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Biomedical subjects

K Gondo

Publications and source records attributed to K Gondo.

At least 19 recordsLinked to original sources

A magnetoencephalographic study on development of the somatosensory cortex in infants.

In order to examine the sensori-motor correlation in infants, we recorded the somatosensory evoked magnetic fields to tactile stimulation by using a 37-channel magnetoencephalograph. Twelve healthy infants were examined at palmar grasp stage and pincers grasp stage. Air-tapping stimulation of the right thumb was performed. Three distinct components (W1-3) emerged, W3, with a latency of approximately 100 ms, being the most prominent. As infants grew up, the correlation coefficient and the amplitude of the equivalent current dipole of W3 for the thumb increased. These developmental changes may be attributable to increases in the stability and reproducibility of the cortex in response to somesthetic inputs. Moreover, this change along with motor development supports the presence of a sensori-motor correlation in infants.

Aging↗

Cerebral ganglioglioma with epilepsy: neuroimaging features and treatment.

Gangliogliomas are an increasingly recognized cause of epilepsy in children. In this study the clinical, neuroimaging, and neurophysiological data of five patients with cerebral ganglioglioma and epilepsy are reviewed retrospectively. The average age of these patients was 4.4 years at onset and the average duration of seizures before diagnosis was 11 months. Tumors were located in the frontal (3), parietal (1), and occipital (1) lobes. While one cystic and four solid tumors showed various densities on CT and MRI, one frontal lesion was not demonstrated by CT scan but clearly shown by MRI. Scalp electroencephalography (EEG) showed neither localized nor epileptiform abnormalities in three patients, while the remaining two had these abnormalities. In one patient, invasive chronic electrocorticography (ECoG) recordings with subdural electrodes revealed an ictal onset zone located in the hand motor area. In all patients, intraoperative ECoG failed to reveal any epileptiform activities, and tumor removal alone was performed. For a mean of 3.4 years after surgery, all patients are alive and seizure-free, with stable imaging findings. Tumor resection may be the most important factor for optimal seizure control and prevention of tumor recurrence despite the fact that EEG and ECoG findings may conflict on tumor location.

Adolescent↗

Age-related changes of the MR appearance of CNS involvement in neurocutaneous melanosis complex.

We report a case of giant congenital melanocytic nevi (GCMN) at risk of developing neurocutaneous melanosis (NCM) with age-related changes observable on MRI of the brain. However, although the usefulness of MR imaging in NCM is well known, age-related changes on MRI have rarely been reported. The prevalence of positive MRI findings and prognosis in GCMN accompanied by epilepsy and/or mental retardation awaits clarification. This case report may suggest the importance of serial brain MRI in cases of GCMN in assessing the risk of NCM.

Age Factors↗

High prevalence of anticardiolipin antibodies in hepatitis C virus infection: lack of effects on thrombocytopenia and thrombotic complications.

Hepatitis C virus (HCV) causes various extrahepatic immunologic abnormalities. Recently, an association between HCV infection and antiphospholipid syndrome, including thrombocytopenia, has been reported. However, the precise relationship between thrombocytopenia and anticardiolipin antibodies in patients with chronic HCV infection is not fully understood; likewise, the association of antiphospholipid syndrome and various liver diseases is not well understood. To evaluate the prevalence and importance of antiphospholipid antibodies in various chronic liver diseases, we determined the levels of anticardiolipin antibodies, platelet numbers, and levels of platelet-associated immunoglobulin G (PA-IgG) and thrombin-antithrombin III complex (TAT) in patients with chronic HCV infection, chronic hepatitis B virus (HBV) infection, and primary biliary cirrhosis (PBC). The prevalence of anticardiolipin antibodies in patients with HCV infection was significantly higher than that in control subjects or individuals with the other liver diseases examined. However, there was no significant correlation between anticardiolipin antibodies and platelet counts or TAT. The frequency of thrombotic complications was similar in anticardiolipin antibody-positive and -negative patients with chronic HCV infection. Further, sera from all but one anticardiolipin antibody-positive HCV patient were negative for phospholipid-dependent anti-beta2 glycoprotein I antibodies. Our results suggest that anticardiolipin antibodies are frequently found in patients with chronic HCV infection, but they do not appear to be of clinical importance. Immunologic disturbances induced by HCV or prolonged tissue damage in systemic organs as a result of the extrahepatic manifestations of HCV infection may induce the production of antibodies to various cardiolipin-binding proteins or phospholipids.

Antibodies, Anticardiolipin↗

Diagnostic usefulness of diffusion-weighted magnetic resonance imaging in influenza-associated acute encephalopathy or encephalitis.

A magnetic resonance imaging (MRI) study was performed for a 20-month-old girl with an influenza type A infection who presented acute encephalopathy. Conventional MRI performed 8 days after the onset of encephalopathy, including T1-weighted, T2-weighted, and fluid-attenuated inversion recovery imaging, revealed only vague lesions in the right frontal, temporal, and parietal lobes. In contrast, diffusion-weighted imaging (DWI) then demonstrated the lesions much more intensively. On the 26th day, the lesions previously observed on DWI had become less discernible. The hyperintensity observed on DWI might reflect cytotoxic edema. Thus, DWI may be useful for evaluation of acute influenzal encephalopathy/encephalitis.

Acute Disease↗

Asymmetric sweating in a child with multiple sclerosis.

A 10-year-old male with multiple sclerosis complained of excessive sweating on the right side of the forehead and shoulder on relapse 3 months after the onset of multiple sclerosis. Because the neurologic evaluation revealed no abnormalities in the sudomotor function, it is likely that the hyperhidrosis resulted from a lesion in the central or preganglionic sympathetic nervous system. Magnetic resonance imaging demonstrated a high-intensity lesion involving the left hypothalamus on T(2)-weighted imaging. Thus hypothalamic involvement might be the reason for the hyperhidrosis in this patient.

Abducens Nerve Diseases↗

Acute disseminated encephalomyelitis in a female with hereditary neuropathy with susceptibility to pressure palsy.

A 7-year-old female presented with fever, urinary incontinence, mental regression, gait disturbance, and lethargy after diarrhea. Magnetic resonance imaging revealed multifocal T(2)-weighted hypersignal lesions supportive of acute disseminated encephalomyelitis. Her mother had been diagnosed with hereditary neuropathy with susceptibility to pressure palsy. The girl was also determined to have hereditary neuropathy with liability to pressure palsy, with a 1.5-Mb deletion in chromosome 17p11.2 encompassing the gene for peripheral myelin protein 22 detected by fluorescent in situ hybridization. Hereditary peripheral neuropathies may be a factor in triggering the autoimmune demyelinating disorder of the central nervous system.

Charcot-Marie-Tooth Disease↗

Reorganization of the primary somatosensory area in epilepsy associated with focal cortical dysplasia.

A 5-year-old boy with focal cortical dysplasia was referred to our hospital because of epileptic seizures. He showed mild weakness of the left hand without sensory disturbance. Brain MRI revealed extensive cortical dysplasia with pachygyria and microgyria around the right central sulcus. On EEG examination, interictal spikes were noted over the right fronto/centro/parietal region. A 37-channel magnetometer revealed that the sources of the spikes were in a small, restricted region of the normal frontal lobe adjacent to the dysplastic brain. Somatosensory evoked magnetic fields indicated that the location of the current source of N2O was in the same area. Our patient shows a unique case of plasticity and reorganization of the somatosensory function due to cortical dysplasia.

Cerebral Cortex↗

Surgical management of intractable epilepsy associated with cerebral neurocytoma.

Neuronal neoplasms of the CNS constitute a rarely encountered group of tumors. This report concerns the surgical management of seizures encountered in four cases (ranging from 2 to 10 years-of-age at onset; consisting of two males and two females) of a recently recognized morphologically unique tumor, called 'cerebral neurocytoma'. All patients were associated solely with intractable complex partial seizures. The tumor involved the temporal lobe in two cases, and the frontal in two. Magnetoencephalography (MEG) clearly demonstrated an accumulation of equivalent current dipoles originating from the interictal spikes on the cortex around the tumor. On intra-operative electrocorticography (ECoG), the epileptogenic zone was topographically distinct from the region of the tumor. No definite ECoG activities were observed at the tumor site, although this tumor did consist of small mature neuronal cells. Either a complete or a subtotal resection of the tumor and the epileptogenic cortex was performed and, post-operatively, universal freedom from seizures was demonstrated in all patients. A histological examination of the epileptogenic cortex revealed the presence of minute cortical dysplasia or tumor involvement in the hippocampus. A resection of the epileptogenic cortex along with the tumor was thus found to improve the seizure outcome in patients with neurocytoma-associated epilepsy without inducing any identifiable neurological deficits attributable to the incremental resection.

Adult↗

Renal tubular acidosis associated with zonisamide therapy.

PURPOSE: We sought to report a previously undescribed adverse effect, renal tubular acidosis associated with zonisamide (ZNS) therapy. METHODS: Ammonium chloride, bicarbonate, and furosemide loading tests were performed in an epileptic patient with metabolic acidosis and episodic hypokalemia who was treated with ZNS. RESULTS: Distal renal tubular acidosis was diagnosed. On reexamination 7 weeks after ZNS had been replaced with phenytoin, the renal tubular acidosis disappeared. CONCLUSIONS: This case indicates, for the first time, that ZNS might be a potential cause of renal tubular acidosis. Blood gases and serum electrolytes should be measured in patients undergoing ZNS therapy.

Acidosis, Renal Tubular↗

Altered expression of CD45 isoforms in differentiation of acute myeloid leukemia.

Specific expression of different CD45 isoforms can be seen in various stages of differentiation of normal nucleated hematopoietic cells. Association of membrane expression of CD45 isoforms and differential levels of leukemia cells was studied in 91 cases with de novo acute myeloid leukemia (AML). Membrane expression of CD45RA and CD45RO was analyzed by flow cytometry and their expression patterns were compared with AML subtypes classified according to the French-American-British (FAB) classification. CD45RA was essentially expressed in all of the FAB myelocytic subtypes (M0-M3). Its expression in percentage was lower in the most differentiated subtype of AML (M3) when compared with other myelocytic subtypes. CD45RO expression was rarely observed in cases with myelocytic subtypes (1/56 cases of M0, M1, M2, and M3) except for the minimally differentiated myelocytic subtype (M0) or those with potential for differentiation to T-cell lineage where three of 12 cases showed CD45RO expression. When leukemia cells of an M3 case were differentiated to mature granulocytes by treatment of all-trans-retinoic acid, they showed increasing expression of CD45RO. In subtypes with a monocytic component (M4 and M5), both of CD45RA and CD45RO expression were observed and mutually exclusive. When 10 cases of M5 were subdivided by the differential level into undifferentiated (M5a) and differentiated monocytic leukemia (M5b), expression of CD45RA and CD45RO was strictly restricted to cases with M5a and M5b, respectively. These results suggest that CD45 isoform expression in AML characterizes differential levels both in myelocytic and monocytic lineages and specifically disturbed in each subtype. The assessment of CD45 isoform expression appears to provide an insight on biological characteristics and a useful supplementary test for differential diagnosis of AML subtypes.

Acute Disease↗

Molecular epidemiology of C9 deficiency heterozygotes with an Arg95Stop mutation of the C9 gene in Japan.

Deficiency of the ninth component of human complement (C9) is the most common complement deficiency in Japan, with an incidence of approximately one homozygote in 1000, but is very rare in other countries. Genetic analyses of Japanese C9 deficiency have shown that a C-to-T transition leading to TGA stop codon for Arg95 in exon 4 of the C9 gene (Arg95Stop) is common in Japanese C9 deficiency. To determine the prevalence of heterozygous carriers of the Arg95Stop mutation in a Japanese population, we collected DNA samples from 300 individuals in two of the four main islands of Japan. Heterozygote detection was performed with an allele-specific polymerase chain reaction (PCR) system designed to detect exclusively only one of the normal and mutant alleles, followed by confirmation with PCR/single-strand conformation polymorphism (SSCP) analysis and direct sequencing. Twenty individuals were heterozygous for the Arg95Stop mutation. None was homozygous. The prevalence of carriers of the Arg95Stop mutation was 6.7% (20/300). An estimated frequency (0.12%) of complete C9 deficiency due to homozygous Arg95Stop mutation was consistent with frequencies determined by serological studies.

Arginine↗

Factors affecting maternal anxiety about child rearing in Japanese mothers.

In order to determine the factors causing anxiety in raising young children, data were collected from questionnaires completed by 1164 mothers of 3-y-old children at a standardized baby check-up. Factors concerning demographic characteristics, delivery and child rearing practice were used in multivariate logistic regression analysis. Stepwise logistic regression analysis showed that mothers rearing first children and toddlers who were susceptible to common illnesses were likely to worry. Improvements in the child rearing environment are needed.

Anxiety↗

Nonsense mutation in exon 4 of human complement C9 gene is the major cause of Japanese complement C9 deficiency.

Deficiency of the ninth component of human complement (C9) is the most common complement deficiency in Japan but is rare in other countries. We studied the molecular basis of C9 deficiency in four Japanese C9-deficient patients who had suffered from meningococcal meningitis. Direct sequencing of amplified C9 cDNA and DNA revealed a nonsense substitution (CGA-->TGA) at codon 95 in exon 4 in the four C9-deficient individuals. An allele-specific polymerase chain reaction system designed to detect exclusively only one of the normal and mutant alleles indicated that all the four patients were homozygous for the mutation in exon 4 and that the parents of patient 2 were heterozygous. The common mutation at codon 95 in exon 4 might be responsible for most Japanese C9 deficiency.

Adolescent↗

Magnetoencephalographic analysis of rolandic discharges in benign childhood epilepsy.

We report the detailed analysis of the generator and propagation of rolandic discharges in benign childhood epilepsy with centrotemporal spikes by means of 37-channel magnetoencephalography with neuromagnetic three-dimensional dipole localization. Equivalent current dipoles of prominent negative sharp waves of rolandic discharges appeared as tangential dipoles in the rolandic region, positive poles being situated anteriorly. These equivalent current dipoles showed a relatively limited localization and regular directions compared with other components. Equivalent current dipoles of preceding small positive waves, positive waves following negative sharp waves, and negative slow waves appeared in the vicinity of negative sharp waves. Equivalent current dipoles of rolandic discharges were located around the generator of somatosensory evoked magnetic fields stimulated at the lower lip. These findings suggest that rolandic discharges are generated through basically a mechanism similar to that for the middle-latency components of somatosensory evoked responses.

Child↗