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Washington University Experience | VASCULAR | Amyloid-Beta Related Angiitis (ABRA) | 4A0 Case 4 History
Case 4 History ---- An elderly lady was referred to the Cognitive Assessment Clinic (CAC) 13 months before death, at the age of 81 years, with a 1 year history of difficulties with short term memory and decreased concentration. Assessment showed that she had poor insight into her problem, with decreased verbal fluency and abstract thinking; she was disoriented to day and date; MMSE score was 22/30. Neurological and clinical examination were normal apart from BP of 170/80. She bad been treated for hypertension and high cholesterol for a number of years and had an angioplasty 4 years previously. Lab tests, apart from raised serum bilirubin, were normal. CT scan showed generalized atrophy with no focal lesions. At the time of first assessment there was still no functional effect on daily life managing her own bank account, entertaining and enjoying playing bridge and golf. At follow up 3 and 6 months later, there was no obvious deterioration; repeat MMSE (at 6 months) was 23/30. Her 4th scheduled visit was to be 12 months from the initial assessment. However, at 10 months she started complaining of headaches, treated with Tylenol 3. At eleven months there had been significant deterioration in her cognitive abilities, she was withdrawn, suffered unexplained falls and was admitted to hospital for treatment of a urinary tract infection. She was admitted to a chronic care facility. Several weeks later she stopped eating, drinking, talking and required assistance with her ADL's. Four weeks prior to death with decreased level of consciousness; she was dehydrated, bad no verbal response, limited gaze towards right; she moved all limbs in response to pain stimulus. Lab tests showed only raised white cell counts (13,100). She was re-hydrated and treated for a UTI and left lower lobe pneumonia. MRI showed mild ventricular dilation, some changes suggestive of possible small cerebral vessel disease and generalized atrophy. Despite antibiotic therapy she continued to deteriorate; a PEG was inserted to assist with feeding. EEG showed diffuse slowing with no periodic complexes. Serial CTs showed progressive enlargement of her ventricles. CSF showed raised white cell count and protein with normal glucose. Blood and CSF cultures were negative. Following an infectious disease consult she was started on amphotericin B. Her respiratory status continued to deteriorate, she died in hypoxic respiratory failure, 13 months after her initial visit to the CAC.
