Table of Contents
Washington University Experience | PRION DISEASES | Prion Diseases | 20A0 Case 20 History
Case 20 History ---- The patient is a 67 year old man who was retired for five years from a pigment factory. He was in good health until 8/93 when he started to complain of visual difficulties, including blurred vision and decreased acuity which progressively worsened. In 10/93 he developed blindness to his left visual space and he began to complain of difficulty with his left arm. The arm was continually held in a semiflexed involuntary position and it sometimes would move on its own, such as an alien hand. He developed a festinating gait culminating in loss of the ability to walk in 10/1993. At that time was having difficulty with his mentation with inappropriate speech and memory difficulties. He was admitted to an OSH in 10/1993 and after admission there was noted to have myoclonic episodes involving all four limbs. During the last week of 10/93 he began developing dystonic movement of his right hemibody. During that admission he stopped eating and developed apusitive speech to the point where he would only answer questions with yes or no. He also became much more lethargic. He was transferred to BH at the end of October 1993. He had no family history of dementing illnesses. All four of his limbs were in contraction, right greater than left. He had increased tone throughout including his neck. His deep tendon reflexes including his jaw. The patient was felt to have a rapidly progressive dementia with components of abnormal movement. The differential diagnosis included subacute spongiform encephalopathy, Leydig encephalitis, carcinomatous meningitis and herpes encephalitis. A lumbar puncture was performed which revealed a CSF protein of 81, glucose of 93 and no white blood cells. A repeat EEG was performed which showed generalized slowing in the setting of excessive amounts of theta and delta activity but no specific correlation with CJD. An MRI scan was performed on 11/1993 which showed mildly enlarged ventricles and sulci. There was no abnormal signal within the grey matter and the study was felt to be normal for the patient' age. The patient continued to slowly deteriorate. The patient spiked a fever on 11/3 and his sputum grew out abundant Staphylococcus 'aureus and, following treatment, he became progressively weaker and expired at the end of November 1993.
