West Syndrome
West syndrome is the term used to describe a type of epilepsy which most typically starts in the first year of life, between four and eight months of age. It may, however, start younger than this.
What are the symptoms?
The hallmark of West syndrome is the occurrence of a particular type of epileptic seizure called a spasm. Spasms typically produce a sudden jerk of the body followed by stiffening of the limbs. Different types of spasms may occur, but most typically a child will suddenly bend forward with elevation of the arms or legs. These attacks usually occur in runs or clusters when one spasm occurs after another at five to 15 second intervals for a period of several minutes. These episodes may occur several times per day. Very commonly, when a child starts to have spasms, there is change in their behaviour and they stop vocalising and making visual contact
What are the causes?
West syndrome is not one condition but is a symptom of many different brain disorders. A cause can be found in most children, but in a small proportion no obvious cause can be identified. (Like in Paige's case) Sometimes West syndrome occurs in children with severe abnormalities of the brain such as congenital infections, severe brain injury due to birth asphyxia (lack of oxygen) or severe malformations of the brain. Sometimes very rare genetic or metabolic diseases cause West syndrome. One of the most common causes of West syndrome is tuberous sclerosis. Chromosome abnormalities may also cause West syndrome. It is therefore very important when a diagnosis of West syndrome is made that an extensive search is carried out to find the underlying cause.Statistically, 5 out of every 100 children withWest syndromedo not survive beyond five years of age, in some cases due to the cause of the syndrome, in others for reasons related to their medication.
How is it diagnosed?
The diagnosis of West syndrome is based on the occurrence of infantile spasms and the presence of a very abnormal EEG (brain wave recording). The most typical EEG abnormality is called 'hypsarrhythmia', however, other abnormal patterns may occur.
Microcephaly
Microcephaly is a neurodevelopmental disorder in which the circumference of the head is more than two standard deviations smaller than average for the person's age and sex. Microcephaly may be congenital or it may develop in the first few years of life. The disorder may stem from a wide variety of conditions that cause abnormal growth of the brain, or from syndromes associated with chromosomal abnormalities. In general, life expectancy for individuals with microcephaly is reduced and the prognosis for normal brain function is poor. The prognosis varies depending on the presence of associated abnormalities
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What are the symptoms?
The hallmark of West syndrome is the occurrence of a particular type of epileptic seizure called a spasm. Spasms typically produce a sudden jerk of the body followed by stiffening of the limbs. Different types of spasms may occur, but most typically a child will suddenly bend forward with elevation of the arms or legs. These attacks usually occur in runs or clusters when one spasm occurs after another at five to 15 second intervals for a period of several minutes. These episodes may occur several times per day. Very commonly, when a child starts to have spasms, there is change in their behaviour and they stop vocalising and making visual contact
What are the causes?
West syndrome is not one condition but is a symptom of many different brain disorders. A cause can be found in most children, but in a small proportion no obvious cause can be identified. (Like in Paige's case) Sometimes West syndrome occurs in children with severe abnormalities of the brain such as congenital infections, severe brain injury due to birth asphyxia (lack of oxygen) or severe malformations of the brain. Sometimes very rare genetic or metabolic diseases cause West syndrome. One of the most common causes of West syndrome is tuberous sclerosis. Chromosome abnormalities may also cause West syndrome. It is therefore very important when a diagnosis of West syndrome is made that an extensive search is carried out to find the underlying cause.Statistically, 5 out of every 100 children withWest syndromedo not survive beyond five years of age, in some cases due to the cause of the syndrome, in others for reasons related to their medication.
How is it diagnosed?
The diagnosis of West syndrome is based on the occurrence of infantile spasms and the presence of a very abnormal EEG (brain wave recording). The most typical EEG abnormality is called 'hypsarrhythmia', however, other abnormal patterns may occur.
Microcephaly
Microcephaly is a neurodevelopmental disorder in which the circumference of the head is more than two standard deviations smaller than average for the person's age and sex. Microcephaly may be congenital or it may develop in the first few years of life. The disorder may stem from a wide variety of conditions that cause abnormal growth of the brain, or from syndromes associated with chromosomal abnormalities. In general, life expectancy for individuals with microcephaly is reduced and the prognosis for normal brain function is poor. The prognosis varies depending on the presence of associated abnormalities
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