High power, low frequency components of cardiac, renal, splenic and vertebral sympathetic nerve activities are uniformly reduced by spinal cord transection

Others have reported that spinal cord transection non-uniformly affects the activities of the cardiac, renal and splenic nerves. We were unable to confirm this finding while recording the wide band (1-1000 Hz) discharges of these sympathetic nerves in baroreceptor-denervated, chloralose-anesthetized cats. Most of the power in nerve discharges was below 6 Hz. There was high coherence between the low frequency discharges of different nerves, and power below 15 Hz was essentially eliminated by spinal transection. Evidence is presented that the disparities between this and past studies are, in part, due to the use of a 30-Hz high-pass filter in the earlier studies.     

Discordant repeat size and phenotype in Kennedy syndrome

Previous reports in the literature have described correlation of increasing repeat length with severity of the phenotype, in Kennedy syndrome. We describe male siblings with different repeat lengths, with lack of expression of the phenotype in the sibling with the longer repeat length. The phenotype was identical to motor neurone disease. There is variability of expression in Kennedy syndrome and repeat length even in siblings cannot be taken as a conclusive indicator of severity. CAG repeat length cannot be used to predict the natural history of Kennedy disease. The diagnosis of Kennedy syndrome should be considered in male patients presenting with atypical motor neurone disease.     

Ureteral pseudodiverticulosis associated with carcinoma of renal pelvis

We describe a patient with bilateral ureteral pseudodiverticulosis of 10 years’ duration before developing transitional carcinoma of the pelvis.     

Diagnosis and management (by subxiphoid pericardiotomy) of large pericardial effusions causing cardiac tamponade.

To determine the clinical features, course and outcome of patients with cardiac tamponade, 57 consecutive patients with new, large pericardial effusions were prospectively studied. Twenty-five patients (44%) developed cardiac tamponade with venous hypertension and a pulsus paradoxus greater than 10 mm Hg. Electrocardiography, radiographic studies and echocardiography did not differentiate patients with and without tamponade. All 57 patients underwent thorough diagnostic evaluation followed by subxiphoid pericardial biopsy and drainage. A diagnosis was obtained in 53 patients (93%). Collagen vascular disease was significantly more frequent in the 25 patients with than in the 32 without cardiac tamponade (24 vs 3%; p less than 0.05). The frequency of malignant and uremic effusions was equal in both groups, whereas radiation-induced effusions seldom produced tamponade. At 1-year follow-up, 3 patients (12%) with tamponade had recurrent effusions, and 1 needed reoperation. This was not significantly different from the 32 patients without tamponade. Twelve-month mortality was also similar in both groups (36 vs 44%). This prospective series disclosed several unexpected findings: (1) Cardiac tamponade occurred in almost 50% of patients with new large pericardial effusions; (2) both malignancy and collagen vascular disease occurred with equal frequency as etiologies, whereas radiation-induced tamponade was unusual; (3) thorough clinical evaluation resulted in few idiopathic etiologies; and (4) subxiphoid pericardiotomy was effective for both diagnosis and therapy of tamponade.     

Asymptomatic renal abscess: Evaluation with gadolinium DTPA-enhanced MRI

Incidental detection of renal mass in a transplant patient is a diagnostic challenge. These patients are at risk for the development of neoplasms and, more commonly, infection with atypical organisms. Symptomatology may be lacking in either, and both conditions may present with similar symptoms. Magnetic resonance imaging (MRI) evaluation with gadolinium DTPA is helpful in the evaluation of asymptomatic renal mass, with enhancement effectively excluding simple and hemorrhagic cysts. Because the MRI appearances of renal cell neoplasm and renal abscess overlap, both must be considered in the differential diagnosis.     

Autopsy-proven Huntington's disease with 29 trinucleotide repeats.

Huntington's disease (HD) is a neurodegenerative disorder associated with expansion of CAG trinucleotide repeats in the huntingtin gene. A minimum of 36 CAG repeats is usually reported in patients with clinical features of HD; 30 to 35 repeats represent an intermediate range. Here we report a 65-year-old male with autopsy-proven HD and 29 CAG repeats.