Evaluation of myocardial disorders in patients with dilated cardiomyopathy and left ventricular eccentric hypertrophy--by 201Tl myocardial SPECT]

201Tl myocardial SPECT was performed in cases of dilated cardiomyopathy and valvular heart disease with left ventricular eccentric hypertrophy, and the two groups were compared from the standpoint of the mechanism of onset of myocardial disorders. Significant coefficients of correlation were seen between the Tl score and LVDd (r = 0.792, r = 0.785) and Tl score and LVEF (r = -0.634, r = -0.555) in both dilated cardiomyopathy and valvular heart disease. In cases of valvular heart disease, significant correlation coefficients (r = -0.756, r = -0.720) between LVDd and r-WR (relative-washout rate), and Tl score and r-WR were observed, but no such correlation was seen in dilated cardiomyopathy. In valvular heart disease, a decrease in myocardial perfusion associated with enlargement of the left ventricle appeared, while in dilated cardiomyopathy, there was a marked decrease in LVEF in proportion to the thallium defect. Therefore, it was assumed that left ventricular wall disorders occur due to myocardial metabolic disorders and coronary microcirculation disorders.     

Significance of positive Queckenstedt test in patients with syringomyelia associated with Arnold-Chiari malformations.

Ten patients with syringomyelia associated with Arnold-Chiari Type I malformations were evaluated. In each patient, a manometric Queckenstedt test was performed with the neck in various positions. No patient showed evidence of a block to the flow of cerebrospinal fluid (CSF) with the neck in the extended position; however, all showed a complete CSF block with the neck in a flexed position. Posterior fossa decompression with a C1-2 laminectomy was performed in nine cases, after which Queckenstedt test demonstrated free CSF communication in all nine with the neck in extension, in a neutral position, and in flexion. Postoperative magnetic resonance imaging showed shrinkage of the syrinx in the patients who underwent surgery. It is suggested that obstruction of the CSF pathway at the foramen magnum produced by neck movement is of importance in the formation and progression of a syrinx.     

Central nervous system lesion in myotonic dystrophy--demonstrated by X-ray computed tomography and magnetic resonance imaging]

We made an attempt to detect organic brain lesions in eight patients with myotonic dystrophy (MyD) using X-ray computed tomography (CT) and magnetic resonance imaging (MRI). The patients comprised seven men and one woman with ages ranging from 41 to 58 years (average 47 +/- 5.3 years). Seven patients had intellectual impairment assessed using an intelligence quotient test (WAIS-R < 70). CT scans were obtained in the axial plane with a slice thickness of 10 mm, and MRI scans were performed on a 1.5 tesla system in the axial and coronal sections with a slice thickness of 8 mm. CT scans displayed both marked sulcal and prominent ventricular enlargement in 6 of the 8 patients, indicating the presence of brain atrophy, and 3 cases had a few paraventricular low density area in the cerebral hemispheres. In contrast to CT, MRI study revealed more widespread brain parenchymatous lesions in all the 8 cases, showing the multiple foci of high signal intensity with varying size in the white matter on T2-weighted images and proton density-weighted images. No visible changes were detected on CT or MRI in the brain stem and cerebellum. The present study demonstrated that there exist organic cerebral lesions, predominantly located in the white matter in patients with MyD, regardless of focal neurological symptoms. The parenchymatous lesions in the brain can be detected with MRI, and lesser extent with CT, and are considered to be intimately correlated with intellectual deterioration commonly seen in patients with MyD.     

Sialolipoma of the hard palate

Sialolipoma is a new variant of salivary gland lipoma, which was first proposed by Nagao et al. (Histopathology 2001; 38: 30) in 2001. We report this rare case of sialolipoma in the hard palate. A 60-year-old Japanese woman was referred to our department complaining of a painless swelling on the right side of the hard palate. Intra-oral examination revealed a soft, elastic, dome-shaped mass with 1 cm in diameter located in the posterior part of the hard palate. Magnetic resonance imaging examination revealed high intensity on T(1)-weighted image and isointensity on T(2)-weighted image. Incisional biopsy revealed that the tumor was encapsulated by fibrous tissue, consisted of adipose tissue, and also contained normal salivary gland tissue peripherally. First diagnosed as an ordinary lipoma of the hard palate, the tumor was excised. According to the recent criteria of histologic findings of sialolipoma, we rediagnosed the tumor as sialolipoma of the hard palate.     

Two-level disc herniation in the cervical and thoracic spine presenting with spastic paresis in the lower extremities without clinical symptoms or signs in the upper extremities.

BACKGROUND CONTEXT: There is no report in the literature of two-level disc herniation in the cervical and thoracic spine presenting with spastic paresis/paralysis exclusively in the bilateral lower extremities. PURPOSE: To identify the clinical characteristics of specific myelopathy resulting from C6-C7 disc herniation through a case with spastic paresis in the lower extremities without upper extremities symptoms due to separate disc herniation in the cervical and thoracic spine, which was surgically removed in two stages. STUDY DESIGN/SETTING: A case report. METHODS: A 48-year-old man developed a gait disturbance as well as weakness and numbness in the lower extremities. Thoracic magnetic resonance imaging (MRI) showed a T11-T12 disc herniation, which was removed under the surgical microscope through a minimally invasive posterior approach. He improved, but 2 months after surgery developed recurrent numbness and spasticity. On this occasion, no evidence of recurrence of the thoracic disc herniation could be identified, but cervical MRI demonstrated a compressed spinal cord at the C6-C7 level. The patient had no neurological findings in the upper extremities. The herniated disc at C6-C7 was removed under the surgical microscope with laminoplasty. RESULTS: The symptoms gradually improved after surgery. At the present time, 2 years and 9 months after the initial operation, the patient had a stable gait and was able to work. CONCLUSIONS: Our experience suggests that in the diagnosis of patients with spastic paresis and sensory disturbances in the lower extremities, spinal cord compression should be explored by imaging studies not only in the thoracic spine but also in the cervical spine, especially at the C6-C7 level, even if the symptoms and abnormal neurological findings are absent in the upper extremities.     

Newborn infant with maternal anti-SSA antibody-induced complete heart block accompanying cardiomyopathy.

Newborn case of maternal anti-SSA antibody-induced congenital complete heart block (CCHB) accompanying cardiomyopathy is presented. Unexpectedly, she died of ventricular tachycardia, not bradycardia, 6 days after birth. Autopsy revealed left ventricular cardiomyopathy with endocardial fibroelastosis. Thus, when evaluating fetal cardiac performance in cases of maternal anti-SSA antibody-induced CCHB, it is necessary to pay attention to myocardial attributes such as endocardial hyperplasia.     

The effects of intranasal application of low dose buserelin in women with hypothalamic amenorrhea]

Low doses of the Gn-RH agonist (buserelin, 30 micrograms) were given intranasally to 14 women with clomiphene ineffective hypothalamic amenorrhea three times daily for three weeks in order to study pituitary responses and to induce follicular maturation and ovulation. Clomiphene ineffective hypothalamic amenorrhea patients were classified into two groups by LH-RH stimulation test before the treatment. Group 1 was defined as having basal serum LH and FSH levels lower than 1.5 mIU/ml, LH and FSH peaks lower than 3mIU/ml by LH-RH stimulation test. Group 2 consisted of cases other than those in Group 1. While a significant increase in basal LH and FSH (p less than 0.01, p less than 0.001) and improvement in pituitary response to LH-RH stimulation test were observed in group 1, the basal levels of LH and FSH did not increase significantly and pituitary response to a LH-RH stimulation test was decreased in group 2. It is suggested that pituitary priming occurred in group 1 and pituitary desensitization occurred in group 2. None of 14 patients showed signs of follicular maturation during or after the treatment. The results demonstrated that the biphasic pituitary response to intranasal buserelin spray and the limit of its therapeutic use for the treatment of hypothalamic amenorrhea.     

Epicardial radiofrequency ablation for chronic atrial fibrillation undergoing simultaneous on-pump beating coronary artery bypass grafting

Off-pump coronary artery bypass grafting has become an attractive surgical alternative for myocar-dial revascularization because of the advantage of myocardial protection and other benefits of patients. However, it is still regarded as a controversial treatment for the coronary artery disease accompanied by atrial fibrillation (AF). A significant number of patients in need of coronary revascularization have chronic AF. Although the Cox-Maze III procedure is the gold standard for the surgical treatment of AF, few of these patients undergo AF operations at the time of their coronary bypass grafting. We report herein a case of the pulmonary vein isolation to eliminate the AF by means of epicardial radiofrequency ablation combined with 2 vessels coronary artery bypass grafting on the beating heart with the aid of cardiopulmonary bypass.     

Clinical and immunological studies on patients with Graves' disease preoperatively treated with corticosteroids and iodides

The purpose of this study was to evaluate the acute effects of corticosteroid and iodide preoperative therapy in patients with Graves' disease in terms of thyroid function and immunological parameters. The above combination was prescribed for 4 patients who had experienced severe side effects from antithyroid drugs (ATD) in order to reduce the possibility of post-thyroidectomy thyroid storm. Corticosteroids were employed daily for four days, and iodides were given daily for two weeks prior to thyroidectomy. The free T₃ values decreased rapidly to euthyroid levels following the administration of both drugs, although the free T₄ values were still much higher than normal in 3 of the 4 patients at the time of surgery. By comparison, 3 of 8 patients treated with ATD also had thyroid hormone levels above normal. Studies of lymphocyte subsets revealed that the percentage of helper T cells was significantly less in the corticosteroidiodide treatment group than in the control and ATD groups. It is thus possible that postoperative thyroid storm might be prevented through corticosteroid-iodide therapy by virtue of the reduction of free T₃ values to within the normal range by the time of surgery. The acute suppression of helper T cells was another result of this form of therapy observed.