An epistatic effect of the female specific loci on the development of autoimmune vasculitis and antinuclear autoantibody in murine lupus.

OBJECTIVE: To identify the genetic loci regulating the incidence and severity of renal autoimmune vasculitis developed in murine lupus. METHODS: Vasculitis of renal arteries was histopathologically evaluated in MRL/Mp-Fas(lpr) (MRL/lpr), C57BL/6-Fas(lpr) (B6/lpr), (MRL/lpr x B6/lpr) F1, and MRL/lpr x (MRL/lpr x B6/lpr) F1 backcross mice. Using genomic DNA samples of the backcross mice, genome-wide scans, association studies, and linkage analyses were carried out based on genotypes of polymorphic microsatellite markers. Correlations of vasculitis grade and levels of various autoantibodies were also evaluated. RESULTS: Two recessive susceptibility loci of the MRL allele were identified on chromosomes 4 and 1, which had previously been defined as the autoimmune related loci termed Arvm1 and Sle-1/Nba2, respectively. The former was epistatic to the latter in a female specific manner. The titre of antinuclear autoantibody (ANA) in IgG class, but not ANA in IgM class or anti-dsDNA in either IgG or IgM class, correlated significantly with vasculitis grade. CONCLUSIONS: The present loci have been reported in previous studies using a different set of murine strains, suggesting that they are of importance in the development of autoimmune vasculitis in murine models. The concomitance of autoimmune vasculitis and IgG ANA suggests a shared genetic factor regulating these traits.

Effects of trimebutine maleate on gastric motility in patients with gastric ulcer.

The effects of trimebutine maleate (TM), a prokinetic drug, on gastrointestinal motility in patients with gastric ulcer were investigated. Twenty patients with active gastric ulcers were allocated to two groups; 10 patients received a proton pump inhibitor alone (PPI group), given orally, and 10 patients received oral TM in combination with a PPI (PPI + TM group), each for a period of 8 weeks. Electrogastrography (EGG) and gastric emptying were measured before and after the treatment period. During the active ulcer stage, tachygastria (more than 0.06 Hz) or bradygastria (less than 0.04 Hz) in the EGG frequency were observed in 9 patients either before or after meals. During the healed ulcer stage, tachygastria or bradygastria was observed in 4 of 10 patients in the PPI group, while in the PPI + TM group, 1 patient had tachygastria and none had bradygastria. Postprandial dip (PD) was observed in 3 of the 20 patients during the active stage, while after treatment, PD was observed in 3 patients in the PPI group and in 6 patients in the PPI + TM group, respectively. Gastric emptying in the PPI group did not show any change between before and after treatment, while that in the PPI + TM group improved significantly after treatment. These results suggest that TM may have an ameliorative effect on abnormal gastric motility in patients with gastric ulcer.

Painful ophthalmoplegia caused by idiopathic hypertrophic cranial pachymeningitis in the cavernous sinus.

A 73-year-old man presenting with painful unilateral ophthalmoplegia is reported. Magnetic resonance imaging demonstrated a mass in the cavernous sinus extending to the floor of the middle cranial fossa. After resection of the lesion and anti-tuberculous chemotherapy his symptoms improved. Pathological examination revealed extensive caseous necrosis.

Computerized tomography in the assessment of brain shifts in acute subdural hematoma.

Computed tomography (CT) was used to characterize brain shifts in 30 adult patients (mean age 51.9 years) with fatal supratentorial acute subdural hematomes (ASH) who were admitted within 3 hours of the head trauma. The Glasgow Coma Scale (GCS) score, hematoma width, and midline shift were tabulated. The width of the cistern surrounding the brainstem (CSBS) and the distance from the floor of the fourth ventricle to the posterior clinoid process (IVv-PC) were measured. Opening (+) or disappearance (-) of the suprasellar cistern (SC) was determined using axial CT. The data were compared with those in agematched controls (n = 26) using the Wilcoxon and Kruskal-Wallis tests. The hematomas in the SC(-) group (n = 22, mean GCS score: 3.8 +/- 1.0) were wider (p = 0.02) than those in the SC(+) group (n = 8, mean GCS score: 4.7 +/- 1.5). The CSBS in the SC(+) group was significantly smaller than that in the controls (p < 0.01). In the SC(-) group the CSBS was smaller and the IVv-PC was significantly larger than those in the controls (IVv-PC; p < 0.01) and the SC(+) group (IVv-PC; p < 0.05). In the evolution of brainstem shifts in ASH, when the SC is open the predominant vector is in the direction of the skull base, while when the SC is not observed, an axial vector shifts the brainstem.

Measurement of local directional pressures in the brain with mass.

In order to understand the effects and the direction of pressure transmitted from a mass lesion through various brain structures, miniature strain gauges were inserted in different brain locations in eight anesthetized monkeys. Mass lesions were created by inflating a balloon in either of two locations--subcortical in four animals (Group I) and deep (lateral to the caudate nucleus) in the other four animals (Group II). Anterior-posterior directed pressures were thus measured from a gauge placed in the parietal lobe, and lateromedially directed pressures (LM-dPs) were measured from gauges in the temporal lobe and midbrain. Intracranial pressure, systemic mean arterial pressure, and cerebral blood flow were also monitored. After balloon inflation was begun, temporal changes in pressure were recorded from gauges as percentage increase or decrease from baseline measurements. In both groups, balloon inflation caused a gradual increase in the parietal lobe anterior-posterior directed pressure with a concomitant increase in intracranial pressure and a decrease in cerebral blood flow. The temporal lobe gauge in Group I recorded an initial negative followed by a positive LM-dP with further balloon inflation. In Group II, this gauge recorded a positive LM-dP throughout. The midbrain gauges in both groups recorded an initial positive followed by a negative LM-dP. This reversal in the direction of pressure in the midbrain occurred just before the supratentorial pressure reached a peak and was noted to be concurrent with a sudden rise in mean arterial pressure and a decline in cerebral blood flow.(ABSTRACT TRUNCATED AT 250 WORDS)

Granulomatous angiitis of the central nervous system--a case with recurrent intracerebral hemorrhage.

Granulomatous angiitis of the central nervous system occurred in a 43-year-old male presenting with recurrent intracerebral hemorrhage confirmed by computed tomography. A surgical specimen obtained at hematoma removal revealed granulomatous angiitis. Systemic investigation found no underlying cause for the hemorrhage. Although the incidence is very low, this condition should be considered in cases of recurrent intracerebral hemorrhage with unknown cause. When surgery is indicated, biopsy of the brain and leptomeninges should be obtained, because involvement of the leptomeninges has occurred in virtually all autopsy cases.

Screening for unruptured asymptomatic intracranial aneurysms in patients undergoing coronary angiography.

In screening for unruptured asymptomatic intracranial aneurysms, an intracranial intra-arterial digital subtraction angiography (IA-DSA), posteroanterior view, was obtained in 72 consecutive patients who underwent coronary angiography for workup of angina pectoris. In cases where an aneurysm was suspected from IA-DSA, conventional intracranial angiography was obtained. Five asymptomatic unruptured aneurysms were detected in four of these cases. The aneurysms ranged from 4 to 16 mm in diameter. There were no complications associated with the coronary angiography nor with the additional intracranial IA-DSA, which required less than 5 minutes to perform in all cases. The value of performing intracranial IA-DSA in patients undergoing coronary angiography to screen for unruptured asymptomatic aneurysm is discussed, as is the possible racial differences in the frequency of aneurysms.

Periodic oscillation of intracranial pressure in ventricular dilation: a preliminary report.

Artificial pressure waves (PWs) were generated by manual inflation of a balloon in the trigonum of the lateral ventricle in seven adult mongrel dogs with normal cerebrospinal fluid (CSF) circulation. In 14 of 16 series of continuous appearances of artificial PWs, local shifts of the brain were successfully monitored using small strain-gauge sensors at the periventricular structures in these animals. Of the 14 series, 13 showed displacements of the periventricular structures, suggesting ventricular dilation. These results did not always correlate with macroscopic findings. They are thought to be due largely to periventricular oedemas and, in part, non-uniform dilations of the ventricles during PWs. We conclude that a water hammer formed by reflection of an increased pulse pressure of PWs at the site of CSF absorption causes a shift of CSF from the ventricle to the periventricular structures through the wall of the ventricle. This phenomenon appears amplified in patients with impaired CSF absorption. Thus, PWs have a pathological role in the progress of ventricular dilation in patients with normal pressure hydrocephalus.

[A case report of neoplastic aneurysm due to metastatic choriocarcinoma].

A twenty-year-old female, who had multi-agent chemotherapy for eighteen months after surgical resection of a choriocarcinoma originating from the left adnexa, was hospitalized because of a cerebrovascular accident. Her complaints were unconsciousness, generalized convulsion and left hemiparesis. Although both plain and contrast enhanced CT scans demonstrated only acute subdural hematoma in the right frontoparietal region associated with midline shift, no tumor shadow was recognized over the cortex or intraparenchymally. A carotid angiogram revealed a large and irregularly shaped aneurysm at a peripheral branching of the angular artery. Evacuation of the hematoma, resection of the aneurysm and revascularization for the defect of the parent artery by transplantation of a part of STA by means of microsurgical anastomosis were done at an emergency operation. Nevertheless the graft did not prove to be patent on the post-operative angiogram. Due to retrograde filling of the distal portion of the angular artery through collateral channels, the patient has been completely free of neurological deficit and her performance of daily activities has been excellent. No appearance of other intracranial metastasis of the tumor has been detected for six months thereafter. Histological examination of the resected aneurysm demonstrated that choriocarcinoma composed of cytotrophoblasts and syncitiotrophoblasts filled the lumen of the aneurysm. The van Gieson's staining clarified the invasion of the tumor into the vascular wall causing disruption of lamina elastica beneath the fairly intact adventitia. Microscopic findings of the specimens taken from adjacent brain tissue and overlying dura mater confirmed absence of metastatic lesion in them.(ABSTRACT TRUNCATED AT 250 WORDS)

[Evaluations of regional cerebral blood flow (rCBF) by 133Xenon i.v. method in patients with occlusions of bilateral internal carotid arteries].

The studies of changes in regional blood flow (rCBF) have been reported on different clinical courses in the five cases (mean 57.4 y.o.) showing bilateral occlusion or stenosis of internal carotid arteries. Values of rCBF using Fg (fast component in gray matter) were closely correlated with their clinical courses. The three patients (2 males and 1 female) showed bilateral focal decreased patterns of rCBF and their uneventful clinical courses except for mild attacks of transient cerebral ischemia. However, the rest two male patients showed bilateral decreased pattern of rCBF and moderate hemiparesis including attacks of loss of consciousness. The one died suddenly because of the thalamic hemorrhage and the other died also suddenly from the unknown etiology. Such differences between the classification of clinical course and hemodynamics evaluated by rCBF was discriminated more clearly by Fg (fast component in gray matter) than by F mean (mean rCBF). The authors concluded that the evaluation by Fg may be valuable to estimate prognosis of patients with bilateral occlusion of internal carotid artery. There are two patterns of decreased rCBF in our study. The patients with bilateral diffuse decreased patterns of rCBF should be followed up more carefully because of the sudden death from cerebral hemorrhage.