The clinical features of immunoglobulin light-chain (AL) amyloidosis with heart involvement

We reviewed clinical presentation, investigations, therapy, prognosis and outcome of 232 patients with primary (AL) cardiac amyloidosis. There were 142 men and 90 women. Median age at presentation was 59 years (range 29-85). AL heart disease was unusual both in patients under the age of 40 (3.0%) and in non-Caucasians (6.5%). Fatigue and weakness were the commonest presenting symptoms. Hallmark features of periorbital ecchymoses and macroglossia were present in 12.5% and 27.2%, respectively. AL cardiac amyloidosis was unusual in isolation (3.9%), and most frequently patients had features of multiorgan dysfunction; heavy proteinuria and features of malabsorption predominating in this respect. Heart involvement represents the worst prognostic indicator, with a median survival from diagnosis of 1.08 years, falling to 0.75 years with the onset of heart failure. Current therapeutic procedures appear to prolong survival, with left ventricular wall thickness, mass and ejection fraction on echocardiography and late potentials on signal averaged electrocardiography of use in prognostic stratification. Cardiac involvement from AL amyloidosis is rapidly fatal. It should be suspected in all patients with heart failure who have wall thickening on echo, normal chamber sizes, low EKG voltages and evidence suggesting a multisystem disease.      

Relation between reaction time and loudness

The loudness of one-third octave bands of noise centered at either 1, 2, or kHz was measured in 10 normal-hearing young adults for sound levels of 50-90 dB SPL. Reaction times (RT) in response to these same stimuli were also measured in the same subjects. A moderate-to-strong correspondence was observed between the slopes for functions depicting the growth of loudness with sound level and comparable slopes for the reaction-time data. The correlation between slopes for the RT-intensity function and the loudness-growth function was comparable in magnitude to the test-retest correlation for the loudness-growth function except at 1 kHz.     

Temporomandibular joint arthrography: comparison of morbidity with ionic and low osmolality contrast media

We compared patient morbidity associated with temporomandibular joint (TMJ) arthrography using both meglumine/sodium diatrizoate (60%) and the new monoacidic dimer, Hexabrix, in a double-blind randomized clinical trial in 31 patients. Patients experienced maximal discomfort from TMJ arthrography with the initial joint filling and joint distension; this rapidly resolved over 10 minutes. Delayed exacerbation of pain is less than described for shoulder arthrography. The newer contrast media promise to decrease patient morbidity with arthrography.