[A case of multiple extracranial vertebral artery aneurysms associated with neurofibromatosis].

An unusual case of multiple aneurysms of the extracranial vertebral artery associated with neurofibromatosis is presented. A 48-year-old woman, complaining of weakness and numbness of the left upper extremity, was admitted to our hospital on July 27, 1987. She had undergone the removal of a left internal thoracic artery aneurysm due to hemothorax on June 12, 1987. On the next day, the symptoms of the left upper extremity appeared. On admission, general physical examination showed multiple cafe-au-lait spots on her body and cervicothoracic scoliosis. Neurological examination revealed left C5, 6 radiculopathy. Cervical CT scans showed enhanced masses at the transverse foramina. Left subclavian angiography demonstrated multiple extracranial vertebral artery aneurysms from the origin of the vertebral artery to the C4 level. This case was treated by proximal and distal ligation of the aneurysms. Postoperative right and left angiograms revealed no aneurysms filled with contrast materials. The weakness and numbness of the left upper extremity disappeared gradually after the operation.

[A case of spinal cord glioblastoma multiforme].

A 35-year-old woman was admitted to our hospital with a 3 month history of progressive paraparesis and impairment of bowel and bladder function. MRI suggested a malignant glioma at the level of T9 to L1. Laminectomy and subtotal removal of the tumor was performed. The surgical specimen was a glioblastoma multiforme. An aggressive adjuvant therapy was scheduled to prevent rapid local regrowth and leptomeningeal dissemination. Radiotherapy with a total dose of 65Gy was delivered with chemotherapy including ACNU (2mg/kg) and vincristine (0.2mg/kg). Lymphokine-activated killer (LAK) cells were given intrathecally with a total dose of 1.6 x 10(9) LAK cells with 3 x 10(4) units of IL-2. MRI taken 6 months after surgery revealed no residual tumor, and no malignant cell was detected in the patient's CSF. After physiotherapy, she became able to walk with a stick and was discharged. Chemotherapy (ACNU 2mg/kg/8 weeks) had been further continued for 2 years. She did well until 14 months after surgery, when paraparesis recurred and rapidly progressed to completism. MRI revealed a spinal cord swelling with marked edema, suggesting delayed radiation necrosis. Two years after surgery, MRI showed a marked atrophy of the spinal cord, and no residual tumor. But 3 years after surgery, a round tumor at the level of T11 and T12 was revealed on MRI, and she was admitted to our hospital again. A spinal cord amputation was performed, and the tumor was totally removed without worsening her neurological symptoms. Surgical specimen of the tumor was glioblastoma multiforme again.(ABSTRACT TRUNCATED AT 250 WORDS)

[Lyme disease with the triad of neurologic manifestations].

A 40-year-old man developed progressive neurologic manifestations following a tick bite with subsequent localized erythema migrans. These manifestations included bilateral sensory radiculoneuritis (T7-12), rectovesical dysfunction, paraparesis, right facial palsy and nuchal rigidity. Both serum and cerebrospinal fluid titers of IgG antibody against Borrelia burgdorferi were 1:8,192 using indirect immunofluorescence assay. No IgM antibody was detected. With high-dose intravenous penicillin and corticosteroid treatment the neurologic symptoms and signs gradually subsided, with a corresponding decrease in the IgG antibody titers. Among the cases of Lyme disease reported so far in Japan, the present case seems to be typical and serious in terms of the severity of nervous system involvement and the intensity of the antibody response.

Surgical removal of giant acoustic neurinomas involving the skull base. Report of two cases.

Neurinomas arising from the peripheral branch of the acoustic nerve distal to the internal auditory canal in the temporal bone are rare. Two advanced skull-base neurinomas are described which were situated mainly in the temporal petrous bone, and extended to the parapharyngeal space anteriorly, to the lateral cervical portion inferiorly, into the sphenoidal sinus medially, and into the middle and posterior cranial fossae compressing the brain stem. Both patients had been deaf for several years without other neurological deficits. The operative findings revealed that the fifth, seventh, and caudal cranial nerves were intact; therefore, it was suspected that these neurinomas originated primarily within the cochlea or the vestibule in the temporal bone. The tumors were completely removed via an extradural approach, with good results. Since the surgical treatment of such advanced skull-base neurinomas is difficult, the operative infratemporal fossa approach is described in detail.

[A case of pituitary adenoma presenting binasal inferior quadrants hemianopsia].

Visual field defect due to pituitary adenoma ordinarily shows bitemporal hemianopsia. But we experienced a case presenting binasal inferior quadrants hemianopsia. A 60-year-old woman was admitted to our hospital complaining of headache and blurred vision. At ophthalmologic examination, the visual acuity on the right was 0.02 and on the left 0.3. Visual field showed a loss of bilateral inferior nasal quadrants. There was neither pallor nor edema of either of the optic disks. A computerized tomography (CT) scan showed an enhancing mass in the intra- and suprasellar region. But despite remarkable suprasellar expansion of the tumor, the straight view of bilateral carotid angiograms revealed no elevation of the first part of the anterior cerebral arteries (ACA). On the lateral view, the terminal portion of the precommunicating part of the left ACA showed rather marked anteroinferior displacement. 2 mm thin sliced CT scans at the suprasellar region revealed that the left internal carotid artery had been touching the lateral portion of the tumor and the ACA had been displaced anteriorly by the tumor. Two weeks after admission, transsphenoidal tumor resection was carried out. Total removal was achieved and histological examination showed that the tumor was nonfunctioning chromophobe adenoma. The postoperative course was uneventful except for transient diabetes insipidus. The patient's visual acuity rapidly improved to 0.8 on the right and 0.5 on the left two weeks after operation. Although there was still a tendency for left inferior nasal field defect, remarkable improvement was obtained subjectively and objectively. According to the findings of CT scans and cerebral angiograms, binasal hemianopsia may have been produced by the mechanism as follows.(ABSTRACT TRUNCATED AT 250 WORDS)

[Transoral transclival approach to vertebral-posterior inferior cerebellar artery aneurysm. Case report].

A 61-year-old male was hospitalized for subarachnoid hemorrhage. Cerebral angiography revealed a small left vertebral-posterior inferior cerebellar artery aneurysm, 5 mm to the right of the midline and in the lower third level of the clivus--the so-called "no man's land." It was felt that surgery through the lateral suboccipital approach would likely result in neurological deficits, since the aneurysm would be obscured by the tortuous parent artery or the basilar artery. Therefore, the transoral transclival approach was undertaken and the aneurysm was successfully obliterated. Postoperatively, the patient developed transient right hemiparesis and mild meningitis without evidence of liquorrhea, but fully recovered with conservative management. Certain specific sites of aneurysms, as well as excessive tortuosity of the parent artery, are considered to be indications for the transoral transclival approach. Meningitis is the most worrisome complication of this approach, and mortality is high. Effective preventative measures, which were taken in this case, include tight closure of the dura mater and the pharyngeal mucous membrane, use of the smallest possible clip, and continuous cerebrospinal fluid drainage.

[A case of radiation induced aneurysm of extracranial carotid artery].

An unusual case of post-irradiation aneurysm of extracranial internal carotid artery is presented. A 70-year-old man, complaining of left cervical throbbing mass with focal pain, was admitted on February 8, 1985. It was noted, from his past history, that he had had surgery of the removal of cervical lymph-nodes and that unknown dosage of irradiation had been added to the cervical region 30 years before. Left carotid angiography (on admission) demonstrated a giant aneurysm in the cervical portion of internal carotid artery. Right carotid angiography with compression of left carotid artery revealed good cross filling through anterior communicating artery. Computed tomography with contrast media showed a ring like enhanced mass, which was thought to showed a ring like enhanced mass, which was thought to suggest that a large part of the aneurysm was filled with intraluminal thrombosis. During 30 days of evaluation, the aneurysm grew larger and his cervical pain became untolerable. Operation, the resection of the aneurysm and the reconstruction (of circulation) with vein graft, was challenged n March 12. It was so difficult with meticulous work that the ligation of left common carotid artery was performed after all. Seven days after the operation, he suffered from the gastrointestinal bleeding, which was enough to lead him to hypovolemic shock. Thereafter, right hemiparesis and aphasia were brought about. Two months later, he die of pneumonia.(ABSTRACT TRUNCATED AT 250 WORDS)

Functional prognosis of surgical treatment of craniosynostosis.

Twenty-three cases of craniosynostosis were studied with regard to age at operation, symptoms, operative methods, and long-term results. Long-term results of 20 cases that were followed up for more than 1 year after surgery showed that mental retardation remained in 8 cases; a slight improvement in mental function was observed in only 2 cases. Of the 8 patients with mental retardation, 7 were operated on more than 6 months after birth, 7 showed striking digital impression on skull X-ray films and 5 were suffering from oxycephaly. Of the 8 patients with mental retardation, 5 had suffered from perinatal asphyxia or had seizures during delivery and subsequent past history. From these observations, factors affecting prognosis are: (1) age at operation; (2) the degree and duration of increased intracranial pressure; (3) the extent and degree of suture closures; (4) perinatal asphyxia and seizure disorders.

Study of the posterior circulation in moyamoya disease. Clinical and neuroradiological evaluation.

Eighty-two cases of cerebrovascular moyamoya disease were studied by cerebral angiography and computerized tomography. Occlusive lesions were demonstrated not only in the anterior circulation but also in the posterior circulation, and they were associated with the development of an abnormal vascular network (moyamoya vessels). Although occlusive lesions do occur in the vertebrobasilar system, the vertebrobasilar system also acts as a source of collateral channels to the anterior circulation in this disease.

Moyamoya disease associated with bilateral occlusion of the vertebral artery.

An unusual case of moyamoya disease associated with bilateral occlusion of the vertebral artery is reported. This case shows several embryologic collateral channels, which are typical of moyamoya disease, around the brainstem, that is, a transdural anastomosis between the superior cerebellar artery and the posterior meningeal artery, a leptomeningeal anastomosis between the superior cerebellar artery and the posterior inferior cerebellar artery, and moyamoyalike collateral vessels via primitive lateral anastomotic channels of Padget . Bilateral encephalomyosynangiosis (temporal muscle graft) were performed. Postoperatively, no ischemic attacks have developed for 6 years.

[Study on the vertebro-basilar system in "moyamoya" disease].

Posterior circulation in 82 children of "moyamoya" disease are studied. Two aspects of "moyamoya" disease, (ie; occlusive lesion of cerebral vessels and the development of abnormal vascular network as collateral channel,) are also detected in the vertebrobasilar system. Among 82 cases, 49 cases showed the occlusion of posterior cerebral artery at their quadrigeminal segment. Twenty-three showed the more proximal occlusive lesions. Vertebral artery occlusion were found in 3 cases. As the occlusive lesion progresses, abnormal vascular network at the posterior portion of skull base developed. This network consists mainly of thalamogeniculate artery, posterior choroidal artery, and also of other thalamoperforators. Visual field defect as an ischemic symptom of occipital lobe was detected in 9 cases (11%). Superficial temporal artery-middle cerebral artery anastomosis and encephalo-myo-synangiosis (temporal muscle graft), which were not considered to be so effective to the ischemia of the posterior circulation, were shown to exert indirect redistribution effect upon the vertebrobasilar system. However, this effect is such an indirect one that these surgical treatments cannot prevent the occurrence of ischemic stroke in the vertebrobasilar system. For this purpose, omentum transplantation to the occipital lobe may be needed as a method of direct revascularization.

Spinal cord arteriovenous malformations associated with spinal aneurysms.

Two patients with spinal cord arteriovenous malformations (AVMs) had coexisting spinal aneurysms on their main feeding arteries. The angiographic disappearance of these coexisting aneurysms after artificial embolization of the spinal cord AVMs suggests that hemodynamic stress is the important factor in the development of spinal aneurysms. The incidence of hemorrhage increases when spinal cord AVM is associated with spinal aneurysm.