The Rolandic epilepsy/ESES/Landau-Kleffner Syndrome and correlation with Language impairment study

In clinical practice language impairment is frequently reported in association
with nocturnal epileptiform activity. There is a spectrum of epileptic
conditions that are characterized by nocturnal epileptiform activity: Rolandic
epilepsy, nocturnal frontal lobe epilepsy, Landau-Kleffner syndrome and
electrical status epilepticus of sleep. The exact characteristic of the
relationship between nocturnal epileptiform activity and language impairment is
yet to explore. We suggest that nocturnal epileptiform EEG discharges and
nocturnal epileptic seizures during development will cause diseased neuronal
networks that involve language. The diseased neuronal networks are less
efficient compared with normal neuronal networks.

Identification of a diseased neuronal network characteristic in children with
nocturnal epileptiform activity, which can explain language impairment in these
children

An observational and clinical comparative case-control study, in children with
nocturnal epileptiform activity and healthy controls. About 25 children
diagnosed with Rolandic epilepsy, LKS, ESES-like ESES will be investigated. 20
healthy matched control children will be tested. Group differences in neuronal
network architecture will be examined, and the MR parameters will be correlated
to the language impairment.
EEG-parameters will be correlated to MR and language impairment.

This study involves minors who are unable to give informed consent. Following
the WMO guidelines, the *not unless* principle applies to granting permission
for this study. The MRI-techniques and neuropsychological assessments that are
applied in this study are non-invasive. We use a 3.0 Tesla MRI instead of a 1.5
Tesla MRI. The risks of a MRI-scan are negligible because it is a magnetic
field, does not involve ionizing radiation and does not require contrast agents
or anaesthetics. The brain imaging will be more detailed by using a 3.0 Tesla
MRI compared with a 1.5 Tesla MRI. Another reason why we use 3.0 Tesla MRI
instead of 1.5 Tesla MRI is that a 1.5 Tesla MRI is not available at the
epilepsy centre Kempenhaeghe. Furthermore, according to the literature, there
are no statistically significant difference between 1.5 Tesla MRI and 4.0 Tesla
MRI for headache, tinnitus, hiccupping, vomiting and numbness. The difference
is not known for 1.5 and 3.0 Tesla MRI, but it is assumable that this
difference is also not statistically significant for less stronger magnetic
field.To minimize the burden, we will start with good education, including an
information folder. Children can get used to the sound of the MRI-scan from a
computer program. Children will be constantly guided by their parents and a
specialized trial nurse. The scanning environment will be made as comfortable
and cosy as possible. A Walt Disney movie will be displayed between the
scanning sessions. In preparation, the children will be familiarized with the
MRI system. The scanning time is 2 times 30 minutes with a half hour break in
between (if necessary longer) and consists of individual programs with an
average duration of 7 minutes. These sessions can be interrupted at any time,
and children are allowed to break up or stop the scanning and leave the MRI
room at any time. The neuropsychological assessment will take one hour in
total. Moreover, we will apply short scan protocols to minimize the magnet time
of all the children.

The 24 hour EEG is carried by the child for 24 hour (electrodes and battery).
This will only be performed in patients, electively. The patient cannot
shower/bathe during the examination.

Recruitment of children in the age of 8-18 years is essential as Rolandic
epilepsy, ESES, ESES-like and LKS are basically diseases of childhood and often
disappears during adolescence (commonly with persistence of the cognitive
impairments). Adults are therefore not representative to unravel the
development of neuronal correlates of language, cognitive comorbidity and
refratoriness in Rolandic epilepsy, ESES, ESES-like or LKS. The study of
possible neuronal correlates of language and cognitive impairment and
refractoriness in Rolandic epilepsy, ESES, ESES-like and LKS requires the
inclusion of an age-matched control group. There are three reasons for doing
so. Firstly, all effects will be relative effects and not absolute effects that
require a comparison with normal development to be able to understand.
Secondly, apart from the secondary changes due to the seizures, brain
development in children with Rolandic epilepsy, ESES, ESES-like and LKS may as
well differ from healthy subjects. Thirdly, MRI derived cerebral properties
such as oxygenation changes due to brain activation and diffusion properties of
white matter depends on age, and changes most strongly in the age category that
makes up our study population. The imaging of the normally developing brain
provides a necessary baseline for comparisons with Rolandic epilepsy, ESES,
ESES-like and LKS.