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Explore a case study of a male fetus following fetal demise, identifying brain abnormalities and predominant pathologies. Discuss brain regions affected, diagnosis of pontosubicular necrosis, and manifestations of hypoxic-ischemic injury.
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Case Study 30 Julia Kofler, M.D.
Question 1 The brain in this case is from a male fetus following intrauterine fetal demise at 40 weeks gestation. Pathologic examination of the placenta revealed chronic villitis of unclear etiology and acute chorioamnionitis due to Strep. B infection. Autopsy of the fetus revealed no significant pathology outside the brain, no congenital anomalies and a normal karyotype. Identify the predominant pathologic process in the following slides and high-power images.
The following powerpoint slides show high-power images of the abnormal brain regions. Each slide also shows gross images from a normal adult brain to help in the identification of the abnormal brain region. The white square indicates the brain region that corresponds to the section in the photomicrographs. Hint: Correct identification of the abnormal brain region may provide a clue for the diagnosis. Click here to view virtual slide.
Answer Karyorrhectic cell death of numerous neurons
Question 2 Which brain regions are primarily affected?
Answer Subiculum and basis pontis
Question 3 What is your diagnosis?
Answer Pontosubicular necrosis
Question 4 What is the predominant mode of cell death in pontosubicular necrosis?
Answer Apoptosis
Question 5 What is the cause of pontosubicular necrosis?
Answer Hypoxia/ischemia
Question 6 During which time period does pontosubicular necrosis occur?
Answer • Pontosubicular necrosis can be seen from 28 weeks gestation to 2 postnatal months • The initiating hypoxic insult may occur in utero, in the perinatal or postnatal period
Question 7 Name other gray matter manifestations of perinatal hypoxic-ischemic injury?
Answer • Cerebral cortex • Basal ganglia and thalamus (the combination of neuronal loss, gliosis and abnormal myelination in these brain regions may result in a white, firm, marbled appearing lesion and is called status marmoratus) • Brainstem nuclei • Cerebellum (granular layer)
Question 8 Which portion of cortical gyri is typically affected?
Answer The depths of sulci
Question 9 Hypoxic-ischemic injury to the sulcal depth gives the damaged gyrus a characteristic mushroom appearance. What is the correct term for this condition? An example can be seen in the following slide. Click hereto view slide.
Answer Ulegyria
Question 10 Which neuronal populations are most susceptible to global hypoxic-ischemic injury in the adult brain?
Answer • CA1 sector of the hippocampus • Purkinje cells in the cerebellum • Cortical neurons in laminae 3 and 5