Akira Takaku

An Attempt to Treat Cerebrovascular ‘Moyamoya’ Disease in Children

Moyamoya formations at the base of the brain are not congenital vascular malformations but represent collateral pathways associated with chronic progressive stenosis of the carotid fork. The authors have studied 44 personal cases, 18 children under 15 years of age, and 26 adults. In children the Moyamoya vessels change through six stages: (1) carotid fork stenosis; (2) progressive carotid stenosis with initial Moyamoya collaterals and dilatations of cerebral arteries; (3) dilatation of Moyamoya collaterals and disappearance of anterior and middle cerebral arteries; (4) thinning of Moyamoya; (5) contraction of Moyamoya and disappearance of posterior cerebral arteries; (6) intracerebral vessels perfused from the external carotid and/or vertebrae. These six stages are not observed in adults. Bilateral cervical perivascular sympathectomy (PVS) was performed in 9 children and superior cervical ganglionectomy (SCG) was added unilaterally in 4 and bilaterally in 3 cases. Angiographic follow-up studies were carried out 1-7 years following surgery. Improvement was observed in most of the cases examined within the first 2 months after surgery. This was not the case in arteriograms performed more than 6 months postoperatively. It would seem that PVS and SCG can improve the progress of Moyamoya vessels but only for a short period of time. Clinical symptoms, however, seem to continue improving over a long period of time. Cerebral blood flow improved 5 weeks following surgery in a 13-year-old boy.

Brain Tumor in Newborn Babies

5 cases of brain tumor in newborn babies under 2 months are presented. 4 of them were supratentorial teratoma and originated from the midline, and 1 was a glioma at the cerebelloponitine angle. 2 cases died before surgery and 2 cases after surgery. In our 5th case a benign teratoma of 150 g was removed from the third ventricle. He was discharged and enjoyed rather good health for 2 years and 1 month before dying of recurrence of tumor. Internal hydrocephalus associated with the huge tumor was responsible for the sudden deterioration of general condition. Therefore, shunting procedures should be performed as the first aid. However, in our experiences, the conditions at admission were the critical and/or too late to be beneficial. Thus early diagnosis is absolutely necessary for surgical treatment. A summary and discussion of the data of 103 previously reported cases are included.

Vasa vasorum of the intracranial arteries.

SummaryMost of the major extracranial arteries have vasa vasorum which play an important role in some pathological conditions. However, in the intracranial arteries, the existence of vasa vasorum and their pathological implication have not been adequately investigated. We examined the distribution and incidence of vasa vasorum in the major cerebral arteries and their relationships to certain clinical factors in 50 autopsy cases performed between 1987 and 1994. By light microscopy, vasa vasorum were found in 36 of 50 patients. Of 36 patients, vasa vasorum in 30 cases were localizedly observed in the tunica adventitia and the in other 6 were distributed in the tunica media accompanied by intramural haemorrhage. Existence of vasa vasorum was more common in the proximal arteries (vertebral, internal carotid, and basilar arteries) than in the distal arteries (middle cerebral and anterior cerebral arteries). Vasa vasorum were found more frequently in aged patients with severe atherosclerosis and those with cerebrovascular diseases. Our results indicated that intracranial vasa vasorum existed with a higher frequency in the tunica adventitia of the vertebral and internal cerebral arteries, and the incidence of vasa vasorum related to severity of atherosclerosis. The development of vasa vasorum in the tunica media may reflect some pathological changes of cerebral arteries.

Changes in the cerebral vascular bed in experimental hydrocephalus: an angio-architectural and histological study.

SummaryThe angio-architectural and histological changes of small cerebral blood vessels in experimental hydrocephalus were studied to assess the changes of the vascular bed in the cerebral mantle.Change of the microvasculature assessed from microcorrosion casts by scanning electron microscopy (SEM) and the histological changes shown by light and electron microscopy were compared before and after shunting for hydrocephalus. The changes of the rCBF were also evaluated by the hydrogen clearance method.In hydrocephalus, a reduction in the number and caliber of the capillaries was noted in both the white and gray matter in the SEM study, but the capillaries were preserved and changes were mild and nonspecific in the electron microscopic examination. Shunting resulted in the reversal of all these changes to normal along with recovery of the rCBF, which had decreased in hydrocephalus.These observations suggest that changes of the vascular bed participate in the alteration of cerebral mantle width in the hydrocephalic process, and that the changes of the microvasculature result not only from damage to the capillaries themselves but also from changes of the perivascular structures.

The use of computed tomography in the prediction of delayed cerebral infarction following acute aneurysm surgery for subarachnoid haemorrhage

SummaryIn order to predict the occurrence of cerebral infarction after aneurysmal surgery in patients with subarachnoid haemorrhage, we measured the amount of subarachnoid blood on initial and on post-operative computed tomograms. We used a reliable grading method to estimate the amount of blood on computed tomograms in 24 patients with infarction due to vasospasm and 45 patients without cerebral infarction, all of whom underwent aneurysmal surgery within 48 hours after the ictus.The total amount of subarachnoid blood on admission and on the day after operation was more in the cerebral infarction group than in the non-infarction group. The clearance rate of subarachnoid blood by surgery was lower in patients with cerebral infarction than in patients without infarction and the predominant site of subarachnoid blood corresponded with the site of the infarct. Of 24 patients with cerebral infarction, 22 (92%) belonged to the group whose initial total blood score was more than 10 on admission and whose clearance rate by surgery was less than 50%.Therefore, we propose this range to be an indication for the occurrence of cerebral infarction in postoperative patients due to cerebral vasospasm.The presence of intracerebral haematoma and the amount of ventricular blood and their clearance by surgery were also estimated for the prediction of delayed cerebral infarction after aneurysmal surgery. However, they had no relation to the occurrence of cerebral infarction due to vasospasm.

Platelet-activating factor and antiphospholipid antibodies in subarachnoid haemorrhage

SummaryThe purpose of this study was to measure plasma platelet-activating factor (PAF) concentration, PAF-acetylhydrolase activities, anti-phospholipid antibody (aPLs) titers, and platelet function in patients with subarachnoid haemorrhage (SAH) and to assess the association of these variables with the development of cerebral vasospasm.Thirty-two patients with SAH due to ruptured cerebral aneurysm were studied. Plasma PAF concentration, PAF-acetylhydrolase activity, platelet count and aggregability, and plasma factor 4 (PF 4) concentrations were measured regularly until approximately 2 weeks after SAH. aPLs, including lupus anticoagulant and anti-cardiolipin IgG and IgM were measured within 3 days after SAH.Plasma PAF concentration in patients with SAH showed the highest value on the occasion during 5 to 9 days after SAH. The concentrations were higher in patients with infarction due to vasospasm than in patients without cerebral infarction on any occasions after SAH. Plasma PAF-acetylhydrolase activities did not change in patients, regardless of the presence of cerebral infarction after SAH. Increased platelet consumption and aggregability and higher concentrations of PF 4 were detected in patients with cerebral infarction and not in patients without cerebral infarction. The patients with cerebral infarction due to cerebral vasospasm had aPLs more frequently than the control volunteers.Our findings indicate that increased plasma PAF and aPLs may contribute to the pathogenesis of cerebral vasospasm after SAH.

Pyogenic osteomyelitis of an invaginated odontoid process with rapid deterioration of high cervical myelopathy: a case report.

A 72-year-old woman with diabetes mellitus presented with a progressive two-week history of neck pain and right hemiparesis. On admission to our hospital, the patient was afebrile but showed slight neck stiffness. Results of the laboratory examinations were normal except for a high fasting blood sugar of 188 mg/dl and two-plus glycosuria. Radiographs showed basilar impression and assimilation of the atlas Fig. 1a). MRI revealed compression of the upper cervical cord by the invaginated odontoid process and a mass lesion at the retro-odontoid space (Fig. 1b, c, d). Right hemiparesis was progressive after admission. So treatment with steroid and insulin was started and the right hemiparesis greatly improved. The patient then underwent transoral surgery two weeks after admission. The anterior arch of the axis was removed and the odontoid process was drilled out. The tip of the odontoid process was eroded and osteomyelitis was diagnosed. However, bacterial cultures of the resected specimens were negative for aerobic and anaerobic bacilli including Mycobacterium tuberculosis. Empirical antibiotic therapy was initiated with cefazolin 4g iv per day. Her neurological status was unchanged immediately after surgery and remained stable until the second postoperative day. Thereafter, the patient deteriorated rapidly with right hemiparesis on the third postoperative day followed by tetraplegia and apnea developing over the next few hours. Emergecy CT suggested compression of the upper cervical cord by a combination of residual odontoid process, body of the axis, and epidural soft tissue material. Urgent transoral re-operation was done and residual odontoid process, and the body of the axis were drilled out. An epidural abscess was noted behind the odontoid process and this was also removed. Pulsatile dural protrusion was observed afterward. The patient subsequently underwent occipitocervical fixation with iliac bone from occiput to C4. Tetraplegia and apnea recovered slowly, but the right hemiparesis persisted.

Turcot's syndrome presenting with medulloblastoma and familiar adenomatous polyposis : A case report and review of the literature

A 19-year-old man initially became aware of decrease in rightsided auditory acuity and tinnitus in May 1993. He presented with headache and right facial nerve palsy in addition to the above symptoms and was admitted to our hospital on December 12th, 1995. The patients grandfather had su ̈ered from colonic polyposis and his father had died from colon carcinoma. The patient himself had undergone colon ®berscopy at 17 years of age, which revealed multiple polyposis (Fig. 1). The pathological diagnosis was tubular adenoma. Neurological examination on admission revealed papill edema, right facial nerve palsy, impairment of right sided auditory acuity, bilateral nystagmus, and right cerebellar signs. T1-weighted MRI disclosed a cystic mass lesion with di ̈use enhancement in the vermis and right cerebellar hemisphere (Fig. 2). Subtotal removal of the tumour was performed on December 14th, 1995. Microscopic examination revealed a highly cellular tumour composed of oval cells with hyperchromatic nuclei. Numerous Hormer Wright rosettes were seen, but the tumour lacked connective tissue (Fig. 3). These ®ndings were compatible with classical medulloblastoma. He was managed with postoperative chemotherapy and radiotherapy, and has been without recurrence for the following 3 years. Acta Neurochirurgica > Springer-Verlag 1998 Printed in Austria Acta Neurochir (Wien) (1998) 140: 631±632

Gastrointestinal bleeding in cases of ruptured cerebral aneurysms.

SummaryAmong 1,000 cases of patients undergoing direct surgery on cerebral aneurysms, two, showed clear signs of preoperative, and 19 cases showed postoperative gastrointestinal bleeding. We have made a clinical analysis of various aspects of the 19 cases in which the bleeding developed postoperatively.1.Gastrointestinal bleeding was most frequent postoperatively in cases of AComA aneurysms (4.3%) and ICA aneurysms (2.0%), and less common in MCA and ACA aneurysm cases.2.Gastrointestinal bleeding was most frequently seen in those cases operated on between the third and seventh days after the last subarachnoid haemorrhage (8.9%) and was more common in cases with a relatively poor preoperative grade.3.The development of such bleeding in cases with a good preoperative grade was due to problems with the surgical operation in most cases, although the influence of vasospasm must not be ignored. The development of bleeding in cases with a poor preoperative grade is thought to be due primarily to vasospasm and transitory brain damage to the hypothalamus and the orbital portion of the anterior lobe due to a haematoma caused by aneurysm rupture.4.First, the location of gastrointestinal bleeding should be determined endoscopically and, if haemostasis is not achieved by coagulation, then the desirability of surgery should be considered early. Abdominal surgery may be performed.

Anti-phospholipid antibodies and cerebral vasospasm following subarachnoid haemorrhage.

SummaryDelayed ischaemic deficits due to cerebral vasospasm contribute to the high morbidity and mortality rates associated with subarachnoid haemorrhage. We evaluated the usefulness of measuring anti-phospholipid antibodies (aPLs) for prediction of the occurrence of symptomatic vasospasm and the outcome after subarachnoid haemorrhage. 32 consecutive patients with subarachnoid haemorrhage due to ruptured cerebral aneurysms were studied. They were admitted and operated on within 72 hours after the onset of subarachnoid haemorrhage. aPLs such as lupus anticoagulants, anti-cardiolipin IgG and anti-cardiolipin IgM were measured repeatedly after admission. Furthermore, platelet count, platelet aggregability and plasma platelet factor 4 were also measured. Eleven among the 32 patients (34.4%) showed positive in the examination for aPLs. Although aPLs could not predict symptomatic vasospasm, once symptomatic vasospasm occurred, patients with aPLs frequently demonstrated cerebral infarction and there-fore their outcome was worse. aPLs were associated with a severe initial clinical grade and SAH grade on CT scan. Therefore it may explain the association of aPLs with worse outcome. aPLs were detected between 1 and 7 days. Four of 6 patients (67%) with aPLs became negative between 7 and 13 days after subarachnoid haemorrhage. The mechanism of transient aPLs is unclear but it is more likely to occur in the severer grade patients. The reduction in platelet count, the increased platelet aggregability, and the increased plasma platelet factor 4 concentration were also observed in aPLs-positive patients with symptomatic vasospasm.