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Acute generalized exanthematous pustulosis (AGEP) is a diffuse pustular disorder that usually begins in intertriginous folds with widespread erythema. The causes in the majority of the cases are drugs. T cells and interleukin (IL)-8 play roles in the development of AGEP, but the mechanism remains to be elucidated. We investigated the involvement of Th17 cells and their cytokine IL-22 in the pathogenesis.Three patients with AGEP were enrolled in this study. The percentages of IL-17(+) Th17 cells, interferon γ(+) T cells and IL-4(+) T cells were measured in the patients' peripheral blood lymphocytes by intracellular cytokine staining and flow cytometry. The concentration of IL-22 in the sera was measured by enzyme-linked immunosorbent assay.The percentages of Th17 cells were markedly higher in all three patients than healthy control individuals. The frequencies of interferon γ(+) T cells were slightly high in the patients compared with the control, and there was no definite tendency in IL-4(+) T-cell frequencies. The concentration of IL-22 was remarkably high in all patients when compared with normal subjects with levels under detection.Th17 cells and their produced cytokine IL-22 were elevated in the peripheral blood of patients with AGEP. As IL-17 and IL-22 cooperatively stimulate keratinocytes to produce IL-8, IL-8 may contribute to the accumulation of neutrophils in the lesional epidermis of AGEP.

We showed that normovolaemic induced hypertension therapy was effective in reducing ischaemic symptoms attributed to cerebral vasospasm in 41 patients after subarachnoid haemorrhage. By inducing hypertension to 25% to 50% above normal systolic arterial blood pressure, we observed that in 17 of 24 cases (71%) neurological deficits improved. In four cases of haemorrhagic infarction, the blood pressure rose to over 50% of systolic arterial pressure, and a low density area was confirmed on computerized tomography (CT) scan prior to vasospasm. Induced hypertension was therefore not considered when a low density area was revealed on CT scan. Restriction of fluid input is usually a factor in producing hypovolaemia after a neurosurgical operation. Intravascular volume expansion has been reported effective in reversing ischaemic deficits. However, according to Poiseuille's equation, increasing blood volume to a state of hypervolaemia can not enhance flow. The cerebral blood flow (CBF) was raised by increasing perfusion pressure, reducing viscosity, or increasing blood vessel diameter. Intravascular volume expansion elevates not only systemic arterial pressure, but also pulmonary artery wedge pressure over 18 mmHg and cardiac index over 2.2. Since pulmonary oedema and congestive heart failure may develop, one should monitor haemodynamic parameters with the Swan-Ganz catheter as a preventive measure. We emphasize that normovolaemic induced hypertension, maintaining haemodynamics subset 1 of the comparable haemodynamic subsets, is effective in raising perfusion pressure of CBF.

We reviewed surgical result of 5 patients with pulmonary metastasis from submandibular gland cancer. Pathological diagnosis was adenoid cystic carcinoma in 2 patients, carcinoma in pleomorphic adenoma in 1, epidermoid carcinoma in 1, and mucoepidermoid tumor in 1. Disease free interval from resection of the primary lesion to diagnosis of pulmonary metastasis was ranging from 7 to 76 months. Lobectomy was performed in 2 patients and partial lung resection in 3. Three patients died of tumor recurrence 12 to 28 months after lung resection and 2 patients with a single metastatic lesion have survived as long as 42 and 150 months after the operation. This study suggests that surgical treatment may be effective for pulmonary metastasis, especially for a single metastatic lesion, from submandibular gland cancer.

Because most of the diseases to which radon (222Rn) therapy is applied are related to activated oxygen, in this study the effect of the radioactivity of radon and the thermal effect were compared under a room or a hot spring condition with the same chemical component using as the parameters the activity of superoxide dismutase (SOD), which is an oxidation inhibitor, and lipid peroxide and low density lipoprotein (LDL)-cholesterol, which are closely involved in arteriosclerosis. Results show that the SOD activity was significantly increased, and the lipid peroxide and LDL-cholesterol levels were significantly decreased on days 6 and 7 of study. The results were about 2-fold larger in the radon group than in the thermo group. This suggests that the anti-oxidation function was more enhanced by radon therapy than by thermo therapy, and suggests that radon therapy may help to prevent the causes of life style-related diseases such as arteriosclerosis. These findings are important in understanding the mechanism of diseases in which radon therapy is used as treatment, and most of which are called activated oxygen-related diseases.

We reported a 35-year-old male patient with acute type adult T cell leukemia(ATL) and human T-lymphotropic virus type I associated myelopathy. He presented meningitis and polyradiculoneuropathy and improved with steroid treatment. He presented headache and sensory ataxia and these symptoms responded to steroid treatment. The usual clinical features of ATL were absent at onset. There existed ATL-like cells in the cerebrospinal fluid and HTLV-I proviral DNA in the blood. Therefore, we surmised that there was monoclonal hyperplasia of the infected cells. He presented ATL after 2 years remission. We pointed out that there was acute type of ATL in patients with meningitis and polyradiculoneuropathy, for which steroid treatment could be effective.

We studied the location of the electric generator of photoparoxysmal discharges using a scalp-skull-brain dipole tracing (DT) method. By this method, the location of epileptic discharges on each scalp EEG was calculated as an equivalent current dipole (ECD) in 8 cases of epilepsy who had photoparoxysmal discharges. We divided these cases into groups A and B (4 cases each), comprising of patients with and without visually-induced seizures, respectively. The ECDs of the 3 cases in group A corresponded to the small area adjacent to the lateral geniculate nucleus on MRI superimposed. On the other hand, the ECDs of all cases in group B located at the corpus callosum. This study suggests the pathways of epileptic discharges from the epileptic focus are different between two groups. It is supposed that neural activity of the lateral geniculate nucleus might be responsible for the generator mechanism of photoparoxysmal discharges which evokes photosensitive epilepsy.

We described a family of dentatorubral-pallidoluysian atrophy (DRPLA). The mother presented with cerebellar ataxia at 35 years of age and thereafter her neurological symptoms became exacerbated. Her daughter had mental retardation during the preschool period and epilepsy at 10 years. Her son presented with epilepsy at 14 years. Their clinical phenotypes demonstrated maternal anticipation in this family. Genetical analysis of their DNA revealed CAG repeat expansion of the DRPLA gene, the number of which was 51 (mother), 65 (her daughter), and 53 (her son). MR imaging showed disappearance of T, shortening of the red nucleus in the mother and her daughter in contrast to the normal appearance in her son. MR imaging was effective in evaluating neuropathological changes in the DRPLA patients.

We describe a patient who presented with isolated retrograde amnesia of 2-year duration after recovery from viral encephalitis. The patient was a 29-year-old right-handed male dentist and was admitted to our hospital with a complaint of generalized convulsion. Cerebrospinal fluid (CSF) showed mononuclear pleocytosis. Neuroradiological examinations (X-ray CT, MRI and 123I-IMP SPECT) revealed no significant abnormality. Immunological method showed no specific increase of viral antibody titers. However, with a tentative diagnosis of viral encephalitis, administration of acyclovir was started. After 3 weeks he became comprehensive, and several kinds of neuropsychological tests were performed. His intelligence and immediate memory were normal, and his procedural memory of dentist was intact. On the other hand, he could not recall any information about events, both personal and public, occurred within 2 years before the onset of present illness. His autobiographical memory disorder was also demonstrated using a series of weekly comic. In isolated retrograde amnesia following viral encephalitis like this case, memory of relatively short time duration, acquired prior to the onset of present illness, tend to be disturbed.

The outcome of cerebrovascular disease in the elderly, defined as persons 70 years of age and over, and in the non-elderly, those under 70 years, was compared. Fifty-one consecutive elderly and 156 non-elderly patients were studied. The patients in each group were classified according to type of cerebrovascular event: intracerebral hemorrhage, cerebral infarction, or subarachnoid hemorrhage. Patients were evaluated by using the Glasgow Outcome Scale both at the time discharge and 6 months later. The elderly patients were more severely disabled at the time of discharge than the non-elderly patients. The number of patients evaluated as "good recovery" or "moderate disability" had increased at the 6-month follow-up examination in the non-elderly patients, whereas the condition of many of the elderly patients had declined. The death rate of the elderly at the time of discharge and 6 months later was 17.6% and 52.0%, respectively, as opposed to 13.5% and 18.8% in the non-elderly group. The outcome of cerebrovascular disease was much worse in the elderly patients than in the non-elderly patients. Patient age and status at discharge were important predictors of the clinical course.

An 11-year-old girl was admitted to Chiba University Hospital because of the infection of jaw cysts. She had many pits on her palms and soles, basal cell nevus, and typical facial features to Gorlin syndrome (nevoid basal cell carcinoma syndrome). Her 14-year-old sister and 44-year-old father also had similar clinical symptoms, thus they were diagnosed as having a familial Gorlin syndrome. Recently, the father had a complication of maxillary cancer. Colony formation tests after X-ray and ultraviolet irradiation in skin fibroblasts derived from these sisters revealed no remarkable hypersensitivity to these agents. It is important to make an early diagnosis and a proper management in Gorlin syndrome, which has cancer predisposition.

To establish reference values for bone mineral density (BMD), a population survey was carried out in a fishing community in Wakayama Prefecture. The BMD measurements of the lumbar spine (L2-L4) and proximal femur (femoral neck, trochanter and Ward's triangle area) were performed by sex and age, using dual-energy X-ray absorptiometry. Four hundred subjects, aged 40 to 79 years, were recruited randomly to give 50 persons in each of eight age-sex strata from a list of 2,261 residents (1,028 men and 1,233 women) living in a fishing village. The validity of sampling methods was assessed using a questionnaire about lifestyle factors, which was used for all residents aged from 40 to 79 years in the baseline survey. There were no significant differences between the subjects for BMD measurements and all the residents of the community in the frequencies of past history of diseases, healthy habits, food intakes, and rates of smoking, alcohol drinking, coffee and green tea consumption. These findings suggested that the present study population could be considered representative of samples obtained from the entire population in the fishing community. The results of BMD measurements in these subjects, who were selected at random, showed that except for a slightly high BMD of the lumbar spine in men in their 60's, the mean BMD of the lumbar spine and the three proximal segments of the femur decreased with increasing age in both sexes.

Anti-GQ1b antibody seems to be a pathogenetic factor in the development of Fisher syndrome (FS). Although several patients received immunoadsorption therapy (IAT), whether it can remove the autoantibody has not yet been clarified. We treated two patients with FS by IAT using tryptophane column (TR-C) and phenylalanine column (PH-C) (TR-C; 9 times altogether in 2 patients, PH-C: twice altogether in 2 patients), and compared the removal ability of IgG anti-GQ1b antibody and immunoglobulin between TR-C and PH-C. TR-C removed the IgG anti-GQ1b antibodies, IgG, IgA and IgM more than PH-C did. TR-C removed the IgG anti-GQ1b antibody more selectively than non-specific immunoglobulin. In practicing IAT on FS, the use of TR-C rather than PH-C is recommended in view of the removal ability of the autoantibody.

A 31-year-old male developed intramedullary tumors in the medulla oblongata and the upper cervical spinal cord. He was first admitted with tetraparesis. Magnetic resonance (MR) imaging revealed a low intensity mass lesion in the medulla oblongata. The tumor was removed and diagnosed as a pilocytic astrocytoma. Nine years later, he was readmitted with motor weakness and dysesthesia in the right arm. MR imaging revealed a mass lesion in the cervical cord. This tumor was removed and diagnosed histologically as ependymoma. We suggest that the displacement of primitive spongioblasts with subsequent differentiation resulted in an astrocytoma and an ependymoma in adjacent areas.

A 32-year-old male presented with subarachnoid hemorrhage following head trauma. Initial carotid angiography revealed an aneurysm protruding from the dorsal wall of the supraclinoid internal carotid artery. Two weeks later, following two additional hemorrhagic episodes, repeat carotid angiography showed severe vasospasm and dramatic expansion of the aneurysm. He died 17 days after the accident. Autopsy and histological examination confirmed the diagnosis of a traumatic, false aneurysm of the internal carotid artery. Improved prognosis requires early recognition and surgical obliteration of such aneurysms.

PURPOSE: To evaluate the utility and possible increased sensitivity of fluid-attenuated inversion recovery (FLAIR) images for the detection of tubers in patients with tuberous sclerosis, compared with standard T2-weighted images, and to evaluate whether the tubers are correlated with neurologic symptoms. METHOD: We examined the number, size, and location of tubers in five tuberous sclerosis patients using T2-weighted and FLAIR images. Their intelligence quotients, ages at the onset of the first seizure, seizure types, and epileptic severity also were studied retrospectively. RESULTS: The number of tubers observed ranged from 4 to 17 on T2-weighted images, and from 10 to 33 on FLAIR images. All the tubers, other than the myelination line on T2-weighted images, were remarkably demonstrated as high-intensity lesions on FLAIR images. No correlation was found between the neurologic outcome and the number, size, or location of tubers on FLAIR images. CONCLUSION: FLAIR images were very sensitive for the detection of tubers, especially small subcortical ones, the number, size, and location of which are not related to the neurologic symptoms.

A 13-year-old boy presented with ophthalmoplegia, cerebella ataxia and areflexia subsequent to watery diarrhea and pyrexia. Campylobacter jejuni (PEN 2: LIO 4) was isolated from the stools. The patient had a high titer of IgG anti-GQ1b IgG antibody titers which decreased during the clinical course. Although C. jejuni has recently been recognized as the most common microorganism causing infection preceding Guillain-Barré syndrome, only six patients with Fisher's syndrome after this bacterial infection have been reported. This is the first report of a patient with Fisher's syndrome in whom C. jejuni was identified as a causative organism in Japan.

We investigated mirror movements observed in two patients skilled in playing the piano and compared these symptoms with those reported in patients associated with frontal lobe, corpus callosum or cervical cord lesion. We found the following common features in our two patients: 1) mirror movements were observed during skilled finger movements such as playing the piano, 2) these were observed in distal parts of the bilateral upper extremities, 3) contralateral imitative associated movements were seen concomitantly, 4) frontal lobe symptoms and callosal disconnection syndrome were not seen, 5) both patients recalled having mirror movements in their infancy, and one had family history. These characteristics of mirror movements in our patients were similar to those in patients associated with cervical cord lesion, but were different from those in patients associated with frontal lobe or corpus callosum lesion. It is suggested that abnormal pathways in the pyramidal tract or cervical cord lesion elicited mirror movements in our patients.

Patients who have undergone allogeneic bone marrow transplantation (allo-BMT) are susceptible to a variety of opportunistic infectious complications in the months to years after engraftment. Impaired in vitro T-cell functions have been documented in these patients, and these T-cell dysfunctions contribute to the prolonged immune deficiency after allo-BMT. In the present study, we examined the expression of CD26 as well as the reconstitution of CD26-mediated T-cell costimulation via the CD3 and CD2 pathways at various times in patients aged greater than 18 years after CD6-positive, T-cell depleted allo-BMT. We found that the percentage of CD26- and CD3-positive cells, as well as the levels of expression of both antigens, was lower than in normal controls during the first 4 months after CD6-depleted allo-BMT. Subsequently, the amount of lymphocytes expressing CD3 and CD26 and the quantitative surface expression of CD3 and CD26 were not significantly different in patients and normal controls. Functional studies showed that CD26-mediated T-cell proliferation via the CD3 pathway was considerably improved and almost reached normal levels by 1 year, whereas recovery of CD26-mediated T-cell proliferation via the CD2 pathway was delayed for at least 2 years after CD6-depleted allo-BMT. As CD26 involvement in the regulation of human thymocyte activation is restricted preferentially to the CD3 pathway--unlike its involvement with both CD3 and CD2 pathways of peripheral T cells--our results suggest that the different effects of CD26-mediated costimulation via the CD3 and CD2 pathways after CD6-depleted allo-BMT may be a reflection of peripheral T-cell immaturity in those individuals, similar to that seen in mature medullary thymocytes or cord T lymphocytes.

We previously have reported that indium-111 labeled anti-glioma monoclonal antibody G-22 (111In-G-22) exhibits diagnostic potential against human glioma xenografts in athymic mice. Herein, we report the selective tumor localization of 111In-G-22 in patients with glioma. Five patients were administered an average of 2.2 mCi of 111In-G-22 whole IgG intravenously. No immediate or delayed side effects were attributable to 111In-G-22 injection. Serial gamma scintigraphy and single photon emission computed tomography (SPECT) were performed, and the distribution in the brain and intratumoral accumulation of 111In-G-22 were evaluated. CT and/or magnetic resonance (MR) images were performed simultaneously and these images were compared. In the case of malignant glioma, tumor-imaging was successfully obtained beginning at 6 h following injection with a maximum uptake tumor/brain ratio at 48 h. The distribution of 111In was selective. It predominantly accumulated in the biologically active areas of the tumor. Furthermore, the tracer uptake appeared to correlate with the histologic tumor grade. This confirms the G-22 monoclonal antibody specifically binds to biologically active and malignant glioma tissue, and tumor-imaging using 111In-G-22 may give support to the diagnosis of malignant glioma.

PURPOSE: To determine whether the depression of cerebral bioenergetic metabolism caused by chronic subdural hematomas can account for neurologic dysfunction and whether the degree of metabolic depression may be useful for clinical assessment and therapy. METHODS: Sixteen patients who had chronic subdural hematomas with hemiparesis and/or mental disturbances underwent phosphorous 31 MR spectroscopy before and 10 to 14 days after surgery. Phosphorous 31 MR spectroscopy was also performed on 5 patients who had chronic subdural hematomas with only slight headaches who were treated by conservative therapy and on 10 healthy volunteers. RESULTS: The peroperative phosphocreatine-to-inorganic phosphate ratio (2.10 +/- 0.36) improved to normal values (2.69 +/- 0.44) after evacuation of hematomas. This improvement was accompanied by complete disappearance of hemiparesis and/or mental disturbance. Brain tissue pH also improved from 7.07 +/- 0.11 to 7.205 +/- 0.13 after surgery. On the other hand, patients who had chronic subdural hematomas with only slight headaches had the same phosphocreatine-to-inorganic phosphate ratio and brain intracellular pH as healthy volunteers. CONCLUSION: The phosphocreatine-to-inorganic phosphate ratio may be useful for determining when to operate on patients with chronic subdural hematomas and to assess the efficacy of treatment.

To clarify the prognosis of patients with intractable diseases, a baseline survey of patients who received financial aid for intractable disease treatment between April 1984 and March 1985 was performed in Wakayama Prefecture, followed by a follow-up survey over a period of 8 years. Based on public welfare-subsidized system in Wakayama Prefecture, all patients with intractable diseases were checked up annually their certificates of financial aid for treatment. The results obtained were as follows: Parkinson's disease had the highest rate of discontinuation of medical care at 65%, followed by idiopathic thrombocytopenic purpura (ITP) at 64%, aplastic anemia at 55%, ulcerative colitis (UC) at 54%, and Behcet's disease at 50%. Systemic lupus erythematosus (SLE) had the lowest rate of discontinuation of medical care at 32%. Discontinuation was due to death in approximately 50% of the patients with SLE, scleroderma, dermatomyositis and polymyositis, and aplastic anemia. Of these, the causes of death in more than 50% were directly related to the primary diseases. However, in patients with SLE and aplastic anemia, 25% discontinued care because of cure or alleviation. Some patients remained in remission even though the prognosis for these diseases is not generally considered to be favorable. Transfer of jurisdiction to the Disabled Persons Welfare Act was seen in 28% of patients with Parkinson's disease, 24% with Behcet's disease, and 23% with ossification of posterior longitudinal ligament, diseases which are believed to cause restriction in daily activities of patients in some cases. On the other hand, cure or alleviation was the reason for discontinuation in 60-70% of patients with Buerger's disease, UC and ITP.(ABSTRACT TRUNCATED AT 250 WORDS)

PURPOSE: To clarify whether there were any differences in MR appearance between the childhood and the adult moyamoya syndromes. METHOD: We compared the cranial MR findings in four children under the age of 6 who had moyamoya syndrome with previously documented adult cases. RESULTS: Moyamoya syndrome in younger children exhibited a significant increase in cortical and subcortical infarction, and a decreased incidence of deep white matter infarction in the centrum semiovale and basal ganglia, in contrast to adult cases. There were no remarkable differences between these two groups of moyamoya cases with regard to the occlusive changes of the internal carotid and middle cerebral arteries, or to the flow void sign on MR. CONCLUSION: These differences in the sites and frequencies of infarctions between the childhood and the adult moyamoya syndromes observed on MR might reflect differences in the cerebral circulation.

Survival rates and several effective factors for the postoperative course of 10 advanced cases of small sized lung cancer (ASLC) were evaluated. Followings were considered as characteristics of these patients: 1) ASLC were observed at the rate of 4.0% of whole lung cancers, 2) ASLC were more often seen in relatively young patients, females and adeno carcinomas than ordinary lung cancer, 3) as factors concerned to postoperative prognosis, stage III A (especially T1N2M0), few numbers of metastatic lymph nodes in N2, and performance of relative curative operation may predict good prognosis, but adenosquamous type will be a sign of poor prognosis. Satisfactory result was obtained even in a case of stage IV when complete resection of metastasis was carried out. There was no significant difference of survivals between 2 cm and 3 cm of diameter of the tumor. In conclusion, above mentioned points should be considered when operative indication of ASLC is evaluated.

Blasts from 5 cases of AML with pseudo-Chediak-Higashi granules were examined ultrastructurally and histocytochemically using peroxidase, acid phosphatase, high iron diamine (HID) and periodic acid-thiocarbohydrazide-silver proteinate (PA-TCH-SP) stainings. Pseudo-Chediak-Higashi granules, which appeared as vacuole-like inclusions by light microscopy, generally contained electron-lucent materials. All pseudo-Chediak-Higashi granules were, positive for peroxidase but some were negative for acid phosphatase. Pseudo-Chediak-Higashi granules were HID positive, indicating that they contained sulfated glycoconjugates. Glycogen-like particles were observed in the pseudo-Chediak-Higashi granules with the PA-TCH-SP method, as occasionally observed in granules in drug resistant ALL blasts. In conclusion, the contents of pseudo-Chediak-Higashi granules, which seems to be formed by fusion of small granules, differed from those of normal azurophillic granules.

Cytogenetic analyses were performed on 12 adult patients with abnormal megakaryoblastic proliferation which was detected by ultrastructural cytochemical study (platelet peroxidase) and platelet-megakaryocytes-specific monoclonal antibodies (TP-80, Plt1, AN51, and KOR-77). The patients consisted of two patients with myelodysplastic syndromes (MDS), three with acute megakaryoblastic leukemia (AMKL), six with megakaryoblastic transformation in Philadelphia-positive chronic myelogenous leukemia (CML-meg-BC), and one case of chronic myeloproliferative disorder (CMPD). Among them, an inversion of the long arm of chromosome 3 [inv(3)(q21q26)] was found in one AMKL patient with a normal platelet count. Chromosome change at band 3q26 was also found in one MDS patient without thrombocythemia. Furthermore, the long arm of chromosome 13, where rearrangements in myelofibrosis are clustered (13q12----q22) was seen in one MDS patient. Trisomoy of chromosome 19 was found in one AMKL patient and three CML-meg-BC patients. These findings indicate that cytogenetic abnormalities involving 3q26, 13q, and trisomy 19 are associated with hematologic neoplasia with megakaryocytic lineage in adult patients, although these abnormalities were not related to the survival of the patients. During the period of this study, two acute myelogenous leukemia patients (AML-M2 and AML-M5b) with chromosome rearrangements at band 3q21 and thrombocythemia were found, indicating that chromosome abnormality at band 3q21 is related to quantitative platelet dysfunction, whereas that at 3q26 is related to hematologic malignancies with a proliferation of megakaryocytic lineage.

The surgical treatment of large pituitary adenomas with suprasellar extensions has been controversial. To elucidate the indications for transsphenoidal surgery of large adenomas and to evaluate the techniques for removing the suprasellar portions of the tumors, surgical procedures on 100 consecutive patients with suprasellar extensions of nonfunctioning pituitary adenomas were retrospectively investigated. Patients were followed up for 1 to 12 years (mean, 4.5 yr). One hundred twenty-five transsphenoidal operations were performed on 100 patients. The removal of each suprasellar tumor was facilitated by the placement of a lumbar subarachnoid catheter and the injection of lactated Ringer's solution or saline. This method was used in 77 operations and was effective on 60 of 72 adenomas with30-mm suprasellar extensions (Hardy's Grades A, B, and C) but not on those that were fibrous or dumbbell-shaped. The descent of the remaining suprasellar tumor was facilitated by keeping the sella and sellar floor open with an intrasellar drain, and the subsequent removal was achieved with staged transsphenoidal operations. Of nine fibrous or dumbbell-shaped adenomas with 10- to 30-mm suprasellar extensions, gross total removal in eight was achieved by the open sella technique and two-stage transsphenoidal operation, whereas one required transcranial surgery. Adenomas with30-mm suprasellar or lateral extensions (Grade D) could not be removed sufficiently by transsphenoidal operations, except one adenoma for which a subtotal removal was achieved in the third staged operation. The disease-free rate 10 years after operation was 74% for all patients: 91% for Grade A, 74% for Grade B, and 61% for Grade C.(ABSTRACT TRUNCATED AT 250 WORDS)

A 34-year-old woman with primary choroid plexus papilloma occurring in the suprasellar region is reported. No connection with the ventricular system was found during intraoperative observations. The findings of pathological examinations such as hematoxylin and eosin staining, transthyretin (prealbumin) immunoreactivity, and electron microscopy were consistent with choroid plexus papilloma. Radiologically, it was extremely difficult to differentiate from tuberculum sellae meningioma. To our knowledge, this is the first case of primary choroid plexus papilloma in this location reported in the literature.

Postnatal development of autonomic nerves of major cerebral arteries was histochemically studied in mice from one day to five months of age. For demonstration of aminergic nerves the glyoxylic acid method was used, while for cholinergic nerves Karnovsky and Roots' technique was utilized consecutively on the same whole mount preparations. The results obtained were as follows: 1) In one-day-old mice a few aminergic nerves were seen while cholinergic nerves were scarcely observed. The cholinergic nerves were clearly observed in one-week-old mice. Then, both nerves increased rapidly in the first 2 weeks with a slight delay of maturation in the latter. They completed development between 3 and 4 weeks. 2) Longitudinal and circular distributional patterns were observed for the both nerves; the former pattern developed earlier than the latter.

Pressure of brain retraction was measured with a strain gauge spatula in 31 cases of 21 tumours and 10 aneurysms. They were operated on via different approaches: subfrontal (10 cases), interhemispheric (5), subtemporal (7) and suboccipital (9). The patients' age ranged from 11 to 74 years (average 50). The recorded data were averaged for every 5 minutes and divided into two groups: the first was data taken in the initial stage during the approach to the lesions, and the second were those taken during procedures at and around the lesion. Significant difference in the pressure among different approaches was found in the tumour group approached by the subtemporal route compared with other routes (p less than 0.005). The retraction pressures in the tumour cases were higher during the approach (31 +/- 15.5 torr) than during the main procedure (12 +/- 9.2 torr) (p less than 0.025). On the other hand, the pressures in the aneurysm cases were the reverse, being lower during the approach (22 +/- 15.2 torr) than during the main procedure (38 +/- 17.7 torr) (p less than 0.01). Early-stage operations for aneurysm required a higher retraction pressure than delayed operations.

We have given intrathecal injections of dexamethasone more than 2000 times for approximately 200 cases with visual disturbance and 20 cases with sciatic pain. An injection of 8 mg was given once or twice a week until a total dose of 80 to 100 mg was given. Six of the 14 cases that were thus treated in their chronic stage for post-traumatic visual disturbance showed various degrees of improvement in visual fields and acuity. There were no serious complications in this series. It is concluded that intrathecal steroid injection is effective in certain cases of post-traumatic visual impairment.

Subdural fluid collections appeared in 15 cases (39%) after removal of 38 intra- and paraventricular tumours in the third or lateral ventricle through 18 frontal and 20 parietal transcortical approaches. Transient fluid collections which disappeared within 2 weeks occurred in 6 cases (16%) and persistent ones in 9 cases (24%). Four of the 9 cases (11%) of collections required surgical treatment because of positive clinical signs and symptoms. Two cases had expansive fluid collections and the other two contained subdural haematomas at surgery. The risk factors likely to contribute to a persistent collection were preoperative ventriculomegaly (frontal horn index greater than 0.38) and a frontal transcortical approach. A symptomatic collection should be considered as a potential complication of the transcortical approach to intraventricular tumours and some methods should be devised to prevent it when intra- or paraventricular tumours with ventriculomegaly are removed.

Five cases of the supratentorial extradural hematomas secondary to the posterior fossa craniectomy were reported. The site of the hematoma was far from the operative field and two cases showed acute course and three were rather mild. The symptoms of this complication were the unsuspected sensorium disturbance, anisocoria and the non-functioning ventricular drainage. Two cases died of this complication and two were severely disabled. The mechanism of this complication is not clearly understood. The lowering of the ventricular pressure by the ventricular tap during the operation may play significant role in the formation of the extradural hematoma. The younger age of the cases and the long history of increased intracranial pressure were stressed in the literature. The significance of the management of the ventricular pressure during the operation was emphasized. The prevention is the best treatment.

A simple method of making a three-dimensional display model of recorded computerized tomography (CT) scan films is presented. The reconstructed display model provides useful information as to surgical approaches to intracranial mass lesions. The actual size and three dimensional shape of a lesion and its topographical relationship to surrounding brain structures are easily grasped by directly handling this model.

We report here a case of multiple sclerosis (MS) showing pure alexia. The patient was a 28 year-old, right-handed male student of medicine. He had been healthy prior to college, but when he tried to read the newspaper one morning in January of 1983, he suddenly became aware that he could not. He could speak fluently and had no disturbances of auditory comprehension. No signs of abnormality were noted in his writing ability. Thereafter, the patient occasionally experienced difficulty in reading, together with right homonymous hemianopsia. These symptoms, however, usually vanished following sleep. In April, 1984, the patient experienced headache and ataxia when walking, followed by repeated remission and exacerbation of the symptoms. Corticosteroid therapy produced complete disappearance of the headache and ataxia. In September of 1985, he was hospitalized at our department due to the clouding of consciousness accompanied by convulsions. Neurologically, no abnormalities of the ocular fundus were noted and Babinski's sign was negative, but left hemiplegia was observed. Neuropsychologically, his intelligence was normal and he had no difficulty in writing, but paralexia was noted. There were no disorders of spontaneous speech or auditory comprehension. Examination by CT scan and magnetic resonance imaging revealed a number of lesions in the white substance of the cerebrum, including the left occipital lobe. The abnormal signs seen radiographically vanished when cortical hormones were administered. It has been thought that the symptoms of multiple sclerosis are due mainly to disorders of the white substance, and that MS seldom produces symptoms of aphasia, due to the fact that the lesions in this disease are generally small.(ABSTRACT TRUNCATED AT 250 WORDS)

A rare case of spontaneous bilateral extradural hematomas probably due to hypofibrinogenemia is reported. On April 17, 1983, a 21-year-old woman was referred to our hospital because of her comatose state. She has complained of her longer duration of menstrual bleeding lasting 10 days to 2 weeks, but has had no history of hemorrhagic diseases. At the last night, she complained of headache and vomited several times, after drinking some beer but she went to bed without therapy. We cannot see a history of her head injury several days before admission. On admission, she was comatose with bilateral dilated pupils and flexed her upper extremities against painful stimuli. There was a tendency for subcutaneous hematomas to form immediately in places where the stimuli were applied. Skull fracture was not found on craniogram and CT scan showed bilateral extradural hematomas (60 ml on the right side and 130 ml on the left side). Laboratory data showed leukocytosis (14800), longer prothrombin time (15.2 seconds with a control of 11.9 seconds), markedly elongated activated partial thromboplastin time (90 seconds with a control of 15-45 seconds) and decreased fibrinogen concentration (114 mg/dl with a control of 200-400 mg/dl). At the bilateral decompressive craniectomies with infusion of 5 gram of fibrinogen and 300 ml of fresh blood, we found neither scalp hemorrhage nor skull fracture and after removal of coagulated hematomas no dural anomaly was found. After operation she improved gradually and laboratory re-examination was normal including bone marrow puncture. Now, 5 months after operation she is clear with slight tetraparesis on her rehabilitating course.(ABSTRACT TRUNCATED AT 250 WORDS)

Microvascular decompression has been widely used as a method for the treatment of hemifacial spasm and trigeminal neuralgia. We have experienced 30 such cases in the last 2 years; 25 of them were hemifacial spasm and 5 trigeminal neuralgia. Excellent results were obtained in 26 cases; the remaining two cases, both hemifacial spasm, were partially cured. Mild facial paresis appeared several days after the operation in 3 patients. In all the cases, the facial paresis recovered completely within several weeks. The cause of the facial paresis was not known. In 2 cases a slight hearing deficits were noticed after surgery, which has been gradually improving over several months. As this operation is functional surgery, operative complications must be avoided as much as possible. It has been our policy that we first try medical treatment and/or some kinds of nerve block and if no effects are obtained, we recommend the microvascular decompression. For microvascular decompression, suboccipital craniectomy is performed in lateral position. From the point of view of surgical technique, we stress several important points as follows: The head is elevated about 30 degrees, and it is kept approximately horizontal and should not be excessively rotated. Craniectomy is made as far laterally as the sigmoid sinus; its shape is elongated oval. Retraction of the cerebellum should not be done in the direction of the cranial nerves to avoid post-operative hearing deficit. Two tapered retractors are effectively used for cerebellar retraction. A third slim, tapered retractor is useful for holding an offending artery when exploring the root exit zone or placing a sponge for decompression.(ABSTRACT TRUNCATED AT 250 WORDS)

A trochlear nerve meningioma in a patient with von Recklinghausen's disease is reported. The tumour appeared to have originated from the trochlear nerve itself, having no connection either with the neurinomas present in the adjacent regions, or with the tentorium. Histological examinations revealed that the tumour was a meningotheliomatous meningioma and the trochlear nerve fibres were placed in the periphery of the tumours. It was noteworthy that diplopia was not detected either before or after the resection of the trochlear nerve with the tumour.

A cured case of superior sagittal sinus thrombosis is reported. The patient, a 26-year-old man, also displayed severe complications such as hemorrhagic infarction and status epilepticus. Although his prognosis was considered to be extremely poor, conservative treatment, with mannitol, steroid, anticonvulsants, etc. was effective, and he was discharged without any neurological deficit. This report discusses the clinical course, CT findings, angiographical findings and regional cerebral blood flow (rCBF). At first, CT showed no abnormal findings, but hemorrhagic infarction was detected on the 3rd day after the onset. Follow-up CT showed subcortical low density area, hemorrhagic infarction with perifocal brain edema, midline shift etc; the focus of hemorrhagic infarction was almost absorbed 2.5 months after the onset. Cerebral angiogram showed not only the obstruction of the superior sagittal sinus but also that of cortical veins of cerebral convexity at first. Follow-up angiogram showed the development of collateral circulations such as deep cerebral, ophthalmic and emissary veins. On CBF study, low rCBF at the bilateral parasagittal region was observed, but marked increase of rCBF was measured in the parasagittal region, especially at the site of hemorrhagic infarction after the administration of 20% mannitol. We, therefore, consider mannitol as an effective agent for the treatment of cerebral sino-venous thrombosis.

The changes in CBF and rCBF through the entire age range include the rapid period in childhood was reported. Sixteen children between the ages of 1 and 15 and 14 adults were studied. These 30 subjects were either volunteers of out-patients without CT and EEG abnormalities. rCBF was measured by the 133Xe intravenous injection method using Varmet rCBF analyzer. Ther relationship between age and CBF, the correlation coefficient was calculated based on the regression equations and the regression curve with the highest correlation was chosen. For the analysis of rCBF, he mean rCBF values (ISI) of 3 channels corresponding to the frontal, temporal, parietal and occipital lobes were expressed as a percentage of the hemispheric CBF. The hemispheric blood flow (ISI and CBF gray) of children less than 5 years of age was approximately twice that found in adults. This value decreased rapidly with age and in the 10-15 years the blood flow was approximately 1.3-fold that of adults. Thereafter, there was a slow decrease and a negative correlation with age was found. The decrease showed the correlation on the following equations; y = 146.5 - 58.5 log x. (r = -0.903) for ISI and log y = 2.26 - 0.29 log x. (r = -0.881) for CBF gray, which was statistically significant. In contrast, the CBF white showed a slightly higher value in the 1-2 years old children, but thereafter the CBF did not show a notable decreases with age. Through the entire age range, a best fit for the Fw values was found with : y = 18.3 + 37.5/x. (r = 0.798), which was also statistically significant.

Polycystic kidney is a familial disorder known to be complicated often by liver cyst, pancreas cyst and cerebral aneurysm. While numerous reports have been made on the concurrence polycystic kidney and cerebral aneurysm in foreign countries, there are only several autopsy cases documented so far in Japan. This paper describes a surgically treated case of proven non-ruptured aneurysms of bilateral middle cerebral arteries associated with polycystic kidney, and discusses the epidermology of cerebral aneurysm accompanying polycystic kidney in Japan. According to the Annual Report of Pathological Autopsy Cases in Japan, 1981, 8.4% of the cases of polycystic kidney were associated with cerebral aneurysms. The patients with polycystic kidney should be investigated for cerebral aneurysms.