Biomedical subjects

K Aso

Publications and source records attributed to K Aso.

At least 37 records · Page 2Linked to original sources

PubMed1999

Epileptic spasms preceded by partial seizures with a close temporal association.

PURPOSE: To investigate the distinctive features of patients with West syndrome who had partial seizures followed by epileptic spasms (PS-ES). METHODS: We examined 45 patients with West syndrome whose epileptic spasms were recorded with simultaneous video-electroencephalography (EEG) monitoring between 1982 and 1996. We investigated the patients who had PS-ES and compared the PS-ES patients with the 37 patients without PS-ES. RESULTS: Of the 45 patients who had epileptic spasms in clusters (ES) and hypsarrhythmia on the interictal EEG, eight (17%) had ES preceded by partial seizures (PS) with a close temporal association. Seven of these eight were female patients. The underlying disorders were tuberous sclerosis (one patient), Aicardi syndrome (one), nonketotic hyperglycinemia (one), and focal cortical dysplasia (one). The etiology was unknown in the remaining four patients, but was suspected to be of prenatal origin in three. Three types of seizure sequence were identified: PS followed several seconds later by ES (two patients), alternating PS and ES starting with PS (three), and PS gradually replaced by ES with overlapping of the two (three). PS-ES disappeared or was replaced by other types of seizures in 1-34 months. Six patients could not walk, and all patients could not speak any sentences at age 3 years. CONCLUSIONS: Compared with patients without PS-ES, those with PS-ES more often had organic brain lesions of prenatal origin, other types of seizures before the onset of ES, asymmetric hypsarrhythmia on the EEG, and poor psychomotor outcome.

Age of Onset↗

PubMed1999

[Evaluation and improvement of medical and nursing service and caregiving for the elderly using MDS. 1. Diagnosis and some problems in dementias].

A Minimum Data Set (MDS) was recorded in two geriatric hospitals every 4 months for about 5 years. The initial records were available in a total of 1,735 disabled elderly. In MDS, about 360 trigger items were recorded. A selection was made to determine whether there were problems of care in 18 areas of Resident Assessment Protocols. We analyzed errors and biases in selection of the diagnosis in the chapter E of MDS disclosing that: 1) they were almost satisfactorily made in diseases except dementias, 2) diagnosis of dementias was biased in several ways, particularly Alzheimer's disease was overselected, and 3) preprinted diagnostic options in MDS were not suited with the Japanese practice and were insufficient for comprehensive medical/nursing service and caregiving.

Aged↗

PubMed1999

[Corneal amyloidosis complicated by trichiasis--immunohistochemical identification of the amyloid light chain protein].

PURPOSE: To identify the amyloid protein of the corneal amyloidosis complicated by trichiasis. METHODS: The two patients were 41-year-old and 38-year-old women with trichiasis. They had gelatinous drop-like corneal change in hemilateral eye. The lesion was excised and examined by light and electron microscopy. Additionally, we performed an immunohistochemical study with immunofluorescence techniques using cryosections. RESULTS: The amyloid deposits were confirmed with light and electron microscopy. Congo red positive staining was not reduced following pretreatment with potassium permanganate. Immunohistochemically, amyloid deposits in the cornea stained positively with serum human light chain kappa and lambda. Pretreatment of the section with 0.05% Tween-20 did not decrease the staining with fluorescence. The deposits stained negatively with serum prealbumin and keratin antibodies. CONCLUSIONS: These findings indicate the protein of the corneal amyloidosis complicating trichiasis to be an amyloid light chain (AL) protein that has never been identified in this kind of corneal amyloidosis.

Adult↗

PubMed1998

Neuropeptide Y stimulates bile secretion via Y1 receptor in the left dorsal vagal complex in rats.

Neuropeptide Y (NPY) injected into the cerebrospinal fluid and the left dorsal vagal complex enhances bile acid-independent and bicarbonate-dependent bile secretion through vagal muscarinic pathways in animal models. NPY binds to and activates six different receptor subtypes, and NPY Y1 and Y2 receptors are distributed in the dorsal vagal complex. We sought to determine which NPY receptor subtypes are involved in central stimulation of bile secretion by examining the effect of microinjection of specific NPY receptor agonists into the dorsal vagal complex. The bile duct was cannulated in urethane-anesthetized and bile acid-compensated rats. After measuring basal secretion, NPY, peptide YY (PYY), [Leu31, Pro34]NPY, NPY(13-36), or NPY(3-36) was microinjected into the either right or left dorsal vagal complex and bile secretion was observed for 100 minutes. Hepatic branch vagotomy was performed 2 hours before the peptide injection. Microinjection of NPY and PYY (8 pmol) into the left dorsal vagal complex increased bile secretion. [Leu31, Pro34]NPY microinjected into the left dorsal vagal complex also dose-dependently (1-8 pmol) stimulated bile acid-independent and bicarbonate-dependent bile secretion. Microinjection of NPY(13-36) into the left dorsal vagal complex did not stimulate and NPY(3-36) dose-dependently inhibited bile secretion. Stimulation of bile secretion by [Leu31, Pro34]NPY was abolished by hepatic branch vagotomy. NPY acts in the left dorsal vagal complex to stimulate bile acid-independent and bicarbonate-dependent bile secretion via Y1 receptor subtype.

Animals↗

PubMed1998

Necrotizing toxoplasmic encephalitis in a child with the X-linked hyper-IgM syndrome.

UNLABELLED: We report on a 9-year-old boy with the hyper-IgM syndrome who presented with rapid impairment of consciousness. The brain CT scan showed multiple round lucencies, and the brain histology revealed necrotizing toxoplasmic encephalitis. This patient, whose CD40/CD40 ligand system was impaired, indicates the importance of this system for defence against toxoplasmic infection. CONCLUSION: Although disseminated toxoplasmosis is a rare complication of the hyper-IgM syndrome, it must be included in the differential diagnosis of infections.

Child↗

PubMed1998

Benign myoclonic epilepsy in infants: video-EEG features and long-term follow-up.

We report video-EEG findings and a long-term follow-up study in 10 patients with benign myoclonic epilepsy in infants (BMEI). A high incidence of a past and family history of febrile convulsions was noted. Six of the 10 patients manifested characteristic vocalization associated with myoclonic seizures. It consisted of a sudden, brief expiratory noise and is considered to be characteristic of BMEI. Afebrile convulsions occurred before the onset of myoclonic seizures or during the clinical course in six patients, but the accurate type of these seizures remains to be clarified. Monotherapy with valproatic acid (VPA) was very effective, but plasma VPA levels over 100 micrograms/ml were initially necessary in most of the cases, although they did not need to be maintained for further seizure control. All patients showed a favorable long-term seizure outcome, although one showed moderate mental retardation.

Adolescent↗

PubMed1998

Efficacy of ursodeoxycholic acid in Japanese patients with type 1 autoimmune hepatitis.

Ursodeoxycholic acid (UDCA) has been shown to have beneficial effects on patients with primary biliary cirrhosis, suggesting that UDCA has immunomodulating effects. We investigated the effect of UDCA in patients with autoimmune hepatitis (AIH) which is characterized by immunological abnormalities. Eight patients with type 1 AIH were treated with 600 mg of UDCA per day for 2 years. Based on the criteria of the International Autoimmune Hepatitis Group, five patients were diagnosed as definite and three as probable type 1 AIH. Liver function tests were performed every 4 weeks, before and during UDCA therapy and the serum levels of anti-nuclear antibodies (ANA), smooth muscle antibodies (SMA), immunoglobulin G and gamma globulin were determined every 3 months. The levels of serum aspartate aminotransferase and alanine aminotransferase significantly decreased from 154 +/- 24 IU/L and 170 +/- 17 IU/L before UDCA therapy to 31 +/- 3 IU/L and 25 +/- 5 IU/L (P < 0.001) after 1 year of treatment and 28 +/- 2 IU/L and 23 +/- 4 IU/L (P < 0.001) after 2 years of treatment. After 2 years of treatment, the levels of serum immunoglobulin G and gamma globulin significantly decreased (P < 0.05) and ANA titres (5/8 patients) were reduced and SMA (3/5 patients) became negative. Furthermore, hepatic histopathological changes of four patients were assessed after 1 year of treatment, and an improvement of intrahepatic inflammation, but not fibrosis, was observed. In conclusion, these results suggest that UDCA has a beneficial therapeutic effect in patients with type 1 autoimmune hepatitis.

Aged↗

PubMed1998

Successful polymerase chain reaction-based diagnosis of fungal meningitis in a patient with chronic granulomatous disease.

Meningitis is not a common complication of chronic granulomatous disease (CGD). Here, we present details of a 3-year-old boy with X-linked CGD, who suffered from fungal meningitis. While 19 samplings using conventional cerebrospinal fluid (CSF) cultures failed to detect any organisms, fungal DNA was identified in the CSF by a new polymerase chain reaction (PCR)-based method. The patient recovered without any sequelae after treatment with a combination of antifungal agents, interferon-gamma and granulocyte infusions. This case report demonstrates that fungal meningitis must be included in the differential diagnosis of infections in CGD patients and that the PCR-based detection of fungal DNA is a powerful tool for diagnosis.

Child, Preschool↗

PubMed1998

Evolutional changes and outcome of West syndrome: correlation with magnetic resonance imaging findings.

The prognosis and evolutional changes of 77 patients with West syndrome (WS) were studied after patients were classified into four groups on the basis of their magnetic resonance imaging (MRI) findings: anomaly, perinatal injury, normal, and the other groups. The average age at onset of spasms was earliest in the patients with anomalies and latest in patients with normal MRI findings. Patients with normal MRI findings had the shortest duration of spasms, and patients with anomalies had the longest duration of spasms. Antecedent seizures were observed in 6 patients (3 patients with anomalies, 1 patient with normal MRI findings, and 2 patients with other abnormalities). Thirty-five patients had subsequent seizures. Patients with anomalies often had partial seizures and patients with perinatal injuries often had generalized seizures. Seizures were infrequent in patients with normal MRI findings. Developmental outcome was best in the patients with normal MRI findings and worst in patients with perinatal injuries. Various types of epileptic syndromes occurred subsequent to WS in patients with anomalies, although nonspecific symptomatic generalized epilepsy was common in patients with perinatal injuries. These results suggest that seizure prognosis, evolutional changes in seizures, and developmental outcome are different among the types of brain lesions.

Adolescent↗

PubMed1998

[Dramatic improvement with immunoabsorption therapy in a 7-year-old girl with severe Guillain-Barré syndrome after unsuccessful gammaglobulin therapy].

We report a 7-year-old girl with severe Guillain-Barré syndrome (GBS) who showed dramatic improvement after immunoabsorption therapy. She had progressive muscle weakness with resultant respiratory failure. On the 7th day of the illness, she was nearly quadriplegic and dependent on mechanical ventilation. In addition, she had autonomic manifestations, areflexia, abolished or delayed peripheral nerve conduction, and increased CSF protein. Administration of a high dose of gammaglobulin failed to improve the symptoms. Immunoabsorption therapy using a tryptophan column was performed 9 times during 17 days. After 5 times of immunoabsorption therapy, the muscle weakness improved dramatically; she was extubated 16 days after the institution of the therapy and walked without assistance on the 41st day. She regained normal muscle power without any sequelae. The immunoabsorption therapy caused hemolysis and decrease of serum fibrinogen, but no serious complications. The clinical course of this patient suggests the efficacy of immunoabsorption therapy in GBS.

Child↗

PubMed1997

Severe myoclonic epilepsy in infancy: evolution of seizures.

Changes in seizure type of severe myoclonic epilepsy (SME) in infancy were reviewed retrospectively in 14 patients (11 males and 3 females) who were followed-up to the age of 7 years or more. The observation period ranged from 5 to 16 years with a mean of 10 years. During the follow-up, three or four types of seizures were seen per patient, but the pattern of appearance and disappearance of each seizure type varied considerably among the patients. Tonic-clonic convulsion, either generalized or unilateral, was seen most consistently through the entire course, and it continued to the end of follow-up in 11 patients (79%). On the contrary, myoclonic seizure, complex partial seizure, and atypical absence often disappeared and reappeared repeatedly during the course. In SME, seizure symptoms varied widely among patients in comparison with other neurological symptoms, and the most consistent core seizure type was tonic-clonic convulsions.

Child↗

PubMed1997

[Neuronal migration disorders and epilepsy].

We retrospectively studied 32 patients who had cortical dysplasia and epilepsy. Cortical dysplasia was classified into diffuse cortical dysplasia (8 patients), bilateral localized cortical dysplasia (5), unilateral diffuse cortical dysplasia (2), and focal cortical dysplasia (17). The onset of epilepsy was younger in patients with more widespread lesions. At the onset, patients with bilateral lesions generally had symptomatic generalized epilepsy, while those with unilateral cortical dysplasia tended to have symptomatic localization-related epilepsy. In patients with focal cortical dysplasia, however, 4 patients had West syndrome, either at the onset of epilepsy or during the follow up period. Seizure outcome was poor in any type of cortical dysplasia. An evaluation of prognostic factors in patients with focal cortical dysplasia did not show any of statistical significance, including gender, age at onset of epilepsy, psychomotor delay and the presence of high intensity areas in T2-weighted MRI.

Adolescent↗

PubMed1996

Recombinant Plasmodium falciparum dihydrofolate reductase-based in vitro screen for antifolate antimalarials.

We describe the system for screening the effective antifolate antimalarials that uses the recombinant Plasmodium falciparum DHFR domain of the bifunctional DHFR-TS expressed in Escherichia coli, and were designed with amino acid alterations found in the DHFR genes of the antifolate resistant strains. The validity of the screen was verified by the subsequent examination of several substituted pyrrolo[2,3-d]pyrimidines for their antimalarial activity. Among the 120 chemical derivatives, 5 compounds were identified by their preferential inhibition of the drug sensitive pfDHFR to that of the mammalian isoenzyme. As compared to the sensitive enzyme, the decrease in response of the cycolguanil-resistant and pyrimethamine-resistant enzymes to the selected compounds were relatively moderate. This gave folds decrease in sensitivity of 0.8-7.5 and 3.6-29, respectively, while those for cycloguanil and pyrimethamine were 400 and 308. The compounds inhibited the growth of drug-sensitive cultured P. falciparum with 50% effective concentrations of the ranged 0.17-30 nM. As contrasted with the sensitive strain, the fold decrease in sensitivity of the resistant parasites were 0.9-2 and 15-50 in the case of the test compounds, while those for cycloguanil and pyrimethamine were 690 and 20,500. Moreover, the most selective pyrrolo-pyrimidine (P-1) showed in vivo activity against P. berghei in mice.

Animals↗

PubMed1996

Cryptogenic localization-related epilepsy of neonatal onset.

We report three patients with localization-related epilepsy of neonatal onset. They exhibited favourable psychomotor development and had no cerebral lesions on neuroimaging studies despite the presence of intractable partial seizures of neonatal onset. Although rare, some cases of epilepsy of neonatal onset may be cryptogenic, i.e. they belong to neither the symptomatic nor the idiopathic group.

Blood Glucose↗

PubMed1996

Cortical hypometabolism and delayed myelination in West syndrome.

PURPOSE: We examined the relation between cortical hypometabolism and delayed myelination in patients with West syndrome (WS). METHODS: Serial positron emission tomography (PET) with [18F]fluorodeoxyglucose ([18F]FDG) and magnetic resonance imaging (MRI) were performed in 18 patients with WS, first at the onset of epileptic spasms and later at age 10 months. The age at onset of seizures ranged from 2 to 7 months. Ten patients were diagnosed as having cryptogenic WS and 8 as having symptomatic WS. RESULTS: Cortical hypometabolism was detected in many patients at onset of epilepsy, but disappeared later, whereas delayed myelination tended to become evident with age. PET showed diffuse or focal cortical hypometabolism in 12 patients at onset, but in only 6 patients at age 10 months. MRI showed delayed myelination in only 2 patients at onset of epilepsy, but the number of patients with delayed myelination increased to 12 at age 10 months. Delayed myelination was more often present in patients with cortical hypometabolism. Delayed myelination was noted in 11 (85%) of 13 patients with cortical hypometabolism on first or second PET scans, but in only 1 (20%) of 5 patients who did not show PET abnormalities. Hypometabolism on the first or second PET scan was positively correlated with delayed myelination at age 10 months. CONCLUSIONS: In patients with WS, assessing myelination with MRI again at age 8-10 months is important even when MRI at the onset of epilepsy appears normal. Serial MRI and PET scans disclose more detailed pathophysiology of WS.

Age of Onset↗

PubMed1996

Chilblain lupus erythematosus of Hutchinson responding to surgical treatment: a report of two patients with anti-Ro/SS-A antibodies.

We report two patients with chilblain lupus erythematosus of Hutchinson (CL) who responded to surgical treatment. One of them was a 72-year-old woman (case 1), and the other a 62-year-old man (case 2). We attempted to treat these patients by excising the lesions and subsequently performing full-thickness free skin grafting, using skin from the abdominal region. No recurrence was seen in the operated area 7 years (case 1) and 3 years (case 2) after surgery. However, lesions persisted in the areas not operated upon, and in the areas where lesions had not been adequately excised. These results suggest that surgical removal of local factors reduces the rash in these cases. In addition, both patients were serologically positive for the anti-Ro/SS-A antibody suggesting that local expression of the Ro/SS-A antigen may be involved in the pathogenesis of the skin lesions. To our knowledge, full thickness free skin grafting has not been used previously to treat CL-associated skin lesions, and is promising as a treatment for patients who do not respond to conventional means.

Aged↗

PubMed1996

[Usefulness of continuous arterial infusion chemotherapy for post operative multiple recurrence and residual hepatocellular carcinoma].

Twenty five patients with postoperative multiple recurrence and residual hepatocellular carcinoma received continuous arterial infusion of CDDP and 5-FU via implanted reservoir. For the next five days, 10 mg/body of CDDP and 250 mg/body of 5-FU using arterial infusion were administered. It was discontinued for two days as one course, and 4 courses were basally administered. The efficacy rate was 60%, and there were 7 (28%) CR (complete response) cases. The survival rate was 76.0% for 1 year and 36.5% for 3 years, which is a favorable result considering their advanced stage. Thus, this treatment seemed to be effective for multiple hepatocellular carcinoma.

Antineoplastic Combined Chemotherapy Protocols↗

PubMed1995

Do ictal, clinical, and electroencephalographic features predict outcome in West syndrome?

Ictal electroencephalographic/video recordings of 42 patients with West syndrome (WS) were reviewed to define the relation between ictal, clinical, and EEG features and etiology or prognosis. The duration and type of spasms, the number of spasms per cluster, and the interval between spasms did not correlate with the etiology or the short-term prognosis. However, eye deviation, asymmetric spasms, and partial seizures concomitant with spasms were observed only in symptomatic WS patients with poor outcome. In particular, all 8 patients with concurrent partial seizures had severe psychomotor retardation. The ictal EEG characteristics did not correlate with etiology and prognosis. There was no significant difference between the patients with persistence or reappearance of hypsarrhythmia and the patients without interspasm hypsarrhythmia.

Electroencephalography↗