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Washington University Experience | VASCULAR | Subcortical Arteriosclerotic Encephalopathy | 1A0 Case 1 History
Case 1 History ---- This 86 year old woman was first seen due to a family history of dementia in her son who carried the clinical diagnosis of FTD. Her past medical history was significant for chronic inflammatory demyelinating polyneuropathy which was treated with steroids, pulmonary embolism S/P inferior vena cava filter placement and an abdominal aortic aneurysm. In 1994, she had a two-hour period of confusion and difficulty writing followed by full recovery, thought to be a TIA vs. a small stroke. At that time there were no changes in memory or thinking (Clinical Dementia Rating, CDR 0). At age 89, she experienced a major decline in judgment and in memory. Her personality had changed in the past year in that she became more quiet and withdrawn (rated by her clinician, as CDR 0.5, DAT [Dementia of the Alzheimer type]). She had been diagnosed with hypertension and had a pacemaker placed due to syncopal episodes. There was a decline in her bridge playing and her reading ability. On examination, there was evidence of bradykinesia but not rigidity or tremor. Her gait was abnormal with marked postural instability. Mild apraxia was noted. At that time she received a diagnosis of (DAT, CDR 1) with idiopathic parkinsonism. In March 2007, she had a lengthy hospitalization for abdominal aortic aneurysm repair. There was a time during her hospitalization when her speech became gibberish and she could not feed herself with her right hand. After many weeks, she became conversant and was steadily improving in September 2007. Her daughter noted a hand tremor at rest but there was no history of hallucinations. She had a significant decline following abdominal aortic aneurysm surgery, followed by a possible stroke. Her family stated that she had gained back much that she had lost when she had a sudden sharp left flank pain, became unresponsive and died in October 2007 at age 91. ---- At autopsy the fresh brain weighed 1,050g. Infarcts were demonstrated in the left posterior temporal white matter (L34), pallor and cavitation in the left centrum semiovale pallor, early cavitation and axon loss, along with severe arteriolosclerosis (L27), right precentral gyrus white matter (R36) and right thalamus (R35).There was moderate to severe accompanying cerebral arteriolosclerosis and atherosclerosis of some cerebral vessels. There are only minimal Alzheimer's disease neuropathologic changes including a few diffuse amyloid plaques (but no neuritic types). The neurofibrillary tangles were limited to the medial temporal lobe (entorhinal cortex, subiculum, amygdala and hippocampus), Braak and Braak Stage III. These neurodegenerative changes are not sufficient to meet the neuropathologic criteria for the diagnosis of Alzheimer's disease according to the Khachaturian, CERAD, NIA-Reagan Institute or NIA/AA criteria. The findings were interpreted as vascular dementia. There was no evidence of cortical or brainstem Lewy bodies, Pick bodies or evidence of any other neurodegenerative disease.
