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Washington University Experience | VASCULAR | Congophilic Angiopathy (CAA) | 7A0 Case 7 History
Case 7 History ---- The decedent was a 74 year old woman with a history of hypothyroidism, hypertension and melanoma. In July of 2015 she began having refractory generalized nonconvulsive seizures and hallucinations. By September, she had developed worsening confusion, right gaze deviation and left-sided neglect. MRI showed cortical atrophy in addition to right temporal, occipital, and thalamic lesions concerning for malignancy. Many microhemorrhages were seen in the cortical gray matter on susceptibility imaging. A paraneoplastic panel was positive for voltage-gated potassium channel (VGKC) antibodies at a titer of 0.42 nM but was initially felt to be an incidental finding. On September 28, biopsy showed cerebral amyloid angiopathy with Alzheimer disease type changes. Around the time of the biopsy, there was clinical concern for refractory status epilepticus secondary to autoimmune encephalitis associated with voltage-gated potassium channel antibody. She was started on IV IgG and prednisone and eventually Versed drip and Klonopin. Anti-epileptics included Topamax, Lamictal, Keppra and fosphenytoin. She was discharged but returned on October 13 with worsening confusion and decreased use of her left arm. Brain MRI demonstrated cortical diffusion restriction of the right temporoparietal lobe concerning for sequelae of seizure. She was admitted to the ICU and had intermittent subclinical seizures throughout her stay. She underwent G-tube placement on Oct 30 and tracheostomy placement on Nov 3 due to prolonged ventilation issues. She continued to have seizures throughout her stay at BJH. She was transitioned to hospice care and expired on November 23. ---- At autopsy her unfixed brain weight was 1215g. She had Alzheimer Disease Neuropathologic Change which was Intermediate in severity. There was multifocal hypoxic ischemic injury ranging from selective neuronal necrosis to frank infarction in the neocortex, hippocampus, basal ganglia and thalamus. Microhemorrhages and microinfarcts accompanied cerebral amyloid angiopathy, especially in the temporal lobes. In no region of the brain did we find evidence for an infectious, vasculitic or auto-immune process.
