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Biomedical subjects

S Wakai

Publications and source records attributed to S Wakai.

At least 127 records · Page 7Linked to original sources
PubMed1991

[Occipital horn syndrome (Ehlers-Danlos syndrome type IX) with severe psychomotor retardation and muscle atrophy--a first Japanese case].

Occipital horn syndrome (OHS; Ehlers-Danlos syndrome type IX) belongs to the category of the copper metabolism disorders and is at present being investigated biochemically as is Menkes disease. We report a case of OHS in a 34-year-old male, which we believe to be the first Japanese case. He had been noted to have psychomotor retardation since his early childhood and now presents severe psychomotor retardation and muscle atrophy. He shows characteristic facial appearance, hyperelasticity of the skin, joint subluxation and generalized muscular atrophy. Laboratory investigations revealed a low serum copper and ceruloplasmin level as well as intestinal non-absorption of copper. Radiologic imagings showed occipital exostoses and bladder diverticula. The activity of lysyl oxidase, a copper-dependent enzyme involved in cross-link formation in collagen, was decreased in a skin-biopsied specimen. Electronmicroscopic investigation of a muscle biopsy showed irregularity of the myofibrillar network and accumulation of the concentric laminated bodies in the subsarcolemmal regions.

Adult↗
PubMed1991

[Anti-cardiolipin antibody and renal microthrombi in lupus nephritis].

To evaluate the relationship between anticardiolipin (ACL) antibody and microthrombi in renal tissue, we examined sera and renal biopsies from 47 patients of systemic lupus erythematosus (SLE) with lupus nephritis (LN). ACL antibody was measured by an enzyme-linked immunosorbent assay (ELISA). Their renal tissues were examined for histological types of lupus nephritis according to the WHO classification and appearance of renal microthrombi. Positive ACL had been shown in 28 of 47 patients (60%) with LN and in 15 of 18 patients (83%) with WHO IV (diffuse proliferative LN: DPLN). Incidences of renal microthrombi were significantly higher in patients with DPLN (61%) than in patients with all LN (34%) (p less than 0.01). The prevalence of renal thrombosis in patients with ACL (46%) was significantly higher than without ACL (16%) (p less than 0.05). Incidence of positive ACL was 81% in the cases with renal microthrombi and 48% in the cases without them. These findings suggest that there is strong association between ACL and the renal microthrombi in active LN.

Autoantibodies↗
PubMed1991

[A case of Moebius syndrome--electrophysiological studies of facial nerve and brainstem].

A five-year old boy was the product of a 40 week pregnancy by vertex presentation complicated only by threatened abortion at approximately 8 weeks gestation. Apgar score was 5 after one minute. At birth he was noted to have a generalized hypotonia associated with facial diplegia, small mandible, weak suck and swallow reflexes. Admission examination revealed small mandible, mask-like facial expression and mild mental retardation. Cranial nerve examination showed bilateral blepharoptosis and facial nerve palsies. Pupil reflexes were normal, but corneal reflexes were impaired bilaterally. Diplopia due to the left abducens nerve palsy was suggested. There was no atrophy of the tongue. Motor tone, strength, and deep tendon reflexes were normal. A normal 46 XY karyotype was present. The other clinical and laboratory findings were normal. MRI of the brain was unremarkable. The characteristics of electrophysiological studies were summarized as follows: 1) Auditory brainstem evoked responses demonstrated waveforms IV-V were abnormal because their amplitudes were less than 30% of wave I bilaterally. 2) Somatosensory evoked potentials documented by central conduction times from cervical region to sensory cortex were prolonged on both sides. 3) Facial nerve conduction velocity was calculated by evoked EMGs of the mentalis muscle electrically stimulated at two distal points over the marginal mandibular branch. MCV of the left side was reduced (34.2 m/sec). 4) The amplitude of the facial muscle potentials evoked by facial nerve stimulation was reduced on both sides. 5) Blink reflex responses documented by the latency difference of R1 responses between the two sides were prolonged.(ABSTRACT TRUNCATED AT 250 WORDS)

Blepharoptosis↗
PubMed1990

Loss of constitutional heterozygosity in chromosome 10 in human glioblastoma.

Gene deletion of chromosome 10 in 11 tumor tissues removed from nine patients with malignant glioma (seven glioblastomas and two malignant astrocytomas) was investigated. Loss of heterozygosity was found in four of the seven (57%) cases with glioblastoma, whereas heterozygosity was preserved in two malignant astrocytome cases. One glioblastoma case showed loss of heterozygosity only in the recurring tumor tissue, although heterozygosity was maintained in the initial tumor tissue. The significance of loss of heterozygosity in the growth and recurrence of glioblastoma is discussed.

Adolescent↗
PubMed1990

Efficacy of closed-system drainage in treating chronic subdural hematoma: a prospective comparative study.

The authors conducted a prospective comparative study on the recurrence rate of chronic subdural hematoma after the use of two different treatment modalities: burr-hole irrigation of the hematoma cavity with (Group A) and without closed-system drainage (Group B). Thirty-eight patients were studied. Patients were assigned to groups sequentially upon admission. There were no significant differences between the two groups for age, sex, preoperative hematoma volume, and density on computed tomographic scan. One patient in Group A (5%) suffered a recurrence as opposed to 6 in Group B (33%). The difference in recurrence rate between the two groups was statistically significant (P less than 0.05). The authors conclude that closed-system drainage after burr-hole irrigation reduces the recurrence rate of chronic subdural hematoma.

Aged↗
PubMed1990

Ruptured cerebral aneurysms missed by initial angiographic study.

The authors reviewed the computed tomographic (CT) scans of patients with subarachnoid hemorrhage whose initial angiograms were negative, to investigate the validity of CT scans in predicting the presence of an angiographically missed aneurysm in such patients. During the past 14 years, additional angiograms have been obtained for 38 of the 45 patients with subarachnoid hemorrhage whose initial angiograms disclosed no aneurysm. Aneurysms were found in 8 patients; 7 on the anterior communicating artery and 1 at the junction of the internal carotid and posterior communicating arteries. CT scans were taken within 4 days after subarachnoid hemorrhage in 31 patients. Analysis of these scans showed that the second angiogram revealed 1) an aneurysm in 21% of the patients with a thin layer of subarachnoid blood and in 63% of those with a thick layer; 2) no aneurysm in the patients without subarachnoid blood; and 3) an aneurysm of the anterior communicating artery in 70% of the patients who showed a considerable amount of blood in the basal frontal interhemispheric fissure. These results suggest that if CT scans show thin or thick subarachnoid blood, angiographic study should be repeated early in the course. If a considerable amount of blood is shown in the basal frontal interhemispheric fissure, it is highly probable that an aneurysm is hidden on the anterior communicating artery, even if the angiogram is negative for an aneurysm.

Adult↗
PubMed1990

Epidermoid tumor with unusual CT and MR findings--case report.

The authors report a case of epidermoid tumor attached to the dura of the frontal convexity and extending into the cerebral parenchyma, with unusual computed tomographic (CT) and magnetic resonance (MR) findings. The tumor appeared as a mixed density intracerebral mass containing dense calcifications on the precontrast CT scan, and the capsule was intensely enhanced on the postcontrast scan. It appeared as a mixed intensity lesion on both the T1- and T2-weighted MR images.

Brain Neoplasms↗
PubMed1990

Successful removal of an angiographically occult arteriovenous malformation of the ventral medulla.

A case with an angiographically occult arteriovenous malformation (AVM) of the ventral medulla presenting with progressive motor and sensory deficits is reported. The AVM, about 1 x 1 x 0.5 cm in size, accompanied by intramedullary haematoma, was successfully removed through a small incision placed in the right ventrolateral medulla between the vagus and the hypoglossal nerves. The patient's neurological symptoms improved after surgery.

Cerebral Hemorrhage↗
PubMed1990

Choroid plexus arteriovenous malformation in a full-term neonate. Case report.

A full-term newborn infant with intracranial hemorrhage secondary to an arteriovenous malformation (AVM) of the choroid plexus is presented. Although angiography was not undertaken before surgery, a vascular nodule containing an AVM nidus was found in the hematoma wall. The authors stress that an AVM should be considered as a cause of intracranial hemorrhage and that the hematoma wall be searched thoroughly in neonates without predisposing factors.

Cerebral Hemorrhage↗
PubMed1990

[The criteria for indication of plasma exchange on lupus nephritis].

Although many reports have been made on the effectiveness of plasma exchange (PE) in active systemic lupus erythematosus (SLE), but there are no clear-cut criteria of indication for lupus nephritis (LN). In order to determine the criteria for indication of LN, 35 patients with LN treated by PE were studied with respect to renal function (F), proteinuria (P), immunological activity (A) and renal histology. The patients were divided into 4 groups according to the severity of renal function: F1 (stable chronic renal failure (CRF) or stable renal function n = 13), F2 (relapse type n = 9), F3 (rapidly progressive LN; creatinine clearance (Ccr) less than 40 ml/min n = 7) and F4 (acute renal failure; Ccr less than 10 ml/min n = 6). Proteinuria was also studied in 4 groups: P1 & P2 (without nephrotic syndrome (NS], P3 (acute type NS n = 15), and P4 (chronic type NS n = 7). These patients were divided into 3 groups to study immunological activity: A1 (chronic stage n = 6), A2 (relapse stage n = 12) and A3 (active stage n = 17). Comparison was made in each parameter. Renal histological classification according to WHO criteria of LN, activity score (AS) and chronicity score (CS) were evaluated and compared. As a result, the following indication was obtained. 1) ABSOLUTE INDICATION: 1. Rapidly progressive LN with high immunological activity; elevated serum creatinine (SCr) greater than 1.0 mg/dl/month or decreased Ccr from normal renal function to less than 40 ml/min within 1-2 months after onset. 2. Acute type NS within 1 year after onset. 3. histological AS greater than 20. 2) RELATIVE INDICATION: 1. Relapse LN with moderate immunological activity, decreased Ccr from normal function to 40-50 ml/min within 3-6 months, the rise in SCr of greater than 1.0 mg/dl/month. 2. Proteinuria is 1.0-3.5 g/day within 1 year after onset. 3. Such complication as CNS, serositis, thrombocytopenia and leukopenia, steroid resistance and/or severe side effects of steroid. 3) NO INDICATION: 1. CRF or stable renal function (Ccr greater than 50 ml/min). 2. Chronic type NS over 1 years with past history of NS and/or edema. 3. Low immunological activity and mild renal histology.

Adolescent↗
PubMed1990

[Benign familial neonatal convulsion: clinical features of the propositus and comparison with the previously reported cases].

A patient with benign familial neonatal convulsions was presented. The patient had the first episode of cyanosis on the second day of life. Thereafter, he also experienced focal clonic and/or multifocal clonic seizures. The interictal EEG showed no definite abnormality. Between the seizures he appeared well and physical examination was essentially normal. Treatment with phenobarbital (4 mg/kg/day, P. O.) was started and subsequently he had no further seizures until 3 months. At the age of 4 months, he was admitted to the hospital again because of generalized tonic-clonic seizures. The interictal EEG showed sporadic spikes dominantly in the right central area. The findings of ictal EEG at that time are characterized by fast spiking of increasing amplitude during the tonic phase. During the clonic phase, there are repetitive+ bursts of spikes and sharps mixed with persisting muscle potential. The termination of the convulsion is characterized by general voltage depression. Clinical characteristics such as seizure types, EEG findings, responses to antiepileptic drugs and recurrence of the seizures found in our propositus were compared with those of the patients previously reported in the literature.

Adult↗
PubMed1989

Preoperative angiographical prediction of the necessity to removal of the anterior clinoid process in internal carotid-posterior communicating artery aneurysm surgery.

During the last 10 years, we had to resect the anterior clinoid process (ACP) before applying the clip in three of the 70 patients with internal carotid-posterior communicating artery (ICPCom) aneurysms. To reveal the angiographic characteristics in these three patients, we measured the following parameters on carotid angiograms in all 70 patients: 1) the angle between the midline of the skull and the axis of the C1 segment of the internal carotid artery (ICA) on A-P view (angle A). 2) the angle between the axes of the C1 and C2 segments of the ICA on A-P view (angle B). and 3) the distance between the posterior wall of the carotid "knee" and the proximal aneurysmal neck on lateral view (distance d). The common angiographic features of these three patients were as follows: 1) angle A was larger than 60 degrees. 2) angle B was less than 90 degrees. 3) distance d was less than 10 mm. and 4) the posterior communicating artery was of the foetal type. The present results suggest that preoperative angiogram can predict whether or not the ACP should be removed during ICPCom aneurysm surgery.

Carotid Artery Diseases↗
PubMed1989

Huge pineal teratoma mimicking a dermoid cyst. Case report.

A case of huge pineal teratoma mimicking a dermoid cyst is reported. Although both computed tomography and surgical findings were entirely similar to those of a dermoid cyst, close histological examinations using the serial sectioning technique of a small solid portion attached to the pineal region revealed tissue components of both mesodermal and entodermal origins among mostly epidermal and dermal components. The importance of serial sectioning of the surgical specimens of seemingly a dermoid cyst is stressed.

Adult↗
PubMed1989

Histological verification of microaneurysms as a cause of cerebral haemorrhage in surgical specimens.

Surgical specimens taken from 14 patients with lobar intracerebral haemorrhage or cerebellar haemorrhage without vascular abnormalities on angiograms were examined histologically. In seven of the 14 patients, arteriovenous malformation or amyloid angiopathy were found by ordinary pathological examinations. Among the remaining seven patients, definite microaneurysms were verified in five and possible ones in two patients by using the technique of serial sectioning of the solid nodular tissues removed from the presumed bleeding site, where an arterial connection between the tissues and the surrounding brain was noted. Four of these seven patients had no history of hypertension and showed normal blood pressure before and after surgery. To verify microaneurysms in surgical specimens, it seems important to search the presumed bleeding site properly by a meticulous microsurgical technique and to section the tissues serially for the histological examination.

Adult↗
PubMed1989

[Effects of serum lipid concentrations and smoking and drinking habits on serum vitamin A and E levels].

Serum retinol and tocopherol concentrations in 419 males and 478 females, aged 10 to 49 years, were determined by an HPLC method. Then their relationships to serum lipid concentrations and smoking and drinking habits were examined. Retinol levels were higher in males than in females but tocopherol showed little difference by sex. The sex differences in age-related serum levels of retinol and triglyceride (TG) were similar to those of tocopherol and total cholesterol (TC), respectively. Retinol had a significant correlation with TC and TG, with coefficients of 0.20-0.29 (p less than 0.001). These were smaller than those of tocopherol (R = 0.32-0.52, p less than 0.001) both in males and females, suggesting that it had a higher susceptibility to factors other than serum lipids than tocopherol did. Both the retinol and tocopherol levels were significantly higher in the groups with smoking and drinking habits than in the groups without them among the males aged 30 years and over. Furthermore, the retinol level was positively dependent on the daily consumption of both cigarettes and alcohol, whereas tocopherol was dependent on the consumption of alcohol. Multiple regression analysis showed that smoking and drinking habits had statistically significant effects on the serum retinol level independent of other factors and that their effects were greater than those of TC and TG. Tocopherol was affected most by TC and TG and then by drinking habit. Less significant but similar results were obtained for the females of the same age group.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1989

[A case of epilepsy with myoclonoic absences].

A case of epilepsy with myoclonic absences is reported. A boy, 3 years and 8 months old, had the first attack at the age of 1 year and 8 months. He was mentally retarded, but had no evidence of organic brain damages. He had been said to have "absence" at another hospital for 2 years until he was referred to our hospital. The attack was characterized clinically by sudden loss of consciousness accompanied with rhythmical massive myoclonias. The ictal EEGs showed synchronous diffuse 3 c/s spike-wave discharges. There was a strict and constant relation between spike-wave discharges and the myoclonia. The polygraphy recording (EEG and EMG of various muscles) was very helpful for the diagnosis of epilepsy with myoclonic absences. We think that epilepsy with myoclonic absences should be considered in any case of "absence" with concomitant 3 c/s spike-wave discharges which is resistant to appropriate treatment, or is accompanied with mental retardation.

Child, Preschool↗