[Risk factor for cerebral hemorrhage for the patient with lacunar infarction: investigation of 5 cases associated with both of symptomatic hemorrhagic and ischemic strokes].

BACKGROUND AND PURPOSE: Microangiopathy is regarded as an important cause of intracerebral hematoma(ICH) and lacunar infarction. Dot-like low intensity spots on T2-weighted echo planar image(EPI) have been regarded as hemosiderin deposit associated with microangiopathy. However, clinical significance of dot-like hemosiderin spot(dotHS) is still debated. Therefore, we analyzed the number of dotHS on EPI of symptomatic lacunar infarction associated with ICH. METHODS: To investigate how the dotHS or risk factors contributed to hemorrhagic strokes for patients with lacunar infarction, the number of dotHS and various risk factors were made a comparison between 20 cases with symptomatic lacunar infarctions(lacunar group) and 5 cases with both symptomatic lacunar infarction and symptomatic ICH(complicated group). In addition to EPI, fluid attenuated inversion recovery image, and T1- and T2-weighted MR images were performed for differential diagnosis of dot HS. RESULTS: EPI demonstrated that asymptomatic ICH was significantly more frequent in complicated group (60%) than in lacunar group(10%), and dotHS were significantly more frequent in complicated group(100%) than in lacunar group(50%). The number of dotHS of complicated group was 14.6 +/- 4.3, which was significantly larger than that of lacunar group(4.1 +/- 9.2). No significant difference between two groups were founded in other risk factors including hypertension, diabetes mellitus, hyperlipidemia, and smoking. CONCLUSION: These results suggested that dotHS was one of the risk factors for ICH for patients with symptomatic lacunar infarction, and an increasing number of dotHS was one of the predictive factors of symptomatic and/or asymptomatic ICH.

Primitive neuroectodermal tumor involving the frontal skull base in an infant.

We report here a case of a primitive neuroectodermal tumor involving the frontal skull base in a 5-month-old male infant. He presented with rapidly progressing exophthalmos on the left side. Preoperative magnetic resonance imaging revealed a well-demarcated, enhanced mass of 6 cm in diameter in the intradural extra-axial frontal region. The tumor was totally removed by surgery. Pathological findings were consistent with a primitive neuroectodermal tumor (PNET). Intradural extra-axial PNET is very rare, and its prognosis is poor.

Gene expression profile in Alzheimer's brain screened by molecular indexing.

Gene expression in the Alzheimer brain and normal brain was compared by molecular indexing, an advanced version of differential display. Using this technique, each gene was represented by a 3'-end cDNA fragment generated by class IIS restriction enzymes. The fragments were divided into 384 groups, and each group was separated by denaturing polyacrylamide gel electrophoresis. Comparison of gel patterns revealed 70 genes exhibiting marked differences in gene expression between AD and normal brain. A similarity search revealed 22 genes already reported, including those considered to be related to the pathogenesis such as G protein, G protein-related, and mitochondrial components. Detailed analysis of one from those only matched to EST sequences revealed a novel protein with leucine-zipper and SH3-binding motifs. Its expression was suppressed in a subpopulation of cortical pyramidal neurons in the AD brain, suggesting a possible relation to the pathogenesis. Thus, genome-scale analysis of gene expression of neurodegeneration is a potentially powerful approach to listing genes related to the pathogenesis.

[A case of granulomatous angiitis of the central nervous system presented with subacute mental deterioration resembling diffuse white matter disease].

We reported a 60-year-old man with granulomatous angiitis of the central nervous system (GACNS) manifesting as subacute mental deterioration. His first symptoms were nausea and vomiting which brought him to a hospital, where no abnormality was found except for gastritis. One month later, he began to feel dizziness and brain tumor was suspected by a neurosurgeon with the MRI findings such as abnormal T2 signal and swelling in his brainstem. While he was followed up, he gradually presented mental change, disorientation and dysmnesia with the abnormal T2 signal spreading over the cerebral white matter bilaterally. Corticosteroid therapy was started based on the suspicion of a lymphoproliferative disease, and his symptoms and the abnormal MRI findings improved. Then he was referred to our department for further evaluation. Because we could not find any evidence of systemic diseases and he had been almost fully recovered, we discontinued the therapy. Soon after that, his mental deterioration as well as the abnormal T2 signal lesions on MRI relapsed. By open brain biopsy, the diagnosis of GACNS was established, and steroid pulse therapy was started. His symptoms and the abnormal T2 signal lesions improved gradually and the steroid was tapered to the maintenance dose without remission. Since the laboratory and imaging findings are not specific for the diagnosis of the angiitis confined to the central nervous system, brain biopsy is recommended for these disorders.

[Common carotid artery dissection propagated from acute aortic dissection: a case successfully treated by PTA].

We reported a rare case, which was successfully treated by PTA, of right common carotid artery dissection propagated from acute aortic dissection (AAD) type A. A 45-year-old male with a past history of hypertension and an artificial graft replacement of the abdominal aorta due to AAD type B, 7 years ago, was brought into our hospital by ambulance 30 minutes after an attack of fainting and left hemiparesis. On admission, the patient complained not of chest pain or left hemiparesis, but nausea. At that time his consciousness level was JCS 1. During examinations, he had the same attack twice and his consciousness level deteriorated to JCS 2. Brain MRI showed no abnormality, but cervical MRA did not visualize the right carotid artery and thoracic CT depicted acute aortic dissection including branches of the aorta. Emergent angiography disclosed that the dissecting 99% stenosis of the right common carotid artery had developed from AAD type A with poor collateral blood flow. PTA was carried out 8 times and reduced the residual stenosis to about 50% with shortened circulation time. The patient's consciousness disturbance improved. After the replacement of the whole aortic arch in an artificial graft, the residual stenosis disappeared. The patient recovered without neurological deficit but right frontal silent embolic infarction caused by the artificial graft replacement was detected. AAD is a catastrophic illness and sometimes accompanied by devastating ischemic cerebral disease (ICD) because of propagation of dissecting to extracranial vessels. This is the first report that shows the efficacy of PTA for treatment of ICD associated with AAD.

Two novel genes, human neugrin and mouse m-neugrin, are upregulated with neuronal differentiation in neuroblastoma cells.

We herein report two new genes, human neugrin and mouse homologue m-neugrin, found by screening the cDNA library for the human spinal cord. The neugrin mRNA encodes 219 amino acids and its deduced amino acid sequence contains an NLS-like domain. No previously known motif is found in it. m-neugrin mRNA encodes 233 amino acids. Neugrin and m-Neugrin are 70% homologous in amino acid sequence. Northern analysis revealed that neugrin was strongly expressed in the heart, brain, and skeletal muscle, and m-neugrin in the liver, kidney, and brain. A transfection study indicated that these proteins are localized in the nucleus. Although the expression of neugrin was found to be ubiquitous in the nervous system, in situ hybridization showed that both neugrin and m-neugrin were expressed mainly in the neurons rather than the glial cells. Their expression was highly upregulated with the neurite outgrowth associated with neuronal differentiation in neuroblastoma cell lines. These results indicate that neugrin and m-neugrin are mainly expressed in neurons in the nervous system, and play an important role in the process of neuronal differentiation.

[Acute surgical and endovascular therapy for stroke: especially patients with brain infarction].

From January 1994 to December 1997, 845 patients with stroke were admitted to Hakodate Municipal Hospital. They consisted of 514 patients with brain infarction, 206 with brain hemorrhage, 121 with subarachnoid hemorrhage and 4 with intracranial hemorrhage from arteriovenous malformation. The clinical categories of brain infarction were as follows; atherothrombotic recognized in 158 patients, cardioembolic in 114, lacunar in 217 and other categories in 25. With regard to the cures of brain infarction in the acute phase, direct percutaneous transluminal angioplasty (direct PTA) was carried out on three patients with atherothrombotic infarction, immediate PTA on two, superselective fibrinolytic therapy on two, and STA-MCA anastomosis on three. In all, ten atherothrombotic patients (6.3%) were treated by acute surgical or endovascular therapy. On the other hand, superselective fibrinolytic therapy was carried out on 35 patients (30.7%) with cardioembolic infarction. There were no patients in the lacunar infarction group who were given acute surgical treatment. Neurological improvement after 24 hours was recognized in 4 patients (40%) of 10 with atherothrombotic infarction, and in 9 patients (25.7%) of 35 with cardioembolic infarction. However, symptomatic intracerebral hematoma was recognized in 4 patients (11.4%) with cardioembolic infarction. Indication for acute surgical or endovascular treatment for brain infarction was very limited because of the time factor from the onset to admission. It is suggested that neurosurgeons might enlighten citizens about the necessity for acute surgical or endovascular therapy for stroke.

[A patient with choreoathetosis of the left upper extremity due to acute cerebral infarction].

A 71-year-old woman was admitted to our hospital because of involuntary movement of the left upper extremity. MR image of the brain 15 days after the onset revealed the low intensity in right posterior limb of internal capsule. The lesion was surrounded by thalamus, subthalamic nucleus, and globus pallidus with enhancement by Gd-DTPA. Surface EMG revealed irregular grouped discharge in short duration and grouped discharge in long duration in the left upper extremity. Those features are compatible with one of choreoathetosis. Choreoathetosis due to cerebral infarction in acute phase is rare. We discussed pathophysiology of this involuntary movement due to lacunar infarction of posterior limb of internal capsule in acute phase.

[Differential diagnosis between aneurysm and infundibular dilatation in the IC-PC region with 3D-CTA].

In cases of asymptomatic internal carotid-posterior communicating artery (IC-PC) protrusions, it is sometimes difficult to differentiate infundibular dilatation (ID) from aneurysm by digital subtraction angiography. We applied three-dimensional CT angiography (3D-CTA) in 32 cases of these IC-PC protrusions. SOMATOM PLUS 4 was used under such conditions as to provide images with high spatial resolution. The shaded surface display (SSD) method was adopted to reconstruct the 3D images because of its advantage in separating overlapped vasculature. We also made reference to source images and maximum intensity projection (MIP) to make sure of our diagnoses. In all cases including 4 aneurysms and 28 IDs, we were able to distinguish between ID and aneurysm. The accuracy of 3D-CTA was confirmed by 9 surgical cases. Our technique was as follows: 1) To inject a high dose of diluted contrast medium rapidly to smaller arteries for opacification of contrast medium. 2) To exclude neighboring useless structures except for the very close structures such as posterior clinoid process from the target image focusing on the IC-PC region. 3) To observe the reconstructed image of MIP and SSD from various angles. The contralateral and craniocaudal view were valuable. 4) To change the threshold level gradually and observe the configurational changes of the apex of protrusion. Poorly developed PcomA was mostly delineated at the optimum threshold level. Otherwise, the apex of protrusion remained spherical in an aneurysm and became pyramidal in shape in an ID when the threshold level was gradually decreased. In conclusion, 3D-CTA was a useful modality for IC-PC protrusions to distinguish between ID and aneurysm.

[Clinical training and home therapy in our clinic].

Since 1986, we have practiced clinical training. In this training, the physician trainee acts as the head of our clinic, managing programs including the medical teams for outpatient clinics and home therapy. Our home therapy started in 1975 and successive resident trainees have held conferences, cooperation between hospitals and clinics between clinics, and also held home patient and family parties. In our clinical training, therefore, the resident can effectively gain generalized clinical abilities.

[Prevention of MRSA spread in the neurological field: intranasal application of mupirocin calcium ointment].

From September 1997 to March 1998, forty patients with cerebral disorders were investigated. They were divided into two groups: one treated and the other untreated. Mupirocin calcium ointment (MCO) was applied three times a day for three days into the nasal cavities of the patients in the treated group. In order to check the growth of MRSA (methicillin-resistant Staphylococcus aureus), bacterial isolation culture from the nasal cavity was carried out on admission, one week after admission and one month after admission. MRSA was nor detected in isolation culture of any of the cases on admission. One week later MRSA was detected in isolation culture of one case of the 20 MCO treated patients and in three of the 20 untreated patients. There was no significant difference between treated and untreated groups. In isolation culture after one month, MRSA was recognized in four cases of 16 in the MCO treated group (three patients were discharged and one expired). On the other hand, it was recognized in eight cases of thirteen in the untreated group (seven cases were discharged). MRSA infection of the nasal cavity decreased significantly due to MCO treatment (p < 0.05). It is suggested that the nasal carriage of MRSA was prevented by intranasal application of MCO on admission.

[Surgical indication and problems of patients aged over 70 years with unruptured aneurysms].

Surgical indication and problems of patients aged over 70 years with unruptured aneurysms were investigated. Clinical features of eighteen cases of unruptured cerebral aneurysms were analyzed. The location of the cerebral aneurysms were in the internal carotid artery in five cases, in the middle cerebral artery in ten cases, in the anterior cerebral artery in 2 cases and in the basilar artery in 1 case. The size of the aneurysms was less than 10 mm in diameter in 17 cases and giant in one case. Treatment of these aneurysms was classified into two groups as follows; the conservative treatment group (four cases) and the surgical treatment group (14 cases). The therapeutic results of the conservative group were good recovery in 2 cases, and death in 2 cases. On the other hand, the results of surgical group were good recovery in 12 cases and fair in 2 cases. Operative complications were recognized in two cases. Consciousness disturbance and left hemiparesis was recognized in one case. Right hemiparesis was recognized in the another case. Postoperative MR imagings or CT scan presented small cerebral infarctions in the corona radiata in both cases. The cause of infarction was thought to be the occlusion of lenticulostriate arteries. From these data, in patients aged over 70 years with unruptured cerebral aneurysms, surgery should be considered not only from the aspect of aneurysmal size and its site, but also from the aspect of cerebral blood flow of the patient.

[Visual acuity outcome and recurrence in large or huge pituitary adenomas operated with transcranial approach: comparison between frontotemporal and interhemispheric approaches].

Pituitary macroadenomas which required transcranial removal were reviewed concerning visual acuity outcome as well as recurrence. We encountered 173 pituitary adenomas of which 27 (15.6%) were removed transcranially during the past 13 years. Eight cases were excluded due to inadequate information and improper utilization. Thus, a total of nineteen cases were reviewed in which the frontotemporal (FT) approach was utilized for seven cases and the interhemispheric (IH) approach for 12 cases. The mean size and volume of the tumors in the FT group were 3.0 x 3.7 x 3.1 cm and 18.2 cm3. The main reason for utilizing this approach was the fact that the tumors extended laterally involving the unilateral cavernous sinus or that unilateral preoperative visual acuity was obstructed. The visual acuity outcome was as follows: In six cases showing useful visual acuity on both sides before surgery, no apparent aggravation on either side was found in four cases, while in two cases there was complete obstruction on the operative side. The remaining case showed aggravation on both sides, though the approach side was decided upon because of the obstructed vision on that side. The mean size and volume of the tumors in the IH group were 4.6 x 4.8 x 4.9 cm and 71.1 cm3. This approach was used due to the extreme suprasellar extension because of the large size of the tumors. Although the tumors were relatively large and the visual acuity was assessed as fair prior to surgery, visual acuity showed no significant deterioration after the operation and was found to be satisfactory in 11 out of 12 cases. The complications in the FT group were oculomotor palsy in 3 cases, hemorrhage in one case, and frontal infarction in one case. On the other hand, three cases suffered hemorrhage in the tumor cavity of the IH group, though none needed surgical evacuation. Most of the cases in the IH group showed pituitary hypofunction following the surgical removal of the tumors as compared to the cases in the FT group. Recurrence had occurred, in some cases, several years after the operation. Furthermore, some of the tumors are still growing larger following only partial or subtotal removal. The prime aim of the treatment for huge pituitary adenomas which require the transcranial removal is to retain as much visual acuity as possible. In conclusion, the IH approach has been shown to be preferable in this situation. The FT approach was found to be more dangerous in terms of visual outcome than had been expected, even if the tumors were not particularly large.

Treatment of acute superior sagittal sinus thrombosis by t-PA infusion via venography--direct thrombolytic therapy in the acute phase.

BACKGROUND: Dural sinus thrombosis is a relatively rare syndrome, often with a very poor prognosis. Systemic anticoagulant therapy has produced poor results; therefore rapid recanalization of the affected vessels is essential. The recent advancements in angiographic technique and catheter technology enable us to perform direct selective venography. CASE REPORT: We observed a case of acute superior sagittal sinus thrombosis in a pregnant woman. The patient's consciousness level and motor function gradually deteriorated. Direct thrombolysis was performed via venography. RESULTS: The patient was treated successfully by thrombolysis with infusion of t-PA via selective venography within 2 days of rapid clinical deterioration and sustained a dramatic improvement of her neurological deficits. CONCLUSIONS: Direct thrombolysis via selective venography is considered a safe and useful treatment for dural sinus thrombosis in the acute phase.

Transcondylar approach for dural arteriovenous fistulas of the cervicomedullary junction.

BACKGROUND: Spinal dural arteriovenous fistulas are abnormal arteriovenous connections on the surface of the dura. The site of the fistula is most commonly in the thoracic and lumbosacral regions and they are rarely located in the cervical region. CASES: The patients had two asymptomatic dural arteriovenous fistulas of the cervicomedullary junction fed by the left posterior meningeal artery and draining to the dilated coronal venous plexus and the radiculomedullary vein. RESULTS: The lesions were successfully treated by surgical interruption of the intrathecal vein with coagulation via a suboccipital transcondylar approach and a condylar fossa approach. Both patients left the hospital without significant deficits. CONCLUSIONS: We recommend that dural arteriovenous fistulas in the cervical region be surgically treated.

Successful Treatment of Superior Sagittal Sinus Thrombosis. Direct Thrombolysis with Local Infusion of t-PA via Venography in the Acute Phase.

SUMMARY: A 22-year-old pregnant women showed a rapid deterioration in her clinical condition. Superior sagittal sinus thrombosis was diagnosed, and direct transvenous infusion of t-PA into the superior sagittal sinus was performed. This treatment resulted in recanalization of the occluded superior sagittal sinus and in a dramatic improvement of neurological deficits.

[A case of endodermal cyst in front of the pontomedullary junction].

We reported a rare case of an endodermal cyst on the posterior fossa. The patient was a 51-year-old woman with tinnitus, ear blockage and a mild swallowing disturbance. MR images revealed a cystic mass 5 cm in diameter in front of the pontomedullary junction. Gadolinium-enhanced images showed some spotty lesions in the cystic mass. The brain stem was compressed strongly by the mass. The left vertebral artery was involved in the mass. Total removal of the tumor was performed via the transcondylar approach. During the operation the lower cranial nerves, the bilateral vertebral artery and the vertebral union were recognized. The cystic mass consisted of a yellow colored thin wall and a watery fluid which contained some small hard lesions. Histopathological examination of the cyst wall revealed a single layer of ciliated columnar epithelium, and that of the small lesions showed histiocytes and granulation. Immunohistochemically, the cyst wall was stained by an epithelial membrane antigen and a carcinoembryonic antigen. From these histopathological findings, the final diagnosis of the cystic lesion was an endodermal cyst. Twenty such cases have been reported to date since Afshar reported a case of an endodermal cyst on the posterior fossa in 1981. There are many types of cystic lesions in the intracranial region, and immunohistochemical studies are necessary for diagnosis of these intracranial cysts.

[Seizures as a late complication of bromocriptine therapy in patients with prolactin-producing macroadenomas: correlation between lateral extension of the adenoma and seizure onset].

Seizures have been reported as a late complication of medically treated prolactin-producing macroadenomas with lateral extension. Nine prolactin-producing macroadenomas with lateral extension to the cavernous sinus were treated with bromocriptine alone. In all cases, rapid decrease of serum prolactin level was recognized and in eight cases, evidence of tumor shrinkage on CT or MRI was demonstrated. During medical treatment, symptomatic seizures occurred in three cases. MRI showed small residual tumors on the medial surface of the temporal lobe in all these patients. Additionally, a hypointense signal on both T1- and T2-weighted images was recognized around the residual tumor. It was consistent with hemosiderin, a result of intratumoral hemorrhage caused by bromocriptine. On the contrary, the remaining six patients without seizures during treatment did not show abnormal hypointense signals on the medial surface of the temporal lobe. Seizures occur with a high incidence as a late complication of medical treatment of prolactin-producing macroadenomas with intradural supracavernous extension. The patients with seizure show hemosiderin deposit, which may be the trigger of the seizure, within the medial surface of the temporal lobe after bromocriptine therapy.

[Prognosis after total removal of craniopharyngiomas via the frontobasal interhemispheric approach].

Prognosis after total removal of craniopharyngiomas via the frontobasal interhemispheric approach is reviewed. Seventeen patients with craniopharyngiomas were operated on in Sapporo Medical University Hospital between January, 1985 and December, 1993. In eleven patients, lamina terminalis was incised and in the last six patients, it was left intact. Tumors were completely resected in all patients. After removal of the tumor, hypothalmic--pituitary functions, visual functions and psychometric functions were examined. Two of the 17 cases showed hypernaturemia and fourteen (82%) had permanent DI. Fourteen patients are receiving DDAVP and all are receiving endocrine replacement. Of fourteen patients who had disturbance of their visual acuity, nine (64%) improved. Five of six patients (83%) who presented visual field defect showed improvement in their deficits. Thirteen patients had a psychometric assessment at the time of follow-up examination. Full-scale intelligence quotient scores were distributed as follows: three above 120, five between 90 and 109, three between 70 and 79 and two below 69. Four (32%) had some impairment of memory. Concerning the QOL after total removal of craniopharyngiomas via the frontobasal interhemispheric approach, thirteen patients (76%) are leading normal lives, and three (18%) are leading nearly normal lives but require some help to overcome mild deficits. One (6%) has suffered a significant handicap.

[Clinicopathological findings in symptomatic Rathke's cleft cyst: correlation between enhancement effects on MRI and histopathology of the cyst wall].

We have studied MR images and the histopathology of eight patients with symptomatic Rathke's cleft cysts. Six cases showed visual disturbance and two showed galactorrhea. In five, the cyst fluid had low signal intensity on T1-weighted images and high intensity on T2-weighted images; in 2, the cyst fluid had high intensity on both T1 and T2-weighted images; in 1, the cyst fluid had high intensity on T1-weighted images and low intensity on T2-weighted images. Enhancement of the cyst wall by Gd-DTPA was able to be distinguished in six cases: two patients showed no enhancement, two showed thin enhancement and the remaining two, thick enhancement. Fluid aspiration and total resection of the cyst wall was performed in all patients (three cases by the transcranial approach and five by the transsphenoidal approach). Normal pituitary glands were found in all cases during the operations. Histopathologically, ciliated epithelium with goblet cells was recognized in three cases. Non-ciliated epithelium was recognized in the other five. Stratified squamous component was recognized in one case and secondary inflammation, in another. Normal pituitary tissue was recognized in five cases. Immunohistochemically, ciliated and non-ciliated epithelium was successfully stained for detecting antibody against epithelial membrane antigen and/or carcinoembryonic antigen. Two cases with no enhancement of the cyst wall by Gd-DTPA showed only ciliated epithelium. Two cases with thin enhancement of the cyst wall had single layer epithelium with normal pituitary tissue. Two cases with thick enhancement of the cyst wall showed single layer epithelium with its stratified squamous component or with secondary inflammation. A close relationship was suggested between the enhancement effect on MRI and histopathology of the cyst wall.