Circadian variation of basal total vascular tone and chronotherapy in patients with vasospastic angina pectoris.

Vasospastic angina pectoris (VSA) is an anginal attack which occurs characteristically between night and early morning. The aim of this study was to clarify the cause of VSA. The subjects consisted of 16 patients with VSA, 18 patients with effort angina (EAP) and 15 healthy individuals, who were used as the control group. Subjects were attached to an ambulatory blood pressure monitor and a non-invasive continuous cardiac output monitor concurrently, over a 24-hour period. Mean blood pressure (MBP), and cardiac index (CI) were measured. Then basal total vascular tone (TVT) was calculated as follows: basal TVT = (MBP/CI) x 1,332 dyne/sec/cm5. The decrement of CO was greater during sleeping hours as compared with the decrement of the MBP in the VSA group. Nocturnal basal TVT was significantly greater in the VSA group than in the EAP group or the control group. The increased nocturnal basal TVT was significantly suppressed by long acting calcium antagonists to the level of the EAP and the control groups. The treatment also decreased the frequency of ischemic attacks.

[An autopsy case of atypical Friedreich's ataxia with chronic idiopathic intestinal pseudo-obstruction].

We report a 58-year-old man with slowly progressive muscle atrophy and weakness in the four extremities, accompanying cerebellar ataxia and sensory impairment of all modalities. He was a product of consanguineous marriage. His neurological manifestations began in childhood. He was admitted to our hospital because of marked abdominal distension and pretibial edema with hypoalbuminemia and hyperlipidemia. Neuroimaging studies showed marked atrophy of the cerebellum and spinal cord. Nerve conduction studies presented with slowing and sural nerve biopsy revealed demyelination with onion-bulbs. Abdominal distension was interpreted to be caused by chronic idiopathic intestinal pseudo-obstruction (CIIP), leading to protein-losing gastroenteropathy and hypalbuminemia caused by the CIIP. He died of DIC by myelodysplasic syndrome and DIC, two years later. Postmortem study demonstrated with severe loss of anterior horn cells and gliosis in the spinal cord. The Clarke's column was also affected. There was symmetrical degeneration in the dorsal column and corticospinal tracts. The cerebellum showed atrophy of molecular layer, prominent loss of Purkinje's cells and sparse granular cell layer, but no obvious change in the dentate nucleus. Neuronal loss in the dorsal root ganglia was remarkable. There were no alternations in the cerebral cortex, striatum, thalamus, subthalamic nucleus, and pontine nucleus, except for mild changes in substantia nigra and inferior olivary nucleus. This case was clinically suspected either of variant of Friedreich's ataxia or an early onset ataxia associated with hypoalbuminemia (EOAHA), although marked autonomic dysfunction was atypical. But the postmortem study, demonstrated with marked neuronal loss in anterior horn cells and cerebellan cortex and rather suggested an independent category of this case.

Regional variations in the progression of bone loss in two different mouse osteopenia models.

Differences in trabecular and cortical bone loss have been demonstrated clinically, but differences in bone loss at different skeletal sites remain unclear. We examined regional variations in bone loss histomorphometrically in two strains of mice in which osteopenia progresses spontaneously: tiptoe-walking Yoshimura (twy) mice (from 4 to 37 weeks of age) and senescent ICR mice (from 4 to 88 weeks of age). Morphometrical measurements were obtained to investigate the changes with age in trabecular bone area and anterior cortical bone width in the lumbar vertebral body, trabecular bone area in the tibia, bone area in the parietal bone, and the cortical index in the humerus. Results showed that, in twy mice, trabecular turnover was higher than in ICR mice, and bone loss progressed in the following order: tibial trabecular bone, lumbar trabecular bone, parietal bone, lumbar anterior cortical bone, and the humerus. In ICR mice, bone formation declined after 60 weeks. Bone loss progressed in tibial trabecular bone and the parietal bone at 60 weeks of age, followed by lumbar trabecular bone, lumbar anterior cortical bone, and the humerus at 88 weeks of age. Bone loss varied at each site and between the two mouse strains, with different bone turnover rates. The findings of the present study indicate that special attention should be paid to regional variations in the progression of bone loss associated with differences in pathologic features.

Cervical myelopathy caused by pseudoarthrosis between the atlas and axis associated with diffuse idiopathic skeletal hyperostosis.

STUDY DESIGN: This is a report of a patient with severe cervical myelopathy due to pseudoarthrosis between the posterior tubercle of the atlas and the spinous process of the axis, associated with diffuse idiopathic skeletal hyperostosis. Radiographs of 170 patients with neck pain were reviewed to identify lesions involving abnormal contact between the atlas and axis. OBJECTIVE: Based on an analysis of 170 radiographs, the prevalence of the reported condition was estimated. SUMMARY OF BACKGROUND DATA: A number of histologic studies on pseudoarthrosis involving the lumbar spine have been reported. In contrast, lesions between the posterior tubercle of the atlas and the spinous process of the axis have not been reported in association with cervical myelopathy. METHODS: Clinical and pathologic features of a patient with pseudoarthrosis between the posterior tubercle of the atlas and the spinous process of the axis were investigated. Radiographs of 170 Japanese patients over 40 years old were examined, and abnormal contact between the atlas and axis was classified into two groups based on the degree of spinal hyperostosis. RESULTS: Of 170 patients, 53 showed abnormal contact between the atlas and axis. The prevalence of abnormalities in the pronounced hyperostosis group (Forestier's stages II and III) was much higher than in the group with normal or slight hyperostosis. Two men had radiographic patterns showing osteophytes projecting into the spinal canal and associated with marked cervical myelopathy. CONCLUSION: In the group with pronounced hyperostosis, pseudoarthrosis or a variant can cause serious problems in the upper cervical spine that should not be overlooked.

Alteration of elastin in aorta from diabetics.

The differences in desmosine, isodesmosine (DID), hydroxyproline and cholesterol in the human thoracic aorta from diabetic (n = 16) and non-diabetic (n = 17) autopsy subjects were investigated. DID was analyzed by the use of high performance liquid chromatography. The amount of DID, and total DID (DID+reduced DID) tended to be lower in the diabetic than in non-diabetic subjects. The ratio of DID or total DID to hydroxyproline was significantly decreased in diabetic compared to non-diabetic subjects. Amount of DID, reduced DID and total DID were significantly lower in aorta with plaque formation than that without plaque or ulcer. Multiple regression analysis showed that amount of cholesterol, DID, reduced DID and age were significantly associated with dry weight per area of the aorta. A similar association was not observed in non-diabetic subjects. Compositional changes of aortic cholesterol and elastin have a closer relationship with atherosclerosis in diabetic than in non-diabetic subjects.

Long-term follow-up study of anterior surgery for cervical spondylotic myelopathy with special reference to the magnetic resonance imaging findings in 52 cases.

A series of 151 patients with cervical spondylotic myelopathy (CSM) were surgically treated through an anterior approach in the period from 1960 to 1987. Particular attention was given to the results of the 52 patients who were operated on more than 12 years ago. Satisfactory short-term results were obtained in all but the few who had incomplete decompression. In these patients, function deteriorated within five years. A disturbing incidence of progression of myelopathy was noted ten years after surgery. Magnetic resonance imaging identified many of the causes (e.g., newly developed intervertebral disk herniation and progression of spondylosis associated with spinal malalignment in both cephalad and caudal directions). Other adverse changes were hypertrophy of the yellow ligament and ossification of the posterior longitudinal ligament. The new and improved techniques consist of decompression to a width of 16 mm or more with intraoperative ultrasonography in addition to extirpation of the posterior longitudinal ligament. This new measure may reduce the incidence of late neurologic deterioration.

[Experimental study on the spinal lesions in hyperostotic mouse (twy/twy): special reference to the pathogenesis of the ossification of the spinal ligaments and to the action of ethane-1-hydroxy-1, 1-diphosphonate (EHDP)].

The hyperostotic changes in the spine of the twy mouse and the action of ethane-1-hydroxy-1, 1-diphosphonate (EHDP) on those hyperostotic changes were studied histomorphometrically. The periosteal bone formation and the endosteal bone resorption in the anterior cortex of the vertebral bodies of the twy mouse continued even after maturation, resulting in the anterior cortex shifting to the anterior direction. In parallel with these changes, calcification in the enthesis (to which spinal ligaments and the outer layer of disc fibrosis are attached) continued, ending in ankylosis of the whole spine. It was similar to a membranous ossification. Moreover calcification in these regions was most sensitively inhibited by EHDP and reappeared as soon as the discontinuation of EHDP. These findings showed that the pathogenesis of the twy mouse was the hyperostosis which continued even after maturation in the genetical region of a membranous ossification, especially in the enthesis. Further they appeared to be similar to the initial changes in ankylosing spinal hyperostosis or the ossification of the spinal ligaments. In addition, our results provided evidence for the usefulness of EHDP in the twy mouse.

[A report of an atypical case of Degos' disease with multiple perforations of the stomach and small intestine].

A 47-year old male complaining of severe abdominal pain associated with distention was admitted to our department on August 5, 1986. His first admission to our hospital was 18 days previously for leg pain and fever. He underwent emergency operation with a preoperative diagnosis of acute peritonitis due to perforation of gastric ulcer. Operative findings showed one perforation of the stomach and two of the ileum. Distal gastrectomy, enterectomy and peritoneal drainage were carried out. Resected specimen revealed six ulcers, two of them in the stomach, four in the ileum. Microscopic examination disclosed intimal proliferations of small arteries in the mucosal layer. The vessels near the ulcers were most severely involved but the same changes were also found in the subserous layer and mesentery. It was suggested that the multiple ulcers were secondary to vascular lesions identical to the gastrointestinal lesion of Degos' disease. Postoperative examinations revealed one ulcer in the jejunum and another in the descending colon. Ten months after operation he lives with no complaint on the gastrointestinal tract. Only 80 cases of Degos' disease have been reported in the western countries and 10 cases in Japan. In those atypical cases of Degos' disease without papulosis were only Manuel's and ours.