Adult-Onset Neuronal Ceroid Lipofuscinosis in a Shikoku Inu.

A two-year-and-eleven-month-old male Shikoku Inu was referred for evaluation of progressive gait abnormality that had begun three months prior. Neurological examination revealed ventral flexion of the neck, a wide-based stance in the hindlimb, wide excursions of the head from side to side, tremor in all four limbs, hypermetria in all four limbs, proprioceptive deficits in all four limbs, reduced patellar reflex in both hindlimbs, and postural vertical nystagmus. Later, behavioral and cognitive dysfunction, ataxia, and visual deficits slowly progressed. Magnetic resonance imaging revealed symmetrical progressive atrophy of the whole brain and cervical spinal cord. Bilateral retinal degeneration was observed, and both flush and flicker electroretinograms were bilaterally non-recordable at the age of five years and eight months, and the dog was euthanized. Histopathologically, faint-to-moderate deposition of light-brown pigments was frequently observed in the cytoplasm of neurons throughout the cerebrum, cerebellum, and nuclei of the brainstem. The pigments were positive for Luxol fast blue, periodic acid-Schiff, and Sudan black B, and exhibited autofluorescence. Electron microscopic examination revealed the accumulation of membranous material deposition in the neuronal cytoplasm. Small foci of pigment-containing macrophages were frequently observed around the capillary vessels. Based on these clinical and pathological findings, the animal was diagnosed with adult-onset neuronal ceroid lipofuscinosis.

Discontinuation of Cardiac Resynchronization Therapy for Heart Failure Due to Dilated Cardiomyopathy in a 61-Year-Old Female "-Super-Responder" with Return of a Reduced Left Ventricular Ejection Fraction to Normal.

BACKGROUND Although cardiac resynchronization therapy (CRT) is widely used, it has been validated only during active pacing. "Super-responders" are patients with normalized or markedly improved left ventricular (LV) systolic function with CRT who may experience a decline in cardiac function with CRT discontinuation. CASE REPORT A 61-year-old woman with a nonischemic cardiomyopathy was admitted to our hospital in September 2008 for the treatment of heart failure (HF). Cardiac assessment revealed impaired LV function with an ejection fraction of 18%, LV dilatation, and left bundle branch block (LBBB). Despite optimized medical treatment, her HF progressed, with a rapid increase in LV chamber size, mitral regurgitation, and widening of the QRS complex. In July 2011, the patient initially refused CRT, but later consented to the procedure; CRT pacemaker implantation was subsequently performed. Thereafter, the LVEF improved from 27% to 46%, LV diastolic dimension decreased rapidly from 79 mm to 56 mm, and LVEF (65%) and LV size (47 mm) normalized within 1 year later. As of August 2012, battery exchange was needed within 1 year because of high LV pacing thresholds. In October 2012, although CRT discontinuation was not recommended, we discontinued CRT to conserve battery life with the patient's consent, hoping to maintain her condition with pharmaceutical treatment. She remained stable through January 2020, with no indication of re-exacerbation. CONCLUSIONS We describe a female patient with a nonischemic cardiomyopathy and LBBB who demonstrated a super-response to CRT and maintained improvement in LV function and functional status for 8 years after discontinuing CRT.

Thoracolumbar intradural disc herniation in eight dogs: clinical, low-field magnetic resonance imaging, and computed tomographic myelography findings.

Intradural disc herniation is a rarely reported cause of neurologic deficits in dogs and few published studies have described comparative imaging characteristics. The purpose of this retrospective cross sectional study was to describe clinical and imaging findings in a group of dogs with confirmed thoracolumbar intradural disc herniation. Included dogs were referred to one of four clinics, had acute mono/paraparesis or paraplegia, had low field magnetic resonance imaging (MRI) and/or computed tomographic myelography, and were diagnosed with thoracolumbar intradural disc herniation during surgery. Eight dogs met inclusion criteria. The prevalence of thoracolumbar intradural disc herniation amongst the total population of dogs that developed a thoracolumbar intervertebral disc herniation and that were treated with a surgical procedure was 0.5%. Five dogs were examined using low-field MRI. Lesions that were suspected to be intervertebral disc herniations were observed; however, there were no specific findings indicating that the nucleus pulposus had penetrated into the subarachnoid space or into the spinal cord parenchyma. Thus, the dogs were misdiagnosed as having a conventional intervertebral disc herniation. An intradural extramedullary disc herniation (three cases) or intramedullary disc herniation (two cases) was confirmed during surgery. By using computed tomographic myelography (CTM) for the remaining three dogs, an intradural extramedullary mass surrounded by an accumulation of contrast medium was observed and confirmed during surgery. Findings from this small sample of eight dogs indicated that CTM may be more sensitive for diagnosing canine thoracolumbar intradural disc herniation than low-field MRI.

A construction of 4,4-spirocyclic γ-lactams by tandem radical cyclization with carbon monoxide.

A straightforward synthesis of 4,4-spirocyclic indol γ-lactams by tandem radical cyclization of iodoaryl allyl azides with CO was achieved. The reaction of iodoaryl allyl azides, TTMSS and AIBN under CO pressure (80 atm) in THF at 80 °C gave the desired 4,4-spirocyclic indoline, benzofuran, and oxindole γ-lactams in moderate to good yields.

Influence of osmolarity of contrast medium and saline flush on computed tomography angiography: comparison of monomeric and dimeric iodinated contrast media with different iodine concentrations at an identical iodine delivery rate.

PURPOSE: To evaluate the influence of osmolarity of iodinated contrast media and saline flush on the contrast effect in thoracic computed tomography angiography (CTA) at an identical iodine delivery rate (IDR). MATERIALS AND METHODS: Seven beagles were used in a cross-over experiment. The contrast media used were iohexol 350 mgI/ml (IOH350; osmolarity 844 mmol/kg) and iodixanol 320 mgI/ml (IDX320; osmolarity 290 mmol/kg). Each contrast medium was administered to groups with and without saline flush at 40.0 mgI/kg/s for all experiments. Dynamic CT scanning was performed at the ninth thoracic vertebra level. The peak value, area under the curve (AUC), and time to peak (TTP) were calculated from the time attenuation curves of the pulmonary artery and aorta. RESULTS: There was no significant difference between IOH350 and IDX320 with or without saline flush in the peak values for the pulmonary artery and aorta. AUC was significantly higher in groups with saline flush for both contrast media and arteries (p<0.05) with no significant difference between contrast media. TTP was significantly longer in groups with saline flush than without saline flush for both contrast media and arteries (p<0.05), with no significant difference between contrast media. CONCLUSIONS: There were no significant differences in the contrast effects of monomeric IOH350 and dimeric IDX320 in thoracic CTA when used at an identical IDR. Moreover, saline flush prolonged the peak duration at 600 mgI/kg.