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Biomedical subjects

H Tohgi

Publications and source records attributed to H Tohgi.

At least 91 records · Page 5Linked to original sources

[Trace elements analysis of serum and cerebrospinal fluid with PIXE--effect of age and changes in parkinsonian patients].

The concentrations of Br, Cu, Fe, Se, Zn, Mg were determined by particle-induced X-ray emission (PIXE) in serum and cerebrospinal fluid (CSF) of 13 untreated, 7 L-dopa treated parkinsonian patients and 25 controls. There were significant positive correlations between serum concentrations and CSF concentrations of Br, Cu, Fe. The Cu/Zn ratio in the serum and the CSF/serum ratio of Br were significantly increased with age in normal controls. The mean CSF concentration of Mg in both treated and untreated parkinsonian patients was lower than in controls. PIXE is a valuable method for multielemental analysis of the serum and CSF.

Adult↗

[A case of progressive supranuclear palsy showing improvement of rigidity, nuchal dystonia and autonomic failure with trazodone].

A 63-year-old man was admitted to the hospital with a 1,5-year history of progressive dementia, supranuclear ophthalmoplegia, pseudobulbar palsy, rigidity and dystonia in the neck and the upper trunk. Magnetic resonance imagings showed severe atrophy of the frontal lobe and the brainstem. He was diagnosed as having progressive supranuclear palsy (PSP). Rigidity, nuchal dystonia, frequent micturition, and profuse sweating ameliorated after trazodone administration. Furthermore, additional administration of L-dopa and droxidopa improved his pseudobulbar palsy, akinesia, and lack of initiative. Single photon emission tomography using IMP after medication showed increased IMP-uptake in the frontal areas and the basal ganglia compared with that before medication. This patient illustrates a substantial role of impairments in the serotonin system in the production of some PSP symptoms.

Autonomic Nervous System Diseases↗

[Genomic analysis of Japanese patients with adult-type metachromatic leukodystrophy].

We performed the genomic analysis of arylsulfatase A (ASA) gene in five Japanese patients with adult-type metachromatic leukodystrophy (MLD) including two sibling cases. Sequencing of amino acid coding region of ASA gene of proband case of family A disclosed 426Pro (CCG)-->Leu (CTG) mutation, which was reported to be frequently found in Caucasian patients with late-onset MLD. We developed mismatch primer PCR/RFLP method for detection of this mutation. If 426Pro-->Leu mutation exists in genomic DNA, Pst I site is newly created by PCR with a 3'-primer mismatched at one nucleotide. Genomic analysis of family A members using this method revealed that younger patient was homozygote of 426Pro-->Leu mutation and patient's parents and her younger brother were heterozygotes, which were confirmed by sequencing of exon 8 of ASA gene. Screening of this mutation using mismatch primer PCR/RFLP method was performed in one sibling case and one autopsy case. This point mutation was found in the sibling case. These results showed the possibility of world-wide spread of 426Pro-->Leu mutation in late-onset MLD patients and usefulness of our mismatch primer PCR/RFLP method for screening of this mutation.

Adult↗

[In situ expression of IL-1 beta and IL-2 mRNA in thymuses from patients with myasthenia gravis].

The expression of IL-1 beta mRNA and IL-2 mRNA was analyzed in seven thymuses from patients with myasthenia gravis (MG) and in three normal thymuses using in situ hybridization techniques. Cells containing IL-beta mRNA were detected in all seven cases. These IL-1 beta producing cells were mainly located and clustered in the connective structures of thymic septae and peri-lobuler area like anti-keratin AE3 positive cells. The distribution of anti-CD68 positive cells was different from that of IL-1 beta producing cells in thymus of myasthenia gravis patients. IL-2 producing cells were also found in all seven cases, and their frequency (number of cells/mm2) had a tendency to correlate with the severity of the disease. On the other hand, we could not find both IL-1 beta mRNA and IL-2 mRNA in three normal thymuses. These results suggested a possible role of high IL-1 beta and IL-2 production to activate T cells in myasthenia gravis thymus. Furthermore, in situ production of IL-2 might be reflected in the severity of the disease.

Adolescent↗

[Three siblings of painful muscle cramps (generalized muscle cramp disease) with alopecia and endocrinological disorders].

Three siblings with generalized painful muscle cramps, generalized alopecia, and endocrinological abnormalities are presented. Their clinical features are very similar to those in sporadic cases reported as having generalized muscle cramp disease. Autosomal recessive inheritance was suggested in our patients. Abnormal laboratory tests include hypersecretion of insulin after glucose loading, elevated levels of luteinizing hormone (LH) and follicular stimulating hormone (FSH), and hypersecretion of LH in the LH-RH test. An elevation of IgG and IgG index in the cerebrospinal fluid (CSF) of two patients suggests IgG production in the CSF. Oral administration of 75 to 150 mg of dantrolene sodium decreased the frequency, intensity, and duration of cramps in all cases. Autoimmune mechanisms based upon hereditary abnormalities are suggested as a cause of their disease.

Adult↗

Alterations in the concentration of serotonergic and dopaminergic substances in the cerebrospinal fluid of patients with Parkinson's disease, and their changes after L-dopa administration.

In untreated patients with Parkinson's disease (PD), the total (free and conjugated) serotonin (5-HT) and dopamine (DA) concentrations in the cerebrospinal fluid decreased significantly. While the 5-HT concentration displayed a non-significant trend of negative correlation with the DA concentration in controls, it had a significant positive correlation with the DA concentration in untreated PD patients. In L-dopa-treated patients, the DA concentration increased remarkably, whereas the 5-HT concentration decreased further compared with untreated patients. The tryptophan, 5-hydroxyindolacetic acid (5-HIAA), and 3-OH kynurenine concentrations had significant positive correlations with L-dopa doses. The 5-HT concentration had a significant positive correlation with scores of psychomatric testing in L-dopa-treated patients.

Aged↗

Concentrations of serotonin and its related substances in the cerebrospinal fluid of parkinsonian patients and their relations to the severity of symptoms.

We evaluated the concentrations of free and total serotonin (5-hydroxytryptamine, 5-HT) and its related substances in the cerebrospinal fluid from patients with Parkinson's disease (PD). The concentrations of total 5-HT, 5-hydroxytryptophan, kynurenine and 3-hydroxykynurenine decreased significantly in PD patients compared with controls. The concentration of total 5-HT had significant negative correlations with Hoehn and Yahr's stages, the severity of rigidity, akinesia and gait freezing; the correlation with gait freezing was most conspicuous.

5-Hydroxytryptophan↗

The effect of age on concentrations of monoamines, amino acids, and their related substances in the cerebrospinal fluid.

We studied age-related changes in the concentrations of monoamines, amino acids, and their related substances in the cerebrospinal fluid on 144 neurologically normal subjects. The concentrations of tyrosine, 3-O-methyldopa, dopamine (total), norepinephrine (total), homovanillic acid, p-hydroxyphenylacetic acid, and 5-hydroxytryptophan increased significantly with age (p < 0.05), and the concentration of 3.4-dihydroxyphenylacetic acid displayed a non-significant trend to decrease, whereas concentrations of other monoamine precursors and metabolites were unchanged. We found the significant positive correlations between the concentrations of HVA and 5-HIAA (p < 0.001), between tyrosine and tryptophan (p < 0.001), and between tyrosine and 3-O-methyldopa (p < 0.001). The concentrations of asparagine, glycine, taurine, and alanine increased significantly with age (p < 0.05), while glutamine, arginine, and threonine concentrations did not change with age. The aspartate, glutamate, and GABA concentrations displayed the non-significant trends to decrease in the elderly subjects. The concentrations of aspartate, glutamate, and GABA had mutually significant positive correlations (p < 0.05), but had significant negative correlations with the concentrations of some neutral amino acids. The urate and xanthine concentrations increased significantly with age (p < 0.01). These findings suggest that the concentrations of monoamine and amino acid transmitters and their related compounds in the cerebrospinal fluid reflect age-related changes in the synthesis, release, and reuptake mechanisms of the transmitters and their transport mechanisms across the blood-brain barrier.

Adult↗

Monoamine metabolism in the cerebrospinal fluid in Parkinson's disease: relationship to clinical symptoms and subsequent therapeutic outcomes.

We correlated monoamine concentrations in the cerebrospinal fluid from de novo (untreated) patients with Parkinson's disease with their clinical symptoms and therapeutic outcome after two years of L-dopa with/without other anti-parkinson medication. A significant correlation was found between the severity of some parkinsonian symptoms and the reduction in particular monoamines: Hoehn and Yahr's stage with dopamine, norepinephrine, and homovanillic acid: rigidity with dopamine; akinesia with dopamine and norepinephrine; freezing of gait with norepinephrine; and dementia with dopamine and homovanillic acid. Tremor had no correlations with the concentrations of the monoamines measured. Patients with dementia had a significantly increased level of epinephrine concentrations. Insufficient therapeutic responses of individual symptoms were associated with significantly decreased concentrations of particular monoamines before treatment: Hoehn and Yahr's stage with norepinephrine and epinephrine; akinesia with homovanillic acid and 5-hydroxyindoleacetic acid; and freezing of gait with dopamine, norepinephrine, homovanillic acid, and 5-hydroxyindoleacetic acid. These results suggest a significant correlation between the reduction in particular monoamines and the severity of some parkinsonian symptoms and their subsequent responses to L-dopa.

Aged↗

The effects of L-threo-3,4-dihydroxyphenylserine on the total norepinephrine and dopamine concentrations in the cerebrospinal fluid and freezing gait in parkinsonian patients.

We studied the effects of L-threo-DOPS (L-DOPS) on the concentrations of total (conjugated and unconjugated) dopamine (DA) and norepinephrine (NE) in the cerebrospinal fluid (CSF) of parkinsonian patients with freezing phenomenon. The NE concentration increased remarkably and dose-dependently after administration of L-DOPS in both L-dopa/carbidopa-pretreated and untreated patients. The DA concentration also increased mildly but significantly in L-dopa/carbidopa-untreated patients. Freezing phenomenon improved in 6 out of 8 patients at Hoehn and Yahr's stage III, and 1 out of 5 patients at stage IV. These results indicate that L-DOPS administration increases the NE concentration dose-dependently, and is effective for freezing of gait of moderate severity.

Aged↗

The urate and xanthine concentrations in the cerebrospinal fluid in patients with vascular dementia of the Binswanger type, Alzheimer type dementia, and Parkinson's disease.

We determined the urate and xanthine concentrations in the cerebrospinal fluid (CSF) in patients with vascular dementia of the Binswanger type (VDBT), Alzheimer type dementia (ATD), and Parkinson's disease (PD). We found that the urate concentration was significantly increased in VDBT patients, but significantly decreased in ATD patients compared with controls. The ratio of the concentrations of uric acid (UCSF) to xanthine (XCSF) in the CSF (UCSF/XCSF) had a significant correlation with the ratio of the UCSF to the urate concentration in serum (U(serum)) (UCSF/U(serum)) in ATD and PD, whereas UCSF/U(serum) increased independently of UCSF/XCSF in VDBT. We concluded that the significant increase in the urate concentration in VDBT is mainly due to an impairment of the blood-brain barrier (BBB), and its significant reduction in ATD may reflect impaired brain metabolism.

Aged↗

Cerebellar infarction. Clinical and neuroimaging analysis in 293 patients. The Tohoku Cerebellar Infarction Study Group.

BACKGROUND AND PURPOSE: We performed this multicenter study to explore the full spectrum of the clinical characteristics and neuroimaging findings of cerebellar infarction, including patients with mild to severe illnesses. METHODS: We studied 293 consecutive patients with cerebellar infarction diagnosed by computed tomography and/or magnetic resonance imaging who were admitted to 36 hospitals during 5 years. RESULTS: Cerebellar infarcts constituted 2.3% of the total patients with acute brain infarction. The backgrounds and risk factors were similar to those in patients with infarctions of the cerebral hemispheres. At least 24% were embolic, and the diagnosis of embolism could not be ruled out in 27%. Infarcts involving the superior cerebellar artery (SCA) region (52%) and the posterior inferior cerebellar artery (PICA) region (49%) were far more frequent than those involving the anterior inferior cerebellar artery (AICA) region (20%). Patients with SCA infarcts exhibited obtunded consciousness and ataxia more frequently than those with PICA infarcts (P < .05). Infarcts in the PICA regions were associated with abnormalities of the PICA (64%) or the vertebral arteries (57%), whereas infarcts in the SCA and AICA regions were associated with abnormalities in the SCA or AICA, respectively, in approximately 30% of patients, in the basilar artery in approximately 16%, and in the vertebral artery in more than 60% of patients. Outcomes were poorer with SCA infarcts than with AICA and PICA infarcts. CONCLUSIONS: These data indicate similar frequencies of SCA and PICA infarcts and illustrate the difference in clinical presentation and outcomes between SCA and PICA infarcts. They also indicate that not only in situ thrombosis but also cardiogenic or artery-to-artery embolism and the insufficiency of collateral circulation play important roles in the pathogenesis of cerebellar infarction.

Aged↗

Coagulation-fibrinolysis system in poststroke patients receiving antiplatelet medication.

BACKGROUND AND PURPOSE: We studied the activities of the coagulation-fibrinolysis system in the chronic stage of poststroke patients and the effect of antiplatelet medication on the system. METHODS: We determined fibrinogen, antithrombin III, thrombin-antithrombin III complex, tissue plasminogen activator antigen, plasminogen activator inhibitor-1, plasmin-alpha 2 plasmin inhibitor complex, and D-dimer in plasma from 153 poststroke patients in the chronic phase (ie, 33 patients not receiving antiplatelet medication, 78 patients receiving 200 mg/d ticlopidine, and 42 patients receiving 40 mg/d aspirin), and compared the results in control subjects and among the treatment groups. RESULTS: The concentrations of fibrinogen, thrombin-antithrombin III complex, antithrombin III, plasmin-alpha 2 plasmin inhibitor complex, and tissue plasminogen activator were slightly but significantly increased in all treatment groups compared with control subjects (P < .01) but did not differ among the treatment groups. The plasminogen activator inhibitor-1 levels were significantly elevated in patients not receiving antiplatelet medication compared with control subjects (P < .01), whereas they were in the normal range and significantly lower in patients receiving ticlopidine or aspirin than in patients not receiving antiplatelet medication (P < .01). The plasminogen activator inhibitor-1 levels were significantly lower in patients whose platelet aggregation was inhibited by antiplatelet medication than in patients with uninhibited platelet aggregability (P < .05). CONCLUSIONS: These findings suggest that coagulation-fibrinolysis markers are mildly increased in poststroke patients in the chronic phase and that antiplatelet medication is effective in reducing the elevated plasminogen activator inhibitor-1 levels.

Aged↗

[Two siblings with metachromatic leukodystrophy of adult and juvenile onset].

We reported two siblings with metachromatic leukodystrophy of adult and juvenile onset. Patient 1, a 24-year-old female had been unremarkable until 23 years, when she began to develop loss of spontaneity, bradykinesia and gait disturbance. Nine months later, she became unable to walk and mentally deteriorated. Neurological examination disclosed dementia, frontal signs and hyperreflexia. Patient 2, a 22-year-old male, brother of patient 1, whose fetal development and birth were uneventful, developed muscle weakness at 12 years old, had difficulty in walking and writing at 13, mental deterioration at 16 and became bedridden and presented with tonic-clonic seizures at 19. Neurological examination at 24 revealed dementia, frontal signs, hyperreflexia and ataxia. In both patients computed tomography scans displayed low density in the cerebral white matter, and the nerve conduction velocities were decreased. Arylsulfatase A activities in urine (33% of normal) and leukocytes (8% of normal) were markedly reduced. Molecular genetic analysis identified the mutation 426Pro --> Leu (allele 426) as has been reported in adult type metachromatic leukodystrophy in Germany. As far as we know, only one sibling with adult type or juvenile type metachromatic leukodystrophy has been reported in Japan. This is the first report of siblings with 426Pro --> Leu mutation in Japan.

Adult↗

[Lateral medullary syndrome due to cavernous malformation in the brain stem].

A 59-year-old female was admitted with complaints of vertigo, dysarthria and dysphagia. On neurological examination, right-sided cranial nerve signs included ptosis, Bruns's nystagmus, decreased corneal sensation, diminished facial pain and temperature sensation, decreased palatal excursion and loss of gag reflex. There was no evident motor weakness, but deep tendon reflexes were slightly exaggerated on the left extremities. Coordination testing showed right cerebellar signs. Sensory examination of the remaining parts of the body was quite normal. X-ray CT scan showed multiple high density areas in the right medulla, right pons, right temporal and frontal lobes. T2 weighted MRI demonstrated these lesions as mixed signal intensity areas with marked low signal intensity rim. There were multiple black dots in the bilateral frontal and temporal lobes, cerebellar hemispheres on T2-weighted images. Carotid and vertebral angiograms showed no abnormality. This is the first report of the cavernous malformation presenting as lateral medullary syndrome.

Cavernous Sinus↗

A selective reduction of excitatory amino acids in cerebrospinal fluid of patients with Alzheimer type dementia compared with vascular dementia of the Binswanger type.

We determined the concentrations of the putative transmitter amino acids in the cerebrospinal fluid of patients with Alzheimer type dementia (ATD) and vascular dementia of the Binswanger type (VDBT). In ATD, aspartate and glutamate concentrations were significantly and selectively reduced, while in VDBT, concentrations of aspartate, glutamate, gamma-aminobutyric acid (GABA) and many other amino acids were decreased non-selectively. In both ATD and VDBT, we found a tendency for all amino acids to increase with progression of the disease, and this reached statistical significance for some amino acids.

Aged↗