The neonatal arterial switch operation with combined circulatory arrest and low-flow bypass is associated increasingly with age, with reduced neurodevelopmental outcome but not with cognitive dysfunction. In our experience, the risk of long-term neurodevelopmental impairment after neonatal corrective cardiac surgery is related to deleterious effects of the global perioperative management and to special adverse effects of prolonged bypass duration. Severe preoperative acidosis and hypoxia and postoperative hemodynamic instability must be considered as important additional risk factors.

40, 1-14-year-old children suffering from congenital hemiparesis were re-examined neurologically and admitted to CT. According to our morphological results we found three different types of CT patterns: 1. unilateral enlargement of the lateral ventricle or parts of it (20 patients), 2. cavity in the cortex and subcortical white matter within the supply area of the middle cerebral artery (17 patients), 3. normal CT scans (3 patients). Patients with a cortical and subcortical cavity consistently had a moderate to severe hemiparesis and suffered more often from epilepsy and intellectual impairment than patients with unilateral ventricular enlargement and those with normal CT findings. Most patients with cortical defects had a history of perinatal complications, while abnormal pregnancies and prematurity prevailed in patients with unilateral ventricular enlargement. We believe that a cavity in the cortex and subcortical white matter is of arterial-ischemic origin, whereas unilateral ventricular enlargement with destruction of the deep white matter is related to venous hemorrhage. But it must be emphasized that CT cannot detect the causes, mechanisms and timing of the underlying brain lesions in congenital hemiparesis.

Using a recently developed noninvasive EMG recording technique with multi-electrode arrays we investigated the pattern and distribution of motor unit action potentials (MUAP) following maximal voluntary contraction of the musculus abductor pollicis brevis. An additional parameter, i.e. muscular conduction velocity (CV) in single motor units, was calculated from the multi-electrode array EMG recordings. From 63 healthy children of various age the normal EMG pattern and CV were derived and compared to the EMG of diagnosed patients known to suffer from Duchenne muscular dystrophy and from spinal muscular atrophy. In normal individuals the muscular CV in neonates was lowest at 1-2 m/s and gradually reached a plateau of 2.9-4 m/s from the age of 4 years onwards. The EMG in 31 children with Duchenne muscular dystrophy showed an abnormal pattern with low amplitude action potentials. In 30 out of 31 patients a significantly lower muscular CV was found. In 10 children with spinal muscular atrophy the EMG showed action potentials of abnormally large amplitude and a reduced recruitment of firing motor units. The muscular CV remained within the normal range. Compared to classical needle EMG the application of this new noninvasive EMG technique in children is painless and offers an easy-to-handle diagnostic tool to differentiate between neuromuscular diseases of denervating or of myopathic origin.

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