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Juvenile myoclonic epilepsy is a relatively newly recognized syndrome unfamiliar to many physicians who do not work in the field of epilepsy. It is easily recognized, if you know what to look for and know what questions to ask of the patient. It is also easily treated.Epilepsy of Janz starts in late childhood or adolescence, often about the time of puberty. Its hallmark is mild myoclonic jerks, most common as the person is going to sleep or awakening in the morning. An adolescent will describe jerking of the arms or legs, a feeling of being very “jumpy.” Some patients have told us that they set their alarm clocks to wake up early and then stay in bed for one half hour to an hour, until the jumpiness wears off. They say that if they get up more quickly the jerking gets much worse.If a person has early morning seizures, informing your doctor about the jerks that precede them may make it easier to diagnosis this particular form of epilepsy.Occasionally, the jerking builds up and becomes sufficiently severe so that the person experiences a clonic or a tonic-clonic seizure. In addition, people may experience absence seizures.The EEG between seizures, in this form of epilepsy, often shows a fast, multiple- or double-spike pattern followed by slow waves, with fast rapid spikes occurring during the jerks. When the diagnosis is suspected, the best way of confirming it is a sleep EEG, continued for ten or fifteen minutes after the person awakens. It is during this time that the jerks and the characteristic EEG pattern are most likely to be seen.Diagnosis is important because although this form of epilepsy responds poorly to many medications, it is usually easily controlled with valproic acid. The seizures often recur when this medication is withdrawn. A familv history of epilepsy may occur in as many as 40 percent of siblings of those with the epilepsy of Janz. Studies of these families are beginning to provide clues to its genetic basis.*92\208\8*

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An electroencephalogram (EEG) records the electrical activity of the brain; your ‘brain waves’. Abnormalities of electrical activity show up on the EEG recording in about 75 per cent of people who have epilepsy. But an abnormal EEG does not necessarily mean that you have epilepsy, and neither does a normal EEG prove that you do not have epilepsy.
An EEG recording takes about one hour. Small silver discs (electrodes) will be attached to your scalp with a special glue. This does not involve any shaving of the hair, although your hair may have little tacky bits in it afterwards and need to be washed. During the recording you will be asked to do various things which are likely to provoke epileptic activity, including opening and closing your eyes, overbreathing and sitting in front of a flickering disco-like lamp (a strobe).
Sleep enhances epileptic activity in some people who have perfectly normal EEGs while they are awake. If your sleep EEG is to be recorded, you will probably be asked to take a sleeping tablet (seconal) to help you sleep during the day in the unfamiliar laboratory surroundings.
The specialist’s report on your EEG may mention the following terms:
Paroxysmal activity
Spike and wave
These first two observations are both suggestive of generalized epilepsy.
Focal spikes These indicate that there is abnormal activity in one part of the brain, suggesting partial epilepsy.
Generalized slow activity This often indicates that there is some underlying brain dysfunction, though it is not necessarily epilepsy.
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