Pediatr Neurol

Acute hepatic failure in a child treated with lamotrigine.

Year 1998
Arnon R. DeVivo D. Defelice AR. Kazlow PG.
Department of Pediatrics, Columbia University School of Medicine, New York, New York 10032, USA.
An 8-year-old boy with a known seizure disorder that was being treated with lamotrigine developed acute hepatic failure. The patient, who had been well previously, presented with jaundice, elevated liver enzymes, and a coagulopathy. After discontinuation of lamotrigine and aggressive resuscitation, the patient made an uneventful recovery. We believe that the hepatic failure in our patient was secondary to the use of lamotrigine. We recommend careful monitoring of liver function when lamotrigine is administered.

3-Hydroxyisobutyric aciduria in two brothers.

Year 1998
Sasaki M. Kimura M. Sugai K. Hashimoto T. Yamaguchi S.
Department of Child Neurology, National Center Hospital for Mental, Nervous, and Muscular Disorders, National Center of Neurology and Psychiatry, Kodaira, Tokyo, Japan.
Two Japanese brothers with 3-hydroxyisobutyric aciduria (3HiB-uria) are studied. The elder brother died of a ketoacidotic episode at the age of 4 years; the younger brother also manifested repeated episodes of ketoacidosis after 1 year of age. He is diagnosed as having 3HiB-uria by gas chromatography/mass spectometry analysis, using the unique fragment ions of 3HiB. Magnetic resonance imaging reveals focal white matter abnormalities. Protein restriction is effective for preventing the ketoacidotic episodes, although carnitine therapy seems less effective.

Outcome of children with cerebral edema caused by fulminant hepatic failure.

Year 1998
Alper G. Jarjour IT. Reyes JD. Towbin RB. Hirsch WL. Bergman I.
Department of Pediatrics and Neurology, Children's Hospital of Pittsburgh, University of Pittsburgh School of Medicine, Pennsylvania 15213, USA.
Mortality is high in patients with fulminant hepatic failure (FHF). Neurologic complications of encephalopathy and cerebral edema are major contributors to mortality. Orthotopic liver transplantation has improved survival in these patients. However, the complexity of medical and surgical problems in this patient population, coupled with a severe shortage of organs, requires careful patient selection. The aim of this study was to describe the neurologic outcome of children with FHF who developed radiologically apparent cerebral edema. The hospital and outpatient records and radiologic studies of 20 children with FHF admitted to Children's Hospital of Pittsburgh from 1981-1995 who developed encephalopathy and computed tomographic evidence of cerebral edema were reviewed. Fourteen patients died (70%), three were left with severe neurologic deficits (15%), and three were left with moderate deficits (15%). Survival was correlated with a lesser degree of coma. Histopathologic examination of eight brains demonstrated cerebral edema and widespread ischemic neuronal necrosis in all eight. The presence of radiographic cerebral edema in children with FHF is an objective measure that indicates a very poor prognosis. Termination of care is a reasonable option. Comprehensive monitoring of cerebral function and intracranial pressure is required in children with FHF. Orthotopic liver transplantation should be performed in children with severe and worsening encephalopathy before the development of radiographically apparent cerebral edema.

Rotavirus encephalitis mimicking afebrile benign convulsions in infants.

Year 1998
Hongou K. Konishi T. Yagi S. Araki K. Miyawaki T.
Department of Pediatrics, Faculty of Medicine, Toyama Medical and Pharmaceutical University, Sugitani, Japan.
A 2-year-old-boy had rotavirus gastroenteritis and demonstrated afebrile partial seizures with secondary generalization. The presence of rotavirus genome and anti-rotavirus IgG was detected in the cerebrospinal fluid. The genotype of VP4 was P8 and that of VP7 was G4. The imaging analysis by computed tomography and single photon emission computed tomography implied encephalitis. The seizures were well controlled by standard antiepileptic therapy, and the child experienced normal development with neither physical nor neurologic sequelae, which appeared to mimic benign infantile partial epilepsy.