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Washington University Experience | VASCULAR | Hypoxia-Ischemia, neonate, Multicystic Encephalopathy (MCE) | 1A0 Case 1 History

1A0 Case 1 History
Case 1 History ---- The patient was twin B born after 34 weeks gestation with a birth weight of 1565 grams. Pregnancy was complicated by maternal tobacco abuse. Apgar scores of 8 and 9 and her initial post-natal course was uneventful. At 2 weeks of age she developed Group B streptococcal sepsis and meningitis culminating in shock and lethargy and required intubation. She recovered after appropriate treatment but was left with severe, diffuse residual brain injury. CT scan showed marked diffuse hemispheric lucencies and, 2 months later, severe atrophy and parenchymal calcifications. EEG showed diffuse burst suppression patterned with absence of normal background activity. Additional sequelae of her meningitis included hypothalamic dysfunction, diabetes insipidus, hypoadrenalism, cortical blindness as well as optic nerve damage with left afferent pupil and bilateral hearing loss. She was also noted to have hypertrophic cardiomyopathy with a left ventricular outflow obstruction. Follow-up neurological examination revealed severe microcephaly, severe spastic quadriparesis, and persistent bilateral asymmetric tonic neck reflex. She developed a chronic seizure disorder and recurrent episodes of hypernatremic dehydration. On the day of expiration she had fever associated with lethargy and poor feeding and was then found breathless and pulseless in bed by her mother. She was brought to the ER where during intubation a brown fluid was aspirated from the trachea. Prolonged CPR attempt failed and she expired at 9 months of age.



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