms and frequent generalized high-amplitude 3-5 Hz spike waves or poly spike
and wave complexes. Sometimes, normal background EEG can be observed be-
tween generalized spike and wave discharges (see Figs. 19.1 and 19.2 for EEG ex-
amples). Studies have shown increased background slowing of EEG or no change
in patients with more advanced ULD stages [4, 9]. Generalized slowing of EEG
Fig. 19.1: Five-second baseline EEG recording.
