Biomedical subjects

I Goto

Publications and source records attributed to I Goto.

At least 163 records · Page 9Linked to original sources

PubMed1989

Neurological and radiological studies in painful ophthalmoplegia: Tolosa-Hunt syndrome and orbital pseudotumour.

Twelve patients with idiopathic painful ophthalmoplegia were studied by CT, which was found to be normal in 6 patients, but with a high density area in the orbit in the others. This abnormal high density area was not found when CT was performed at a relatively early stage of the disease, but was seen when it was performed more than 1 month after the onset of symptoms. Orbital phlebography was performed in 6 of 12 patients; 3 with normal and 3 with abnormal CT findings. An occlusion of the superior ophthalmic vein was seen in 3 patients with abnormal CT, but not in 3 patients with normal CT. All 12 patients improved with administration of corticosteroids. The results suggest that the intraorbital abnormality revealed by CT may be due to an inflammatory process in the chronic stage and/or the oedematous and infiltrating processes in soft tissues which can be caused by the occlusion of the superior ophthalmic vein or cavernous sinus.

Adult↗

PubMed1989

Biosynthesis of galactosylsphingosine (psychosine) in the twitcher mouse.

In attempts to elucidate the origin of accumulated galactosylsphingosine in the twitcher mouse, a murine model of human globoid cell leukodystrophy (Krabbe's disease), UDP-galactose:sphingosine galactosyltransferase activity was assayed in tissues from normal and twitcher mice. Among several tissues from normal, 20 day postnatal mice, the highest galactosyltransferase activity was found in the brainstem and spinal cord, followed by cerebrum, kidney and liver, in that order. Chronologically, the enzyme activity in the central nervous tissue increased with age, reached a maximum at 25 postnatal days, and declined thereafter. In the kidney and liver, however, the activity remained much the same during development. In the twitcher mouse, developmental change in the enzyme activity was similar to that seen in control mouse, but the decrease in activity in the central nervous tissue after the 25 postnatal days was more rapid. The galactosyltransferase activity and the accumulation of galactosylsphingosine in the tissue of the twitcher mouse were closely related; where and when the enzyme activity was higher, the greater was the accumulation of galactosylsphingosine in the tissue of the twitcher mouse. These results strongly suggest that the accumulated galactosylsphingosine in the twitcher mouse is synthesized mainly by UDP-galactose:sphingosine galactosyltransferase.

Aging↗

PubMed1989

Metabolism of exogenous galactosylceramide in the twitcher mouse brain.

The in vivo metabolism of galactosylceramide (gal-cer) in normal mice and in twitcher mice, a model of human GLD, was examined following intracerebral administration of gal-cer containing [1-14C] stearic acid. In normal mice, gal-cer was hydrolyzed to ceramide within 6 hours and ceramide was hydrolyzed to sphingosine and fatty acid. Most of the released fatty acid was immediately incorporated into other lipids. About 75% of injected gal-cer was hydrolyzed 80 hours after the injection, while in the twitcher mouse, only 17% of gal-cer was hydrolyzed. These results show that degradation of gal-cer is impaired in the twitcher mouse brain, but contradict to the fact that there was no evidence of any accumulation of gal-cer in the brain. This discrepancy may be due to the different sorting routes of biosynthesized and exogenously-administered gal-cer in the mouse brain. Most of the biosynthesized gal-cer is incorporated into myelin, while the injected gal-cer is incorporated into lysosomes.

Animals↗

PubMed1989

Cerebral blood flow, oxygen and glucose metabolism with PET in progressive supranuclear palsy.

Cerebral blood flow, cerebral oxygen metabolic rate and cerebral glucose metabolic rate were measured with positron emission tomography (PET) in four patients with progressive supranuclear palsy (PSP). Decreased blood flow and hypometabolism of oxygen and glucose were found in both subcortical and cortical regions, particularly in the striatum including the head of the caudate nucleus and the frontal cortex. The coupling between blood flow and metabolism was preserved even in the regions which showed decreased blood flow and hypometabolism. These findings indicated the hypofunction, as revealed by decreased blood flow and hypometabolism on PET, both in the striatum and the frontal cortex, and which may underlie the pathophysiological mechanism of motor and mental disturbance in PSP.

Adult↗

PubMed1989

A comparison of erythrocytes, lymphocytes and blood plasma as samples in fatty acid analysis for the diagnosis of adrenoleukodystrophy.

We studied the very-long-chain fatty acids of blood plasma, erythrocyte membranes and lymphocytes in 4 adrenoleukodystrophy patients, 5 adrenoleukodystrophy obligate carriers, 12 normal controls and 81 patients with various neurological disorders by high-performance liquid chromatography and compared the reliabilities in the diagnosis of adrenoleukodystrophy of these 3 components of peripheral blood. Of 81 patients with various neurological disorders, 2 myotonic dystrophy and 2 spinocerebellar degeneration patients showed increased ratios of C26:0 to C22:0 in erythrocyte membranes, but not in blood plasma and lymphocytes. None of the 12 normal controls showed increased ratios of C26:0 to C22:0 in erythrocyte membranes, blood plasma and lymphocytes. These results suggest that fatty acid analysis for the diagnosis of adrenoleukodystrophy is more reliable when blood plasma and lymphocytes are used than when erythrocyte membranes are used.

Adolescent↗

PubMed1989

Positron emission tomography in epilepsy: correlative study.

Positron emission tomography (PET) was performed with the 18F-fluoro-deoxy-glucose method on 29 patients with epilepsy (generalized epilepsy, 4; partial epilepsy, 24; undetermined type, 1). The subjects were restricted to patients with epilepsy without focal abnormality on X-CT. All the patients with generalized epilepsy showed a normal pattern on PET. Fourteen out of the 24 patients with partial epilepsy and the 1 with epilepsy of undetermined type showed focal hypometabolism on PET. The hypometabolic zone was localized in areas including the temporal cortex in 11 patients, frontal in 2 and thalamus in 1. The location of hypometabolic zone and that of interictal paroxysmal activity on EEG were well correlated in most patients. The patients with poorly-controlled seizure showed a higher incidence of PET abnormality (12 out of 13) than those with well-controlled seizures (2 out of 11). The incidence of abnormality on PET and MRI and the location of both abnormality were not necessarily coincident. These results indicated that the PET examination in epilepsy provides valuable information about the location of epileptic focus, and that the findings on PET in patients with partial epilepsy may be one of the good indicators about the intractability of partial epilepsy, and that PET and MRI provide complementary information in the diagnosis of epilepsy.

Blood Glucose↗

PubMed1989

Decreased fatty acylation of myelin proteolipid protein in the twitcher mouse.

We examined chronological changes of myelin proteins of the brainstem and spinal cord of the twitcher mouse (15, 20, and 30 days old), a murine model of human globoid cell leukodystrophy caused by a genetic deficiency of galactosylceramidase I activity. The yield of myelin was normal until postnatal day 20, whereas galactosylsphingosine (psychosine) accumulated with age in myelin. The protein profiles of myelin and the activity of 2',3'-cyclic nucleotide 3'-phosphodiesterase in the myelin remained normal throughout the experimental period. Fatty acylation of proteolipid protein (PLP) was examined in a cell-free system by incubation of myelin with [3H]palmitic acid, CoA, and ATP, and was normal at postnatal day 15, but decreased after postnatal day 20. Decreased fatty acylation of PLP was also observed in the twitcher mouse at postnatal day 20 when the isolated myelin was incubated with [14C]palmitoyl-CoA in the absence of ATP and CoA, or the slices of brainstem and spinal cord were incubated with [3H]palmitic acid. The activity of fatty acid:CoA ligase was reduced in myelin. These data suggest that decreased acylation of PLP in twitcher mouse myelin is probably due to reduced activities for both activation and transfer of fatty acid into PLP and that metabolic disturbance is present in myelin because acylation of PLP has been shown to occur in myelin membrane. Although psychosine (200 microM) inhibited only 17% of the acylation in vitro, it may be responsible for the reduced acylation of PLP in vivo.

2',3'-Cyclic-Nucleotide Phosphodiesterases↗

PubMed1989

Second interim report of the nation-wide collaborative study on the long-term effects of bromocriptine in the treatment of parkinsonian patients.

The interim data obtained up to the end of the 2nd year of the nation-wide multicentric cooperative study on the long-term effects of bromocriptine in parkinsonian patients revealed the following results. Firstly, the bromocriptine monotherapy could be continued in about 40% of the patients at the mean maintenance dose of 10.8 mg daily. Secondly, the additional use of bromocriptine in parkinsonian patients already treated with levodopa had a favorable influence on late side effects of levodopa such as on-off phenomenon and dyskinesia. No significant difference has been found so far as to the management of the wearing-off phenomenon between the patients with levodopa alone and those with levodopa and bromocriptine.

Adult↗

PubMed1989

[Acute disseminated encephalomyelitis affecting medulla and upper cervical cord--comparison of the clinical signs and MRI findings].

Correlation of MRI abnormalities and clinical manifestations was studied in a case of acute disseminated encephalomyelitis. A 54-year-old man developed Brown-Séquard syndrome affecting the right upper cervical cord, bilateral bulbar palsy, right Horner syndrome, and rotatory nystagmus. After the admission, right facial numbness and weakness of left upper and lower limbs were added. He was treated with glucocorticosteroid successfully. Painful tonic seizures of left upper and lower limbs were noted in the convalescence. T1-weighted MRI of the brain and spinal cord revealed low signal intensity areas in the medulla oblongata and right upper cervical cord. T2-weighted images showed a high signal intensity in the same areas. The distribution of MRI abnormalities well corresponded to the clinically expected lesions. The MRI abnormalities gradually decreased and disappeared with his recovery. The MRI abnormalities appeared to reflect edema accompanying the acute inflammatory process. MRI seems to be a very useful test for verifying lesions in the brainstem and spinal cord in a case of acute disseminated encephalomyelitis.

Acute Disease↗

PubMed1989

[Studies on antigenicity and contact sensitivity of miporamicin].

Experiments were performed to assess antigenicity and contact sensitivity of miporamicin (MPM) with the results leading to the conclusions as follows: 1. In the test to find out if an active systemic anaphylactic reaction would occur in guinea pigs, none of the animals developed any typical signs of anaphylactic reaction. 2. All the sera samples obtained from guinea pigs which had been given sensitizing injections of MPM proved negative for homologous PCA reaction. 3. In a test to see if PCA reaction would occur in rats, all the sera samples obtained from mice which had been given sensitizing injections of MPM did not cause positive reactions. 4. MPM was negative for contact sensitivity tested in guinea pigs; thus, no evidence of contact sensitizing potential was observed. Thus, MPM has proven to be not antigenic in guinea pigs or mice, nor to have any contact sensitivity in guinea pigs under the testing conditions employed.

Anaphylaxis↗

PubMed1989

[Local irritation study of miporamicin in male rabbits].

Local irritation effect of miporamicin (MPM), a new macrolide antibiotics for animal use, on skin and ocular tissues was determined in rabbits by the primary skin irritation test and ocular irritation test, respectively. In the former test, P.I.I. and inflammatory findings increased with increasing exposure time to MPM. A short-time contact with MPM caused relatively little effect on ocular tissues. Contrarily, a long-time contact with MPM caused medium or high degree of inflammatory reactions on cornea, iris and conjunctiva tissues. The increased inflammation was naturally accompanied with increases of the DRAIZE score. All these results suggest that MPM per se has local irritation effect on both skin and ocular tissues.

Animals↗

PubMed1989

[Causalgia in the upper limbs following neck trauma: existence of widespread dysfunction of sympathetic postganglionic fibers].

The functions of autonomic nervous system were systematically evaluated in a case of causalgia in the upper limbs after neck trauma. A 14-year-old boy had had hard blow on his nucha in a rough fight. After one week, a sustained burning pain, swelling and skin color change developed in the left upper limb. These symptoms also appeared in the right upper limb after 6 weeks. The physical examinations disclosed edema, reddish moist skin, and atrophic nail in the upper limbs. The neurological examinations showed a radiating pain to the upper limbs caused by the neck movement or pressure on the supraclavicular fossae, weakness of the upper limbs and left lower limb, and loss of sensation in the 5th to 8th cervical and first thoracic dermatomal segments. Deep tendon reflexes were diminished in the upper limbs and exaggerated in the lower limbs. Neither Horner syndrome nor sphincter disturbance was observed. He was diagnosed as being the cervicothoracic radiculopathy and cervical myelopathy due to the mechanical force. The burning pain disappeared with oral administration of guanethidine. On the examinations of the autonomic functions, the sweating response to the thermal stimulation was absent above the 5th thoracic dermatomal segment. The sweating response to the intradermal acetylcholine was decreased in the second and third thoracic dermatomal segments. The systolic hypotension with increasing pulse rate occurred on standing. The reactive elevation of the blood pressure to the intravenous tyramine was absent. The excessive elevation of the systolic blood pressure was induced by the subcutaneous injection of epinephrine. These results indicated the dysfunction of the sympathetic postganglionic sudomotor and vasomotor fibers.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

PubMed1989

[Flexion myelopathy due to tic of neck].

A 22-year-old male developed a tic of neck-flexion at the age of 14. The tic occurred 40 to 50 times per minute on its peak at age 16. Since then he noticed the atrophy and weakness of his both upper limbs. His right leg became weak at age 22. On admission, neurological examination revealed tic of lip and neck, severe muscle atrophy and weakness of bilateral upper limbs, mild muscle weakness and spasticity of right lower limb and hyperreflexia in four limbs. Needle EMG studies revealed fibrillation, positive sharp wave and giant MUP in the biceps, triceps and first interossei muscles. There were no abnormal findings suggesting cervical spondylosis or disc herniation on neck roentgenogram and neck MRI in neutral position. Neck MRI in the ventro-flexed position showed a flattening of the lower cervical cord and a band-like isointensity lesion in the posterior epidural space at C4-6. This isointensity lesion was considered to represent a congestion of the internal vertebral venous plexus. These findings suggest that frequent neck flexion by itself causes the injury of the lower cervical cord through (1) over-stretching of the cord, (2) compression of the cord by dural sac, (3) arterior ischemia, and/or (4) stagnant hypoxia due to venous congestion. Flexion myelopathy may represent one of the most important mechanisms of cervical cord injury accompanied with involuntary movement of neck.

Adult↗

PubMed1989

[Recurrent unilateral painful oculomotor nerve palsy with an ipsilateral posterior ethmoidal mucocele].

We presented a case of 6 episodes of the right painful oculomotor nerve palsy for 10 years, accompanied by a posterior ethmoidal mucocele on the same side. No destructive bone lesions were shown on every recurrence with the brain CT. For the pathogenesis of neurological involvement in cases of mucocele, either the direct compression by an expanding mucocele or the inflammatory infiltration from a mucocele has been considered. In this case the latter mechanism is considered to be responsible for the etiology of the painful oculomotor nerve palsy. Cases of the painful oculomotor nerve palsy with paranasal lesion or history of parasinusitis are characterized by (1) predominant in male, (2) neurological manifestations unilaterally on the same side of paranasal lesion, and (3) frequent recurrence of neurological symptoms.

Adult↗

PubMed1989

[Familial scapuloperoneal-type myopathy associated with a marked elevation of serum creatine kinase and hepatomegaly].

The proband, a 17-year-old boy, was admitted to our department because of the difficulty in standing on heel. Physical examination revealed a marked weakness and atrophy of bilateral lower legs, especially anterior tibial muscles. Patellar and Achilles tendon reflexes were abolished. Marked hepatomegaly and moderate splenomegaly were noted on abdominal echogram and CT scanning. Serum creatine kinase, lactate dehydrogenase, GOT and GPT were markedly increased. There were no abnormal findings in thyroid function, serum lipid analysis and serum lactate level after ischemic forearm exercise test. EMG of anterior tibial and calf muscles showed a mixture of myogenic and neurogenic patterns and biopsy specimen of calf muscle was compatible with a dystrophic change. Liver biopsy specimen revealed no noticeable change except a slight ballooning of hepatocytes in light microscopy. However, electron microscopic examination showed a marked increase of intracellular vesicles and enlarged smooth ER in which low-density, cotton-like materials were contained. In family study, both his father and paternal uncle were also affected with advanced scapuloperoneal-type myopathy associated with a marked elevation of serum creatine kinase and hepatomegaly. The disorder differs from Miyoshi's distal muscular dystrophy, which shows an early involvement of flexor muscles in lower extremities and is inherited as an autosomal-recessive trait. Although the etiology of hepatomegaly in this case remains to be elucidated, the special findings on electron microscopic study imply the possibility of some unknown metabolic disorder involving both muscle and liver. This disease seems to be a new type of scapuloperoneal-type myopathy, probably having an autosomal-dominant inheritance.

Adolescent↗

PubMed1989

[A female case of carnitine palmitoyltransferase deficiency].

A 17-year old woman noted myalgia after taking a long distance walk at the age of 10. In adolescence, she had several episodes of myalgia and pigmenturia after athletic activity or infection. At age 17, myoglobinuria and abnormally increased serum creatine kinase were documented after one of these episodes. The neurological examination revealed mild proximal muscle weakness of upper extremities. Electromyography showed myogenic patterns, such as brief, small abundant potentials on them. Venous lactate was raised normally on the ischemic exercise test. During prolonged fasting, plasma ketone bodies increased normally but there were abnormal elevations of plasma creatine kinase and myoglobin. Morphometric analysis of electron microscopy in muscle showed few lipid deposits and that of light microscopy revealed no abnormality. CPT activity in muscle was only 15% of normal value by the isotope-exchange assay. These results were consistent with the diagnosis of CPT deficiency. Although several cases of CPT deficiency with recurrent myoglobinuria have been reported in Western countries, our patients is the first case of Japanese showing recurrent myoglobinuria. CPT deficiency should be considered as a differential diagnosis in cases of recurrent myoglobinuria.

Acyltransferases↗

PubMed1988

The twitcher mouse: accumulation of galactosylsphingosine and pathology of the sciatic nerve.

Morphological and biochemical changes were investigated in the early developmental stages of sciatic nerve of the twitcher mouse, a murine model of human globoid cell leukodystrophy. The concentration of galactosylsphingosine (psychosine) and the chronological changes of the twitcher mouse peripheral nerve pathology correlated well. Galactosylsphingosine had already accumulated at birth and dramatically increased with age. Characteristic inclusions were observed in Schwann cells and macrophages of the twitcher mouse on the 5th postnatal day. Endoneurial edema developed after 10 postnatal days and the hypomyelination was pronounced at 15-20 postnatal days. These findings suggest that galactosylsphingosine is cytotoxic for myelin-forming cells and is closely related to pathogenetic events in the twitcher mouse.

Aging↗

PubMed1988

A high-performance liquid chromatographic assay for acid ceramidase activity in cultured fibroblasts from patients with Farber's disease and from controls.

The high-performance liquid chromatographic (HPLC) method we devised for assay of acid ceramidase activity involves coupling of a fluorescent probe to the enzymatically released sphingosine in the reaction mixture and detection of the fluorescent sphingosine derivative by reverse-phase HPLC. Using the method, acid ceramidase activity in fibroblast homogenates was accurately assayed, with or without the addition of exogenous ceramide, as the substrate, and the patients and carriers of Farber's disease could be readily diagnosed.

Acid Ceramidase↗