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Biomedical subjects

A Mitsudome

Publications and source records attributed to A Mitsudome.

At least 37 records · Page 2Linked to original sources
PubMed1999

Application of rapid random stimulation (RRS) to visual evoked potentials in children.

The present study was performed to examine the effects of regular (1 Hz) and modified rapid random stimulation (RRS) (6 and 12 Hz) on visual evoked potentials (VEPs), by simultaneously recording negative waves around 100 ms, wave IV-latency, positive waves around 60 ms, wave III-latency, and amplitudes calculated from peak to peak, without causing impairment of visual acuity, in 44 patients aged 5-17 years. The wave IV-latencies of VEPs obtained by 6 and 12 Hz RRS were easily determined, and the latencies were not significantly changed compared to those obtained by previous 1 Hz regular stimulation. On the other hand, the amplitudes decreased in a frequency-dependent manner (1 < 6 < 12 Hz). These results were found to be similar in both preschool and school children. The examination time of VEPs determined by RRS is one-tenth shorter than that of 1 Hz regular stimulation. Thus, this method has the benefit of shortening the examination time, which decreases fatigue and inattention of the subjects, suggesting that modified RRS is a practically useful method for children.

Adolescent↗
PubMed1999

Development of the temporal lobe in infants and children: analysis by MR-based volumetry.

BACKGROUND AND PURPOSE: Recent advances in data-processing techniques have allowed more accurate MR-based volumetric measurement than was possible in the past. The purpose of this study was to use this technique to evaluate the development of the temporal lobes in childhood. METHODS: The study group consisted of 42 subjects aged 3 weeks to 14 years (mean age, 5 years), all with normal findings on a routine MR study and none with a history of epilepsy. MR images were acquired on a 1.0-T system using a T1-weighted 3D ultrafast gradient-echo sequence. The volumes of the hippocampal formations and temporal lobes were measured by using a workstation, and the percentage of hippocampal formations in the temporal lobes was calculated. Myelination in the limbic system and related structures was also evaluated. RESULTS: The volume of the hippocampal formations increased sharply until the age of 2 years, and continued to increase slowly thereafter. However, the percentage of hippocampal formations in the temporal lobes showed a negative correlation with age. The hippocampal formations on the right side were larger than those on the left in 38 cases (91%), and the anterior temporal lobes on the right were larger than those on the left in 32 cases (76%). This right-left asymmetry of the hippocampal formations and anterior temporal lobes was observed from early infancy, and these differences were statistically significant. A longitudinal fasciculus of high signal intensity was seen in the white matter beneath the subiculum by about 3 months of age. CONCLUSION: MR-based volumetry established developmental characteristics of the temporal lobe, such as a hippocampal growth spurt, a growth difference between the hippocampal formation and the rest of the temporal lobe, and right-left asymmetry. Knowledge of these characteristics may aid in the understanding of hippocampal and temporal lobe abnormalities in children.

Adolescent↗
PubMed1998

[A case of encephalitis due to Mycoplasma pneumoniae: detection of specific DNA from cerebrospinal fluid and elevation of interleukin-6].

A 9-year-old female was admitted to our hospital due to a generalized seizure and consciousness disturbance. The patient had a fever and rash four days before admission, but she had no respiratory symptoms. The seizure and consciousness disturbance was prolonged and intractable. We diagnosed the patient as having encephalitis because of the increase in the cell count in the cerebrospinal fluid (CSF) and a diffuse slow EEG wave. The computed tomography of the head was normal. The causative agent was identified as Mycoplasma pneumoniae because of the increase of antibodies, and the detection of a specific DNA with a polymerase chain reaction. The interleukin (IL)-6 level of CSF was high (384 pg/ml). In spite of intensive treatment she had severe neurological sequelae. The invasion of Mycoplasma pneumoniae to the central nervous system appeared to have a role in the development of encephalitis in the patient. We speculated that there is a possible relationship between the IL-6 levels of CSF and clinical severity of encephalitis.

Child↗
PubMed1998

Hypothalamic hamartoma associated with multiple congenital abnormalities. Two patients and a review of reported cases.

We report 2 patients with hypothalamic hamartoma associated with multiple congenital abnormalities and analyze 42 (including our own) reported cases, including our 2 cases, of hypothalamic hamartoma or hypothalamic hamartoblastoma with multiple congenital abnormalities, to understand the timing of their occurrence and clarify the prognosis. To this end, we classified them into lethal and nonlethal cases. We found poly- and syndactyly, cleft or high-arched palate and nose abnormalities to be important manifestations of this syndrome. Major organ abnormalities and CNS and endocrine abnormalities occurred frequently among the lethal cases, very likely indicative of a disturbance of embryogenesis between gestational days 34-37 and thus implicated in a negative prognosis.

Abnormalities, Multiple↗
PubMed1998

Valproate therapy does not deplete carnitine levels in otherwise healthy children.

OBJECTIVE: To determine whether children with epilepsy undergoing valproate (VPA) antiepileptic therapy and who are otherwise healthy have a lower serum level of carnitine (CAR) and a higher plasma level of plasma ammonia than do normal children. METHODOLOGY: A total of 45 children with epilepsy, 6.3 to 21.7 years of age, who were treated solely with VPA and were free of abnormal neurologic findings or nutritional problems were randomly selected (VPA-treated group). An age-matched control group (n = 45) was selected from subjects without epilepsy (control group). Total (T) and free (F) serum CAR, serum VPA concentration, and the plasma ammonia level were measured and analyzed. RESULTS: Serum VPA concentration exhibited a weak negative correlation with both T- (r = -0.34) and F-CAR (r = -0.41). The T-CAR levels were 55.7 +/- 12.4 and 57.6 +/- 12.1 mM, and the F-CAR levels 42.7 +/- 9.9 and 44.4 +/- 9.9 mM in the VPA-treated and control groups, respectively. Thus, there was no significant difference in T- or F-CAR levels between the VPA-treated and control groups. Plasma ammonia levels were the same in the two groups: 26 +/- 9.2 and 29.4 +/- 11.8 mM in the VPA-treated and control groups, respectively. There was no significant correlation between blood ammonia and either T- (r = +0.024) or F-CAR (r = -0. 026). CONCLUSION: Children on a regular diet ingest a sufficient amount of CAR that more than meets their daily CAR requirement. The level of neither T- nor F-CAR in patients with epilepsy and without severe neurologic or nutritional problems being treated with VPA appeared to be affected by VPA therapy. Because the blood CAR level depends on nutritional condition rather than on blood VPA concentration, CAR deficiency caused by VPA is not likely to occur in this population. The usefulness of supplementation of CAR for this type of patient with epilepsy, therefore, must be reevaluated carefully.

Adolescent↗
PubMed1997

Japanese monozygotic twins with Rett syndrome.

We present a study of 28-year-old Japanese monozygotic female twins with Rett syndrome (RS). To our knowledge, this is the first report of monozygotic twins with RS from Japanese family. There are some differences between twins about seizures, scoliosis and stereotypical hand movements during adolescence. Monozygosity was confirmed by both blood typing and HLA titers.

Adult↗
PubMed1997

The effectiveness of clonazepam on the Rolandic discharges.

Rolandic discharge (RD), noted in the electroencephalography (EEG) of patients with benign epilepsy in childhood with centrotemporal spikes (BECCT) has several unique features. One feature is that the amount or frequency of RDs does not correlate well with the incidence of seizures in BECCT although it is a key finding in the diagnosis of this epileptic syndrome. In this study, we examined the efficacy of antiepileptic drugs focusing on the disappearance of RDs in relationship with seizure control. Forty patients with BECCT who were not medically treated prior to this study were randomly sorted into three groups. Twenty patients were assigned for clonazepam (CZP) treatment, 10 patients for valproate (VPA) and the remaining 10 patients for carbamazepine (CBZ). Each drug was administered for 4 consecutive weeks. EEGs were recorded twice during the study, before and 4 weeks after the medication trial. The effects of each treatment on RDs were assessed. RDs disappeared in 15 of the 20 cases treated with CZP (75%) within 4 weeks while the same was observed in only one of the 10 cases treated with VPA (10%). CBZ failed to demonstrate any effect on RD. In the group treated with CZP, there were no differences in seizure incidence, seizure type and blood concentration of CZP between the patients whose RDs disappeared and those whose RDs remained.

Anticonvulsants↗
PubMed1997

Kabuki make-up syndrome associated with West syndrome.

A Japanese boy with Kabuki make-up syndrome associated with West syndrome is reported. He developed periodic tonic spasms at 6 months of age while his electro-encephalogram also revealed hypsarrhythmia. Although only a few previously reported cases of Kabuki make-up syndrome have been associated with epilepsy, it is likely that epileptic seizures are another primary feature of Kabuki make-up syndrome.

Electroencephalography↗
PubMed1997

Rhythmic slow activity in benign childhood epilepsy with centrotemporal spikes.

In benign childhood epilepsy with centrotemporal spikes (BCECT), focal rhythmic slow EEG activity is occasionally observed in the same region where the centrotemporal spikes or Rolandic Discharges (RD) are seen. This slowing appears especially when RDs are frequent. Holmes therefore hypothesized that these slow waves in BCECT were not the manifestations of structural lesion of the brain but secondary slowing accompanying RD. In order to clarify the origin of the slow activity, clonazepam was administered to four BCECT patients who had the focal slow waves. Clonazepam has recently been found by us to selectively diminish RD in BCECT. After the administration of clonazepam for 4 consecutive weeks, not only RDs but also the slow activity disappeared completely. This finding strongly suggests that these focal rhythmic slow waves in the same region of the RD are a part of the morphology of RD.

Child↗
PubMed1997

[An operated case of intractable occipital lobe epilepsy associated with calcification in the occipital lobe].

We reported a 20-year-old female of intractable occipital lobe epilepsy associated with calcification in the left occipital lobe. She developed seizures since 2 years old and electroencephalography showed paroxysmal activities on the left occipital area. At 8 years old, partial resection of the lesion and interictal spike focus under intraoperative corticography guidance was performed, but the favorable seizure outcome was not obtained. At the age of 20 years old, the frequency of her seizures has increased and admitted to our department. Preoperative evaluation with chronic invasive subdural recording showed that the ictal zone was located in the inferior (tentorial) surface and convexity of the left occipital lobe. Functional mapping involving cortical stimulation and cortical recording of pattern-reversal visual evoked potentials demonstrated that the ictal onset zone was not in the visual area. Following cortical resection of the ictal onset zone and multiple subpial transection on the surrounding cortex, she had good relief with less than one minor seizure per 1-3 months. Histological findings of the resected specimen were consistent with those of Sturge-Weber syndrome. Thus chronic invasive subdural recording may improve seizure outcome in patients whose epileptogenic area was located near the visual field.

Adult↗
PubMed1996

[Werdnig-Hoffmann disease type I with progressive ophthalmoplegia and ptosis].

Although pathological changes are observed in both the oculomotor nucleus and abducens nucleus in autopsied cases of infantile progressive spinal muscular atrophy, external and internal ocular palsy and ptosis have not been previously reported clinically. We presented here two long-surviving cases on respirators which gradually developed ophthalmoplegia and ptosis were presented. From our observation of these cases, it was suggested that there are certain periods of latencies between the occurrence of pathological changes and their clinical manifestation and that the lack of clinical signs of upper cranial nerve involvement in cases with Werdnig-Hoffmann type I is due to their short survival length.

Blepharoptosis↗
PubMed1995

A severe muscular dystrophy patient with an internally deleted very short (110 kD) dystrophin: presence of the binding site for dystrophin-associated glycoprotein (DAG) may not be enough for physiological function of dystrophin.

We report a 4-yr and 5-month-old boy with severe clinical features of an early-onset Duchenne muscular dystrophy, who had a very short (110 kDa) dystrophin at the sarcolemma. The patient had a large deletion (exons 2-44) of the dystrophin gene which was predicted to cause a reading frame shift. Sequence analysis of dystrophin mRNA in muscle revealed an alternatively spliced gene product from exons 1 to 51 that caused restoration of the reading frame, in addition to an mRNA corresponding to the DNA deletion. A consistent result was obtained by immunocytochemical analysis of muscle; i.e. positive staining for dystrophin at the sarcolemma using antibodies against the C-terminus, cysteine-rich region and last three of 24 repeat units of the central rod-domain, but not for the remaining antibodies for dystrophin that recognize the N-terminal and proximal rod-domains. Immunostaining for dystrophin-associated glycoproteins (DAGs: 43 and 50 K) and merosin were preserved. Utrophin staining was positive but fainter than other DMD muscles. These results suggest that an extremely short dystrophin lacking the entire actin-binding site in the N-terminus cannot function properly even if the protein possesses the putative DAG-binding cysteine-rich and the C-terminal domains, and still has an ability to associate with sarcolemmal membrane.

Base Sequence↗
PubMed1994

Symptomatic Chiari malformation and associated pathophysiology in pediatric and adult patients without myelodysplasia.

The clinical characteristics of eight pediatric and five adult patients with Chiari malformation were evaluated. Six pediatric and five adult patients had associated syringomyelia. All patients initially underwent a suboccipital craniectomy with upper cervical (C-1 and/or C-2) laminectomy and duraplasty, and/or shunting procedures. The clinical characteristics of the pediatric and adult groups were compared. The mean interval between onset of symptoms and operation was shorter in the pediatric group (3 yrs 6 mos) than in the adult group (7 yrs 1 mo). Pediatric patients without syringomyelia had the shortest mean interval of 1 year 8 months. Preoperatively, the clinical features were more severe in the adult patients than in the pediatric patients. Postoperatively, seven of eight pediatric patients improved and one stabilized, while two of five adult patients improved, one stabilized, and in two the disease continued to progress despite multiple corrective procedures. Cine magnetic resonance imaging revealed correction of the abnormal cerebrospinal fluid (CSF) flow at the craniovertebral junction and decreased to-and-fro movement in the syrinx after posterior fossa decompression, which were closely correlated with the improvement of clinical features in pediatric patients. However, adult patients required further procedures because of the multifactorial nature of the disease. Evaluation of abnormal CSF pathways at the craniovertebral junction is important for investigating the pathogenesis of Chiari malformation and associated syringomyelia.

Adolescent↗
PubMed1993

[MRI hippocampal volumetric measurements in benign childhood epilepsy with centrotemporal spike(s) and temporal lobe epilepsy of childhood onset].

Benign childhood epilepsy with centrotemporal spike(s) (BCECT) is characterized by rolandic discharges (RD) on EEG. As the origin of RD, the lower rolandic cortex was proposed by Lombroso, but recently the hippocampus was proposed by some reports, based on the character of RD or the pharmacological effects of anticonvulsants. On the other hand, MRI hippocampal volumetric measurements revealed the relationship between the hippocampal atrophy and EEG foci in intractable temporal lobe epilepsy (TLE) of adults, but there are a few reports of the hippocampal volumetric study in children. Therefore, in this paper, we reported about MRI hippocampal volumetric measurements in BCECT and TLE of childhood onset. The statistical examination was performed by t-test. MRI hippocampal volumetric measurements were performed in 27 cases with BCECT (6-19 years, 9.9 +/- 2.7 years, 11 males, 16 females), 22 cases with TLE (5-22 years, 12.9 +/- 5.5 years, 15 males, 7 females) and 17 normal control subjects (6-15 years, 10.4 +/- 2.7 years, 10 males, 7 females). The age was not significantly different among the 3 groups. The clinical course of TLE group was significantly longer than BCECT group, but the onset age of epilepsy was not significantly different between the 2 groups. The hippocampal volume and the right-side minus left-side volume were not significantly different in 3 groups, but these values of TLE were more scattered than the others. There was no abnormal signal intensity in hippocampus on MRI, or no specific abnormality in the surrounding structures in 3 groups.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1993

[Electrophysiological study of Bell's palsy in children].

An electrophysiological study of Bell's palsy in children was reported. Direct response to facial nerve stimulation was tested in 28 patients. Of these, 17 patients were further tested for electrically elicited blink reflex. The reduction in amplitude of direct response on affected side was characteristic of Bell's palsy. The direct response was evoked on affected as well as nonaffected side. The amplitude of the direct response on the affected side was expressed as percentage of that on the nonaffected side (ratio of M response amplitude:RMA). 13 patients showed incomplete recovery after 3 months, and RMA of the 11 patients of them were reduced below 30%. In the patients showing good clinical recovery within 2 months, RMA returned to normal promptly. In the blink reflex, R1 R2 components were found to be persistent on the first examination, suggesting a satisfactory functional outcome in Bell's palsy. As these electrophysiological methods are conventional assessing facial nerve function, it seemed to be valuable for the follow up of children with Bell's palsy.

Adolescent↗
PubMed1992

Electrophysiological study of four patients with Fisher syndrome: a mechanism of areflexia.

Electrophysiological studies were performed on four patients with the Fisher syndrome (two 4-year-old boys, a 5-year-old boy and a 9-year-old girl). Motor nerve conduction velocity (MNCV), nerve action potential, F wave, H reflex and T reflex were measured at the stage when areflexia was present but ataxia and ophthalmoplegia had recovered. MNCV and SNCV by compound mixed nerve potential were normal in all our patients. The amplitude of the muscle responses (M-responses) and compound mixed nerve action potentials were also within normal limits. The latencies of the F waves and F wave conduction velocities were normal. However, H-reflexes and T-reflexes were absent in all four cases. Our results suggested that areflexia in the Fisher syndrome is attributed to the desynchronization of the impulses or the partial conduction block of GIa fibers.

Ataxia↗
PubMed1992

A case of myasthenia gravis complicated with hyperthyroidism and thymic hyperplasia in childhood.

We report here a case of myasthenia gravis complicated with hyperthyroidism and thymic hyperplasia. The patient was a 13-year-old girl with struma and hyperthyroidism which began at age 12. Two weeks following the initiation of treatment against hyperthyroidism she developed left blepharoptosis, diplopia, and dysphagia, which responded promptly to edrophonium administration. An increase of the anti-acetylcholine receptor antibody was found in the serum. A chest CT showed a large soft tissue mass in front of the ascending aorta, which was proven histopathologically as thymic hyperplasia. The patient underwent an extensive thymectomy and was placed on combination therapy with an anti-thyroid drug, glucocorticosteroid, and an anti-cholinesterase drug. Her symptoms and signs have been well controlled by this treatment. Coexistence of myasthenia gravis, hyperthyroidism, and thymic hyperplasia in childhood have never been documented in literature.

Adolescent↗