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Washington University Experience | VASCULAR | Infarct - Embolic | 14A0 Case 14 History
Case 14 History ---- The patient was a 59-year-old man with a history of COPD and a family history of Huntington disease involving several siblings; he, himself, showed no symptoms of Huntington disease. A week prior to his death, he presented to an OSH ED, where he was diagnosed with an upper respiratory infection, and was treated with antibiotics. A few days later, he presented to a second ED with a 2-3 day history of weakness and fever. His neurological examination identified no focal findings, but while in the ED, he developed a headache and decreased level of consciousness. Head CT showed multiple intraparenchymal hemorrhages, the largest, 2.7 cm, was located in the temporal lobe. A hemorrhage in the left midbrain measured 1.3 cm. He was intubated for airway protection and transferred to BJH NICU on 7/3. Upon arrival, CT of the head demonstrated enlargement of the multiple brain hemorrhages, as well as development of intraventricular hemorrhage, and hydrocephalus; the midbrain hemorrhage extended into the pons. He was not arousable to painful stimuli. His pupils were fixed and dilated with no Doll’s eye reflex, but gag and cough reflexes were intact. He had an extensor motor response in both upper extremities and a triple flexion response in both lower extremities. Clinical laboratory studies were remarkable for: D-dimer of >20, hematuria, leukocytosis of 17,100 (93% neutrophils), PTT 39, INR 1.2, and 18K platelets. He received two platelet transfusions, which increased his platelet count to 74K. The patient’s spontaneous intraparenchymal hemorrhages without trauma in the setting of fever and laboratory evidence of sepsis was concerning for septic embolization. Death was declared on 7/3. ---- Autopsy showed left thalamic lacunar hemorrhage with intraventricular extension into cerebral aqueduct and numerous hemorrhagic infarcts associated with septic emboli as well as Huntington's disease, Vonsattel grade 2. These findings were consistent with sequelae of bacterial endocarditis, which was identified in the general postmortem examination, accompanied by septic emboli involving the kidneys and spleen. Blood cultures initiated on 07/03 identified Staphylococcus aureus as the causative agent. Most critical for definitive diagnosis of Huntington disease were intranuclear inclusions with immunoreactivity for ubiquitin and polyglutamine, present within neurons of the moderately atrophic caudate and putamen.
