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Biomedical subjects

H Narabayashi

Publications and source records attributed to H Narabayashi.

At least 19 recordsLinked to original sources
PubMed1992

Juvenile parkinsonism: ventricular CSF biopterin levels and clinical features.

Total biopterin (T-BP) levels in the ventricular cerebrospinal fluid (CSF) and clinical features of 19 patients with juvenile parkinsonism (JP: Parkinson's disease manifesting below the age of 40) were evaluated and compared with 61 patients with classical Parkinson's disease (classical PD: symptoms developing at the age of 40 or above). The JP patients were divided into two subgroups: JP-I; those with good response to levodopa followed by marked motor fluctuations and dopa-induced dyskinesias (DID), JP-II; those with milder response than JP-I with less fluctuations and DID being more similar to classical PD. Both of the mean ventricular CSF T-BP concentrations in the JP and classical PD patients were significantly lower than that in neurological controls. Moreover, the mean T-BP level in the JP-I was markedly lower than that in the JP-II or classical PD. Total biopterin levels revealed a gaussian distribution in the classical PD. However, a bimodal distribution was noted in the JP, with the lower peak consisting of only JP-I patients. These results seem to indicate that JP-II represents early-onset classical PD, while JP-I represents a distinct subgroup having a different physiopathology from classical PD.

Adult↗
PubMed1992

Analysis of intention tremor.

A marked effect of stereotaxic thalamotomy on intention tremor is described and a neurophysiological interpretation is offered. Tremor-generating activity seems to start in the ventral intermediate nucleus (VIM) of the thalamus, as revealed by recording of the unitary activity through a microelectrode at the tip of the insertion needle, after diminution of facilitatory input due to pathology of the cerebellum or its efferent pathway to the cerebrum. This secondary change within the VIM and the loss of facilitatory input leads to an intention tremor as one of the cerebellar symptoms seen in various neurological diseases.

Adult↗
PubMed1991

Destruction of norepinephrine terminals in 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP)-treated mice reduces locomotor activity induced by L-dopa.

There is little information concerning the effects of norepinephrine (NE) depletion on clinical features of patients with Parkinson's disease. By inducing two types of experimental parkinsonism, one with a dopamine (DA) deficiency alone and the other with both a DA and NE deficiency, we attempted to evaluate the differences in locomotor activity and behavioral responses between the two groups after L-DOPA administration. The results of the study revealed that increases in locomotor activity were markedly suppressed in the DA and NE deficient group. This may suggest that with striatal DA deficiency the central NE terminals play a significant role in the increase in locomotor activity after L-DOPA administration.

1-Methyl-4-phenyl-1,2,3,6-tetrahydropyridine↗
PubMed1991

Total biopterin levels in the ventricular CSF of patients with Parkinson's disease: a comparison between akineto-rigid and tremor types.

Total L-erythro-biopterin (T-BP) levels in the ventricular cerebrospinal fluid (CSF) were measured in 43 patients with Parkinson's disease (PD) and 12 age-matched neurological controls. In 5 of the PD patients and 1 control, lumbar CSF T-BP values were also measured. The mean ventricular CSF T-BP level in the PD patients, 15.6 +/- 0.5 pmol/ml (mean +/- SE), was significantly lower than that in the controls (21.3 +/- 1.4 pmol/ml, P less than 0.0001). The mean T-BP concentration in the ventricular CSF was 1.9 times higher than that in the lumbar CSF (P less than 0.0005), indicating a rostrocaudal gradient for the T-BP value in the CSF. When the PD patients were classified according to their predominant clinical features into 24 akineto-rigid (A-R) type and 19 tremor (T) type, there was a significant negative correlation between the T-BP levels and duration of illness only for the A-R type patients (rho = -0.605, P less than 0.005). No such significant correlation was found in the T type patients. These results may indicate a difference of pathophysiological changes in the brain between the 2 types of PD.

Biopterins↗
PubMed1991

New pathologic observations in juvenile onset parkinsonism with dystonia.

A patient with hereditary juvenile onset parkinsonism with dystonia died at age 39. There were Lewy bodies and regionally selective neuronal damage in the substantia nigra pars compacta. These changes closely resemble those seen in Parkinson's disease, and emphasize the selective vulnerability of the ventral tier of the pars compacta in these degenerations.

Adult↗
PubMed1991

[Analysis of L-threo-3, 4-dihydroxyphenylserine effect on motor and psychological symptoms in Parkinson's disease].

Improvement of motor and psychological symptoms by L-DOPS (L-threo-3,4-dihydroxyphenylserine) in totally 20 cases with Parkinson's disease (PD), including 5 cases of juvenile or early onset parkinsonism (JP) and one case of pure akinesia was analysed. Improvement was obtained in about two thirds of the cases on symptoms of freezing in gait, difficulty of postural control, depressive mood and bradyphrenia. Severity of freezing in gait and that of the depressive mood were graded in five stage (from 0 to 4) scale and the improvement was evaluated by A (three stage improvement), B (two stage improvement), C (one stage improvement) and D (no change or worsened). Improvement of psychological symptoms was seen parallel to that of motor symptoms. It seems important that marked effect on both motor and psychological symptoms was obtained mostly in PD cases but not in the cases of JP. In MMPI test, depressive score (D) and hypochondriac score (Hs) were normalized in PD cases but not changed in JP, indicating differences in psychological traits between two groups. It was suggested that JP is a condition of mainly DA deficiency in nigro striatum but PD presents wider spectrum of symptoms covering both DA and NE deficiency. Importance of the role of aging of the brain in each individual patient is discussed and interpreted in relation to the difference of clinical pictures.

Aged↗
PubMed1990

Studies on DNA markers (D4S10 and D4S43/S127) genetically linked to Huntington's disease in Japanese families.

This is the first full report on the genetic linkage between Japanese Huntington's disease and the DNA markers D4S10 and D4S43/S127. With use of the HindIII, BglI, and EcoRI polymorphisms detected at D4S10, and the combination of all these polymorphisms to give composite haplotypes, nine Japanese Huntington's disease families were found to be informative. Three recombinants for D4S10 were detected in these families, giving a maximum lod score of 1.662 at a theta of 0.10. Similarly, when we used the MspI and PvuII polymorphisms detected by D4S43/S127, five families gave informative results. No recombinant was detected in these families, giving a maximum lod score of 3.348 at a theta of 0.00. These results clearly support the view that the Japanese Huntington's disease gene may be identical with the Western gene, in spite of the lower prevalence rate in Japan.

Adolescent↗
PubMed1990

[The effect of L-threo-DOPS on P-300 in parkinsonism].

The P-300 has been recently utilized as an objective electrophysiological index of cognitive function in some neurological disease. Hansch et al first described prolonged latency for the P-300 component in Parkinson disease patients and suggested these measures reflects a common, disrupted aspect of cognitive function in this disease. This cognitive disorder may be caused not only from the dysfunction of dopaminergic system but from non-dopaminergic lesions such as norepinephrine ones. On the other hand, L-threo-DOPS, a non-physiological precursor of norepinephrine, has been used for the treatment of some neurological illness such as the freezing phenomenon in Parkinsonism. In the present study we investigate the effect of L-threo-DOPS on cognitive function in Parkinsonism by measuring P-300 component succeedingly. Twenty-four patients, i.e. 19 cases of Parkinson disease (PD), 3 Juvenile Parkinsonism (JP) and 2 cerebrovascular Parkinsonism, are studied. The latencies of P-300 are significantly shortened after treatment of DOPS compared with those of the previous treatment and placebo administration respectively (p less than 0.05, p less than 0.01). The main factors which have influence upon these shortened latencies are analyzed multivariate analysis. The factor analysis and principal component analysis suggest three main factors such as DOPS, age (onset of age), and duration of illness. The patients' groups are divided into three by cluster analysis. The most effective group mainly consists of JP. The least effective one mainly consists of older, long-duration Parkinson disease patients. And there is another group which consists of younger, short-duration PD cases with moderate effect of DOPS. This tendency seems to suggest the different pathological findings between JP and PD. It is postulated that L-threo-DOPS would raise the level of attention and arousal mediated by norepinephrine neuronal system which may improve the cognitive disorder in Parkinsonism.

Aged↗
PubMed1989

Beta 2-microglobulin decrease in cerebrospinal fluid from parkinsonian patients.

A sandwich enzyme immunoassay (EIA) was established by using purified beta 2-microglobulin (beta 2-MG) as a standard protein and a polyclonal antibody raised against human beta 2-MG. The EIA was applied for the measurement of beta 2-MG levels in human cerebrospinal fluid (CSF) from parkinsonian patients and control patients devoid of neurological diseases. beta 2-MG contents in CSF of the control group and the parkinsonian group were 1.81 +/- 0.11 micrograms/ml CSF and 0.63 +/- 0.09 microgram/ml CSF, respectively. Thus, beta 2-MG content in CSF was reduced in parkinsonian patients to less than 35% of the control value (P less than 0.005). We had previously reported that the activity and content of dopamine beta-hydroxylase (DBH) were decreased in CSF from parkinsonian patients. A significant positive correlation (r = 0.87) was observed between the beta 2-MG content and DBH activity for CSF from 45 patients. These results suggest a probable link between an immunological change and the changes in catecholaminergic neurons in Parkinson's disease.

Adult↗
PubMed1989

Difference in recovery patterns of striatal dopamine content, tyrosine hydroxylase activity and total biopterin content after 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP) administration: a comparison of young and older mice.

Striatal dopamine (DA) content, tyrosine hydroxylase (TH) activity, and total biopterin content were measured as parameters of recovery, after administration of 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP) in 7-week-old (young) and 28-week-old (older) C57 BL/6 mice. After 10 consecutive days of injection of MPTP (30 mg/kg/day) young mice were sacrificed at one day and 2, 4, 8, 12 and 20 weeks; older mice (20 mg/kg/day) at one day and 4, 8, and 12 weeks. All 3 parameters were markedly reduced one day after the last injection of MPTP. During the observation period, the parameters showed a gradual and partial recovery. The recovery rates of the 3 parameters differed significantly, especially during the early phases of 2-8 weeks. Total biopterin content showed a greater rate of recovery than TH activity and TH activity, a greater rate of recovery than DA content. In the older mice group, the recovery of all 3 parameters was retarded significantly, and dissociation of the recovery rates between the 3 parameters was more prominent. The results of our present study suggest that, following neurotoxic injury, the recovery of biopterin may play a significant role in dopaminergic terminal regeneration.

1-Methyl-4-phenyl-1,2,3,6-tetrahydropyridine↗
PubMed1989

Unresponsiveness to L-DOPA in parkinsonian patients: a study of homovanillic acid concentration in the cerebrospinal fluid.

In this study, concentrations of homovanillic acid (HVA), a principal metabolite of dopamine (DA), in the cerebrospinal fluid (CSF) were measured in 17 patients with Parkinson's disease (PD) who had been effectively treated with L-DOPA and in 8 patients clinically diagnosed as having striatonigral degeneration (SND) and for whom treatment with L-DOPA had proved ineffective. L-DOPA administration, consisting of a daily dosage of 600 mg plus 150 mg aromatic L-amino acid decarboxylase inhibitor was continued in all cases for at least 3 months. In the PD group, the HVA value was 511.6 +/- 196.5 pmol/ml and in the SND group, 144.9 +/- 83.4 pmol/ml. This remarkably low value for the SND group suggests that there may exist severe disorders in the uptake and decarboxylation of exogenous L-DOPA.

Aged↗
PubMed1989

Microphysiological recordings at CT gantry site during stereotactic thalamotomy.

Neural unit recording was performed during CT-guided stereotactic thalamotomy in 2 patients referred for severe cerebellar ataxia and dysmetric movements. Periodic CT scanning was performed during the recording in order to verify probe location and trajectory. Our experience with electrophysiological recordings at the CT gantry site demonstrates the feasibility of acquiring satisfactory neural unit recordings in spite of the high-voltage environment.

Brain↗
PubMed1989

Striatal symptoms.

Classical striatal symptoms were interpreted through identification of the underlying projection system, by physiological analysis of neuronal activity of each subnucleus in the base of the thalamus during stereotactic procedures for various involuntary movements. Rigidity, levodopa-induced dyskinesia, idiopathic dyskinesia, symptomatic athetoid movement and symptomatic choreic movement, as well as that in Huntington's disease, belong to the category of striatal symptoms. These symptoms may be alleviated by surgery on the pallido-Vo-complex projection, which is the main output affected by disorders due to striatal dysfunction.

Brain↗
PubMed1989

Adrenoleukodystrophy presenting as spinocerebellar degeneration.

The clinical features of 3 patients from a kindred with adrenoleukodystrophy and the analysis of their plasma sphingomyelin are described. Onset of symptoms was between the ages of 33 and 54 years. Ataxic gait and spasticity were the only symptoms noted during the early stage of the disorder. Dementia and optic atrophy were present in two of the cases. Baseline plasma cortisol was normal, but adrenocorticotropic hormone was elevated. Analysis of plasma sphingomyelin demonstrated an increase in very-long-chain (C24-C26) fatty acid. This study demonstrates that adrenoleukodystrophy may present with spinocerebellar symptoms.

Adrenocorticotropic Hormone↗
PubMed1989

Stereotaxic Vim thalamotomy for treatment of tremor.

Pathological tremor is alleviated almost totally and immediately, also sustainedly, by a selective surgical lesion within the ventral part of the Vim thalamus. Since the Vim receives its proprioceptive sense from the periphery, tremor is interpreted as the phenomenon based on the circuit involving both central and peripheral mechanisms. The importance of the role of the spinal reflex mechanisms is discussed.

Humans↗
PubMed1988

Hemiparkinsonism in monkeys after unilateral caudate nucleus infusion of 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP): behavior and histology.

Systematically administered 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP) is biotransformed into 1-methyl-4-phenylpyridinium ion (MPP+), which enters dopaminergic neurons via the dopamine uptake system to destroy nigral cells. Either MPP+ is retrogradely transported to the cell body after being taken up at the nerve terminals, or the dopamine uptake sites on the cell body and its dendritic processes are responsible for the toxin directly entering the neuron. Using a 200 microliter osmotic minipump, we administered 4 mg of MPTP HCl directly into the unilateral caudate nucleus, i.e., the dopamine nerve terminal area, of monkeys for 14 days. Persistent hemiparkinsonism began to appear in a week. Each monkey exhibited a flexed posture and hypokinesia of the contralateral limbs and circling toward the MPTP-treated side. These disturbances developed within 3 months and maintained a plateau for 3 months until the day of sacrifice. After treatment with apomorphine, there appeared a striking circling away from the MPTP-treated side. Selective cell loss in the MPTP-treated side of the substantia nigra pars compacta was found along the entire rostrocaudal extent relative to the untreated side. In conclusion, MPP+ uptake only at the dopamine nerve terminals and retrograde axonal transport to the cell body seemed sufficient to destroy nigral dopamine cells in the monkey.

1-Methyl-4-phenyl-1,2,3,6-tetrahydropyridine↗