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Washington University Experience | VASCULAR | Sickle cell anemia | 10A0 Case 10 History - Copy
Case 10 History ---- This patient was a 44 year-old African-American man with sickle cell trait with a history of left thalamic hemorrhage with right hemiplegia, aphasia and abulia 11 years prior to death. He also had a distant history of motor vehicle accident with occipital craniotomy. In 2002 he developed seizures at his nursing home and in the ER he was hypoxic (O2 saturation 70%), febrile, severely acidotic (pH=6.89) and required intubation. He was unresponsive to voice, had symmetric pupils with minimal reaction, and a spastic right upper extremity. Head CT revealed diffuse cerebral atrophy and multiple hemorrhages in the right parietal lobe and right and left basal ganglia. He required pressors for severe hypotension. Lumbar puncture was unremarkable. During 30+ days in the NeuroICU, he had multiple medical complications (bullous drug eruption, acute tubular necrosis) without significant changes in his neurologic examination (eyes open, did not follow commands, pupils reactive, withdrew to pain). A tracheostomy and G-tube were placed. After placement on a regular floor and awaiting transfer, he was found pulseless and was unable to be revived. ---- At autopsy the weight of the unfixed brain was 1150g. Multiple resolving and remote hemorrhages, hemorrhagic infarcts and pale infarcts were identified in the cerebral hemispheres, deep gray nuclei and brainstem. A microaneurysm with extravasation of RBC was noted in the left thalamus. Neither amyloid angiopathy nor ongoing vasculitis was found; no thrombi were found in the vascular lumina. Coronal sections showed an incidental intraventricular subependymal giant cell astrocytoma (SEGA); however, no areas of candle guttering, frank cortical tuber formation or extracerebral manifestations were noted in the case. Neuron loss and astrocytosis were marked in the hippocampal endfolium and Sommers sector. ---- The small vessel disease in this patient is most reminiscent of arteriolosclerosis associated with hypertensive disease. The nephrosclerosis and microvasculopathy in the kidney and increased heart weight with left ventricular hypertrophy also supports the presence of significant hypertension in this patient. Additionally, there is a likely contribution of sickle cell trait which could worsen hypertensive microvasculopathy, especially since there were sickled cells in areas of local hypoxia, worsened by a superimposed seizure disorder which may have contributed to hippocampal sclerosis. Addition of superimposed coagulopathy with DIC may have also contributed terminally. This case illustrates the difficulty of interpreting the pathologic findings in a complex case with multiple contributors to ischemic and hemorrhagic lesions; however, multiple pathways very likely participated.
