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Washington University Experience | VASCULAR | Respirator Brain (In Vivo Autolysis) | 3A0 Case 3 History
Case 3 History ---- This 6 year old girl was admitted with obtundation of unknown etiology. At age 20 months she had Hemophilus influenzae meningitis and her subsequent development was delayed. At age 2 1/2 she began having staring spells. An EEG at that time showed multiple spikes especially in the left hemisphere and she was started on anti-convulsants. At age 4 years she had a generalized tonic clonic seizure more on the left which lasted 1 hr. and required parenteral anti-convulsants. An X-ray at that time showed metallic objects in the bowel, but a lead level was only 45 ug/100 ml. An EEG at that time showed right occipital slowing. The patient remained on anti-convulsants. She continued to progress but more slowly than expected and she was having some difficulties in regular school. On October 7 she developed a generalized seizure (right>left) which lasted for over l hr, prompting a visit to the ER and treatment with intravenous anti-convulsants. Over 2 days in a local hospital she had a fever between 102-103oF and never regained consciousness. On October 9 she began having gastrointestinal bleeding. She had elevated prothrombin and partial thromboplastin times accompanied by elevated serum transaminase levels. Her salicylate level was 39. Because of the bleeding and coma she was transferred to SLCH. Admission exam at Children's Hospital showed temperature of 38.9°C; BP 90/60; Respirations 32; and Pulse 150. The general physical exam was unremarkable except the liver was 8 cm below the right costal margin. On neurologic exam, the patient was deeply stuporous. The pupils were 4 mm bilaterally, round and reactive to light. The fundi showed slightly distended veins. Extraocular movements were full to dolls head maneuver. Corneal reflexes were symmetric and depressed. The patient was intermittently spastic more in the legs than in the arms. The deep tendon reflexes were also increased more in the legs than in the arms and the toes were bilaterally upgoing. The patient was intubated on Oct. 10 and subsequently placed on a respirator. Over the period between 10/12 and 10/18 the patient's neurologic condition did not improve and she would decerebrate to noxious stimuli. She was intermittently hypotensive and required treatment with blood, fluids, antibiotics and pressors. On Oct. 10, an EEG showed bilateral slowing and low voltage and two days later an EEG showed no electrocerebral activity. On Oct. 21, the patient had a cardiac arrest and died. ---- Significant laboratory studies from the hospitalization included Hct 33.2; platelet count 75,000; and WBC 14,800. The initial blood glucose was 33, but all subsequent values were within normal limits. Lumbar puncture showed an opening pressure of 180 and a protein of 22; glucose of 25; and no white blood cells. Salicylate level was 11.8. SGOT on admission was 15,000, but after 1 week dropped to 107. Alkaline phosphatase was initially 840, falling to 360 after one week. Maximum bilirubin approximately 1 week after admission was 9.3 total and 5.7 direct. Prothrombin time on admission was 21.9 sec. and partial thromboplastin time was 52 sec. The urine and serum amino acids were within normal limits and the urine organic acids were within normal limits. EMI and skull X-rays were normal. ---- At autopsy the brain weight was 1110 grams. By report, sections from the cerebral cortex showed fragmentation of the gray matter and transcortical necrosis. Neuronal cytoplasm was distinctly eosinophilic in most areas, and nuclei were pyknotic or karyorrhectic. Pallor of the neuropil in the digital white matter was noted. Vascular congestion and thrombosis were prominent, including some meningeal vessels. The general autopsy showed centrilobular hemorrhage and necrosis involving the liver but it did not show microsteatosis. Reye disease was considered, particularly in a patient given salicylates with marked brain edema but the diagnosis was not compelling.
