A case of acute encephalitis with refractory,repetitive partial seizures (AERRPS) showing transient disappearance of the seizure with occurrence of choreo-ballistic movement

We present here a 5-year-old girl with acute encephalities with refractory, repetitive partial seizures (AERRPS), a new clinical entity defined by the following five criteria: 1. acute encephalitis with a prolonged acute phase of more than 2 weeks, 2. persistent partial seizures with identical phenotype both in the acute and recovery phase, 3. seizures frequently evolving into convulsive status especially during the acute phase, 4. extremely intractable, and 5. no causative lesion or agent is identified. Interestingly, her seizures had completely diminished from the fifty-sixth day of her illness with concomitant appearance of choreo-ballistic involuntary movements. After the 120th day of the illness, seizures evolved again, though the involuntary movements persisted. This transient disappearance of intractable seizures might provide a clue to the pathophysiology of seizures in AERRPS.     

Interobserver discrepancy using the 1998 World Health Organization/International Society of Urologic Pathology classification of urothelial neoplasms: practical choices for patient care

PURPOSE: Morphological classifications designed by experts to stratify neoplasms according to biological potential must define categories that are reproducible among practitioners or the schemes actually create the heterogeneous populations that they seek to avoid. The application of the 1998 World Health Organization/International Society of Urologic Pathology scheme for urothelial neoplasms was studied in a community practice setting. We documented interpretive discrepancies for each category of neoplasm and determined whether a period of pathologist education may have a positive effect on the frequency of discrepant interpretations. The results suggest that patients may benefit from modifying the classification system. MATERIALS AND METHODS: A consecutive series of specimens was divided into learning and study sets that were each independently examined by 3 pathologists. Specimens in the learning set were interpreted without previous structured education, while those in the study set were interpreted immediately after intensive education. Interpretations for each specimen were compared and interpretive discrepancies were analyzed. RESULTS: Case distribution after education was similar among the pathologists but interpretations for any particular specimen often differed. The level of interpretive discrepancies varied according to the morphological similarity among categories in the classification scheme and was not necessarily decreased by education. When pathologists were required to discriminate between papillary urothelial neoplasm of low malignant potential and low grade carcinoma, the discrepancies were 50% after education compared with 39% before education. In contrast, there were no discrepancies when the discrimination was between papillary urothelial neoplasm of low malignant potential and high grade carcinoma or carcinoma in situ. Eliminating categories with poor reproducibility markedly improved the likelihood of unanimous agreement among practitioners but a probably irreducible level of 10% discrepancies remained. CONCLUSIONS: The 1998 World Health Organization/International Society of Urologic Pathology classification of urothelial neoplasms requires certain discriminations that cannot be reliably made by practitioners. Modifying the scheme to create categories of low grade neoplasm and high grade carcinoma would markedly increase its practical value to patients without significantly altering patient care.     

Oxidative stress and disturbed glutamate transport in spinal muscular atrophy

Spinal muscular atrophy (SMA) is a hereditary motor neuron disease, and three clinical subtypes of autosomal recessive SMA, including Werdnig Hoffmann disease (type 1), have been shown to be induced by deletion within the same genes. In order to clarify the pathogenesis of motor neuron degeneration in SMA, we immunohistochemically examine the expressions of oxidative stress-related materials (oxidative products) and glutamate transporters, which can prevent glutamate neurotoxicity, in five autopsy cases of SMA type 1. Age-matched controls did not show any deposition of oxidative products in the brain. In contrast, the abnormal deposition of 4-hydroxy-2-nonenal-modified protein, a product of membrane lipid oxidation, was observed in the spinal motor neurons in three cases, although the motor neurons did not show an increase of nitrotyrosine, which was observed in adult-onset amyotrophic lateral sclerosis. In addition, the nuclei of neurons and glial cells in the precentral gyrus, thalamus or cerebellar cortex were immunoreactive for 8-hydroxy-2′-deoxyguanosine in two cases, which was one of the most commonly used markers for oxidative DNA damage. Regarding glial glutamate transporters, three of five cases of SMA type 1 showed a reduction in immunoreactivity for excitatory amino acid transporter-1 (GLAST) in the ventrolateral nucleus of the thalamus, in which there was neither neuronal loss nor gliosis in routine histochemistry. One case, having mechanical ventilation, demonstrated a reduced expression of another glial glutamate transporter (GLT-1) throughout the central nervous system. These data suggest that oxidative stress and disturbed glutamate transport can partly be involved in the motor neuron devastation and/or latent thalamic degeneration in SMA type 1.     

A case of medial temporal lobe epilepsy associated with occult focal cortical dysplasia in the lateral temporal neocortex]

A 29-year-old male with medial temporal lobe epilepsy(MTLE) was revealed to have "occult" focal cortical dysplasia(FCD) in the lateral temporal neocortex. He had no history of febrile convulsion and developed complex partial seizure at the age of 14 year, which became intractable. Although MRI failed to reveal structural abnormality in the temporal lobe, even retrospectively, the findings of non-invasive preoperative examination, such as video-EEG monitoring and interictal ECD-SPECT and FDG-PET, were consistent with those of the left MTLE. Intraoperative electrocorticography(ECoG) demonstrated almost continuous paroxysmal activities on the anterior part of the inferior temporal gyrus(ITG). Anterior temporal lobectomy(ATL) with hippocampectomy was performed. Histological examination revealed FCD in the small area with 0.8 mm in diameter of the resected ITG. In the ATL without preoperative invasive examination such as chronic subdural electrode recording, intraoperative ECoG recording is mandatory.     

Socioeconomic factors and cancer incidence, mortality, and survival in a metropolitan area of Japan: a cross-sectional ecological study

Cancer mortality is generally high in people of low socioeconomic status compared with people of high socioeconomic status (SES). Although these differences in mortality may be caused by differences in cancer incidence and survival, analysis of these factors has rarely been conducted. The objective of our cross-sectional ecological study was to analyze socioeconomic differences in cancer incidence, mortality and survival in a metropolitan area of Japan. The age-adjusted cancer incidence rates, age-adjusted mortality rates, relative 5-year survival, and proportions of early stage cancer were calculated for 67 municipalities in Osaka, Japan. For area-based socioeconomic variables, we used the percentages of male unemployment, college or graduate school graduates, home ownership, households receiving government assistance, and households below the subsistence habitation level in each municipality. We performed linear regression taking each municipality's population as weight to examine the relationships between measurements relating cancer and socioeconomic variables. Factor analysis of socioeconomic variables was carried out to determine whether a particular socioeconomic variable tended to be associated with another. Cancer incidence, cancer mortality, 5-year cancer survival, and proportion of early stage cancer were highly correlated with each socioeconomic variable at the municipality level. Five area-based socioeconomic variables could be explained by three factors: economic status, housing characteristics and educational attainment. Despite the major limitation of a lack of individual information about socioeconomic characteristics and outcomes related to cancer, we hypothesize that a municipal area's socioeconomic status might be a predictor of individual incidence, mortality, and survival of cancer.     

Duration of cerebrospinal fluid drainage in patients with aneurysmal subarachnoid hemorrhage for prevention of symptomatic vasospasm and late hydrocephalus

The optimal duration of cerebrospinal fluid (CSF) drainage after acute aneurysm surgery is unclear. The association between the occurrence of symptomatic vasospasm or late hydrocephalus and the duration of CSF drainage was investigated using multiple logistic analysis in 95 consecutive patients with aneurysmal subarachnoid hemorrhage who underwent surgery within 72 hours after onset. The duration of drainage was significantly related to the occurrence of symptomatic vasospasm and late hydrocephalus. The cut-off values of the duration of drainage for preventing symptomatic vasospasm and late hydrocephalus were 11 days (adjusted odds ratio 0.347, 95% confidence interval 0.135-0.889, p = 0.0274) and 6 days (adjusted odds ratio 4.86, 95% confidence interval 1.46-16.2, p = 0.0099), respectively. Prevention of both symptomatic vasospasm and late hydrocephalus is not possible without additional procedures such as cisternal irrigation using fibrinolytic agents.     

Health characteristics of elderly Japanese requiring care at home

The number of elderly subjects requiring care is rapidly increasing, however, their health status has not been well studied. The purposes of this study were to describe the health characteristics of the elderly at home who were using long-term care insurance, and to clarify factors that influence dependence for activities of daily living (ADL). The subjects were 194 elderly people living at home, who were approved for care. The items surveyed were the demographic characteristics, care level (or level of dependence for ADL), Barthel index, grip strength, thigh muscle volume, cognitive impairment using the mini-mental state examination (MMSE), depressed mood using the geriatric depression scale (GDS-15), and serum albumin and hemoglobin levels. The grip strength (p=0.0001), thigh muscle volume (p=0.0030), MMSE score (p=< 0.0001) and serum albumin level (p= < 0.0001) decreased, while the GDS-15 score (p=0.0142) increased with deteriorating care levels. The proportion of subjects not requiring assistance for the items "bathing" and "stair-climbing" in the Barthel index was markedly low in the subjects belonging to the relatively low (mild) care levels. The logistic regression analysis showed that factors associated with dependence for "bathing" and "stair-climbing" were the use of day-services, male sex, decline of grip strength, and a high GDS-15 score. In conclusion, it is important to strengthen the muscles of the upper half of the body, and to correct depressed moods for maintaining levels of ADL in the elderly requiring care at home.     

AN AUTOPSY CASE OF NIEMANN-PICK DISEASE

An autopsy case of Niemann-Pick disease whichμ was characterized by hepatosplenomegaly and lymphadenopathy is reported. On microscopic examination, numerous foamy cells were observed in the liver, spleen, kidneys, bone marrow, lungs, thymus, intestine and general lymph nodes. These cells were generally negative for periodic acid-Schiff (PAS) stain and positive for staining of phospholipid. Based upon the morphologic characteristics of Niemann-Pick disease, its relation to other lipidosis is discussed.     

Brief Atonia Associated with Electroencephalographic Paroxysm in an Infant with Infantile Spasms

The loss of muscular tone or muscular inhibition associated with sharp waves observed during abnormal tonic posture in a patient with infantile spasms of early onset is described. The latency between the beginning of a sharp wave and the muscular inhibition varied from 30 to 700 msec, and the duration of the inhibition ranged from 100 to 400 msec. These brief muscular inhibitions may sometimes precede tonic seizures, and these phenomena might be noticeable only in the state of steady tonic muscular contraction.