[Acute neurologic dysfunction associated with a hypodensity of the basal ganglia]. / Disfunción neurológica aguda asociada a hipodensidad de los ganglios basales.

A previously healthy five years old boy, following a mild nonspecific upper respiratory infection developed, fever (39 degrees C), vomiting, clouding of consciousness and focal seizures. The CSF showed a mononuclear cell reaction with negative bacterial and viral cultures. A cranial CT scan on the 4th day of admission showed bilateral low density lesions on the basal ganglia region. After 30 days of severe involvement of muscle tone (rigidity) which kept the patient immobilized in bed and without a meaningful communication with his surroundings, improvement was noticed. A repeated CT scan 40 days after admission, was considered normal. Two months after the beginning of disease, patient's physical examination was normal. This case shows striking clinical and radiological similarities to the ones described by Aicardi and Goutieres in 1982 and most likely is explained by bilateral basal ganglia edema complicating viral encephalitis. Mumps virus, being so far, the most commonly implicated.

[Neonatal cholestatic icterus simulating atresia of the bile ducts in a patient with alpha-1-antitrypsin deficiency]. / Ictericia colestásica neonatal simulando atresia de vías biliares en un paciente con déficit de alfa-1-antitripsina.

A 40 days old infant with cholestasis is described. The liver was enlarged at 3 cm below the costal margin. No bile ducts were seen at the liver scan (IDA Tc 99) neither bile was collected after cholecystokinine IV administration. Fibrosi, bile ducts proliferation, and cholestasis without intracellular PAS positive material were seen at liver biopsy. Serum alpha-1-antitrypsin level was 42 mg/100 ml. Follow-up was satisfactory after phenobarbital and cholestiramine treatment. Cholestasis decreased and two weeks later bile excretion was obtained after cholecystokinine administration. This stress the importance of alpha-1-antitrypsin determination in cholestasis in infancy.