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There are many different types of childhood
epilepsy syndrome, five of which are outlined
below. When diagnosing an epilepsy syndrome,
factors such as the type of seizure the
child is having, their age at seizure onset,
their gender and whether or not they have
learning difficulties are very important.
Once a doctor has diagnosed a particular
syndrome, he/she will be able to consider
the best course of treatment and the likely
prognosis.
Rolandic epilepsy Rolandic epilepsy
is the most common of the childhood epilepsy
syndromes, affecting approximately one in
4,800 children under 15 years. It is characterised
by partial seizures that usually occur during
sleep or just before wakening, and involve
the face, mouth and speech organs (although
in some cases seizures may be more generalised).
Rolandic epilepsy normally occurs between
the ages of three and 15 years (most commonly
between six and eight years), but the cause,
although suspected to be genetic, is not
fully established. Most children grow out
of the condition when they reach adulthood,
and because seizures occur at night and
learning is not usually impaired, neurologists
often refrain from treating it with anti-epileptic
drugs. It is often referred to as 'benign'.
Epilepsy Research UK is currently funding
a study at Bristol University, using a restricted
grant from the Waterloo Foundation. This
is looking at the link between sleep disturbance
and learning difficulties, and whether treatment
of Rolandic epilepsy as standard practice
would improve the learning outcomes of affected
children.
West's syndrome (infantile spasms)
West's syndrome is a disorder of the brain that occurs in approximately one in every 5,000 babies. It is characterised by infantile spasms (a form of epilepsy that usually develops before the age of one, often at about six months), a specific EEG pattern, developmental regression and, often, learning/physical disabilities. Many of the conditions that cause West's syndrome are genetically inherited, but this is not always the case; so it is very important to establish the exact cause in each child.
Treatments for West's syndrome include the anti-epileptic drugs and oral steroids. Children's responses to these medications a highly variable, however, and cannot be predicted. In cases of drug resistance, epilepsy surgery may be considered.
Rett syndrome
Rett syndrome is a neurodevelopmenal disorder that affects girls almost exclusively. It is characterised by normal early growth and development, followed by a slowing of development, loss of purposeful use of the hands, distinctive hand movements, slowed brain and head growth, problems with walking, seizures, and intellectual disability.
It is estimated that one in 10,000 to 15,000 female babies will be affected by Rett Syndrome, sometimes as early as six months of age. Nearly all cases genetic, but it is thought that 99% of genetic cases are due to spontaneous mutations, not defects inherited from the parents.
There is no cure for Rett Syndrome and most treatments are symptomatic, for example anti-epileptic drugs for seizures and other medications for breathing/movement irregularities.
Angelman syndrome Angelman syndrome
is a genetic neurological disorder that
occurs in approximately one in every 25,000
children. It is characterized by intellectual
and developmental delay, sleep disturbance,
seizures, jerky movements (especially hand-flapping),
frequent laughter or smiling, and usually
a happy demeanor. It is usually diagnosed
between the ages of three and seven, when
the clinical symptoms become apparent; but
developmental delay may begin as early as
six months. There is no specific therapy
for Angelman syndrome, but treatment for
seizures is usually needed. Physical and
occupational therapies, communication therapy,
and behavioral therapies are important in
allowing individuals with Angelman syndrome
to reach their maximum developmental potential.
Lennox-Gastaut syndrome
Lennox-Gastaut syndrome is a 'difficult-to-treat'
form of childhood-onset epilepsy that affects
approximately one in 50,000 children, and
usually appears between the second and sixth
years of life. It is characterised by frequent
seizures of different types and is often
accompanied by mental retardation, psychological
and behavioral problems. In up to a third
of cases, no cause can be found. Lennox-Gastaut
is notoriously resistant to anti-epileptic
medication and often multiple drugs. in
high doses must be prescribed. If there
is still no improvement, alternative treatments
such as the ketogenic diet or epilepsy surgery
may be considered.
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