In 90% of cases, crises appear before one year of age, 10% before the third month. The maximum distribution is between the 4th and 7th month, more frequently in the 6th month. The later start (after 18 months), are nosological errors. A very early onset (before the third month) can be seen especially in the symptomatic forms.
In 9% of cases they were found a history of seizures and convulsions. It is strange (of 4 to 6%) the presentation of several cases of West syndrome in the same family. Transmission is autosomal recessive, rarely being linked to sex.
Infantile spasms that occur in salvos.
Impairment of psychomotor development.
A E.E.G. particularly intercritical: The hypsarrhythmia.
These three elements appear over several weeks in a child previously normal or already submitted crises or deficient neurological signs. In 85% of cases starts with spasms, sometimes with the arrest of psychomotor development. The layout may be missing hypsarrhythmic initially discovered or after the onset of spasms is well advanced. In typical cases, the syndrome set is completed in 4 to 6 weeks.
SEMIOLOGY OF THE CRISIS: The crisis in the West Syndrome have been called in several ways: flexion spasm, myoclonus Mass in flexion, Infantile Myoclonus, Lightning Seizure, Salaam Attack, Nodding Spasm or Blinking, Jerk of Infancy Epileptic, Blitz Krampf, Saalam Krampf, Nick Krampf and Propulsiv Petit Mal.
The clinical expression of spasms depends on the affected muscles and the duration of each spasm. The use of video methods has allowed a more detailed and rigorous analysis.
TYPICAL CRISIS. Much of the authors admitted that in most cases (68 to 80%) spasms occur in bending. They can be more or less extensive and affect the muscles of the neck, trunk and limbs; the child bends "as a razor to close": flexion of the head and trunk, upper limbs intertwine over the chest, lower limb triple flexion.
If the spasm is performed in extension (22.5% of cases), neck and trunk are extended and the members are placed in extension-abduction, separating crosshair. However, most often they are mixed-in flexion / extension-then pretending massive myoclonus.
ATYPICAL CRISIS. Often limited or frustrated in expression, risk being ignored or misunderstood: shake of the head, brief contraction of the abdominal muscles, lift your shoulders, simple adduction or adduction of the arms; more rarely spasm may be asymmetric (0.6%). Exceptionally crisis can be expressed by a akinesia and decreased reactivity (1%).
In general, a single child has several types of spasms.
Certain phenomena may accompany crises: ocular deviation (55%), respiratory distress (59.2%), abnormal heart rhythm. A cry or smile unmotivated conclude the crisis. Due to the short spasm, it is difficult to assess the level of consciousness; in the longer a temporary or atypical crises clouding supported.
If at the start of the condition spasms can be isolated, sporadic, preferably emergence or awakening during sleep, in the phase of state are characteristic saved (78.3% of cases). During a particular saved, the intensity and frequency increase progressively, to then decrease and disappear. They are favorable factors postprandial phase, a sudden noise or a contact.
PSYCHOMOTOR Regression: This is the second characteristic feature of the syndrome. Starts disease in approximately 15% of cases.
Its beginning is manifested by a modification of humor. In several days, the child falls into total indifference: loss of smile, reactions to sensory stimuli. Often the child is qualified deaf or blind, loss of facial expression and motor absolute immobility.
In the older child, selflessness extends to objects that are presented. This indifference, this lack of activity, sometimes give rise to stereotyped activities; more rarely, reactions of aggression or irritability are observed.
Then the impairment is confirmed, the child does not progress, it does not present any new acquisition. By contrast, activities that had lost at the start of his illness: he no longer feels not holding the head, he does not smile more ...
The loss of the most basic and widespread contacts sluggish own stigmas are an obvious regression if they occur in a child whose psychomotor development was already disturbing.
Diagnosis
The diagnosis is based on the triad of infantile spasms, impaired psychomotor development and EEG hypsarrhythmic.
In typical, complete forms, it is easy not done, because the clinical history and EEG rule out:
Misinterpretation of the parents, including doctors, spasm in flexion: flexion abdominal colic can simulate a child or an attempt to sit.
The Tic Sleep or Jactatio Capitis Night: lateral movements of the head, swinging, appearing in normal children during drowsiness or sleep.
The Spasmus Nutans, rhythmic oscillations of the head during the day, even in normal children.
The slow rhythmic swing of some deep backward (Tic Salaam).
Certain conditions must be differentiated West Syndrome:
Specific secondary generalized epilepsies can be expressed at the start by infantile spasms;
The Lennox-Gastaut syndrome, by the fact that the evolutionary continuation of some syndromes West, but its onset is later; there are others associated with those crises, like infantile spasms, where the EEG is different.
Certain conditions described recently seem more or less integrated within West syndrome. They are described at the end of this chapter.
Finally, Lombroso has described a benign myoclonic epilepsy of infancy. Crises are fully comparable to those of the West Syndrome but not altered mental state, EEG It is normal and crises disappear without sequelae before 2 years of age.
In my opinion it is vital that we as nurses know how to act in a case of West syndrome because it is a disorder uncommon but very traumatic for both the patient and the family because in this case it comes to young children besides all are sick, so we can understand their needs and meet them to provide better care to the patient.
Recovered of http://www.apiceepilepsia.org/Sindrome-de-West-o-Espasmos-Infantiles on 23th november, 2015 at 18:35 by. Rebeca Guzman 2719140
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