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Washington University Experience | PRION DISEASES | Prion Diseases | 5A0 Case 5 History
Case 5 History (AANP DSS 1980-08) ---- This 77 year-old woman was admitted to MGH because of progressive visual disturbance. One month earlier she developed a distortion in her usually excellent penmanship, and then complained of seeing letters abnormally elongated and widened. She found it difficult to recognize her friends. When examined at the Mass. Eye and Ear Infirmary, her visual acuity was 20/40 O.U., and her color recognition was intact. There was a right homonymous hemianopia. Her memory was good, but she had slight difficulty following 3-step commands. She copied complex geometric figures poorly and had difficulty identifying photographs of prominent persons. A CT scan showed cortical atrophy with slightly enlarged lateral ventricles. For several days before admission, she stumbled around her house, frequently breaking dishes, and apparently no longer able to discriminate forms or shapes. She could detect light but had no color vision. She described the image as "plaid". On examination at MGH, she could recall none of three objects after three minutes. Her speech was slow, fluent, and without paraphasic errors, but there was marked perseveration. Muscle tone was increased. Sensation was intact, but tactile localization, graphesthesia, and stereognosis were poor. Deep tendon reflexes were brisk, and the plantar reflexes were flexor. Within one week, she was mute and unresponsive. EEG one month later showed almost no background with periodic bursts of polyphasic sharp waves. The patient was transferred to another hospital where she remained in a vegetative state and died one year after the first symptoms of her illness appeared.
