Faculty Bibliography

Echocardiography was performed on 11 patients with Friedreich's Ataxia. Eight of 11 had asymmetric septal hypertrophy and systolic anterior motion of the anterior leaflet of the mitral valve at rest or after inhalation of amyl nitrite. Two patients had concentric left ventricular hypertrophy. In view of this high incidence of hypertrophic cardiomyopathy, echocardiography is suggested as part of the routine evaluation of the patient with Friedreich's Ataxia

Two patients with Fisher's syndrome of ophthalmoplegia, ataxia, and areflexia experienced severe weakness and respiratory distress. Both patients required tracheostomy and assisted ventilation, but both made a complete recovery. Fisher's syndrome is generally considered to be a benign variant of acute infectious polyneuropathy (the Guillain-Barre syndrome). Our two patients demonstrate that the condition is not always benign

Echocardiograms were obtained in 10 normal patients and in 11 patients with respiratory insufficiency due to chronic obstructive lung disease (8) and to thoracic poliomyelitis (3). Only the eight patients with obstructive lung disease had paradoxic pulse. No patient had pericardial disease. The left ventricular internal dimension, stroke volume, and the mitral valve E-F slope and D-E excursion were measured. The inspiratory to expiratory ratio of each measurement was significantly lower in patients with obstructive lung disease than in normal subjects. The patients with thoracic poliomyelitis demonstrated almost no respiratory change in these measurements. The magnitude of the change in the measured factors probably relates to the degree of negativity of intrathoracic pressure during respiration. The inspiratory reduction of mitral valve motion and left ventricular internal dimension is not specific to tamponade but may be seen in patients who exhibit paradoxic pulse due to other conditions

Chest wall stimulation by electrical stimuli was employed to suppress permanent demand (ventricular-inhibited) pacemakers in two patients with competitive ventricular rhythms. The stimuli were nor perceived by either patient, and were continued until surgical intervention was effected. In selected patients with permanent pacemaker malfunction, chest wall stimulation may be appropriate and necessary initial therapy until definitive correction is performed