Abnormalities of the oculomotor and nystagmus are often observed, along with ataxia, choreoathetosis, dystonic and tonic movements, and hemiplegic episodes. Hemiplegia may occur with a shift from one side to another, with the arms being more severely affected than the lower limbs. The child is fretful and irritable, and consciousness is preserved during an attack. Episodes may last from several days to a few minutes, with variable frequencies. Hemiplegia may affect both sides, with one side often progressing or starting abruptly over several minutes. In its typical clinical manifestation, hemiplegic episodes gradually assume a gradually inconsistent and light onset. Autonomic disturbances and eye deviation, along with ataxia and dystonic movements on the right side, were observed in both twins, who showed typical signs of acute encephalopathy after a previous episode 30 months ago. Noisy breathing and guttural sounds were observed. These episodes ceased after crying and lasted a few seconds to 3-4 minutes, with hypertonia and head deviation to one side. The body was sprinkled with hot water or immersed in warm bath water, eliciting unprovoked irritability and a smile. Monozygotic twins who complained of reflex water hot seizures until the age of 3 were described in et al.’S study. Autonomic changes such as vasomotor phenomena may also be observed. Ocular abnormalities mainly consist of nystagmus affecting only one eye, often in association with dystonic or tonic signs. Paroxysmal episodes appearing unilateral with the extension of one limb and the side of the body are rare. The most frequent initial clinical manifestations are episodes with autonomic abnormalities, ocular abnormalities, and dystonic or tonic movements, often linked to trigger events. Episodes are often preceded by trigger events and usually have an early onset in the first few months of life. Clinical manifestations vary in type, severity, grade, and associations with other morbidities and complications.
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