Brain Pathology Case of the Month - May 1998

Contributed by Lawrence C. Kenyon, MD, PhD,1 Herbert I. Goldberg, MD,2 Dennis L. Kolson, MD,3 PhD, Ronald G. Collman, MD,4 and Ehud Lavi, MD1
1Division of Neuropathology, Department of Pathology and Laboratory Medicine, 2Division of Neuroradiology, Department of Radiology, and 3Department of Neurology and 4Medicine, University of Pennsylvania Medical Center, Philadelphia, PA 19104


This was a 36 year old man known to be infected with HIV for 3 years but was clinically well until he presented with decreased visual acuity, back pain and mild leg weakness. He was diagnosed with CMV retinitis and cauda equina polyrediculitis and was treated with intravenous daily gancyclovir. He remained stable for 4 months when he developed high spiking fevers and headache. CSF examination revealed glucose of 30 mg/dL, protein 145 mg/dL, 90 RBC's, and 369 WBC's (70% segmented neutrophils, 30% monocytes). Gram stain, bacterial culture, India ink preparation, cryptococcal antigen, AFB smear, and cytology of the CSF were all negative. Therapy was isoniazid (INH), rifampin, ethambutol and pyrazinamide (PZA) for possible tuberculosis was added to his regimen of gancyclovir. Several days later he developed severe bilateral radicular leg pain, leg weakness and urinary retention which slowly improved over the next few months. He then developed two weeks of episodic fevers of 1020F and severe headaches. Over the course of two days, he developed severe dysphagia to both liquids and solids resulting in profound dehydration.

On admission he was tachycardic, and normotensive. He had oral thrush. The remainder of his general physical examination was normal. He was alert and fully oriented with dysarthric speech but normal language function. His memory was mildly impaired. Fundoscopic examination revealed bilateral retinal changes consistent with acute and chronic CMV retinitis and HIV retinopathy. He had a bilateral course horizontal end gaze nystagmus, with both upward and downward gaze. He had right peripheral 7th nerve palsy, and diminished gag reflex bilaterally. On motor examination, the arms were normal, however, he had bilateral leg weakness of 4 to 4+/5 with the right leg slightly stronger than the left. Cold sensation was diminished in a patchy distribution over the right leg but tactile and vibratory sensation were normal. Tendon reflexes were diminished to absent throughout and his gait was unsteady. Routine laboratory studies revealed anemia, mild leukopenia, and moderately abnormal LFT's.

On the fifth hospital day the patient had a barium swallowing study to evaluate the patient's dysphagia. He suddenly developed severe respiratory distress due to massive barium aspiration. Despite broad spectrum antibiotic therapy the patient expired two days later.




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