Pathophysiologic Findings in a Patient with Shy-Drager and Alveolar Hypoventilation Syndromes: Pulmonary centrilobular emphysemaAverage inspiratory time, average cycle length, and the coefficient of variation (SD/M) for respiratory timing increased from wakefulness to sleep. In contrast, the coefficient of variation for heart rate was similar during wakefulness and sleep. There were no significant changes in respiratory pattern with medroxyprogesterone acetate (120 mg, qd for three weeks) or with acetazolamide, administered once (250 mg intravenously). The patient received positive pressure ventilatory support only during sleep. Two months after first evaluation, the patient was found pulseless, without blood pressure or respirations one-half hour before his customary bedtime.

Autopsy demonstrated mild pulmonary centrilobular emphysema without evidence of pulmonary hypertension. There was atherosclerosis of the aorta, and coronary and renal arteries. There were multiple thyroid adenomas, a pituitary microadenoma, and parathyroid chief cell hyperplasia. A small nodule of prostatic tissue showed a well differentiated adenocarcinoma with extension only into the seminal vesicles. buy actos online
In the cerebral hemisphere, two small cystic infarcts were present, one in the left angular gyrus and the other involving only a few folia of the cerebellum. The substantia nigra was pale on visual inspection. In the substantia nigra and locus ceruleus, the neuronal population was decreased, pigment was scattered throughout the tissue or within macrocytes, and there was a microglial reaction. No Lewy bodies were present. Multiple sections of brainstem and serial sections of the medulla including myelin and Holzer stains revealed no gliosis or decrease in nuclear masses. Cell counts of neurons in the intermediolateral columns at eight thoracic levels of the spinal cord were performed. There was a loss of cells, when compared to counts from identical levels from a normal patient of similar age. The thoracic ganglia were the sites of a mononuclear cell infiltrate. The vagus, phrenic, peroneal, and femoral nerves showed axonal loss and disruption. Samples of skeletal muscle as well as diaphragm showed changes consistent with denervation atrophy.