Contemporary Neurology by M.J.G. Harrison (Eds.)

By M.J.G. Harrison (Eds.)

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Myoclonic jerks may follow anoxic brain damage or uraemia and may occur in rare progressive degenerative forms of brain disease and in Jakob-Creutzfeldt disease. A salaam attack, infantile spasm, hypsarrhythmia, or West's syndrome describes a type of seizure that affects babies and commonly starts between the ages of 3 and 9 months. The baby flexes the body in a salaam, drawing up the legs. These attacks are repeated. There are a number of prenatal, perinatal, and postnatal causes but there is a high incidence of mental retardation in these children and many of them develop other forms of epilepsy.

Motor disturbances include simple facial movements such as grimaces, lip smacking, swallowing, or chewing. Patients may drop what they are holding, pluck at their clothes with their hands, or show brief limb shaking. Sometimes they walk about or perform more complex movements. Usually they break off speech or make nonsensical utterances. These features are very brief but may be followed by a longer period of confusion. Ictal and postictal motor automatisms may occur: patients perform actions, usually out of context, of which they have no recollection.

It can lead people to seek medical attention, particularly when they are predisposed to conversion reactions, hypochondriasis, or psychosomatic illnesses; I have found a history of such problems in more than three quarters of HVS patients. Young women aged 15-30 seem to be particularly susceptible; preadolescents and elderly patients appear to be less prone.

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