Enrique J. Carrazana

Developmental and Neurologic Status of Children after Heart Surgery with Hypothermic Circulatory Arrest or Low-Flow Cardiopulmonary Bypass

Background Deep hypothermia with either total circulatory arrest or low-flow cardiopulmonary bypass is used to support vital organs during heart surgery in infants. We compared the developmental and neurologic sequelae of these two strategies one year after surgery. Methods Infants with D-transposition of the great arteries who underwent an arterial-switch operation were randomly assigned to a method of support consisting predominantly of circulatory arrest or a method consisting predominantly of low-flow bypass. Developmental and neurologic evaluations and magnetic resonance imaging (MRI) were performed at one year of age. Results Of the 171 patients enrolled in the study, 155 were evaluated. After adjustment for the presence or absence of a ventricular septal defect, the infants assigned to circulatory arrest, as compared with those assigned to low-flow bypass, had a lower mean score on the Psychomotor Development Index of the Bayley Scales of Infant Development (a 6.5-point deficit, P = 0.01) and a hig...

A comparison of the perioperative neurologic effects of hypothermic circulatory arrest versus low-flow cardiopulmonary bypass in infant heart surgery

Background Hypothermic circulatory arrest is a widely used support technique during heart surgery in infants, but its effects on neurologic outcome have been controversial. An alternative method, low-flow cardiopulmonary bypass, maintains continuous cerebral circulation but may increase exposure to known pump-related sources of brain injury, such as embolism or inadequate cerebral perfusion. Methods We compared the incidence of perioperative brain injury after deep hypothermia and support consisting predominantly of total circulatory arrest with the incidence after deep hypothermia and support consisting predominantly of low-flow cardiopulmonary bypass in a randomized, single-center trial. The criteria for eligibility included a diagnosis of transposition of the great arteries with an intact ventricular septum or a ventricular septal defect and a planned arterial-switch operation before the age of three months. Results Of 171 patients with D-transposition of the great arteries, 129 (66 of whom were assign...

Facilitation of Infantile Spasms by Partial Seizures

Summary: We report 16 patients with infantile spasms in whom onset of the clusters of spasms appeared to be triggered by close temporal association with partial seizures. Common features included the presence of focal cerebral lesions in 12 infants (3 were classifiable as cryptogenic); all had partial seizures with EEG localization, clusters of bilateral spasms always preceded by partial seizures, and response to adrenocorticotropic hormone (ACTH) and traditional antiepileptic drugs (AEDs) generally was poor. Three had complete agenesis of the corpus callosum, which argues against interhemispheric callosal spread of focal discharges resulting in the generalized spasms. Surgical cortical resections were performed in 6 of the infants, with good outcomes. This group of patients supports a model in which the spasms, although probably generated at a subcortical level, are facilitated or possibly induced by focal discharges from cortical pathology.

Perioperative electroencephalographic seizures in infants undergoing repair of complex congenital cardiac defects.

Many infants with cardiac anomalies undergo repair early in life. Both commonly used support techniques, deep hypothermic circulatory arrest (DHCA) and low-flow cardiopulmonary bypass (LFB), may be associated with adverse neurological outcomes, including seizures. In a single center study, 171 infants undergoing correction for D-transposition of the great arteries were randomized to one of these support techniques. Incidence and onset times of EEG seizures during continuous EEG-video monitoring in the first 48 h postoperatively and clinical seizures in the first postoperative week were compared. EEG seizures were characterized by time, duration, and localization of onset. Incidence of EEG seizures (20%) was more than 3 times that of clinical seizures (6%). Most infants with EEG seizures had multiple seizures beginning between 13 and 36 h postoperatively. Durations ranged from 6 s to 980 min. Onset of EEG seizures occurred most commonly in frontal and central regions. Factors associated with EEG seizures included randomization to DHCA, longer duration of circulatory arrest, and diagnosis of VSD. In this study EEG seizures were common following this type of cardiac surgery, illustrating the importance of EEG monitoring in detecting seizures. This data adds insight into mechanisms of seizures in infants undergoing cardiac surgery.

Patterns of Seizures Observed in Association with Neurofibromatosis 1

Summary: Clinical features of seizures occurring in a neurofibromatosis clinic population were examined in a retrospective study. Twenty‐two individuals in a population of 359 persons with neurofibromatos 1 (NF1) had had one or more seizures. Age at first seizure ranged from 4 days to <20 years. Patients were classified into five groups on the basis of medical history, seizure type, and EEG findings. Two patients had seizures in the first 3 months of life: 1 was born prematurely and had perinatal encephalopathy, and the other developed infantile spasms (IS) and had extensive growth of a plexiform neurofibroma. Two males had seizures in association with aqueductal stenosis. Six individuals had one or more generalized seizures with fever, and 3 had primary generalized epilepsy. Nine patients had complex partial seizures (CPS), which varied in clinical manifestations and response to antiepileptic drugs (AEDs). In none of the individuals with seizures in this study was a structural lesion in the brain visible by neuroimaging. Seizures appear to be relatively uncommon in individuals with NF1, and when they occur have a natural history similar to that of seizures in the general population.

Infantile spasms provoked by partial seizures

Abstract A case of infantile spasms that were persistently preceded by a partial seizure is presented. The absence of normal interhemispheric communication through the corpus callosum excludes rapid secondary generalization via this route as a possible explanation for bilateral spasms. A mechanism implicating the brainstem as the common source of infantile spasms and their characteristic hypsarrhythmic EEG pattern is discussed.

Cerebral toxoplasmosis in the acquired immune deficiency syndrome.

We examined the clinical and pathological features of 26 patients who presented with a diagnosis of CNS toxoplasmosis. Patient data was analyzed with respect to demographics, clinical presentation, treatment course and pathology. Patients presented with a wide variety of signs and symptoms. All patients had positive serum antitoxoplasma IgG; ring enhancing lesion(s) were present on all but one brain CT scans. A series of guidelines in the management of CNS toxoplasmosis in AIDS patients are presented. Prior to biopsy, patients with positive serology and characteristic CT scans should receive two weeks of treatment. Biopsy is indicated in those cases with negative serology, atypical presentation, progressive clinical deterioration, or differential response of lesions to empiric therapy.

Uncal herniation secondary to bacterial meningitis in a newborn.

A newborn with bilateral uncal herniation secondary to acute bacterial meningitis is reported. The findings of previous neuropathologic studies of neonatal bacterial meningitis are reviewed and the factors most likely responsible for the relative rarity of herniation in this disease in newborns are discussed.

Kluver-Bucy Syndrome Following Recovery from Transtentorial Herniation

Kluver-Bucy syndrome is an uncommon constellation of behavioral abnormalities resulting from bilateral temporal lobe damage. The syndrome is rare in humans. In this paper we present a patient who developed the Kluver-Bucy syndrome following recovery from an intracerebral haemorrhage and transtentorial herniation.