Mental deterioration in childhood epilepsy

Mental retardation is detected in 20-30% of children with epilepsy at hospitals specializing in treatment of childhood epilepsy. However, the incidence of mental deterioration in childhood epilepsy is not high. In this study, mental deterioration was found in 52 (1.8%) of the 2,880 children with epilepsy at Okayama University Hospital. The patients showing mental deterioration mostly suffered from specific epileptic syndromes, such as West syndrome, Lennox-Gastaut syndrome, severe myoclonic epilepsy in infancy and epilepsy with continuous spike-waves during slow wave sleep. These types of epilepsy show generalized electroencephalographic (EEG) abnormalities. It is presumed that mental deterioration is caused by the total effects of prolonged diffuse EEG abnormalities and the age of the patients. Antiepileptic drugs exert a relatively minor effect on mental deterioration.</p

Long-term follow-up of an individual with vitamin B6-dependent seizures

We report on a 31-year-old female with vitamin B6-dependent seizures whose seizure onset was in the neonatal period. Her elder brother had the same disorder and died in infancy. Administration of vitamin B6 was initiated in the postnatal period. At the age of 12 years 1 month, 2 months after withdrawal of vitamin B6, visual seizures began to occur frequently. Myoclonic seizures and occasional generalized convulsive seizures were also observed. At the same time, photoparoxysmal response and spontaneous diffuse spike–wave bursts were seen on her EEG. Myoclonic seizures were provoked by intermittent photic stimulation during the EEG. It is distinctive that visual seizures were one of the main seizure types in this patient, that her clinical course was relatively benign, and that she has normal intellectual outcome.</p

Long-term follow-up of an individual with vitamin B6-dependent seizures

We report on a 31-year-old female with vitamin B6-dependent seizures whose seizure onset was in the neonatal period. Her elder brother had the same disorder and died in infancy. Administration of vitamin B6 was initiated in the postnatal period. At the age of 12 years 1 month, 2 months after withdrawal of vitamin B6, visual seizures began to occur frequently. Myoclonic seizures and occasional generalized convulsive seizures were also observed. At the same time, photoparoxysmal response and spontaneous diffuse spike–wave bursts were seen on her EEG. Myoclonic seizures were provoked by intermittent photic stimulation during the EEG. It is distinctive that visual seizures were one of the main seizure types in this patient, that her clinical course was relatively benign, and that she has normal intellectual outcome.</p

A Pilot Study: The Beneficial Effects of Combined Statin-exercise Therapy on Cognitive Function in Patients with Coronary Artery Disease and Mild Cognitive Decline.

Objective Hypercholesterolemia, a risk factor in cognitive impairment, can be treated with statins. However, cognitive decline associated with "statins" (HMG-CoA reductase inhibitors) is a clinical concern. This pilot study investigated the effects of combining statins and regular exercise on cognitive function in coronary artery disease (CAD) patients with prior mild cognitive decline. Methods We recruited 43 consecutive CAD patients with mild cognitive decline. These patients were treated with a statin and weekly in-hospital aerobic exercise for 5 months. We measured serum lipids, exercise capacity, and cognitive function using the mini mental state examination (MMSE). Results Low-density lipoprotein cholesterol levels were significantly decreased, and maximum exercise capacity (workload) was significantly increased in patients with CAD and mild cognitive decline after treatment compared with before. Combined statin-exercise therapy significantly increased the median (range) MMSE score from 24 (22-25) to 25 (23-27) across the cohort (p&lt;0.01). Changes in body mass index (BMI) were significantly and negatively correlated with changes in the MMSE. After treatment, MMSE scores in the subgroup of patients that showed a decrease in BMI were significantly improved, but not in the BMI-increased subgroup. Furthermore, the patients already on a statin at the beginning of the trial displayed a more significant improvement in MMSE score than statin-naïve patients, implying that exercise might be the beneficial aspect of this intervention as regards cognition. In a multivariate logistic regression analysis adjusted for age &gt;65 years, sex, and presence of diabetes mellitus, a decrease in BMI during statin-exercise therapy was significantly correlated with an increase in the MMSE score (odds ratio: 4.57, 95% confidence interval: 1.05-20.0; p&lt;0.05). Conclusion Statin-exercise therapy may help improve cognitive dysfunction in patients with CAD and pre-existing mild cognitive decline

Proton Magnetic Resonance Spectroscopy in Patients with Migration Disorders

Proton Magnetic Resonance Spectroscopy (1H-MRS) can be used to detect cerebral metabolites including N-acetylaspartate (NAA)，creatine (Cr) and choline (Ch). Hence，clinical applications of this method for neuropediatric diseases can be expected. However，regarding neuronal migration disorders，there have been only a few reported studies. We therefore examined the lH-MRS in six patients with migration disorders，ages ranged from 8months to 28years 10months with a mean of 10years 10months. Investigation was performed using Magnetom H15 (Siemens) with a repetition time of 1500 msec and an echo time of 270msec. The ratio of NAA/Cr，Ch/Cr were examined. The volume of interest with the size of 2 × 2 × 2 ～ 3 × 3 × 5cm3 was chosen in the area including lesions，and a contralateral area without lesions was also investigated. Results were as follows. 1) The ratio of NAA/Cr was low in the area with lesions in all 6cases; 1.41，1.95，2.27 and 1.71 in cases with heterotopic gray matter，0.99 in one case with polymicrogyria，an d 1.30 in one case with hemimegalencephaly，contrasted with a contralataral area without lesions: 1.89， 2.89，2.87，2.55，3.26，2.03，respectively. 2) The ratio of Ch/Cr showed no consistent difference between the area including lesions and contralataral area without lesions. Our findings of a decreased NAA/Cr ratio can be inferred to reflect the decreased numbers of neuronal cell population，or reduced metabolism in the lesions

DISPERSAL PATTERN OF JUVENILES OF EMERGENT, CANOPY AND SHADE-TOLERANT TREE SPECIES ON FLOOD-PLANE FOREST AREA AT BERAU, EAST KALIMANTAN

Pola penyebaran anakan jenis pohon emergent (Shorea sp.), kanopi (Maduca malaccensis) dan toleran naungan (Aglaia tomentosa) telah diamati pada hutan dataran rendah luapan banjir di Berau, Kalimantan Timur. Hasil penelitian menunjukkan bahwa, penyebaran anakan ketiga jenis pohon tersebut sangat dipengaruhi oleh agen dispersal buah. Anakan Shorea sp., yang mana memiliki buah tipe samara, sebagian besar tersebar pada bagian Utara-Barat pohon induknya, mengikuti arah angin yang menerbangkan buahnya. Anakan dua jenis lainnya, yang mana buahnya yang bertipe berry sangat disukai oleh binatang mamalia, menyebar secara lebih merata. Struktur populasi anakan ketiga jenis tersebut menunjukkan kecenderungan yang berbeda. Shorea sp. menunjukkan struktur populasi berbentuk huruf J terbalik yang sangat tajam, sedangkan struktur populasi anakan dua jenis lainnya cenderung berbentuk garis horizontal. Perbedaan struktur populasi tersebut terkait dengan toleransi jenis-jenis tersebut terhadap naungan. Buah Shorea sp. yang jumlahnya melimpah segera berkecambah setelah jatuh, namun pertumbuhan anakan selanjutnya tertahan oleh naungan kanopy hutan yang sangat rapat. Key words : Dispersal, Juvenil, Emergent, Canopy, Shade-toleran

Association Between Visceral Adipose Tissue Area and Coronary Plaque Morphology Assessed by CT Angiography

ObjectivesWe sought to investigate the association between visceral adipose tissue (VAT) with the presence, extent, and characteristics of noncalcified coronary plaques (NCPs) using 64-slice computed tomography angiography (CTA).BackgroundAlthough visceral adiposity is associated with cardiovascular events, its association with NCP burden and vulnerability is not well known.MethodsThe study population consisted of 427 patients (age 67 ± 11 years; 63% men) with proven or suspected coronary artery disease who underwent 64-slice CTA. We assessed the presence and number of NCPs for each patient. The extent of NCP was tested for the difference between high (≥2) and low (≤1) counts. We further evaluated the vulnerable characteristics of NCPs with positive remodeling (remodeling index >1.05), low CT density (≤38 HU), and the presence of adjacent spotty calcium. Plain abdominal scans were also performed to measure the VAT and subcutaneous adipose tissue area.ResultsA total of 260 (61%) patients had identifiable NCPs. Multivariate analyses revealed that increased VAT area (per 1 standard deviation, 58 cm2) was significantly associated with both the presence (odds ratio [OR]: 1.68; 95% confidence interval [CI]: 1.28 to 2.22) and extent (OR: 1.31; 95% CI: 1.03 to 1.68) of NCP. Other body composition measures, including subcutaneous adipose tissue area, body mass index, and waist circumference were not significantly associated with either presence or extent of NCP. Increased VAT area was also independently associated with the presence of NCP with positive remodeling (OR: 1.71; 95% CI: 1.18 to 2.53), low CT density (OR: 1.69; 95% CI: 1.17 to 2.47), and adjacent spotty calcium (OR: 1.52; 95% CI: 1.03 to 2.27).ConclusionsIncreased VAT area was significantly associated with NCP burden and vulnerable characteristics identified by CTA. Our findings may explain the excessive cardiovascular risk in patients with visceral adiposity, and support the potential role of CTA to improve risk stratification in such patients

Cerebral blood flow velocity in handicapped children

Using a transcranial Doppler blood flowmeter, the blood flow velocity (BFV) ratio of the middle cerebral artery (MCA) to the basilar artery (BA) was investigated in 12 patients with severe motor and intellectual disability syndrome. The BFV of the MCA was also investigated in 58 handicapped children, classified according to the severity of their motor and intellectual disability. The ratio of the MCA to the BA was lower by 2 SD from the mean of our previously reported standard value in 8 out of the 12 cases with severe motor and intellectual disability syndrome, suggesting a more profound decrease in the level of brain activity in the MCA area than that of the BA area. The BFV of the MCA mainly decreased in cases belonging to the category of the most severe motor disability (bed-ridden). Hence, it is suggested that motor disability is the main factor related to the decrease in the BFV of the MCA.</p

Long-term Follow-up of Orthodontic Patient with Cervical Lymphangioma

Aim: To report a treatment case of mandibular deviation caused by congenital cervical lymphangioma with traditional orthodontic techniques, following-up by 10-year retention. Background: Lymphangiomas, developmental anomalies, can induce various disturbances of swallowing, mastication, speech, breathing, and skeletal deformities as well as psychological stress and anxiety for the patient and their family. Lymphangiomas are benign with virtually no possibility of turning into a malignant lesion, so clinical management aims to treat the patient functionally. Case description: A girl, aged 6 years and 4 months, complained about facial asymmetry and anterior crossbite caused by congenital cervical lymphangioma. Her facial profile was the straight type with an adequate lip position. Anterior and right-side posterior crossbites were observed. On the frontal cephalogram, the menton shifted 3.0 mm to the right. A functional appliance with an expander was placed to correct her dental midline deviation and posterior crossbite. After 2-year treatment, the anterior and right-side posterior crossbites were improved. Multibracket treatment began after the growth spurt. After 44-month active treatment, a functional occlusion, including a Class I molar relationship with a proper interincisal relationship, was achieved. A functional occlusion was maintained during a 10-year retention period, while a mandibular downward growth was observed through the retention period. Conclusion: Conventional orthodontic techniques enable functional and stable occlusion even in patients with mandibular deviation caused by congenital cervical lymphangioma, although only using early orthodontic management by itself may have some limitations. Clinical significance: The hybrid technique combining functional appliance and intermaxillary elastics proves to be an effective therapy for correcting occlusal cant and mandibular deviation caused by cervical lymphangioma

Expansion of CpG Methylation in the SFRP2 Promoter Region during Colorectal Tumorigenesis

Secreted frizzled-related protein 2, (SFRP2) is a Wnt inhibitor whose promoter CpGs were recently found to be methylated at high frequency in colorectal cancers (CRCs). We hypothesized that the pattern of SFRP2 methylation may differ throughout the promoter during progressive tumorigenesis. Using combined bisulfite restriction analysis (COBRA), two methylation-sensitive regions (Regions A and B) of the SFRP2 promoter were investigated in 569 specimens of colorectal tissue:222 CRCs, 103 adenomatous polyps (APs), 208 normal colonic mucosa from CRC patients (N-Cs), and 36 normal colonic mucosa from subjects with no evidence of colorectal neoplasia at colonoscopy (N-Ns). Extensive (including both Regions A and B) and partial (either Region A or B) SFRP2 methylation levels were found in 61.7% and 24.8% of CRCs, 8.7% and 37.9% of APs, 3.9% and 39.9% of N-Cs, and 0% and 30.6% of N-Ns, respectively. Extensive methylation of the SFRP2 promoter was present primarily in CRCs, while partial methylation was common in APs. Whereas APs with the KRAS mutant showed no correlation to any pattern of SFRP2 methylation, extensive methylation of the SFRP2 promoter was significantly associated with KRAS mutant CRCs (p＜.0001), suggesting that genetic alteration in the RAS-RAF pathway might precede the spread of CpG methylation through the SFRP2 promoter, which is observed in over 60% of advanced colorectal tumors