[A case of a multiple sclerosis with severe perifocal edema].

We present a case of tumoral multiple sclerosis in a 66-year-old male and emphasize two clinical features: (1) Multiple well-demarcated massive enhanced lesions except for the central area, which was accompanied with severe perifocal edema, occurred within the cerebral hemispheres and should be strongly considered in the diagnosis of mass lesions. The patient underwent partial removal of the mass lesion. The initial histopathological diagnosis of the surgical specimen was astrocytoma grade II. (2) Multiple lesions disappeared completely for one and half months after starting medication using steroid. The reexamination of the surgical specimen was compatible with that of multiple sclerosis. Again, half a month after remission, multiple enhanced lesions without perifocal edema developed in the cerebral and cerebellar hemisphere. The difficulty of the initial diagnosis of multiple sclerosis with severe perifocal edema was discussed, based on our experience.

Basic science and epilepsy: experimental epilepsy surgery.

Epilepsy surgery, as is employed for the management of intractable seizures, was performed in animals harboring a seizure focus induced by a local application of kainic acid (KA). Amygdalo-hippocampectomy failed to stop spontaneous seizures in the contralateral hippocampus. Callosotomy inhibited seizure propagation to the contralateral sensori-motor cortex. However, epileptic activity ipsilateral to the focus, including subcortical structures, persisted even after the callosotomy. Multiple subpial transection (MST) around the epileptic cortical focus suppressed the seizure activity of the cortex. However, seizure propagations in subcortical structures remained, even after MST. Nefiracetam (a new nootropic agent) was tested in these models, and its promising effect on the intractable extratemporal epilepsy is reported.

[A case of the syndrome of the sinking skin flap: case report].

A case of "the syndrome of the sinking skin flap" was presented. A 40-year-old-man had suffered from severe SAH 9 months before. An aneurysm of the anterior communicating artery was successfully clipped and the bone flap was removed for the purpose of the external decompression. Cranioplasty and V-P shunt were performed 1 month after SAH, but both were removed because of postoperative wound infection, viz. epidural and subdural abscess 4 months after SAH. Following this, L-P shunt was performed, and the patient was discharged with mild dementia. A concave deformity of the skin flap developed about 4 months after the L-P shunt. Neurological examination showed progressive left hemiparesis and akinetic mutism. A low CSF pressure was demonstrated, but RI cisternography revealed normal CSF circulation. Intrathecal infusion of the artificial CSF was carried out via lumbar puncture and concavity of the skin flap gradually improved. This procedure resulted in improvement of the neurological deficits. Cranioplasty with artificial bone was performed under continuous intrathecal infusion of the artificial CSF. Postoperative course was satisfactory and neurological examination revealed only mild dementia. The pathological mechanism in our case was probably due to the compression of the brain by the atmospheric pressure following the external decompression. Moreover, L-P shunt exaggerated this pathology by the overdrainage of CSF.

Vascular abnormalities in surgical specimens obtained from the resected focus of intractable epilepsy.

The histopathological features, particularly hypervascularity, were examined in specimens resected from 21 patients, 15 with intractable epilepsy accompanying cortical dysplasia or dysembryoplastic neuroepithelial tumor (DNT), and 6 with benign brain tumors, such as ganglioglioma and low-grade glioma. Hypervascularity was found in resected specimens from 15 of the 21 patients (71.4%) and in 10 of the 12 patients (83.3%) who had double pathology. Counting of numbers of vessels by CD31 immunohistochemistry revealed that hypervascularity was prominent, especially in cases of vascular malformation or cortical dysplasia. However, almost all cases were negative for vascular endothelial growth factor (VEGF) staining, except for some cases of benign brain tumors. Moreover, all cases showed low or no proliferative potential in MIB-1 immunohistochemistry. These results suggest that the etiology of hypervascularity in the dysplastic lesions is one of a variety of cerebral malformations, as is the case with abnormal maturation and differentiation in neuroglial elements.

[A case of a traumatic anterior cerebral artery aneurysm following the penetration of the skull base by an iron rod].

A 61-year-old male fell from a position 1 m high when building a house. An iron rod, which protruded upward from a solid base in cement, penetrated this patient's neck 15 cm to the head and was successfully extracted by himself. On admission, he complained of headache and vomiting. General examination disclosed nasal bleeding, intraoral bleeding, and L figured skin laceration in the left side of his neck at the level of the thyroid cartilage. Mild disorientation (JCS2) was noted. Otolaryngological examination disclosed hyperemia on the left side of the vocal cord as well as at the dome of the superior pharynx. Plain skull film disclosed pneumocephalus and that a piece of bone fragment of the planum sphenoidale had penetrated the brain. CT demonstrated air in the subarachnoid space, ventricular hemorrhage, intracerebral hematoma in the right frontal lobe, and subarachnoid hemorrhage in the anterior interhemispheric fissure. CAG detected neither cerebral vascular abnormalities nor cerebral aneurysm. While staying in our department, he developed mild fever and CSF rhinorrhea. The diagnosis of bacterial meningitis was made from the CSF finding and was well controlled with conservative therapy. CSF rhinorrhea stopped spontaneously with conservative treatment. Sagittal MRI continuously demonstrated contusional hematoma in the base of the right frontal lobe just above the fractured planum sphenoidale and genu of the corpus callosum following the course of the intracranially invading iron rod. The right CAG on Day 10 demonstrated vasospasm on the A1 and a 1 cm sized saccular cerebral aneurysm at the proximal right fronto-polar artery. CAG on Day 17 again showed the persistent presence of the aneurysm. For the purpose of preventing delayed rupture of the aneurysm, radical surgical treatment was planned. Microsurgical dissection disclosed that the aneurysm was located just behind the elevated fracture of the planum sphenoidale. Severe arachnoid adhesion was noted around the aneurysm. The aneurysm was successfully clipped with preservation of the parent artery without inducing new neurological deficits. From the general, otolaryngological, neuroradiological, and operative findings, this aneurysm was diagnosed as a traumatic cerebral artery aneurysm following the penetration of the skull base by the iron rod. The CAG performed at 8 months postoperatively demonstrated the patency of the parent artery and that there was no recurrence of the aneurysm. An unusual case of a traumatic cerebral artery aneurysm following the penetration of the skull base by an iron rod was thus reported.

Image-guided epilepsy surgery.

Interactive image-guided techniques used in conjunction with three-dimensional images allow accurate planning and performance of a variety of neurosurgical procedures. The frameless stereotactic Viewing Wand System was used to provide real-time correlation of the operating field and computerized images in over 22 neurosurgical operations carried out for intractable epilepsy. The overall results of the surgery demonstrated favorable results, with class 1 + class 2 outcomes in 86.4% of the present series. Our experience shows that the Viewing Wand System is most helpful as an adjunctive navigational device in the microsurgical treatment of epilepsy.

Sequential change of capillary permeability in the rat brain after surgical removal of an experimental brain tumor.

Experimental brain tumors were excised from rats for sequential observation of changes in local capillary permeability during the postsurgical period. Experimental brain tumor-bearing rats were prepared by stereotaxic transplantation of cultured tumor cells and the resultant tumor was delineated by administration of a dye. Following excision of the stained tumor by craniotomy, sequential changes in local capillary permeability were quantitatively followed-up by autoradiography, using 14C-amino-isobutyric acid as a tracer. Capillary permeability was enhanced following surgery, reaching a maximum both in the extent and degree on the third day. After undergoing a gradual reduction, it showed a marked increase for the second time in a very small area on the 10th postoperative day. A recurrence of the tumor was responsible for this late but marked increase. For a control group, the caudate nucleus was excised from normal rats, followed by observation of the sequential changes in the local capillary permeability. Due to surgical procedure, capillary permeability reached a maximum both in the extent and degree on the 5th postoperative day (slightly later than in the tumor group). This change in capillary permeability was less pronounced than in the tumor group. The difference in the conditions of surgery--tumor excision and partial excision of a normal brain tissue--appeared to explain this difference. The results of this study indicated that it is more desirable to give water-soluble antineoplastic agents early during the postoperative period for chemotherapy of a malignant brain tumor after surgery.

Delivery of a novel nitrosourea, MCNU, to the brain tissue in glioma-bearing rats. Intracarotid versus intravenous infusion.

We observed the tissue delivery of a novel water-soluble nitrosourea, 1-(2-chloroethyl)-3-(methyl-alpha-D-glucopyranos-6-yl)-1-nitros our ea (MCNU) in rats bearing experimental brain tumors by conducting autoradiography on all. Prior to this study, the development of a streaming phenomenon was ascertained (and thus finding the optimum velocity for intra-arterial infusion) by 14C-iodoantipyrine (IAP) autoradiography. Furthermore, a single pass extraction value of MCNU was measured. At an arterial infusion rate of 0.2 ml/min., the streaming phenomenon was recognized but the tracer was fairly evenly distributed at a rate of 1.0 ml/min. On the other hand, the single pass extraction value for MCNU was 0.18 +/- 0.036 (mean +/- S.D., n = 3, under pentobarbital anesthesia). It was suggested that MCNU is very unlikely to be transported into the normal rat brain. We conducted 14C-MCNU autoradiography to observe tissue distribution of MCNU following its intra-arterial and intravenous infusions in a brain tumor model using rats. The normal side (the side where no infusions were given) and the cerebral cortex at the side affected by the tumor (the side where the infusion was given) showed hardly any uptake of 14C-MCNU in both the intra-arterial and intravenous infusion groups. The tumorous section was divided into the periphery and the center to measure tissue concentration of the tracer in each section. Compared against the cortical section, the periphery and the center showed significant increases in the concentration (approximately 11 to 15 times and 3 to 7 times, respectively, the figure for the cortical region) for both the intra-arterial and intravenous groups.(ABSTRACT TRUNCATED AT 250 WORDS)

[Spontaneous disappearance of aneurysm after total removal of accompanying intracranial arteriovenous malformation. Case report].

A 55-year-old male was hospitalized with severe headache. On admission, neurological examination revealed no abnormal findings. Plain computed tomography (CT) showed a slightly high-density area in the medial surface of the right parietal lobe. A marked enhancement in the same region was noted in enhanced CT. Cerebral angiography showed an arteriovenous malformation (AVM) in the medial surface of the right parietal lobe and two aneurysms on the right pericallosal artery which fed the AVM. In addition, a saccular aneurysm was noted at the anterior communicating artery. It was not possible to treat the AVM, two aneurysms nearby the AVM, and the unruptured anterior communicating artery aneurysm simultaneously with a single craniotomy. It was therefore decided to perform surgery for the AVM and two aneurysms nearby the AVM prior to clipping of the anterior communicating artery aneurysm. Total excision of the AVM and two aneurysms nearby the AVM was performed. Cerebral angiography performed 18 days after surgery revealed no AVM and also reduction in size was noted of the anterior communicating artery aneurysm. Three months later, repeated cerebral angiography showed disappearance of the aneurysm. This was further confirmed 15 months after surgery by angiography. From the literature, 117 cases of coexistence of AVM and aneurysms of the brain were collected and classified into three types according to their anatomical and hemodynamic correlation. It is suggested that hemodynamic stress, due to increased blood flow caused by the AVM, played a major role in the development of the aneurysm.(ABSTRACT TRUNCATED AT 250 WORDS)

Diffuse low-density areas in white matter on CT scans after intracarotid ACNU infusion--report of three cases.

Since 1984, we have treated 11 malignant glioma patients with intracarotid infusion of ACNU [1-(4-amino-2-methyl-5-pyrimidinyl)-methyl-3-(2-chloroethyl)- 3-nitrosourea hydrochloride] in addition to surgical removal and irradiation. We experienced three patients, who showed clinical manifestation of leukoencephalopathy and computed tomographic (CT) findings of diffuse low-density areas in the white matter on the side of ACNU infusion. Two of the three patients showed an additional CT finding of ring enhancement in the temporo-occipital region. The histological diagnosis of the first case was radiation necrosis, while that of the others was recurrent tumor with coagulation necrosis in the surrounding brain. Our experience suggests that intracarotid ACNU infusion increases the hazard of radiation necrosis, and the optimum dose and effective mode of administration should be evaluated.

[Effect of trihexyphenidyl-HCL on posttraumatic prolonged disturbance of consciousness: report of two cases].

Cases involving two patients who presented post-traumatic prolonged disturbance of consciousness (PTPDC), namely akinetic mutism, and recovered from it after treatment with trihexyphenidyl were reported. Case 1: A seventy-one-year-old farmer. Five months after head injury, when he was first admitted to us, he was stable with signs of oligokinesia, katatonic posture, speechlessness, rigid muscle tones and positive cog-wheel phenomenon. One week after administration of the drug, his speech and voluntary movement improved remarkably. Two months after the treatment, he was able to walk, and was discharged from the hospital. Case 2: A forty-six-year-old man sustained major head trauma. In the acute stage, he was comatose with decerebrate posture. On the 15th hospital day, he showed a state of akinetic mutism with normal sleep - wakefulness cycle. Evacuation of the collected subdural fluid was done one month after the injury, which resulted in no change in his clinical state. Five months after the injury, trihexyphenidyl treatment was begun. A few days after the treatment, his motor activity and his facial expression obviously improved. One week after, he mimicked the word 'o-ha-yo (good morning)' after the physician's greeting. CT scan and magnetic resonance imaging in the chronic state of these patients showed bifrontal cerebral white matter lesions, which indicated old cerebral contusion. No brain stem lesions were detected with these examinations. Our two cases clearly did not belong to the category of post-traumatic parkinsonism because of their clinical courses, and their features shown in radiological examinations. However the anti-parkinsonian drug, trihexyphenidyl was effective.(ABSTRACT TRUNCATED AT 250 WORDS)

[Sacral perineural cyst--report of a case].

The presence of cysts within the sacral spinal canal, so-called sacral cysts, is described in literature. These include 'sacral perineural cyst', 'sacral extradural cyst', 'occult intrasacral meningocele' and 'anterior sacral meningocele'. Sacral perineural cyst in these cystic disorders was first described as an incidental autopsy finding by Tarlov in 1938. Since then, several reports have been made describing the sign and symptom, neurological findings, roentgenographic diagnosis and cause and origin of the sacral perineural cysts, although many problems are not yet solved satisfactorily. This cyst occurs on the extradural components of sacral or coccygeal nerve roots. Although most are asymptomatic, these occasionally cause low back pain, sciatic and sacrococcygeal pain, sensory and motor disturbance in the lower extremities, and urinary dysfunction, which symptoms are similar to those brought on by lumbar disc herniation. In 1948, Tarlov reported a case of sciatic pain due to a perineural cyst, the removal of which relieved the symptoms. Symptoms occur because adjacent nerve roots are impinged upon by the thin-walled, fluid-filled cysts, which are formed in a space between the endoneurium and the perineurium. Microscopically, the cyst walls consist of peripheral nerve fibers or ganglionic cells covered with meningeal epithelium. Communication of the cyst with subarachnoid cerebrospinal fluid may be poor, but myelogram and CT myelogram demonstrate the cysts filling with contrast media. With the advent of magnetic resonance imaging (MRI), imaging of the sacral perineural cysts has improved. Recently we had the opportunity to evaluate a patient in whom perineural cysts had caused considerable erosion of the sacrum.(ABSTRACT TRUNCATED AT 250 WORDS)

[An autopsy case of malignant schwannoma in the brachial plexus extending into the spinal canal].

A 40-year-old woman had pain and numbness in the left upper extremity, on Dec. 1982. These symptoms progressed gradually. She was admitted to our department on Jan. 26, 1984 because of neck pains and progressive paraplegia. Neurological examination revealed monoplegia of the left arm with absent DTRs, spastic paraplegia, sensory disturbances below Th 11 level and severe sphincter disturbance. A metrizamide myelogram showed intradural extramedullary mass at C 4-7. Laminectomy was immediately performed from C 3 to C 7. A soft and brown tumor was attached at the ventral aspect of the cord at C 3 and C 7 and it was firmly attached at the root sleeve of the C 5 level. The histological diagnosis of the tumor was malignant schwannoma. One month later, she was discharged on foot. However, she was re-admitted to our department because of intractable pains in left neck and arm in Aug. '84. The second operation was done on Sept. 15. A soft and brown mass was found intra- and extradurally and it invaded into paravertebral muscules. On Sep. 30 '84 she died from respiratory failure. Postmortem examination revealed that intra-extradural tumor was found at C 4-Th 3, and the left brachial plexus was swollen. In the brachial plexus, the nerve fiber was replaced with tumor in the perineurium. The tumor invaded to the vertebral body and the paravertebral muscles. There were numerous metastasis of the tumor in both lungs. The tumor was formed by variable and scanty cytoplasm with many mitotic figures.(ABSTRACT TRUNCATED AT 250 WORDS)