A distinct difference in clinical expression of two siblings with Aicardi-Goutières syndrome.

Two sibs with an encephalopathy, including intracerebral calcification and a white matter disease, are reported. In the younger sister, the cerebrospinal fluid showed chronic pleocytosis and clinically she strictly fits to the diagnosis of Aicardi-Goutières syndrome. Both sisters were affected by a spastic tetraplegia, truncal hypotonia and dystonic posturing, but the clinical course and the neuroradiological findings were milder in the older sister and she showed no cerebrospinal fluid pleocytosis. The present cases and recent reports of intrafamilial variability of Aicardi-Goutières syndrome may raise interesting aspects as to the limits and criteria of this syndrome.

Potential influence of observer variation in thromboprophylactic trials.

In order to investigate the influence of the interpretation of a postoperative venography, used as the scientific tool in a thromboprophylactic trial, twelve different evaluations of the same venography have been compared. Two hundred and nineteen patients, undergoing total hip replacement, fulfilled a randomized trial evaluating two different prophylactic regimens. Bilateral venography was performed 7-11 days after surgery, and the result of venography was defined as with or without deep venous thrombosis (DVT), irrespective of the location of a thrombus. The venograms were evaluated five times by different interpreters, and the criteria for the presence of DVT were recorded whether or not thrombi in minor veins were present. The incidences of DVT in the two prophylactic groups varied from 2.7 to 20.3% and from 13.5 to 34.2%, respectively, according to the diagnostic criteria. However, the risk reduction between the two groups was almost constant, no matter which type of evaluation was applied (7.9-15.1%), and was statistically significant at the 5% level in all but 4 of the 12 evaluations. It is concluded that despite uniformity in patient selection, the incidence of DVT is not uniform, even though it is stated that venography is used as the end point. The obvious interobserver variation did not change the conclusion when two regimens are compared in a specific randomized study.

Phlebography as the gold standard in thromboprophylactic studies? A multicenter interobserver variation study.

In 241 patients with total hip arthroplasty and entering a study on thrombosis prophylaxis, phlebography was adequately performed in 451 legs 7 to 11 days after surgery. The phlebograms were primary evaluated by 4 independent observers, and finally a consensus of the images in which disagreement primarily occurred was obtained. The diagnosis of thrombosis in the 4 primary observations varied between 65% and 83% (mean 70%) and the agreement on a negative diagnosis between 97% and 99% (mean 98%). Taking into account agreement by chance, kappa-values varied from 0.60 to 0.83 when the 6 different pairs of observations were compared. When comparing the primary evaluations with the final consensus, agreements on positive diagnosis varied between 70% and 90% (mean 80%) and on negative diagnosis between 97% and 99% (mean 98%). Kappa-values varied from 0.68 to 0.90. The factor of uncertainty in evaluation of phlebography may have to be considered when studies on postsurgical thromboprophylaxis are planned.

Cerebrospinal fluid outflow resistance measurements in the selection of patients for shunt surgery in the normal pressure hydrocephalus syndrome. A controlled trial.

In an attempt to determine to what extent the measurement of cerebrospinal fluid (CSF) outflow resistance (R(out)) can distinguish patients with normal-pressure hydrocephalus (NPH) who respond to shunt surgery from patients who fail to do so, the authors undertook a controlled trial. Eighteen patients with the diagnosis of NPH entered the study. In connection with the shunt operation all patients who entered the study underwent R(out) measurement by means of the pressure-volume index (PVI) method of bolus manipulation (Marmarou et al.). As a safety measure patients above 70 of age and patients with dementia as the only clinical symptom underwent continuous intraventricular pressure measurement before they entered the final study. As a result a total of fourteen patients underwent PVI test and shunt surgery during the same procedure. Patients with normal R(out) thus served as controls, since all patients underwent shunt surgery irrespective of the result of the R(out) measurement. Eight patients improved following surgery and the outcome did not correlate with result of PVI nor R(out) determinations.

Prevention of thromboembolism in hip-fracture patients. Comparison of low-dose heparin and low-molecular-weight heparin combined with dihydroergotamine.

In a prospective, double-blind, controlled study, we compared the antithrombotic efficacy of low-dose heparin with dihydroergotamine (A), low-molecular-weight heparin with dihydroergotamine (B), and placebo (C). Two hundred and thirteen patients surgically treated for fractures of the hip were randomly divided into three groups. One hundred and sixty-one patients were analyzed. All thrombi were verified by ascending phlebography. Nine patients died within 1 month after operation. A and B proved to be equally safe but failed to provide any protection against deep-vein thrombosis, although B showed a tendency to reduce the incidence. Mortality within 1 month of operation was unaffected by the type of prophylaxis.

Heparin/dihydroergotamine for venous thrombosis prophylaxis: comparison of low-dose heparin and low molecular weight heparin in hip surgery.

In a prospective, double-blind controlled study we have compared the prophylactic efficacy against deep vein thrombosis of low-dose heparin + dihydroergotamine (A), low molecular weight heparin + dihydroergotamine (B) and placebo (C). A total of three hundred and fifty-six patients undergoing total hip replacement were randomized into three groups and 316 patients were analysed. All thrombi were verified by ascending phlebography. One-third of the patients developed deep vein thrombosis in group A and B, differing significantly from group C. The operative blood loss in group B was higher than that in groups A and C. However, the number of patients transfused and their transfusion requirements did not differ. Severe bleeding occurred in one patient in each group. No deaths were registered during the study. Our study indicates that prophylactic treatment against postoperative deep vein thrombosis with low molecular weight heparin + dihydroergotamine once daily is as effective and safe as conventional low-dose heparin + dihydroergotamine twice daily in patients undergoing total hip replacement. The once-daily regimen has the advantage of better patient acceptance and less nursing time.

Computed tomography and hypocycloid tomography in lesions of the nose, paranasal sinuses and nasopharynx.

In 31 patients with lesions mainly arising from the nose, paranasal sinuses and nasopharynx comparative evaluation of the results of CT and hypocycloid tomography indicated that CT generally gave a better representation of both the bony structures and the soft parts and alone has the capacity to evaluate intracranial extension. CT should first be performed in the transverse axial plane. If abnormalities in relation to horizontal bony structures are demonstrated, the examination should be supplemented by CT in the coronal plane or, if CT scanning in this plane is not possible, by conventional tomography. Enhancement after intravenous injection of contrast medium does not as a rule seem to add information of diagnostic importance.

Laryngopyocele with a report of three cases.

Laryngoceles are classified into three types: internal, external or combined. If complicated with infection, laryngopyocele develops, often with dramatic symptoms. Three cases of laryngopyocele are reported. Aetiology, symptomatology and treatment are discussed. It is recommended that all laryngoceles producing symptoms be removed through an external incision and, if necessary through a laryngofissure.

The effect of nonfractionated and fractionated administration of iopanoic acid on gallbladder visualization.

Visualization of the gallbladder after nonfractionated and fractionated administration of iopanoic acid was investigated in a blind experiment. The authors studied 168 patients; 73 were given 3 g iopanoic acid over a period of 6 hours (0.5 g/hour), and 95 received the same amount of contrast medium in a single dose. No differences in visualization were found for the two modes in both normal and pathological cases.

Foreign body embolism of retinal arteries as a complication of carotid angiography.

Two cases of foreign body embolism of the retinal arteries after percutaneous carotid angiography were encountered within a period of 7 months. Both patients were non-arteriosclerotic. Loss of visual function in direct relation to carotid angiography and the observation of a dark foreign body in a retinal arteriole indicated exogenous embolism. No or only slight remission occurred. Careful ophthalmoscopy in cases of visual complaints after carotid angiography is advised, and if foreign body embolism is suspected, treatment with vasodilators (e. g. amyl nitrite) should be given a trial.

Surgical treatment of nasophrayngeal craniopharyngioma. Radical removal by the transpalatal approach.

A case of infrasellar craniopharyngioma in a 14-year-old girl who was subjected to radical operation by a transpalatal approach is reported.