Rollin J. Hawley

Cardiac manifestations in polymyositis

Twenty-one patients with polymyositis were prospectively examined with echocardiography, phonocardiography and electrocardiography. Cardiac performance, estimated with echocardiography, was enhanced as shown by a significant (P less than 0.01) increase in ejection phase indexes of left ventricular function compared with values in a matched control group. Known causes of the high output state, such as anemia or thyrotoxicosis, were not clinically evident. There was no evidence of left ventricular enlargement, left ventricular wall hypertrophy, or left atrial enlargement in the echocardiogram or chest X-ray film. The echocardiogram showed systolic mitral valve prolapse in 11 of 17 patients (65 percent) with an adequately imaged mitral valve; midsystolic clicks were present in 7 of these. One patient, who did not have prolapse, had echocardiographic evidence of a small pericardial effusion. Electrocardiographic abnormalities were present in 11 of 21 patients (52 percent) and included evidence of atrioventricular conduction disturbances, atrial and ventricular arrhythmias and left atrial abnormality. The pathophysiology of mitral valve prolapse and increased systolic left ventricular function in polymyositis remains uncertain; however, the spectrum of cardiac abnormalities, detected noninvasively in 16 of 21 of our patients (76 percent) may represent a high frequency rate of cardiac involvement in this disease.

Myotonic heart disease A clinical follow‐up

We followed 37 patients with myotonic dystrophy for a mean of 6 years. Two developed atrial flutter or fibrillation, 6 developed a new bundle branch block, 1 developed complete heart block requiring a pacemaker, and another with progressive 1st-degree heart block and a widening QRS interval had a sudden death. Most patients had predictable, gradually progressive disease of their cardiac conduction system. We recommend that patients with progressive atrioventricular block or widening QRS interval due to myotonic heart disease have yearly ECGs and be questioned about syncope or presyncope to determine the need for a cardiac pacemaker.

Left ventricular relaxation, mitral valve prolapse, and intracardiac conduction in myotonia atrophica: Assessment by digitized echocardiography and noninvasive His bundle recording

Myotonia atrophica, a neuromuscular disease marked by autosomal dominant transmission and delayed relaxation of skeletal muscle, has been associated with cardiac failure, conduction abnormality and mitral prolapse (MVP). In order to determine the relaxation rate of cardiac muscle, left ventricular (LV) size and function, and the presence of MVP, 30 patients with myotonia atrophica were studied using digitized M-mode echocardiography (MME). Intracardiac conduction intervals were determined by noninvasive His bundle recording (HBR) from surface electrodes using a high-resolution, R-wave triggered, signal averaging computer. Neurologically unaffected first-degree relatives of the patients with myotonia atrophica were also studied to determine if cardiac abnormalities may be present in the absence of neurologic manifestations of the disease. Peak normalized diastolic endocardial velocity in patients with myotonia atrophica (3.7 +/- 0.8 sec-1) did not differ from unaffected first-degree relatives (3.8 +/- 0.8 sec-1) or normal subjects (3.6 +/- 0.8 sec-1). Systolic LV function and LV dimensions on MME were normal in both groups. However, MVP was present in 7 of 24 (29%) of patients who could be evaluated, but not in unaffected first-degree relatives. Despite normal LV systolic and diastolic function, infranodal intracardiac conduction was prolonged in patients with myotonia atrophica (average HV interval 50 +/- 5 SD msec) but not in neurologically unaffected relatives (average HV interval 40 +/- 5 msec). Delay in proximal intracardiac conduction was also found in patients with myotonia atrophica (average PH interval 140 +/- 20 msec) but not in neurologically unaffected relatives (average PH interval 115 +/- 6 msec). Hence cardiac findings in myotonia atrophica include proximal and distal conduction delay by external HBR even in the absence of abnormality of the standard 12-lead ECG. There may also be an increased frequency of MVP; however, early diastolic relaxation of the LV is unimpaired, and cardiac manifestations of myotonia are not transmitted independently of neurologic abnormality.

Ventricular Late Potentials in Myotonic Dystrophy

OBJECTIVE To determine the prevalence of ventricular late potentials, as determined by signal-averaged electrocardiography, in patients with myotonic dystrophy. DESIGN Cross sectional, with blinded analysis of all electrocardiographic data. SETTING Outpatient departments of a Veterans Affairs medical center and a tertiary care private hospital. PARTICIPANTS Twenty-four patients with myotonic dystrophy. Patients were excluded from the study if they had either a history suggestive of significant ventricular arrhythmias or electrocardiographic evidence of a bundle-branch block. Two comparison groups were also formed; one group included 44 healthy employees at the tertiary hospital and the other, 30 cardiac patients with inducible ventricular tachycardia. MAIN RESULTS A time-domain analysis of the signal-averaged electrocardiograms showed that 75% of patients with myotonic dystrophy met one criterion for the presence of late potentials, 67% met two criteria, and 29% met all three criteria. Spectrotemporal mapping in these patients showed markedly abnormal spectral peaks with a mean factor of normality that was significantly lower than that of the normal volunteers; the frequency of electrocardiographic abnormalities approached that seen in patients with known ventricular tachycardia. The presence of late potentials correlated directly with the length of the PR interval and inversely with left ventricular fractional shortening. CONCLUSIONS In our study, the prevalence of late potentials on signal-averaged electrocardiography in patients with myotonic dystrophy approached that seen in cardiac patients with inducible ventricular tachycardia. It is possible that ventricular arrhythmias play a role in the occurrence of sudden death in some patients with myotonic dystrophy.

Cerebrospinal fluid cyclic nucleotides and GABA do not change in alcohol withdrawal.

Abstract Gamma-aminobutyric acid (GABA) and cyclic adenosine monophosphate (AMP) and guanosine monophosphate (GMP) were compared in the cerebrospinal fluid (CSF) of 13 patients suffering from acute alcohol withdrawal, and the same patients during convalescence. No significant changes were found, but CSF norepinephrine (NE) levels were elevated in acute alcohol withdrawal and decreased toward normal during convalescence.

Human brain: Aldehyde dehydrogenase activity and isozyme distribution in different areas

Three human post-mortem brains were dissected into seventeen areas and assayed for aldehyde dehydrogenase (EC 1.2.1.3) activity employing two assay systems: one at 68 microM and another at 13.6 mM propionaldehyde. The levels of activity with 68 microM propionaldehyde were significantly higher in cerebellum and putamen. The same brain areas were also examined by isoelectric focusing. By this procedure two distinct bands of aldehyde dehydrogenase activity (the cytoplasmic E1 and mitochondrial E2) could be readily visualized in cerebellum and putamen while other brain areas contained mainly the mitochondrial E2 isozyme.

Trace element levels in human alcoholic brain

Copper, magnesium, zinc and manganese levels in the temporal cortex of human alcoholic and control brains were measured by atomic absorption spectrophotometry using both the flame and graphite furnace. All of the 21 alcoholics were male with a mean age of 54.1 years; the 19 male controls had a mean age of 60.2 years. The only statistically significant change in ion levels was an increase in manganese concentration (expressed both as microgram/g wet weight and ng/mg protein) in the alcoholic group when compared to the control group. Five of the alcoholics had malignancies, while 16 of the controls had systemic malignancies. Covariance analysis showed there was no effect of age on the level of manganese in the temporal cortex.