It is well established that the reorganizational potential of the developing human brain is superior to that of the adult brain, but whether age-dependent differences exist already in the prenatal and perinatal period is not known. We have studied sensorimotor reorganization in 34 patients with congenital hemiparesis (age range, 5-27 years), using transcranial magnetic stimulation and functional magnetic resonance imaging during simple hand movements. Underlying pathologies were brain malformations (first and second trimester lesions; n = 10), periventricular brain lesions (early third trimester lesions; n = 12), and middle cerebral artery infarctions (late third trimester lesions; n = 12). Of this cohort, eight patients with malformations and all patients with periventricular lesions have been published previously. In all three groups of pathologies, transcranial magnetic stimulation identified patients in whom the paretic hand was controlled via ipsilateral corticospinal projections from the contralesional hemisphere (n = 16). In these patients, the motor dysfunction of the paretic hand correlated significantly with the timing period of the underlying brain lesion. This demonstrates that the efficacy of reorganization with ipsilateral corticospinal tracts indeed decreases during pregnancy.

Right-hemispheric organization of speech has been observed following early left-sided brain lesions involving the language cortex. The authors studied speech organization in hemiparetic patients with pre- and perinatally acquired lesions in the left periventricular white matter using fMRI, and found that right-hemisphere activation correlated with left facial motor tract involvement. This suggests that the impairment of speech motor output from the left hemisphere plays an important role in this alteration of language representation.

Three-dimensional MRI data sets were obtained from 12 young adult patients with congenital spastic hemiparesis caused by unilateral periventricular white matter lesions. The impact of these lesions on corticospinal projections to the upper and lower extremities was assessed on reconstructed semi-coronal planes following anatomical landmarks of somatotopic organization in the precentral gyrus and in the internal capsule: a more anterior plane running through the hand-knob of the precentral gyrus and the anterior portion of the posterior limb of the internal capsule representing projections to the upper extremity, and a more posterior plane running through the top of the precentral gyrus and the middle portion of the posterior limb of the internal capsule representing projections to the lower extremity. In addition, the total lesion extent was determined volumetrically, and Wallerian degeneration was assessed qualitatively in the internal capsule and quantitatively by measuring brainstem asymmetry. We found a strong correlation between motor dysfunction of the upper and lower limb and the lateral extent of the periventricular lesion measured on the respective semi-coronal planes. The total lesion volume and the degree of Wallerian degeneration correlated less strongly, both reaching statistical significance only with motor impairment of the hand.

Background and Purpose: Transcranial color duplex sonographic examinations in children and adolescents without cerebrovascular disease were evaluated retrospectively. Flow velocities and waveform parameters were determined and their side-to-side differences and age dependence analyzed and, finally,

SUMMARY The authors report on the clinical examination and magnetic resonance imaging (MRI) of 41 children (29 term and 12 preterm) whose diagnosis of congenïtal hemiparesis was confirmed after the first year of life. Periventricular leukomalacia was the most predominant MRI finding (found in 15 term and eight preterm infants). The uniform pattern of lesions observed through MRI (without clear correlation with gestational age) suggests a common aetiopathogenesis in this subgroup. Comparison of these MR images with the periventricular leukomalacia observed in preterm children with spastic diplegia supports the thesis that ischaemia occurring between the 28th and 35th weeks of gestation could be the main cause. RÉSUMÉ Hémiparésie congénitale et leucomalacie périventricular: aspects pathogéniques à partir d'IRM Les auteurs rendent compte de l'examen clinique et des données IRM chez 41 enfants (29 nés a terme et 12 prématurés) chez qui un diagnostic d'hémiparésie congénitale avait été confirmé aprés l'àge d'un an. I.a leucomalacie périventriculaire avait été la donnée IRM la plus habituelle (trouvée chez 15 sujets nés a terme et huit prématurés). La distribution uniforme des lésions observées à l'IRM (sans corrélation évidente avec l'âge de gestation à la naissance) suggère une étiopathogcnie commune à ce sous‐groupe. La comparaison de ces IRM avec la leucomalacie périventriculaire observée chez les prematurés porteurs d'une diplegie spastique, est en faveur de la thése selon laquelle une ischémic sutvenam entre la 28éme et la 35cme semaine de gestation pourrait être la cause principale. ZUSAMMENFASSUNG Congenitale Hemiparese und periventrikuläre Leukomalazie: pathogenetische Aspekte der Kernspin‐Unlersuchung Die Autoren berichten über klinische Untersuchungen und Kernspin (MR1) Befunde, bei 41 Kindern (29 reif‐ und 12 frühgeborene Kinder), bei denen die Diagnose der congenitalen Hemiparese nach dem ersten Lebensjahr bestätigt wurde. Der wichtigste MRI Befund war die periventrikuläre Leukomalazie (bei 15 reif‐ und acht frühgeborenen Kindern nachgewiesen). Das übereinstimmende Bild der Läsionen im MRI (ohne eindcutige Korrelation zum Gestationsalter) läßit eine gemeinsame Aetiopathogenese bei dieser Untergruppe vermuten. Der Vergleich dieser MRI Befunde mit der periventrikulären Leukomalazie bei Fruhgeborenen mit spastischer Diplegie spricht für die Hypothese, daß eine Ischämie in der Zeit von der 28. bis 35. Gestationswoche die Hauptursache sein könnte. RESUMEN Hemiparesia congenita y leucomalacia peri‐ventricular: aspectos patogénicos a partir de la IRM Los autores aportan los datos clinicos y de IRM dc 41 niños (29 a término y 12 pretérmino), en los que el diagnóstico de hemiparesia congénita fue confirmada después del primer año de vida. La leucomalacia periventricular era el hallaz. go por IRM mas predominante (fue confirmado en 15 a término y en ocho pretérmino). El partón uniforme de las lesiones observadas por la IRM (sin una clara correlación con la edad gestacional) sugiere que hay una etiopatogenia comiin...

In osteopetrosis, bone healing is complicated by progressive sclerosis. A 2-year-old blind boy with malignant osteopetrosis developed increased intracranial pressure secondary to craniosynostosis. Osteopetrosis had already been treated with bone marrow transplantation (BMT), and bone remodeling seemed to be restored. A craniotomy was performed. The postoperative course and bone healing were uneventful. After successful BMT, craniofacial procedures seem to be possible without a high risk for osteomyelitis.

To date, there are few reports of paroxysmal exercise-induced dystonia associated with familial epilepsy. We describe a family with 4 affected members spanning 3 generations, suggestive of autosomal-dominant inheritance, who exhibited typical exercise-induced dystonia, different types of epilepsy (absence and primary generalized seizures), developmental delay, and migraine in variable combinations. Linkage of the disease to loci on chromosome 2 (paroxysmal nonkinesigenic dyskinesia) and chromosome 16 (paroxysmal kinesigenic choreoathetosis, infantile convulsions with choreoathetosis) was excluded, suggesting an as yet unidentified underlying genetic basis.

Fiberoptic endoscopy is useful to detect a potentially dangerous penetration or aspiration of saliva, secretions and food. Thereby it enables the physician to change airway and feeding management. This is of special importance as children who formerly have been fed orally without suspicion of risks and pneumonia anamnesis turned out to have severe swallowing deficits. A close connection between appearance of drooling phenomena and aspiration of saliva and food has also to be considered in children with CP.