Cerebrovascular disorders associated with von Recklinghausen's neurofibromatosis: a case report.

A 28-year-old woman with von Recklinghausen's neurofibromatosis (NF-1) had a huge hematoma in the left posterior nuchal region. Carotid and vertebral angiograms revealed marked stenosis at the C3 portion of the left internal carotid artery, slight moyamoya staining, occlusion of the left vertebral artery at the atlas level, and a right internal carotid artery aneurysm. The radiographic, clinical, and histological features of this case are discussed together with a review of 42 similar cases found in the literature.

Prevention of cerebral ischemic symptoms in cerebral vasospasm with trapidil, an antagonist and selective synthesis inhibitor of thromboxane A2.

The results of our previous experimental and clinical studies led us to the hypothesis that, in the pathogenesis of cerebral vasospasm, subarachnoid focal acidosis resulting from anaerobic changes of subarachnoid clots may be a factor upsetting the balanced synthesis of both thromboxane A2 and prostaglandin I2 from prostaglandin endoperoxides on the inner surface of cerebral arteries. Thus, there is a higher concentration of thromboxane A2, a prostanoid that causes arterial contraction and platelet aggregation. We tested the administration of trapidil, an antagonist and selective synthesis inhibitor of thromboxane A2, in a series of 20 cases for the prevention of cerebral vasospasm and cerebral ischemia after aneurysmal rupture. Vasospasm was demonstrated by angiography in 9 of these cases, but only 2 of the 9 showed mild signs of cerebral ischemia. Of the 20 patients, 15 were discharged from the hospital as cured and 3 had a neurological deficit at discharge. Our findings suggest the significance in symptomatic vasospasm of thrombus formation by platelet aggregation and the effectiveness of trapidil as a preventive.

Dural sinus pressure as related to neurosurgical positions.

Taking advantage of the exposure of dural sinuses during craniotomies, we measured confluens sinuum pressure by direct catheterization. The influence of various surgical positions and of other factors on confluens sinuum pressure was studied. When the upper half of the body was raised, the confluens sinuum pressure decreased to reach zero at +25 degrees; when the angle was +90 degrees, a marked negative pressure of -12.7 +/- 3.0 cm H2O (mean +/- SD) was observed in adults. In children under 6 years of age, however, such negative pressure was not observed even at an angle of +90 degrees. The confluens sinuum pressure was 2.7 +/- 0.6 cm H2O with the patient in the reverse jackknife position (supine position with the upper and lower halves of the body elevated ca. 20 degrees), 3.0 +/- 0.8 in the sea lion position (prone position with the upper and lower halves of the body elevated ca. 20 degrees and with the neck hyperextended), 5.8 +/- 0.9 in the prone position, and 5.9 +/- 1.7 in the supine position. In the supine and the sitting positions, the confluens sinuum pressure was elevated well enough by jugular compression to prevent air embolism. Positive pressure respiration did not raise the confluens sinuum pressure. The pressure of the jugular bulb measured by percutaneous direct puncture did not always reflect the confluens sinuum pressure, probably because subcutaneous hematoma produced an effect similar to that of jugular compression.

Total removal of a falcotentorial junction meningioma by biparietooccipital craniotomy in the sea lion position: a case report.

The successful total removal of a huge falcotentorial junction meningioma in a 59-year-old woman by biparietooccipital craniotomy with the patient in the sea lion position (prone with a hyperextended neck and with 20 degrees elevation of the upper and lower halves of the body) is reported, with some comments on the advantages of this approach and position. Taking advantage of the exposure of the dural sinus, the confluens sinuum pressure was measured by direct catheterization with the patient in various positions. The pressure was 3.6 cm H2O in the sea lion position, 2.4 cm H2O in the reverse jackknife position (supine with 20 degrees elevation of the upper and lower halves of the body), and -12 cm H2O in the sitting position.

Subepicranial varix mimicking sinus pericranii: usefulness of three-dimensional computed tomography angiography and bone window computed tomography--case report.

A 16-year-old female presented with a rare case of subepicranial varix in the left temporal area manifesting as a soft mass in the left temporal area when she laid down in the left lateral position. Bulging of the mass was observed when intracranial venous pressure was raised by the Valsalva maneuver, the left lateral position, or the prone position. Bone window computed tomography (CT) revealed a tiny hole, 1 mm in diameter, in the outer bone table. Three-dimensional CT (3D-CT) angiography clearly visualized a mass with a diameter of approximately 10 mm connected to the diploic vein. The mass was totally resected by operation. Venous bleeding was observed from the tiny hole. Histological examination revealed a venous lesion mimicking sinus pericranii and containing endothelial cells. No communication with the intracranial venous sinuses was identified, so the diagnosis was subepicranial varix. Radiological examination by direct injection of contrast medium is usually performed to identify subepicranial varix, but 3D-CT angiography is a non-invasive preoperative examination that can visualize this small venous lesion. Adjustment of the CT acquisition conditions may allow 3D-CT angiography to identify sinus pericranii in the future.

Malignant melanoma arising from the sphenoidal sinus--case report.

Malignant melanomas arising from the sella turcica or sphenoidal sinus with bilateral invasion of the base of the skull or cavernous sinus are extremely rare. Whether the sella turcica or sphenoidal sinus is the site of origin is difficult to determine based on neuroradiological findings. An 83-year-old Japanese female presented with headache as the initial symptom. She suffered rapid progression of bilateral obstruction of the nasal cavity, left nasal bleeding, and bilateral visual field defects. The preoperative diagnosis was pituitary adenoma, metastatic tumor, or malignant paranasal tumor. Biopsy was performed. The histological diagnosis was malignant melanoma. Postoperatively, the tumor progressed rapidly. She suffered several cranial nerve pareses and hypopituitarism. She died within 6 months. Tumors arising from the sphenoidal sinus cause obstruction of the nasal cavity or nasal bleeding first, and then cause cranial nerve pareses by invasion of the cavernous sinus. This sequence of clinical manifestations can be attributed to the anatomical relationships between the sphenoidal sinus, nasal cavity, and cavernous sinus. Differential diagnosis of the origin in the sella turcica or sphenoidal sinus appears to be relatively easy based on further observation of the clinical course and symptoms.

[Posterior fossa subdural hematoma in neonates: report of two cases and review of the literature].

Posterior fossa subdural hematoma in neonates caused by birth trauma is rare; only 30 clinical cases can be found in the literature so far. Here we experience two similar cases and review that literature. Case #1 is that of a full-term female born in the pelvic position. At 21 hours after birth she developed convulsions and a CT scan revealed a high-density area, mainly in the right posterior fossa. On the fourth day, a right suboccipital craniectomy was performed and about 15 ml of hematoma was removed. At present her mental and somatic development is appropriate for her age (5 months), despite the fact that moderate brain atrophy was seen in the CT scan taken at 4 months. Case #2 is that of a full-term female also born in the pelvic position. At 38 hours she became convulsive and opisthotonic; a CT scan revealed a high-density area in the posterior fossa. No neurological abnormality was seen thereafter, however, and she was treated conservatively. On the 18th day, the high-density area in the CT scan had almost disappeared. In the CT scan taken at 6 months moderate brain atrophy was seen, but at the present age of two she evidences quite normal development both mentally and somatically. These cases lead us to emphasize the importance of prompt diagnosis followed by removal of hematoma, and the usefulness of CT scan as a diagnostic method in clinical manifestations of this sort.

[The significance of dural sinus pressure in neurological surgery-- correlation with surgical position].

A study was made of the significance of confluens sinus pressure in various surgical positions and of various factors influencing confluens sinus pressure. The following findings were obtained: Confluens sinus pressure in various positions was follows: Reverse jackknife position (supine position with upper and lower halves of the body elevated ca 20 degrees) 1.3 +/- 0.8 (Mean +/- SD) cmH2O Sea lion position (prone position with upper and lower halves of the body elevated ca 10 degrees with neck hyperextended) 2.7 +/- 0.6 cmH2O Prone position 5.8 +/- 0.9 cmH2O Supine position 5.9 +/- 1.7 cmH2O Right lateral position 6.9 +/- 0.7 cmH2O Left lateral position 9.6 +/- 1.2 cmH2O Since confluens sinus pressure is strongly affected by gravity, the determination point, the height of the right atrium, and the central venous pressure were referred to when measuring the surgical position. When the upper half of the body was raised (at angle ranging from--10 degrees to +90 degrees) confluens sinus pressure became zero in adults when the angle was +25 degrees or thereabouts. When the angle was +90 degrees, a marked negative pressure of -12.7 +/- 3.0 cmH2O, was observed, suggesting the danger of air embolism. In 4 children under 6 years of ago, however, negative pressure was not observed even at an angle of +90 degrees, although some changes due to different angles were noted. This suggests some specificity of dural sinus pressure. Intrathoracic negative pressure at inspiration, the contraction of skeletal muscles, and profuse hemorrhage, and sympathetic nervous strain were surmised as other factors influencing dural sinus pressure.

[Possible role of microthrombus in the pathogenesis of cerebral vasospasm (author's transl)].

Our previous experimental and clinical studies yielded the following results: CSF flow around the subarachnoid vessels is often interrupted by subarachnoid clots; anaerobical incubation of CSF-blood mixture led to a marked fall in the pH value; the vasocontractility of anaerobically incubated CSF-blood mixtures was greater than that of aerobically incubated samples; vasocontraction induced by anaerobically incubated samples was inhibited to a far greater extent the prostaglandin synthesis inhibitor meclofenamic acid than by phenoxybenzamine; cases of asymptomatic marked angiographical vasospasm or of cerebral ischemia with relatively slight angiographical vasospasm were not rarely encountered. Those results lead us to a hypothesis that, in the pathogenesis of cerebral vasospasm, subarachnoid focal acidosis resulting from anaerobical changes of subarachnoid clots may be factor upsetting the balance of the synthesis of TXA2 and PGI2 from PG endoperoxides on the inner surface of cerebral arteries, in favor of TXA2--which plays a role in arterial contraction and in thrombosing with platelet aggregation. On this basis we have been testing the administrations of trapidil, an antagonist and selective synthesis inhibitor of TXA2, in 20 consecutive suitable cases so far, for the prevention of cerebral vasospasm after aneurysmal rupture. Angiographical vasospasm was seen in 9 of the 20 cases, but no signs of cerebral ischemia were detected in 7 of the 9 cases, either clinically or in CT scan. The importance of thrombus formation by platelet aggregation in symptomatic vasospasm are thus suggested.

[Traumatic hematoma in the basal ganglia (caudate) with favorable prognosis: report of two cases].

Two cases of traumatic basal ganglial hematoma with fairly good prognosis were reported. Several cases with similarly favorable prognosis could be also found in the recent literature. In these cases, post-traumatic disturbance in consciousness was mostly slight or moderate and the patients were rarely comatose. The inquiries in these cases were commonly associated with superficial injuries such as skull fracture, epi-or sub-dural hematoma, brain contusion, or another traumatic ICH. The hematomas in the basal ganglia usually showed a mass effect causing focal neurological signs such as hemiparesis. Neurological improvement was achieved in the operated cases, and final outcome was mostly fair with some fully recovered cases. These clinical features are quite different from those of most cases of traumatic basal ganglial hematoma reported so far, which closely resemble diffuse axonal injury and whose prognoses are extremely poor. There may be two different categories in the traumatic basal ganglial hematomas, those with fair outcomes, and those with poor outcomes.

[A case of extra-intracranial bypass venous graft for giant internal carotid aneurysm (author's transl)].

A male patient, aged 42, (No. 780624) was admitted to the Department of Neurosurgery of Hirosaki University Hospital, complaining recent weight loss, intolerance to cold and visual disturbance of the right eye. Ophthalmological examination revealed the optic atrophy with decreased visual acuity and concentric visual defect of the right eye. Endocrinological examination showed almost general suppression of adenohypophyseal function except abnormal high level resetting of cortisol diurnal rhythm. Radiological examination revealed the accessory middle cerebral artery and giant internal carotid aneurysm of the right side which was displayed by contrast-enhanced CT scan, with the enlarged sella turcica. Good cross filling was seen in left CAG through the anterior communicating artery. Extra-intracranial end to end anastomosis of the right internal carotid artery was performed with long venous graft under general anesthesia with hypothermia and induced hypotension, on Oct. 26 '78. Unroofing of the right optic canal was very useful to preserve the optic nerve, and the body of the giant aneurysm was opened and sutured tightly to reduce its mass effect. Interlacing suture for the anastomosis of the cervical internal carotid artery was employed successfully. The blood flow of the bypass graft, measured as enough volume with square wave flowmeter during the operation, was also confirmed with postoperative angiography. After the episodes of gastrointestinal bleeding, hypotensive attack and hemorrhagic infarction of right frontal base, the postoperative final result was successful and the patient is doing well, 6 months after the operation.

[Two cases of well-known syndrome due to midbrain-brain stem hemorrhage--Weber's syndrome and one and a half syndrome].

Two cases of well-known syndrome due to midbrain-brain stem hemorrhage without having a history of hypertension were reported. Case 1 is a 62-year-old female, presenting Weber's syndrome due to midbrain hemorrhage and diagnosed as Moyamoya disease angiographically. Case 2 is a 24-year-old-male, presenting One and a half syndrome due to brain stem hemorrhage from a brain stem cavernous angioma, which was diagnosed angiographically and by magnetic resonance imaging (MRI). Left hemiparesis seen in case 1 had improved by conservative therapy but no improvement could be obtained in the oculomotor nerve palsy. In case 2, hematoma was aspirated surgically for the purpose of decompression and his neurological symptoms improved markedly. Their pathogenesis and pathophysiology were also discussed.

Role of platelet function in symptomatic cerebral vasospasm following aneurysmal subarachnoid hemorrhage.

To evaluate the role of platelet function in the pathogenesis of cerebral vasospasm, we compared sequential changes of platelet aggregability and beta-thromboglobulin and thromboxane B2 concentrations in blood samples from the internal jugular and peripheral vein of 13 patients with aneurysmal subarachnoid hemorrhage. Platelet function in blood from the internal jugular vein tended to be enhanced during days 0-1 but recovered to the normal range during days 2-4. After day 5, platelet function showed various patterns depending on the presence of symptomatic vasospasm. In patients without symptomatic vasospasm, sequential changes were relatively minor, with normal or slightly high values. Patients with symptomatic vasospasm already showed high platelet aggregability during the early stage of vasospasm. The concentration of beta-thromboglobulin increased several days after the onset of vasospasm, reaching 80 ng/ml or more in patients with a poor prognosis. Two of the five patients with symptomatic vasospasm showed markedly high concentrations of thromboxane B2 after day 8. These results suggest that vasospasm activates platelets and promotes aggregability and that the resulting increased tendency for thrombus formation may affect the patient's prognosis during the advanced stage.

Anaerobic change of bloody CSF in subarachnoid haemorrhage. Its relation to cerebral vasospasm.

In our in vitro study of subarachnoid haemorrhage, the anaerobic incubation of CSF-blood mixture led to marked fall in the pH value thereof, which suggested to us that intracranial focal acidosis may play some role in the pathogenesis of cerebral vasospasm or disturbance of consciousness after the haemorrhage. To test this hypothesis, we treated 16 clinical cases of such disorders with carotid injection of 7% sodium bicarbonate solution. The treatment resulted in considerable improvement of the disturbance of consciousness by dilating the cerebral peripheral arteries; this we could observe angiographically 15 minutes after carotid injection. We found no morphological changes, however, in the spastic vessel itself up to 30 minutes after the injection. Given the result both of this study and of our previous experiments, we offer the hypothesis that the synthesizing process of thromboxane A2 and intracranial focal acidosis might play an important role in the pathogenesis of cerebral vasospasm.

Dural sinus pressure: various aspects in human brain surgery in children and adults.

To prevent air embolism and minimize neurosurgical venous hemorrhage, the dural sinus pressure (confluens sinuum pressure, CSP) was examined under various conditions in 47 cases, 11 of whom were children. Either the extracranial (group A) or catheter type (group B) pressure transducer was used. The latter gave approximately 30% higher values than the former. In any surgical position, children showed a tendency toward higher pressure than did adults. This was particularly the case in the sitting position; adults showed negative pressure [-8.6 +/- 2.3 (SD) mmHg, group A], whereas all eight children less than 9 yr of age (group A, 5 cases; group B, 3 cases) showed positive pressure. The youngest with negative CSP in a sitting position was a 9-yr-old boy. When the upper half of the body was raised, the CSP decreased linearly and became zero at approximately 25 degrees. In anteflexion of the neck, the CSP decreased significantly, and even with inclination of the upper half of the body of only 15-20 degrees or more upward, negative pressure was observed in adults. In children, right and left rotation of the neck showed remarkable increase of the CSP. In both supine and sitting positions, CSP was elevated sufficiently by bilateral jugular compression to prevent air embolism. Positive-pressure respiration did not raise the CSP, contrary to widely accepted knowledge. This study was originally performed in relation to brain surgery, but the results also seemed to be valuable in physiology.

A simple self-retaining cerebral retractor. Technical note.

A flexible copper spatula, usually used by us as retractor in brain surgery, was easily converted to become self-retaining without any other apparatus, by a simple minor modification.