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We report a case of conversion surgery for a locally advanced unresectable(UR-LA)pancreatic cancer that was radically resected after S-1 therapy. A 65-year-old man visited a referral physician because of fatigue and liver dysfunction. A CT scan revealed a mass in the pancreatic uncinate process that was suspected to be superior mesenteric artery(SMA)infiltration and was diagnosed as UR-LA pancreatic cancer. GEM nab-PTX therapy was initiated but was discontinued after 2 courses because of adverse events. The treatment was switched to S-1 monochemotherapy. After that, the tumor did not progress for around 1.5 years, and the patient was referred to our hospital for surgical treatment. As the contact between the tumor and the SMA was considered to be less than half-round, we made a diagnosis of borderline resectable(BR-A)pancreatic cancer. Subsequently, we performed a pancreaticoduodenectomy with partial resection of the portal vein and achieved R0 resection. The patient received adjuvant chemotherapy with S-1 and showed no signs of recurrence for 10 months after surgery.

Objective: We retrospectively investigated the efficacy and complications of surgical closure of the larynx (SCL) for recurrent aspiration pneumonia in comparison with tracheoesophageal diversion., Methods: The subjects were persons with severe motor and intellectual disabilities (SMID) who had undergone surgery for recurrent aspiration pneumonia between 1994 and 2011: A 8 SCL patients group and a 16 tracheoesophageal diversion patients group. We investigated two groups the lower respiratory infection incidence, length of hospital stay for the surgery, postoperative complications, and rate of cannula withdrawal, by reviewing medical records., Results: Both the SCL and the tracheoesophageal diversion group showed a reduction in the incidence of infection after surgery, indicating that the efficacy of SCL was equivalent to that of tracheoesophageal diversion in preventing aspiration pneumonea. The SCL group showed a reduction in the length of hospital stay and an increased rate of cannula withdrawal as compared with the tracheoesophageal diversion group., Conclusion: The efficacy of SCL was equivalent to that of tracheoesophageal diversion in preventing aspiration for SMID. We consider SLC to have potential for reducing the burden on patients.

A retrospective analysis of the clinical and MRI features in 20 Japanese children diagnosed with central nervous system inflammatory demyelinating disorders was performed. Using the new criteria proposed by International Pediatric MS Study Group, half of children were reclassified into clinical isolated demyelinating syndrome (CIS). Presence of seizures and a pattern of diffuse bilateral lesions on brain MRIs are more frequent in children with ADEM than in CIS. However we suggest these features and encephalopathy may be associated with the age of patients. Furthermore, though persistence of abnormal MRI lesions is significantly more likely in the group of CIS, none of these patients had a subsequent recurrence or developed MS during the follow-up period. The prediction of patient prognosis seems to be difficult even based on the new criteria, and the nationwide multicenter analysis may be necessary in Japan for acquiring the definite conclusion.

We reported the clinical and neuroradiological findings of 8 patients (4 males; 4 females; age range; 3 - 14 years) with posterior leukoencephalopathy syndrome (PLES). Previous case reports suggested that one of the major factors leading to PLES was severe hypertension. We divided the patients into two groups with or without severe hypertension, and each group was divided furthermore into two corresponding to the reversibility of brain lesions. The four cases of PLES with severe hypertension were all due to renal hypertension, and half of them resulted in irreversible outcomes, which were considered to be associated with inappropriate treatment. On the other hand, the four cases of PLES without severe hypertension showed reversible change, suggesting intravascular endothelial dysfuncton in respect of their causing factors. We consider that PLES could be caused without sever hypertension, particularly in children. Those with severe hypertension must be treated immediately to prevent irreversible brain damage.

It is difficult to differentiate astatic seizures among atonic, myoclonic and tonic seizures without the help of ictal polygraphic recordings. We described a girl with epilepsy presenting periodic astasia caused by epileptic spasms. Her seizures had occurred in clusters since 1-year and 7-month of age. Her interictal electroencephalogram (EEG) showed intermittent diffuse (poly) spike (s) and wave discharges, without a trace of hypsarrhythmia. She had a diagnosis of epilepsy with myoclonic-astatic seizures at a previous hospital, and her seizures had been resistant to multiple antiepileptic agents. After she was referred to our hospital, the ictal video-EEG recordings were made before and after ACTH administration, which revealed that her astatic seizures were epileptic spasms with presumed cortical origin. We should carefully evaluate the astatic seizures during early childhood which may be an atypical presentation of epileptic spasms.

We examined the effectiveness of naftopidil in 81 patients with lower urinary tract symptoms (LUTS) caused by benign prostatic hyperplasia (BPH). We examined quality of life (QOL) and determined which symptoms improved as a result of naftopidil administration. The findings indicated that storage symptoms, voiding symptoms, total International Prostate Sympotom Score (IPSS), QOL index, Qmax and residual urine volume were significantly improved after treatment when compared to baseline. Improvement of nocturia and incomplete emptying by naftopidil contributed to improvement in QOL, odds ratio between the good response group and poor response group were 3.6 and 2.3, respectively. During naftopidil treatment, two of the 81 patients complained of adverse events. The results show that naftpidil is effective for LUTS caused by BPH, and that improvement of nocturia and incomplete emptying contributed to the improvement in QOL.

Enterovesical fistula is a very rare complication of primary urological malignancies. A case of ileovesical fistula caused by a bladder carcinoma is presented. A 66-year-old male was referred with complaints of urinary pain. On admission, fecaluria and urinary tract infection with bladder stone were detected. Cystography revealed the passage of contrast medium into the small bowel. Under the diagnosis ofileovesical fistula due to suspected inflammatory disease, sigmoidectomy and segmental small bowel resection with partial cystectomy were performed. Histological evaluation revealed a poorly differentiated urothelial carcinoma. Without further treatment, the patient died from cancer five months after operation. However, it is hard to assess the effect of fistulas on prognosis. Since it has been reported that about 40% of the patients with T4 bladder tumors could be potentially cured with radical resection, we recommend a thorough examination to confirm the diagnosis of primary disease to obtain the best results.

Multiple sclerosis (MS) is rare in children and the efficacy and safety of interferon beta 1b (IFN-beta 1b) treatment in childhood MS has not yet been established. We started to treat a boy who suffered from relapsing remitting MS with IFN-beta 1b at 8.5 years of age, because he had severe neurological disability in consequence of frequent relapses and incomplete remission. After initiating IFN-beta 1b treatment, his clinical course moved to secondary progressive (SP) MS, and he demonstrated poor improvement in degenerative progression and his disability continued to worsen. We could speculate that IFN-beta 1b was not effective regarding the degenerative component of childhood MS as in that of adult MS. SPMS is extremely rare in children, and this case would provide a very important implication to predict the tolerability of IFN-beta 1b treatment depending on the type of clinical course in childhood MS.

We studied thirty-one children with hemiplegia to elucidate the relationship of intelligence, the laterality of a lesion, and epilepsy, in these children. We investigated the influences of the age at the onset of the seizures, the number of anti-convulsants, and the duration of medication, on the DQ/FIQ. The results indicated a significantly higher DQ/FIQ and VIQ for right hemiplegic children than for left hemiplegic children. But the PIQ of the patients with either a left or right hemiplegia was not significantly different. A significantly higher DQ/FIQ, VIQ, and PIQ was also revealed for the children without epilepsy. In addition, there was also a tendency that the DQ/FIQ of children who suffered from epilepsy before the age of one year was lower than that of children who suffered from epilepsy after the age of one year. The present results provide evidence which supports the crowding hypothesis.

We investigated the sequence of the administration, the efficacy and the safety of antiepileptic drugs (AED) given intravenously for the treatment of status epilepticus and frequent seizures in children. Our institute has a recommended sequence of AED administration for treatment of status epilepticus: the first-line agent is diazepam (0.3 - 0.5 mg/kg administered intravenously, once or twice). The second-line drugs include midazolam (0.15 - 0.4 mg/kg intravenously, once or twice, and if necessary, followed by continuous infusion at 0.06 - 0.18 mg/kg/hour), lidocaine (1 - 2 mg/kg intravenously, once or twice, and if necessary, followed by continuous infusion at 2 - 4 mg/kg/hour) and phenytoin (10 - 20 mg/kg, infused slowly). For those patients who previously experienced a seizure which was refractory to diazepam but responsive to the second-line agent, it was recommended to use the second-line agent as a first-line agent. When seizures were refractory to the first and second-line agents, thiopental was administered (3 - 10 mg/kg intravenously, and if necessary, followed by continuous infusion at 2 -5 mg/kg/hour). The etiologies of 177 occasions of status epilepticus and frequent seizures were categorized into two groups:epilepsy (n = 95) and situation-related seizures (n = 82). Situation-related seizures included febrile seizures (n = 44), acute encephalopathy/encephalitis (n = 31) and benign infantile convulsions (n = 7). The ages of the patients ranged from 0.1 to 18.4 years (average +/- SD:3.69 +/- 3.15 years). Diazepam was administered as the first-line drug on 157 of 177 occasions (88.7%). On 116 occasions the second-line agents were administered. Midazolam and lidocaine were injected as the second-line agent on 54 (46.6%), and on 33 (28.4%) occasions, respectively, although both midazolam and lidocaine injections were off-label use for seizure control in Japan. Thiopental was used as the third to fifth-line agent. Effective ratios (effective occasions/total occasions) of each drug were the following: thiopental 19/21 (90.4%), midazolam 57/99 (57.6%), lidocaine 25/60 (41.7%), phenytoin 16/41 (39.0%), diazepam 59/164 (36.0%). Thiopental was statistically more effective than midazolam, lidocaine, diazepam or phenytoin (p < 0.01), and midazolam was statistically more effective than diazepam (p < 0.01) or phenytoin (p < 0.05). Administration of thiopental caused complications more frequently than the other agents (p < 0.01): The complications by thiopental were severe in some cases requiring intratracheal intubations and artificial ventilation. From the viewpoint of both efficacy and safety, midazolam should be recommended as one of the first-line agents for status epilepticus.

Encephalopathy associated with human herpes virus 6 infection (HHV-6 encephalopathy) is not rare. The pathophysiology of HHV-6 encephalopathy has not been clearly established and diagnosis in the early stages is difficult. To elucidate the diagnostic value of MRI in the early stages of HHV-6 encephalopathy, we performed MRI including diffusion weighted imaging (DWI) on 6 patients. No abnormalities were found in the T1, T2 weighted images and fluid attenuated inversion recovery (FLAIR) images of any of the patients. DWI revealed an increase of signal in the subcortical white matter of the frontal lobes during the acute phase in all 6 patients. Follow-up MRI in two patients showed lesions expanded to the cortical regions, and disappearance of high signal-lesions followed by atrophy of the corresponding areas in 2 patients. To our knowledge, these findings have not previously been reported in acute encephalopathy and encephalitis and may be characteristic of HHV-6 encephalopathy. DWI may be a useful diagnostic procedure in the early stages of HHV-6 encephalopathy.

Single photon emission computed tomography (SPECT) was performed 3 times during attacks and performed 4 times during postictal periods on a case of alternating hemiplegia of childhood. Hyperperfusion of the corresponding hemisphere to hemiparesis was suggested by asymmetric increase RI uptake during the ictal scans, whereas interictal scans showed symmetric topography of cerebral blood flow. Manifestations except hemiplegia included loss of consciousness and vomiting. These manifestations and ictal SPECT findings are the same evidence as hemiplegic migraine. It suggests that these two disorders have a similar pathophysiology. There are two interesting findings regarding this patient. The first finding is magnetic resonance imaging showed progressive cerebellar atrophy. Second finding is interictal SPECT showed a progressive decrease of cerebral perfusion, especially in cerebellar hemispheres. These two findings suggest alternating hemiplegia of childhood may be a chronic progressive disorder.

We retrospectively studied the efficacy of intravenous and intravenous-drip infusion administration of lidocaine on seizures treated in Saitama Children's Medical Center during the period of 1997-2002. Thirty eight seizures of 29 children (12 boys and 17 girls, average age 41.4 months) were treated with lidocaine. The overall rate of effectiveness was 53%. The response to lidocaine appeared within 5 minutes in all episodes. Good response to lidocaine was noted, in 36% of patients with epilepsy, in 72% with CNS infection and in 80% with benign infantile convulsions; in 42% with generalized seizures and in 63% with partial seizures. Thus there was no significant difference in efficacy among the underlying disorders and seizure types. The adverse reaction of SpO2 decrease was found in one patient. We conclude that lidocaine can be used as a second-line, anti-convulsive drug in status epilepticus based on its prompt effectiveness and low risk of side effects.

To evaluate treatment of Bell's palsy (acute idiopathic peripheral facial nerve paralysis) of children, the authors analyzed 38 cases (18 females, 20 males) of Bell's palsy in children aged below 16 years old. The mean age of all cases was 6.8 +/- 6.2 years old. All cases resulted in complete recovery within 6 months. Clinical score of facial motor functions were adapted to 17 patients who were more than 5 years old. They were divided into two groups: early recovery group (clinical symptoms recovered within 3 months; 10 cases) and later recover group (over 3 months; 7 cases). Clinical scores evaluated in the first week from the onset were not significantly different. Steroid therapy was used for 9 patients of early group and 6 patient of later group. All patients of this study were recovered, thus we could not evaluate effect of steroid therapy for Bell's palsy in children. Use of steroid therapy for Bell's palsy needs more concretely administration. We consider how the region locates near to the center is an important prognostic factor.

A 1-year-8-month-old patient developed cerebellar ataxia following a prodromal infection. Despite initial diagnosis of acute cerebellar ataxia, his symptoms lasted for more than 30 days. High-dose intravenous immunoglobulin and steroid pulse therapy failed to ameliorate his cerebellar symptoms, which fluctuated in association with infections. At the age of 3 years and 8 months, he had mental retardation with cerebellar symptoms. Findings of MR imaging and single photon emission computed tomography were normal. Neuron-specific enolase of cerebrospinal fluid (CSF) ranged from 10.4-17.6 ng/ml, correlating with the cerebellar symptoms. Serum and CSF anti-glutamate receptor delta 2 antibodies were detected in the serum and CSF. We diagnosed him as having chronic cerebellitis associated with anti-glutamate receptor delta 2 antibody.

We report an 11-year-old boy with Bickerstaff's brainstem encephalitis (BBE). He had gait disturbance, disturbed consciousness, and diplopia after upper respiratory tract infection. On admission, he showed multiple cranial nerve palsy, muscle weakness of arms, cerebeller ataxia and generalized areflexia. The cerebrospinal fluid on day 7 revealed albuminocytologic dissociation. IgG antibodies against GQ1b and GT1a were detected in the serum. Immunoglobulin was administered intravenously from day 11, and then his symptoms gradually diminished. When he was discharged on day 27, he had neither conscious disturbance nor limb weakness. There still were mild ophthalmoparesis and diminished deep tendon reflexes, but they disappeared by 10 months after the onset. Effective therapy for BBE has yet to be established. Our case had features of Guillain-Barré syndrome (GBS) and Miller Fisher syndrome, such as an acute monophasic course, limb weakness with areflexia, albuminocytologic dissociation in the cerebrospinal fluid, detection of serum anti-ganglioside antibodies and efficacy of intravenous immunoglobulin, indicating that BBE and GBS are closely related. Our case suggested that intravenous immunoglobulin therapy, an established treatment for GBS, should be considered in some patients with BBE.

We investigated the efficacy and safety of midazolam given intravenously for the treatment of status epilepticus in children. Patients received one to three bolus injections of midazolam (0.15 to 0.40 mg/kg) until seizures disappeared completely. In other patients, continuous infusion of midazolam (0.06 to 0.48 mg/kg/hr) followed a bolus of midazolam or other anti-epileptic agents. The dose was increased every 15 minutes until the seizures were brought under control. The etiology of 62 episodes of status epilepticus was epilepsy in 43, acute encephalopathy/encephalitis in 11, febrile seizures in 7 and hypoxic encephalopathy in 1. The age of the patients ranged from 0.2 to 18.4 years (average: 3.7 years). Bolus injections were administered in 53 episodes. The average loading dose was 0.35 +/- 0.22 mg/kg (range 0.15 to 0.90 mg/kg). Of the 42 episodes in which seizures disappeared, 13 required no further continuous infusions. Of the remaining 29 in which continuous infusion was done at the rate of 0.06 to 0.60 mg/kg/hr (mean 0.30 mg/kg/hr), 21 ended in cessation of the seizures. The duration of continuous infusions ranged from 4 to 288 hours (average 49.0 hours). In 6 episodes there were adverse effects:transient hypoxemia (5) and agitation during withdrawal (1). No intervention was needed except oxygenation by mask for less than 72 hours. Midazolam is effective and safe, and can be used as the first line drug in the treatment of status epilepticus in children.

We reported the MRI findings and clinical course of two patients with ophthalmoplegic migraine. Both patients presented with unilateral oculomotor nerve palsy. Contrast enhanced MR imaging revealed unilateral enhancement and thickening of the oculomotor nerve in one patient. Prednisolone was effective in both patients, but only could transiently. On the other hand, cyproheptadine hydrochloride could completely prevent recurrent attacks of ophthalmoplegic migraine. Thus, MR imaging with of contrast enhancement is useful in the diagnosis of ophthalmoplegic migraine. Cyproheptadine hydrochloride is better than prednisolone to prevention recurrent attacks and to avoid adverse effects.

We described a child who developed paroxysmal abnormal eye movement. At the age of 12 months, she had a high fever and a febrile seizure. On the next day she showed tonic upward deviation of the eyes for 1 to 2 seconds, and downward saccades on attempted downward gaze. The upward deviation was repeated for a period of 2 to 3 hours, and disappeared during sleep. The administration of L-dopa was not effective. The symptoms subsided gradually over 10 months without other neurological impairment. She walked alone at 1 year and 6 months. At the beginning of her walking she showed truncal ataxia, but it gradually disappeared and her development was normal at the age of 2 years and 6 months. These abnormal eye movements and another symptoms were similar to paroxysmal tonic upgaze of childhood (PTU) that has been first described by Ouvrier and Billson (1988) as intermittent upward deviations of eyes. In Japan there was only one report of this syndrome with periventricular leukomalacia and hypomyelination.

We investigated the clinicopathological features of 109 patients with urothelial tumors of the renal pelvis and ureter who underwent surgery at four institute from April, 1975 to September, 1997. The patients consisted of 71 males and 38 females, and the mean age was 66.8 years, ranging from 41 to 92 years. Mean followup period was 43 months. The prognostic significance of the pathological factors; pT, Grade, INF, tumor size, pL, pV and pN were evaluated. All these factors affected the survival rates significantly in univariate analysis. Multivariate analysis showed the most influential prognostic factors to be pT and pN.

We reported a 19-year-old woman with multiple sclerosis (MS) with recurrent episodes of psychiatric symptoms at the age of 10 and 19, as the first and 11th relapse, respectively. Her first episode of MS resembled acute encephalitis at the age of 9 and she relapsed 12 times. The psychiatric symptoms were not mood disorders but altered states of consciousness, such as the delirous state. She was treated with high doses of intravenous methylpredonisolone and her symptoms were almost resolved, but her anxiety neurosis remained. Her MRI showed demyelination in the white matter of the frontal lobe and the hippocampus, which suggested that psychiatric symptoms of MS would be related to the frontal lobe lesion.

Epileptogenesis was evaluated in 60 patients with acute encephalitis and in 10 patients with acute encephalopathy. Forty-seven patients have been seizure-free during for more than three years' follow-up (Group III). On the other hand, 23 patients developed epilepsy. Among them, 18 patients developed epilepsy after a latent period of 1 month to 2 3/12 years (Group I). In Group I, a younger age of the onset, a long period of disturbed consciousness and a high activity of CSF neuron-specific enolase (NSE) was associated with refractory epilepsy. The other five patients had continuous seizures from the acute phase of encephalitis without a latent period (Group II). They had more than 2 types of partial motor seizures which occurred frequently during the acute phase of encephalitis. The NSE activity in the CSF of patients in Group II was less than 50 ng/ml, being similar to those in Group III. The epilepsy in Group II, however, was the most refractory. The reason for the development of this continuous refractory epilepsy remained obscure.

This report describes a primary testicular carcinoid. A 41-year-old male was hospitalized with an asymptomatic right testicular mass. A high inguinal orchiectomy was done after the diagnosis of the testicular tumor. Pathologically, the tumor showed the typical appearance of a carcinoid tumor. A computed tomographic scan and other studies could not demonstrate any metastasis elsewhere. He has remained well and without any evidence of recurrence.

Patients with Stage D2 prostate cancer were treated with surgical or medical (LHRH analog) castration combined with either estrogen, chlormadinone acetate or flutamide as initial therapy. The effect of each medication was compared. The overall survival, cause-specific survival and relapse-free survival were not different among the three medications. Patients given each medication were divided into two groups each according to grade, extent of diseases on bone metastases, and levels of tumor marker. Survivals of the corresponding two groups were compared with each other among different medications. No differences were revealed with any medication. There were no serious side effects in whole patients, except that grade 2 liver dysfunction was accompanied in 12% of flutamide-treated group. It is concluded that the three drugs used with castration did not make any difference in the survival of stage D2 patients, and differences between medications were seen in the frequency of side effects.

The development of the human ventral cochlear and medial superior olivary nuclei was studied. We made serial sections of the brain in 10 fetuses at 16-40 weeks of gestation (WG), an infant at 2 months of age and an adult of 63 years using an electronic planimeter with a computer. Although the shape of neurons of the two nuclei was different, our morphometric analysis showed that the development of both of them accelerates between 18 and 21 WG in terms of the columnar length and volume, neuronal size and circularity ratio and the amount of Nissl bodies increases gradually.

The electroconductive lithotripter (ECL) is a new concept for shockwave generation in which a highly conductive solution channels the discharge between the anode and cathode. Out of 152 patients treated, complete follow up data were available on 151 patients. The average number of shocks per treatment was 2,138. At 3 months the overall stone-free rate was 73.5%. Success rate, defined as stone-free or asymptomatic residual fragments measuring 4 mm or less, was 84.9% for renal and 94.9% for ureteral calculi. The overall success rate for all calculi was 89%.

To reveal the role of cortical tubers in the pathophysiology of West syndrome associated with tuberous sclerosis, we studied their numbers and topography, focusing on the differences between patients with infantile spasms and without infantile spasms. We reviewed the clinical date on the seizure types, seizure evolution and developmental status of 13 patients: 8 patients with infantile spasms (the West syndrome group) and 5 patients without infantile spasms (the non-West syndrome group). The number, size and location of the cortical tubers were evaluated on 5 mm-thick T2-weighted MR images. The average number of cortical tubers of the West syndrome group was 11.4 per patient, being larger than that of the non-West syndrome group (7.8). The West syndrome group included two patients with only one tuber. The average number of the cortical tubers in the occipital lobes of the West syndrome group was 2.4, which was larger than that of the non-West syndrome group (0.8). From these results, a large number of cortical tubers, which suggest involvement of larger cerebral cortical regions, increase the possibility of the West syndrome in patients with tuberous sclerosis. On the other hand, some patients with only one or few cortical tubers developed West syndrome, which may suggest the presence of a region critical on the development of West syndrome. Our results also suggest that lesions in the occipital lobes might be more significant than those in other lobes. However, the results of this study are diverse, to some extent, which may have resulted from variable epileptogenicity of the cortical tubers. It is necessary to conduct further study, including a greater number of patients, to reveal topographic pathophysiology in West syndrome.

Lissencephaly type I is a diffuse type of migration disorder that contains agyria and/or pachygyria on the brain surface. We experienced 5 cases of this disease and evaluated their electroencephalographic findings and seizure types based on the neuroradiological classification of lissencephaly. Ages at seizure onset ranged from 2 months to 4 months (mean 3.2 months). The patients with complete agyria had generalized tonic seizures, and those with pachygyria partial seizures or tonic spasms. The characteristic findings of complete agyria in electroencephalogram were high-voltage alfa activity. The amount of high-voltage slow waves increased with the ratio of pachygyria on the brain surface. The appearance of multifocal spikes and sharp waves suggested irregular arrangement of pachygyria on the brain surface.

We report five cases of multiple sclerosis (MS) and three cases of allied demyelinative diseases starting during childhood. Three of the MS patients presented with atypical initial symptoms, such as acute encephalitis or myelitis, making an early clinical diagnosis difficult. Ophthalmologic symptoms were noted in four of MS children, and in two with allied demyelinative diseases. Therefore, if a child shows ophthalmologic symptoms (i.e. optic neuritis, ophthalmoplegia), brain magnetic resonance imaging (MRI) should be conducted for the differential diagnosis of MS and other demyelinative diseases. Cerebrospinal fluid analysis is not useful for the initial diagnosis of MS, because pleocytosis and increase of oligoclonal IgG band in cerebrospinal fluid are seen in both MS and other demyelinative disorders. However, neuron specific enolase (NSE) is slightly higher in the latter than in the former. T2-weighted MRI of multiple sclerosis showed multiple high intensity areas in the white matter of the cerebrum and cerebellum, capsula interna, and crus cerebri etc. Most of these lesions were clinically silent, being characteristic of MS. In two MS cases, however, initial MRI revealed no abnormal findings. Thus, the diagnosis of MS can not be made by initial MRI only.

Patients with prostate cancer treated during 1986-95 were divided into two groups: the first five years as early period and the recent five years as late period. Background factors, therapeutic modality and prognosis were compared between these two groups. The number of patients during the late period (245) was about 1.5 fold that during the early period (161). The average age for each stage are similar within the same period, and also similar between early and late periods. Almost the same ratios were noticed on stage distribution and on histological grade between early and late periods. Therefore, the increase in the number of patients in recent years was due mainly to expansion with the same proportion of background factors. Patients with T2 and T3 during the late period received more aggressive treatments with/without endocrine therapy. Subsequently these patients seemed to show better prognosis than the patients during the early period. No differences in survival of patients with metastosis was revealed between these two periods. Therefore, the outcome was not improved by recent endocrine therapy.

To predict the prognosis of tuberous sclerosis with West syndrome, we studied the relation between the cortical tubers and the neurological evolution. We reviewed the clinical data on the seizure evolution and developmental status of 7 patients (3 males and 4 females) and estimated the number, size and location of cortical tubers on 5 mm-thick T2-weighted MR images. The cortical tubers were grouped into categories: small (10 mm or less in maximum diameter), medium (10 to 25 mm) and large (25 mm or more). The first MRI study was performed at ages form 3 months to 18 years, and the follow-up study was performed on 6 out of the 7 patients. We also estimated the interval change of cortical tubers. The average number of cortical tubers was 12.1 per patient, being larger than the values previously reported for the patients of tuberous sclerosis without West syndrome. However, the numbers varied widely from 1 to 29. Two patients with good outcome had more than 10 tubers, whereas two patients with poor outcome had less than 5 tubers. All the patients with good outcome were female. Follow-up MRI in one patient revealed a marked increase in the number of cortical tubers, from 15 (at the age of 5 months) to 23 (at 4 years 2 months), which probably resulted from physiological hypomyelination during infancy. Some tubers corresponded to a electroencephalographic focus, whereas others did not. There was no difference in the topography of cortical tubers between the patients with good prognosis and those with poor prognosis. Thus, it was hard to make the prognosticate a case of tuberous sclerosis based solely on the number and topography of cortical tubers on MRI.

We reported serial clinical, radiological, and neurophysiological findings of a patient with PEHO (progressive encephalopathy with edema, hypsarrhythmia and optic atrophy) syndrome. The case was a 4-year-and-8-month-old boy. He had no apparent problems during pregnancy, but after his birth severe hypotonia and developmental delay were evident. Infantile spasms appeared at 3 months of age, and progressive opisthotonic posture and loss of his visual acuity at 8 months. Eye fixation was lost, and optic atrophy was observed. Seizures and progressive atrophy of the cerebellum and brainstem developed during the same period. These findings of our case support the hypothesis that brainstem lesions are related to infantile spasms.

We describe a 3-year-old boy with subacute sclerosing panencephalitis (SSPE) who died 4 months after its onset. His initial symptoms were drowsiness and left hemiplegia. He became comatose in 10 days, and developed a decortical posture after 45 days. He suffered from multiple cerebral hemorrhage and infarction 3 months later. Oligodendrocytes were positively stained by immunocytochemical stain with a complement-fixing measles antibody. Light microscopy revealed glial nodules, perivascular cuffing and reactive gliosis. Small arteries showed intimal thickening with resultant occlusion and occasional recanalization. These findings suggested vascular involvement in SSPE. This case illustrates the difference between the fulminant and chronic forms of SSPE.

Clinical effects of estramustine phosphate (EMT) on hormone refractory prostate cancer were studied. Prostate cancer relapsed in 70 of the 259 patients with stage C and D diseases who had initially responded to endocrine therapy. After cancer relapse, endocrine therapy was changed to oral administration of EMT in 21 patients, while initial endocrine therapy was continued in 14 and additional radiation therapy was given in 35. A partial response or no change was observed in 11 of the 21 patients (52%) given EMT therapy, the mean duration of the response being 14.2 months. The 21 patients given EMT therapy survived significantly longer than the 14 patients with continued on endocrine therapy, and those responding to EMT therapy tended to have a better survival than those unresponsive. Side effects of EMT included loss of appetite in 2 patients and edema of the lower limb in 1, but they were not severe enough to require discontinuation of the drug. EMT may be a useful drug for patients with advanced prostate cancer with relapse after endocrine therapy.

An 85-year-old female, who had had a total hip replacement for osteoarthritis in 1985, presented with gross hematuria in June 1995. Cystoscopy revealed a non-papillary sessile tumor in the right side wall of the urinary bladder with a crateriform opening on the top. Computed tomographic (CT) scan and excretory urography demonstrated a fistulous communication between the bladder and the acetabular bed filled with cement. The biopsy of the mass lesion suggested inflammatory granuloma with powdered cement. Since the lesion was composed of internal fistula, and was not accompanied with infection, the patient was treated conservatively. No external fistula had formed during the 5 months of observation.

We reported two cases of anterior spinal artery syndrome in childhood evaluated by MRI of the spinal cord. The T2-weighted MRI revealed high signal intensity in the anterior region of spinal cord in both cases. These findings might be related with edema of the ischemic region. Several years later, the regions of the spinal cord showed atrophic changes. The high signal intensity on the T2-weighted MRI was characteristic of the anterior spinal artery syndrome.

A case of 1-month-old female infant with hemimegalencephaly was reported. This disorder is a rare malformation characterized by congenital hypertrophy of one hemisphere and ipsilateral ventriculomegaly. Clinical signs of the patient included a left sided macrocephaly, intractable seizures and delayed development. Ictal single photon emission computed tomography (SPECT) on the 49th day using 99mTc-HMPAO showed hyperperfusion in the left occipital and frontal lobes, when ictal EEG showed a sharp wave-burst appearing continuously in the left occipital lobe that spread to the left frontal lobe. Interictal SPECT on the 48th day demonstrated and increased tracer accumulation in the left hemisphere, especially in the left frontal lobe. In this case, the megalencephalic hemisphere might have an important role for epileptogenesis and its spread according to the ictal SPECT findings.

Sequential examinations of single photon emission computed tomography (SPECT) were performed on a 5-month-old boy with acute infantile hemiplegia from the acute ictal stage. SPECT was performed with technetium-99m-hexamethyl-propylene-amineoxime during the status when the patient had left-hemiconvulsions (status epilepticus) and on the 3rd, 7th and 10th day after the status. During the ictal stage and the 3rd day after the status, diffuse hyperperfusion was revealed in the right hemisphere, while diffuse hypoperfusion was exhibited in the right hemisphere on the 7th and 10th day after the status. Hypoperfusion in the corresponding hemisphere, after the status, has been reported in patients with acute infantile hemiplegia. There has, however, been no report of ictal brain perfusion. The pathogenesis of this hyperperfusion is not clear, but alteration of brain perfusion in this patient, especially in acute phase, may help to elucidate the etiology of acute infantile hemiplegia.

The first nationwide research into adult bronchial asthma in Japan proposed a new classification of adult asthma. Adult asthma was categorized into child onset asthma, adult onset asthma and adult relapse asthma. The frequency of child onset asthma, adult onset asthma and adult relapse asthma in adult asthma was 11.2%, 77.3% and 3.7%, respectively. The frequency of child onset asthma decreased markedly in the older age group. On the other hand, the frequency of adult onset asthma increased, and reached more than 90%, in the older age group. The frequency of the following factors: atopic asthma, complications with other atopic diseases, mild asthma, male patients, experience of mechanical ventilation, visits to night clinics and oxygen therapy on acute attack, was significantly higher in the child onset asthma group than in the adult onset asthma group. The frequency of infectious type, aspirin intolerance, steroid dependent asthma, severe asthma and regular medication was significantly higher in the adult onset asthma group. Adult relapse asthma seemed to fall between these two groups. Based on the above observations, we proposed a new classification of adult asthma which includes child onset asthma, adult onset asthma and adult relapse asthma.

From September through November 1990, an outbreak of hemorrhagic colitis associated with Escherichia coli O 157: H 7, occurred in a kindergarten in Saitama, Japan. Some of the patients suffered from neurological symptoms such as stupor, deep coma and/or convulsions in the acute stage, and/or action tremors, nystagmus, incontinence, phrenic nerve palsy in the later stage. Serum complements decreased more in the patients with neurological symptoms than in the patients without them. The verotoxin elaborated by E. coli O 157: H 7 was considered to cause the neurological symptoms, on the basis of their clinical courses and laboratory findings, especially the cerebrospinal fluid findings.

We studied the effect of tranilast on the growth of carrageenin-induced granulation and the increase in capillary permeability induced by inflammatory agents in rats. In the carrageenin-induced granulation model, tranilast (50 or 100-200 mg/kg, p.o.) decreased significantly and dose-dependently the weight and the hydroxyproline content of the granulation tissue. Tranilast, however, showed no effect on the healing day of locally wounded dorsal skin of rats. Triamcinolone (10 mg/kg, p.o.) also showed an inhibitory effect on the carrageenin-induced granulation model. Tranilast (50-400 mg/kg, p.o.) dose-dependently inhibited the enhancement of capillary permeability induced by the Ca ionophore A23187, bradykinin and xanthine oxidase. Moreover, tranilast (30 and 300 microM) suppressed superoxide production induced by FMLP in human neutrophils, but did not act as a superoxide scavenger. Considering that hypertrophic scar and keloid are conditions characterized by abnormal cell proliferation and excessive collagen accumulation accompanied with itch and pain, these results suggest that tranilast is useful as a therapeutic drug for hypertrophic scars and keloids.

We studied the inhibitory effects of tranilast, an anti-allergic drug, on the human keloid tissues implanted into the dorsal skin of athymic nude mice and on the growth of keloid fibroblast in vitro. In the keloid tissue-implanted model, tranilast (50-200 mg/kg, p.o.) decreased the weight of the keloid tissue as triamcinolone (25 mg/kg, p.o.) did. Tranilast (200 mg/kg, p.o.) reduced the hydroxyproline content of implanted tissues. Tranilast (3-300 microM) also inhibited the collagen synthesis by keloid fibroblast in vitro. Only a high concentration of tranilast (300 microM) suppressed the glycosaminoglycan synthesis and cell proliferation of keloid fibroblasts. Moreover, tranilast scarcely affected the fibronectin production. Triamcinolone (10 microM) also inhibited glycosaminoglycan synthesis and cell proliferation. These results suggest that the inhibitory effect of tranilast on the keloid tissues is related to its inhibition of the collagen synthesis of fibroblasts. Tranilast would be useful as a therapeutic drug for the treatment of keloids.

Effects of low molecular weight heparin (FR-860) on experimental disseminated intravascular coagulation (DIC) models in rats were compared with those of conventional unfractionated heparin (UF-heparin) and other anticoagulants. In the endotoxin-induced DIC model, FR-860 (12.5-200 U/kg/hr) and UF-heparin (25-200 U/kg/hr) inhibited dose-dependently the decreases in platelet counts, fibrinogen, antithrombin III activity and alpha 2-plasmin inhibitor activity, and they also inhibited the increases in fibrin de-products and thrombus formation in the glomerular capillary bed. Neither gabexate mesilate (FOY, 10 mg/kg/hr) nor nafamostat mesilate (FUT, 0.1 mg/kg/hr) improved endotoxin-induced DIC. FR-860 showed comparable potency to UF-heparin in plasma anti-factor Xa (F.Xa) activity. However, FR-860 was weaker than UF-heparin in prolongation of activated partial thromboplastin time. In the thrombin-induced DIC model, both FR-860 and UF-heparin significantly improved, as seen in the endotoxin-induced DIC model, the changes in coagulation and fibrinolytic parameters and suppressed the production of pulmonary thrombus. On the other hand, both FOY and FUT showed significant but weak improvement in this model. In addition, FR-860 inhibited the enhancement of fibrinolysis and the production of pulmonary thrombus in the lactic acid-induced DIC model. These results suggest that the efficacy of FR-860 on DIC in rats is comparable to that of UF-heparin and that the efficacy can be attributed to its anti-F.Xa activity. FR-860 can be expected to be a useful therapeutic drug for DIC.

Acute polyradiculoneuritis relapses rarely, and autonomic nerve involvement is less frequent than motor cranial nerve involvement. An 11-year-old boy exhibited acute relapsing polyradiculoneuritis associated with two types of autonomic dysfunction of pupils 6 months after the onset of the disease. Parasympathetic dysfunction of pupils with ocular palsies preceded neuropathy of limbs at the onset. Thereafter, sympathetic dysfunction of pupils without ocular palsies was revealed by pupillography at the 2nd attack of limb palsies.

Crossed cerebellar diaschisis (CCD) in twenty five children with hemiplegia were studied using single photon emission computed tomography (SPECT) with N-isopropyl-p-I-123-iodoamphetamine. Seven of twenty-five patients had cerebral palsy, and the others were impaired by acquired brain injury between ten months and fourteen years of age. CCD was demonstrated in five patients (20%), who were impaired by acquired brain injury after seven years of age. CCD could never be detected in patients with cerebral palsy. Ipsilateral cerebellar diaschisis was also demonstrated in two patients with cerebral palsy and three with early acquired brain injury before three years of age. It is suggested that diaschisis presents itself as a different form in a contralateral and ipsilateral cerebellum before three years of age from a form which presents after seven years of age.