Spastic diplegia as a complication of interferon Alfa-2a treatment of hemangiomas of infancy.

We report the development of spastic diplegia in infants during the course of interferon Alfa-2a (IFN) therapy for potentially life-endangering hemangiomas. Five infants who displayed diplegia were selected from a group of 26 infants treated with IFN. Diplegia persisted in three infants, and in the remaining two significant recovery occurred after IFN was discontinued. Magnetic resonance imaging showed no significant brain or spinal abnormalities except minor to moderate delayed myelination in two patients. Myelination was normal on subsequent radiographic examination in all five patients. We conclude that IFN can adversely affect the immature central nervous system and produce spastic diplegia, which is potentially reversible. We recommend careful clinical assessment of neurodevelopmental status during IFN therapy.

Selective injury of the globus pallidus in children with post-cardiac surgery choreic syndrome.

Occasionally children undergoing cardiac surgery using cardiopulmonary bypass with deep hypothermia and cardiac arrest develop a postoperative syndrome of acute chorea. The authors report the neuropathological findings in two such children surgically treated for congenital heart disease. Examination of the brain showed neuronal loss, reactive astrocytosis and degeneration of myelinated fibers (without frank necrosis) in the globus pallidus, primarily the outer segment, with sparing of other regions commonly susceptible to hypoxic-ischemic necrosis. The localization and relative mildness of the brain damage suggest a susceptibility of the globus pallidus to injury in this setting and implicate disruption of pallidal pathways in the pathogenesis of post-cardiac surgery choreic syndrome.

Regional cerebral perfusion abnormalities after cardiac operations. Single photon emission computed tomography (SPECT) findings in children with postoperative movement disorders.

Despite the clinical and pathologic indicators implicating injury to the basal ganglia in children with hyperkinetic movement disorders, we were previously unable to identify lesions in these structures by means of cranial computed tomography or magnetic resonance imaging. We evaluated regional cerebral perfusion measured by single photon emission computed tomography (SPECT) with technetium 99m hexamethyl propylene amine oxime as a technique to localize functional cerebral abnormalities in eleven children who had a movement disorder after hypothermic cardiac surgery. Perfusion defects of the deep gray matter were noted in six of these eleven patients and cortical perfusion defects in nine. For both cortical and subcortical defects a strong right-sided predilection was present. Our findings suggest functional brain injury not detectable by conventional cranial computed tomography and magnetic resonance imaging in these patients. We speculate that these perfusion defects might relate to the behavioral and developmental sequelae in survivors of this syndrome. SPECT may identify subclinical injury in patients at risk for future neurodevelopmental problems and contribute to our understanding of the mechanisms of cerebral injury in the patient operated on for cardiac disease.

Migraine in the infant and toddler.

This report consists of 13 patients who on retrospective analysis had had periodic symptomatology in the first 2 years of life, and who on later evaluation were determined to have juvenile migraine. The commonest early expression consisted of vomiting, accompanied by behavioral change. Seven of 13 showed some indication of headache. Other symptoms such as sleep relief, pallor, and vertigo or ataxia were relatively common. A family history of migraine, primarily maternal, was found in 11 of 12 patients in whom it could be evaluated.

A syndrome of autosomal dominant alternating hemiplegia: clinical presentation mimicking intractable epilepsy; chromosomal studies; and physiologic investigations.

We report the familial occurrence and apparent autosomal dominant inheritance of alternating hemiplegia of childhood. The proband, a 9-year-old boy, presented with developmental retardation, rare tonic-clonic seizures, and frequent episodes of flaccid alternating hemiplegia that had been presumed to represent postictal paralysis. The hemiplegia spells, which started in his first year, did not respond to multiple antiepileptics. Between attacks, there was choreoathetosis and dystonic posturing. Father, brother, paternal uncle, and paternal grandmother had similar histories of alternating hemiplegia. Investigations included negative CT, metabolic, and coagulation studies. EEG and SPECT 99mTc exametazime scanning failed to reveal any significant slowing or any major changes in cortical perfusion during hemiplegia as compared with nonhemiplegic periods. The karyotype revealed a balanced reciprocal translocation, 46,XY,t(3;9)(p26;q34) in the patient, in all the affected living relatives, and in one apparently unaffected sibling. The asymptomatic mother had a normal karyotype. Analysis of DNA markers was consistent with the karyotype results. Both affected siblings were treated with and responded to flunarizine therapy, with a greater than 70% decrease in attack frequency. Documented flunarizine trough serum concentrations were 28.9 ng/ml in the proband and 6.6 ng/ml in his brother.

Factors associated with choreoathetosis after cardiopulmonary bypass in children with congenital heart disease.

BACKGROUND: Choreoathetosis (CHO) after congenital heart surgery has been described since 1960. Risk factors and patient outcome have not been well defined. METHODS AND RESULTS: As our complexity of cases increased and management of pH on cardiopulmonary bypass (CPB) evolved, we noted the appearance of CHO among patients beginning in 1986. We reviewed the hospital course and follow-up of all 19 affected children, including eight younger patients (median age, 4.3 months) who developed a mild transient form of CHO, all of whom survived and had complete resolution of CHO; seven of these eight patients had deep hypothermic circulatory arrest (DHCA). Eleven older patients (median age, 16.8 months) developed severe persistent CHO; 11 had DHCA, 10 were cyanotic, seven had anatomic pulmonary atresia, and three others were physiologically analogous with a systemic to pulmonary artery shunt-dependent circulation. Five of six patients who had pertinent preoperative angiography had systemic to pulmonary collateral vessels arising from the head and neck arteries. Mortality in severe patients was 36% (four of 11); the seven survivors showed improvement, but only one had a normal neurological examination after 60 months of follow-up. When severe CHO patients were compared with 17 age- and diagnosis-matched patients without neurological complications, no differences were found in CPB or DHCA times, arterial blood gases, or hematocrits. Time from onset of CPB to onset of DHCA (time to shutoff) was shorter in the severe persistent CHO group than for comparison patients (22 +/- 7 versus 40 +/- 29 minutes, p = 0.053). CONCLUSIONS: Factors that may be associated with the development of severe persistent CHO include 1) age beyond early infancy, 2) cyanotic heart disease with systemic to pulmonary collaterals, particularly those arising from the head and neck vessels, and 3) the duration of the cooling period used in conjunction with deep hypothermic circulatory arrest. We advocate earlier reparative surgery, precise preoperative diagnosis and preoperative or intraoperative control of systemic to pulmonary artery collaterals, and further study of pH effects during CPB on development of CHO.

CSF dynamics in hydrocephalus--with special attention to external hydrocephalus.

Cerebrospinal fluid (CSF) physiology, and the pathophysiology of hydrocephalus is briefly reviewed, with emphasis on the arachnoid villae and the CSF-venous interface. Five cases of benign "external hydrocephalus" are recorded and the literature of similar cases is reviewed. It is proposed that anatomical or functional obstruction of various etiologies at the level of the arachnoid villae accounts for the dilated subarachnoid pathways in these patients.

Spontaneous intracerebral haemorrhage in patients suspected of multiple sclerosis.

Two cases of spontaneous intracerebral haemorrhage in adolescent girls suspected of having multiple sclerosis are reported. Surgical evacuation of haematomas in the left thalamus and left side of the pons, respectively, was performed with excellent recovery in both cases. Some clinical and pathological aspects of small arteriovenous malformations are discussed and the pertinent literature reviewed.