Severe haemodynamic stress in selected subtypes of patients with moyamoya disease: a positron emission tomography study.

BACKGROUND: The optimum management of patients with moyamoya disease remains controversial. OBJECTIVES: To examine retrospectively the correlation between the degree of haemodynamic stress and the clinical presentation by measuring cerebral haemodynamics and metabolism using positron emission tomography (PET). METHODS: 57 patients with moyamoya disease (mean age 32 years, range 12 to 64), classified into five groups according to clinical manifestations, underwent PET measurement of cerebral blood flow (CBF), cerebral blood volume (CBV), cerebral metabolic rate for oxygen (CMR(O2)), and oxygen extraction fraction (OEF) using (15)O labelled gases. The regional values in patient groups were compared with a normal group. RESULTS: CBF in non-symptomatic patients, patients presenting with transient ischaemic attacks (TIA), and patients with haemorrhagic onset (H) was not significantly lower than in normal controls in any region. CBV in the TIA group and in patients with infarction associated with TIA (I/TIA) was significantly higher than in the controls in most regions. OEF in the frontal, parietal, and temporal cortex was significantly higher in the I/TIA group than in the controls. Patients in the H group and those with a permanent deficit with infarction (PD group) had decreased metabolism with normal OEF. Multivariate analysis to test the distribution of the three dimensional vector (CBF, CBV, OEF) showed significant differences between every possible pair among the six groups except NS v H and H v PD in the frontal cortex. CONCLUSIONS: The haemodynamic status of moyamoya disease is not uniform, and severe haemodynamic stress occurs in selected subgroups of patients.

[Thoracic arachnoid cyst presenting incomplete features of Brown-Séquard syndrome: a case report].

Spinal intradural arachnoid cyst presenting incomplete features of Brown-Séquard syndrome is very rare. Only 6 cases have been reported. We report one in a thoracic lesion. A 42-year-old man noticed thermohypesthesia in his right leg, and monoparesis in his left leg. On admission, he presented incomplete features of Brown-Séquard syndrome below the Th7 level. MRI showed the spinal cord to be displaced antero-laterally to the right at Th6-7 level. Myelography and CT myelography failed to show the cyst wall, but an arachnoid cyst was totally removed with T5-8 osteoplastic laminotomy. Sensory disturbance has not changed postoperatively, but motor weakness in his left leg recovered within one month after the operation. No cystic lesion has been detected by MRI during the ten months since the operation. We investigated all seven cases in the literature including our case which showed incomplete features of Brown-Séquard syndrome. As regards to the location of the cyst, all cases were at the mid-thoracic level. And four cases were at the midline. These results suggest that the mechanism of Brown-Séquard syndrome associated with spinal arachnoid cyst may be related not only to the laterality of the lesion but also to the asymmetrical circulation in the watershed area.

[Clinical evaluation on etiology and surgical outcome in syringomyelia associated with Chiari type I malformation].

Etiology of syringomyelia associated with Chiari type I malformation has been unknown. Moreover, the surgical procedure of foramen magnum decompression for this type of syringomyelia has not been standardized yet. No one procedure has been always successful, leading to many alternative procedures. The purpose of the present study is to elucidate pathway of cerebrospinal fluid into the syrinx cavity and to find out the best procedure for this disease. Fourty two patients with syringomyelia associated with Chiari type I malformation, which were diagnosed with magnetic resonance imaging (MRI), underwent surgical treatment. In all patients, craniocervical junction anomalies, cervical disc herniation and other spinal diseases were ruled out. There were 26 men and 16 women, ranging from 6 to 72 (mean: 42.3) years in age. The size, length and position of upper end of each syrinx cavity and the degree of the tonsillar herniation were measured on preoperative T1-weighted image and were compared each other. There were no significant relationship between the degree of tonsillar herniation and the size, length and position of syrinx cavity. No case showed that the upper end of syrinx cavity communicated to the 4th ventricle. The results suggest that the pathway of cerebrospinal fluid into the the syrinx cavity was not central canal from the 4th ventricle but microcanals in the spinal cord. All patients were carried out with foramen magnum decompression, which was divided into 4 groups according to the degree of decompression: 1) tonsillectomy group: 12 patients underwent subocciptital craniectomy (SOC) with patcy-graft dural plasty using lyophilized dura mater and tonsillectomy, 2) lysis group: 7 underwent SOC, dural plasty and microsurgical lysis of arachnoidal trabecula and fibrinoid filament around herniated tonsil, 3) plasty group: 17 underwent SOC and dural plasty and 4) dural group: 6 underwent SOC and removal of the outer layer of the dura mater. The mean follow-up periods were 3.7 years in tonsillectomy group, 3.6 years in lysis group, 2.3 years in plasty group, 1.8 years in dural group, respectively. Evaluation of the result following four types of surgical treatments was performed on clinical symptoms and the volume of syrinx cavity on sagittal MRI. The ratio in the area of the syrinx and spinal cord on preoperative and postoperative sagittal MRI were measured. There was no significant difference among 4 groups on the degree of reduction of syrinx in the sagittal plane as evaluated on MRI, whereas with regards to improvement of the clinical symptoms, dural group was significantly worse than the other three groups. The surgical procedure of dural plasty was clearly less invasive than those of tonsillectomy and lysis of subarachnoidal trabecula. These results suggest that we should select dural plasty as a primary surgical procedure for syringomyelia associated with Chiari type I malformation.

Occult intrasacral meningocele associated with spina bifida: a case report.

We present a rare case of occult intrasacral meningocele associated with spina bifida and tethered cord syndrome in a 13-year-old female. The etiology is thought to be due to a dysraphic disorder. The pertinent literature is reviewed.

[Long-term evaluation of radiographic changes following cervical anterior fusion with hydroxyapatite ceramic spacer].

Cervical anterior fusion with iliac bone crest has become a popular surgical technique for cervical spondylotic disease. Since about 10 years ago, we have substituted hydroxyapatite ceramic spacer for autologous graft because of postoperative painful hip syndrome. Fourteen patients who underwent cervical anterior fusion with ceramic spacer were evaluated by plain radiographs for over eight years postoperatively. In six among the fourteen patients plain films demonstrated minimal stenosis of intervertebral disc height, of which the ratio ranged from 15% to 28% (mean 22%). The increase in mobility of the adjacent segment after fusion was noted in ten of the fourteen patients, although the ratio ranged only between 12% and 24% (mean 18%). In none of all the patients did dynamic plain films show cervical instability such as slippage and swan neck deformity. It seems that anterior fusion with ceramic spacer is a useful and safe method for a cervical spondylotic disease.

[Spondylodiscitis after operation for lumbar disc herniation].

Postoperative spondylodiscitis (POD) is a rare but severe complication of lumbar disc surgery. There were five patients with POD among 875 patients undergoing surgery for herniated lumbar discs, in the last 16 years. For detecting risk factor of discitis, a POD group of 5 patients was matched to a control group of 870 patients with respect to sex, age, disc type and operation. In five patients with POD follow-up evaluation of clinical symptoms, laboratory data were obtained, and magnetic resonance (MR) imaging was performed. A significant difference between the POD group and the control group was confirmed in sex, operation time and the volume of bleeding during operation. However, there was no significant difference confirmed due to age, disc type, disc level and operative procedure. In the case of males or prolonged operation time or increase of the volume of bleeding, POD may be more frequently observed. All five patients had a period of pain relief after their operations and then reported increasing low back pain with no focal signs. At diagnosis of POD all patients had an erythrocyte sedimentation rate (ESR) greater than 30 per hour and C-reactive protein (CRP) higher than 2.5. After treatment by antibiotics, low back pain gradually receded along with decrease of ESR and CRP. About 40 days later, these patients were almost free of back pain and ESR and CRP were within normal range. MRT1-weighted image during the acute phase demonstrated remarkably decreased signal intensity with loss of distinction between vertebral body and disc space. T2-weighted image showed increased signal intensity in the adjacent vertebral bodies and end-plates. Gadolinium-enhanced T1-weighted image had homogenous enhancement of vertebral body and disc space. During the subacute phase, however, T1-weighted image demonstrated moderately decreased signal intensity noted in the posteroinferior portion of the L5 vertebral body and the narrowed L5/S1 disc space. T2-weighted image showed iso signal intensity in L5 and S1 vertebral bodies. Gadolinium-enhanced T1-weighted image had mild homogenous enhancement in the posteroinferior portion of the L5 vertebral body. Three months after treatment of POD, there was major signal change in neither T1 nor T2 weighted images, though T2 showed subtle abnormalities with decreased signal intensity adjacent to the L5/S1 disc space.

Postauricular response in motor paresis with intracranial lesions.

The clinical usefulness of the postauricular response (PAR) in the evaluation of motor paresis was studied in 105 patients with intracranial lesions and 25 normal volunteers. Click stimuli at 90 dB hearing level were delivered in each ear, and PARs recorded from bilateral posterior auricular muscles with reference to the vertex (Cz). No volunteer demonstrated PAR in the relaxed posture, but in tensed postures increasing muscle tone PARs were elicited with a latency of 11.5 +/- 0.7 msec and an amplitude of 6.2 +/- 2.8 muV. Sixty-two patients (59%) had high-amplitude PARs in the relaxed posture. PARs were enhanced more frequently in paretic patients than in non-paretic patients (p less than 0.01). In subcortical lesions, the PAR latency had a significant correlation with the degree of motor paresis (p less than 0.005). In patients, the enhanced PAR amplitude suggested the presence of motor paresis associated with deep-seated mass lesions. The enhanced response is thought to be associated with dysfunction of the pyramidal and extrapyramidal tracts.

[Intraarterial injection of low molecular weight dextran and urokinase for acute cerebral infarction].

Strategic recanalization of the occluded cerebral vessels has shown promise as a therapy for embolism and thrombosis in the acute stage. A single-dose of urokinase (UK) administered by intravenous and intraarterial routes was usually designed to restore patency of the infarct-related arteries and reperfuse the area of infarction. However, thrombolytic agents which have been available to date may lack resoluvability, limiting the amount of doses, because overdosage may induce hemorrhagic complication. This newly-designed therapy, intraarterial injection of UK-low molecular dextran (LMWD) complex was introduced in order to overcome the danger shown in the previous study. A high-resolvent allows cut-down of urkinase doses. Patients with acute cerebral infarction were selected for treatment with the resolvent if they satisfied the following conditions: 1) up to 79 years old without serious systemic diseases, 2) less than 12 hours from the onset, 3) better than the score of 8 in GCS, 4) no abnormality in CT scan, 5) apparent neurological deficit and 6) responsible pathology in angiography. LMWD of 15ml, UK of 240000IU and 15ml saline-complex was injected as one course at 2.0-2.5ml/min for 11 cases. Recanalization was observed in seven cases of embolism, and lack of reperfusion in five cases of thrombosis. The minimum effective dose was determined as 480000IU of UK in two courses. In terms of time lag from the onset, six hours may be the inferior limit, within which five of six cases (83%) succeeded in the recanalization.(ABSTRACT TRUNCATED AT 250 WORDS)

[A case of acute myeloblastic leukemia associated with multiple intracerebral hematomas].

A case of acute myeloblastic leukemia associated with multiple intracerebral hematomas is presented. A 19-year-old woman with a two week's history of mild fever suddenly lost consciousness, and was afflicted right severe hemiparesis, left mild hemiparesis and motor aphasia. A CT scan revealed bilateral thalamic hyperdense lesions and paraventricular small hematoma in the right hemisphere. Hematology showed marked leukocytosis (450,000/mm3), mild anemia and no coagulopathy including disseminated intravascular coagulation syndrome. Cytology showed myeloblasts with positive stain in peroxidase and negative in esterase both in cerebrospinal fluid and blood. These findings indicated M 1 type, myeloblastic leukemia without maturation, according to FAB (French-American-British Co-operative group) classification. CT scan on the second day demonstrated expansion of the hematoma in the right thalamus, and nine brand-new small hematomas in different locations. The patient deteriorated into brain death soon after this examination. The pathology of this case was supposed to be "hyperleukocytosis", which is defined as a leukocyte count greater than 100,000/mm3. Severe leukostasis due both to dense leukocytes and lack of mobility of the myeloblast brought about an increase in permeability because of local impairment of nutrition to the walls of the vessels. As a result, the following histological changes occurred: 1) cellular exudation into Virchow-Robin space, 2) the appearance of leukemic nodule, admixtures of leukemic cells and erythrocytes, 3) mechanical compression of the capillaries and venules by the enlarging mass of the leukemic nodules. CT scan showed these characteristics as follows: 1) multiplicity, 2) small-size, 3) cerebral hemisphere, especially in white matter.(ABSTRACT TRUNCATED AT 250 WORDS)

[Three-dimensional display of structures and lesions in the brainstem using a personal computer].

The method of three-dimensional reconstruction of structures and lesions in the brainstem was developed by using a personal computer to visualize the affected sites in some cranial nuclei and long tracts. Outlines of brainstem structures and lesions were digitalized manually by tracing the atlas of the human brainstem and CT or MR images. Four outlines of the brainstem, the medulla, lower pons, upper pons and midbrain, and the outlines of any parenchymal structures were taken at every point 2 mm in thickness. These were used as the standard visualization of the atlas. The CT and MRI, which show outlines of the lesions, were taken on the vertical planes to the floor of the fourth ventricle, one of which included the fastigium, at every point 3 to 5 mm in thickness. These two kinds of outlines, taken from the atlas and the CT/MRI, were reconstructed three-dimensionally with commercial 3-D software. It took only 20 to 25 minutes for the digitalization and the 3-D reconstruction. Three-dimensional display of small structures, which were difficult to identify on CT or MRI, and lesions would enable the visualization of their anatomical relations and make possible the detection of the affected sites, not only in the axial but in the longitudinal direction. Correspondence between the affected sites on 3-D and the neurophysiological examinations (ABR, SSEP) confirms that our 3-D method is adequate for use in the anatomical diagnosis of affected sites in any small structure in the brainstem.

Binding of F-actin to a region between SH1 and SH2 groups of myosin subfragment-1 which may determine the high affinity of acto-subfragment-1 complex at rigor.

Covalent cross-linking reaction between SH1 and SH2 groups in myosin subfragment-1 (S-1) by N,N'-p-phenylenedimaleimide (pPDM) was followed by the degree of inactivation of NH4+-EDTA ATPase activity. The rate of the cross-linking reaction decreased to less than a 20th in the presence of F-actin. The inhibitory effect of F-actin was not observed in the presence of MgATP. Binding of F-actin to S-1 was measured using ultracentrifugation. S-1 whose SH1 and SH2 were covalently cross-linked by pPDM or 5,5'-dithiobis(2-nitrobenzoic acid) (DTNB) did not bind F-actin. After the DTNB-cross-linked S-1 is reduced by dithiothreitol, the ability to bind F-actin is recovered. These results suggest that S-1 has a binding site for F-actin in the region between SH1 and SH2. This site appears to determine the high affinity of acto-S-1 complex at the rigor while decreasing the affinity more than 10(2) times in the presence of MgATP.