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Washington University Experience | NEURODEGENERATION | Progressive Supranuclear Palsy (PSP) | 18A0 Case 18 History
Case 18 History ---- This 66yo male was re-admitted to Barnes Hospital for intermittent confusion and deterioration following previous surgery. In the week prior to admission, he developed paucity of movements; he was no longer able to walk without support and was incontinent of urine and feces. He was also unable to feed himself, his movements were slow and he was extremely confused with very little speech. His movements were slow, halting and rigid. He had virtually no horizontal or vertical eye movements. He was now re-admitted to Barnes because of progressive ophthalmoplegia, blunting of affect and increased mental confusion. Past medical history included multiple myeloma since December 1975. In June 1976, he was found to have IgG lambda light chain paraprotein. His treatment was Melphalan and prednisone. In February 1980, he was not responding to this treatment and there was a significant increase of urinary excretion of this paraprotein. His treatment was changed to Cytoxan, BCNU and prednisone. Under this therapy, the myeloma seemed to be reasonably well controlled. He had mild renal insufficiency, moderate cytopenia and a question of normal pressure hydrocephalus with cerebral atrophy. A ventricular atrial shunt with a medium pressure Hakim valve was inserted in September 1980, and he was discharged on his last admission essentially unimproved. CT scan on the afternoon of admission showed the ventricles to be collapsed to a small size and there were large bilateral isodense subdural hemorrhage. The patient was submitted to a revision of the ventricular atrial shunt and to drainage of the bilateral subdural hematomas . First, the shunt was revised. Then, the subdurals were drained through ventriculostomy catheters. The subdural fluid was thin and had a darkish-brown appearance. Immediately postoperatively, he seemed to be more alert, responding to painful stimuli and to commands. Repeat CT scan on 10/ 31 showed the subdurals to be only slightly smaller and the ventricles only slightly larger. His mental status remained unchanged, however. In November 1980 he was submitted to a repeat drainage of the subdural hematomas. Postoperatively, there was also no improvement in his mental status. He essentially had a progressive downhill course with progressive obtundation. He became febrile and then passed away.
