Moyamoya disease in patients of Finno-Ugric origin.

The terms moyamoya disease, moyamoya syndrome and moyamoya phenomenon can be found dispersed throughout the literature. The diagnostic criteria for moyamoya disease are: (1) stenosis or occlusion of the anterior cerebral, middle cerebral and internal carotid arteries, (2) an abnormal vascular network near these arteries and (3) bilateral findings. When only the two first conditions are present, the term moyamoya syndrome is used. The incidence of moyamoya disease is high in the Mongol race, although the moyamoya syndrome is more frequently reported among Caucasians. In the last two decades 41 cases of moyamoya were diagnosed in two Hungarian and two Scandinavian hospitals, respectively. Thirty-one patients were operated on 12 unilaterally and 19 bilaterally--either with extracranial/intracranial bypass (29 cases) or with encephalomyosynangiosis (2 cases). After a mean follow-up of seven years, 67.7% of the operated cases were symptom-free or neurologically improved. The majority of the patients had moyamoya disease and turned out to be of Finno-Ugric or Lapplandish ancestry. These people originally migrated from the East and belong to the Ural-Altaic family. Our findings suggest that while the moyamoya syndrome is found in different races, the moyamoya disease may be limited to people of Eastern (Mongol) origin. Revascularization surgery may be of benefit to patients with moyamoya.

Combined use of bone grafts and implants in the severely resorbed maxilla. Postoperative evaluation by computed tomography.

Combined horseshoe-shaped iliac bone grafts and Brånemark fixtures were used to rehabilitate patients with severely resorbed maxillae. Twenty patients were followed-up by computed tomography (CT) examination with axial slices to assess the fixture sites and to study the changes in height and width of the bone graft 3 weeks and 3, 6, 12, and 24 months postoperatively. The mean height of the bone graft at the 3-week postoperative examination was 8.2 mm; after 2 years the mean value had decreased to 6.2 mm. The height reduction occurred mainly between the 3-month and 1-year examinations. The mean width of the bone graft at the 3-week postoperative examination was 12.2 mm, and it decreased to 8.6 mm after 2 years. Most of the width reduction took place during postoperative months 1-3. From 1 year after the grafting procedure, the rate of reduction of both height and width was very low.

CT assessment of subarachnoid haemorrhage. A comparison between different CT methods of grading subarachnoid haemorrhage.

A number of different CT classifications of subarachnoid haemorrhage (SAH) were applied to a consecutive series of 50 patients with aneurysmal SAH. The best correlation with delayed ischaemic deficits (DID) was obtained with a score formed by the sum of the individual cisternal grades except that of the cortical subarachnoid space. The findings emphasize the significance of the extent of the SAH, rather than the presence of a localized cisternal clot with regard to the development of DID.

Subarachnoid blood on CT and memory dysfunctions in aneurysmal subarachnoid hemorrhage.

Ninety patients with a previous subarachnoid haemorrhage (SAH) were given a set of memory tests comprising immediate free recall of words (indexing long-term memory, LTM, and short-term memory, STM), final free recall of words (indexing LTM), final cued recall of words (indexing LTM), and a digit span test (indexing working memory, WM). Patients with a large amount of blood on CT, carried out within 72 h of the bleed, showed LTM as well as STM dysfunction, whereas patients with a small amount of subarachnoid blood evidenced only STM dysfunction. Patients with the ruptured aneurysm located on the anterior cerebral artery, however, constituted an exception with dysfunction of both LTM and STM together with intact WM, independent of the amount of subarachnoid blood. Also, patients with internal carotid artery or middle cerebral artery aneurysms and large volume SAH displayed LTM dysfunction, but differed concerning STM, the former showing intact STM and the latter showing STM dysfunction. Thus, it appears, that the combined information from factors such as the amount of subarachnoid blood and the location of the ruptured aneurysm is of vital importance for explaining the different patterns of memory dysfunctions after SAH.

Features of Sjögren's syndrome in patients with primary biliary cirrhosis.

Twenty-six consecutive patients with primary biliary cirrhosis (PBC) from northern Sweden were studied regarding the occurrence and features of Sjögren's syndrome (SS). In more than 50% of the patients the rose bengal dye test showed conjunctival and/or corneal staining. In six patients keratoconjunctivitis sicca (KCS) was present with positive rose bengal and Schirmer tests. In a further three patients only the results of the Schirmer tests were abnormal. Radiological findings of sialectasia were demonstrated in six patients, five of whom had KCS. Two of the seven patients who fulfilled our criteria for Sjögren's syndrome were HLA-B8 positive. A high prevalence of increased immune globulins and rheumatic factor was found, but this did not correlate with the presence of Sjögren's syndrome. Some features of Sjögren's syndrome were found in 73% of PBC patients, and keratoconjunctivitis sicca and/or sialectasia were found in 27% of PBC patients. This constitutes a high frequency of secondary manifestations of the liver disease.

Vertical odontoid fracture. Case report.

A case of vertical odontoid fracture is presented in which a concomitant fracture of the skull indicated possible pathogenetic mechanisms. The radiological diagnosis and the management of this case are discussed.

Hypercortisolism revealed by the dexamethasone suppression test in patients [corrected] with acute ischemic stroke.

Using the dexamethasone suppression test, we studied the activity of the hypothalamic-pituitary-adrenal axis within the first week after onset in 62 patients with acute ischemic stroke. Compared with two control groups (one comprising 25 elderly patients with various acute medical disorders and the other comprising 33 80-year-old volunteers), stroke patients had higher postdexamethasone cortisol levels (p = 0.08 and p = 0.001, respectively). By multiple regression analysis, high postdexamethasone cortisol levels in the stroke patients were significantly associated with proximity of the lesion to the frontal pole of the brain (p = 0.008) and with disorientation (p = 0.03), whereas no association with major depression was seen. Many stroke patients are exposed to hypercortisolism, which may have negative consequences upon organ functions. The extent to which dexamethasone administration suppresses cortisol levels seems to be determined mainly by the site of brain lesion and cannot be used as an indicator of major depression early after stroke.

Verbal memory function after subarachnoid haemorrhage determined by the localisation of the ruptured aneurysm.

A total of 219 patients who had suffered from subarachnoid haemorrhage (SAH) were given a set of memory tests. The tests comprised immediate free recall (indexing long-term memory, LTM, and short-term memory, STM), final free recall (LTM) and final cued recall (LTM) of words as well as a digit-span test (an index of working memory, WM). STM was highly sensitive to brain damage caused by SAH whereas WM was not. Localisation of the ruptured aneurysm, angiographic vasospasm or mass effect did not have any selective influence on the SAH group's STM or WM. The final free recall test was the most sensitive measure of LTM whereas the LTM part of the immediate free recall test was the least sensitive. LTM impairment was most pronounced for patients with ruptured left anterior communicating artery aneurysms or for patients with angiographic vasospasm or mass effect. It is suggested that, in the SAH patients, a passive phonological short-term store is impaired, but the active maintenance rehearsal mechanism is intact. For patients with LTM dysfunction, deeper, more elaborate, active processing is disrupted, hindering efficient long-term storage.

Co-existence of abdominal aortic aneurysms and intracranial aneurysms.

The occurrence of abdominal aortic aneurysms (AAAs) and intracranial aneurysms (IAs) in the same patient and in the same family was studied among 89 patients with AAAs and 485 patients with IAs. Among the AAA-patients two had IAs themselves and five had IAs in the family, whereas three IA-patients had AAAs themselves and eight had AAAs in the family. Moreover, one of the patients with both AAA and IA had a blood relative with AAA, and in six of the families with both types of aneurysms there were more than two subjects with aneurysms. The results indicate, that AAAs and IAs may have a common aetiologic factor.

Antifibrinolysis with tranexamic acid in aneurysmal subarachnoid hemorrhage: a consecutive controlled clinical trial.

A randomized controlled clinical trial was carried out to study the effect of tranexamic acid (AMCA, Cyklokapron; AB Kabi, Stockholm, Sweden) in the prevention of early rebleeding after the rupture of an intracranial aneurysm. The incidence of vasospasm, hydrocephalus, cerebral ischemic and thromboembolic complications, morbidity, and mortality was also evaluated. The series comprises 59 patients, 30 treated with tranexamic acid and 29 controls. The treatment was stopped if there was rebleeding, operation, or discharge from the hospital. There were 6 recurrent hemorrhages in 6 patients in the tranexamic acid-treated group and 11 recurrences in 7 patients in the control group. Recurrent hemorrhages occurred later in tranexamic acid-treated patients than in controls. Five patients in each group died from rebleeding. Five additional treated patients and 2 controls died from cerebral ischemic dysfunction. The results suggest that tranexamic acid may protect patients with ruptured aneurysms from rebleeding for 1 or 2 weeks, but that it also may produce cerebral ischemic complications.

Occlusion of the carotid artery using catheter with detachable balloon in pigs.

A new catheter with detachable balloon for vascular occlusion was successfully used for permanent occlusion of the internal carotid artery as well as branches of the external carotid artery in 5 pigs. The technique is intended for clinical use in man.

Computed tomography of the paranasal sinuses.

The diagnostic value of computer tomography in lesions of the paranasal sinuses has been investigated in three cases with maxillary carcinoma and in five cases with fractures of the facial bones. In most of the cases, particularly in carcinoma, computer tomography was found to provide additional information to that obtained with conventional X-ray techniques. In fractures, however, a supplementary plain film examination was found to be indispensable.

Computer tomography of the neurocranium.

The experience with computer tomography of the neurocranium in 300 patients submitted for computer tomography of the brain is reported. The more appropriate projections which may be obtained with the second generation of scanners in combination with an elaborated reconstruction technique seem to constitute a replacement of conventional skull films.