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Biomedical subjects

K Mokuno

Publications and source records attributed to K Mokuno.

At least 19 recordsLinked to original sources
PubMed2000

Mutations in the peripheral myelin protein zero and connexin32 genes detected by non-isotopic RNase cleavage assay and their phenotypes in Japanese patients with Charcot-Marie-Tooth disease.

Mutations of myelin protein zero (MPZ) and connexin32 (Cx32) genes were examined in 70 unrelated Japanese patients with Charcot-Marie-Tooth disease (CMT) without PMP22 gene duplication. A new method, which could detect base pair mismatches with Rnase cleavage on agarose gel electrophoresis, identified 5 and 4 mutations of the MPZ and Cx32 genes, respectively, including one novel mutation (Ser128Ter) of Cx32. This non-isotopic RNase cleavage assay (NIRCA) employed in the present study is very suitable for exploring mutations of MPZ and Cx32 genes in a large number of CMT patients, as the phenotype of patients with CMT is greatly divergent from demyelinating to axonal pathology.

Adolescent↗
PubMed1999

[The clinical usefulness of MRI diffusion weighted images in herpes simplex encephalitis-like cases].

We examined the serial MRI diffusion weighted images (DWIs) in two patients with acute viral encephalitis similar to herpes simplex encephalitis (HSE). Patient 1. A 27-year-old woman was admitted to the psychiatry ward for her confusional state and convulsions. Because of abnormal CSF findings she was transferred to the neurology ward, and the infusion of acyclovir was started. On disease day 5. MRI demonstrated high signal intensity in the left lateral lobe both on T2 weighted images (T2WIs) and DWIs. On day 18, MRI showed progression of the lesions, so acyclovir was changed to ara-A. On day 26, no improvement was seen clinically or radiologically. Then, combination therapy with acyclovir, ara-A and gamma-globulin was began. On day 36, she recovered completely, and abnormal intensity in MRI disappeared both on T2WIs and DWIs. Therefore, antiviral agent therapy was discontinued. Patient 2. A 31-year-old man was admitted for headache, fever and aphasia. On the next day, acyclovir was started and both DWIs and T2WIs of MRI demonstrated a high signal intensity in the left temporal lobe. Ten days later, he became perfectly well, and the increased signal intensity disappeared on DWIs, but not on T2WIs. Treatment was therefore discontinued. No relapse was in either patient. We concluded that serial MRI DWIs may be useful to determine when to discontinue the treatment in encephalitis.

Acyclovir↗
PubMed1998

[Features of MRI diffusion weighted image in early stage of lateral medullary infarction presenting Wallenberg syndrome].

We described early features of MRI diffusion weighted images (DWIs) in three patients with lateral medullary infarction. Patient 1. A 65-year-old woman who complained of vertigo was admitted. Then, DWIs showed a high signal intensity in the right lateral medulla, though T2 weighted images (T2WIs) did not show any abnormalities. On the next day, when the typical symptoms and signs of lateral medullary infarction appeared, the lesion was recognized on both DWIs and T2WIs. Patient 2. A 48-year-old man was admitted 9 hours after he had dizziness, nausea, and repeated vomitings. When a diagnosis of Wallenberg syndrome was made on the neurological examination, MRI DWIs demonstrated a high signal intensity in the right lateral medulla. The lesion became apparent on T2WIs 82 hours after the onset. Patient 3. A 71-year-old man was admitted for nausea, dizziness, and repeated vomitings. A diagnosis of Wallenberg syndrome was made fifty eight hours after the onset, a lesion with high signal intensity in the right lateral medulla was evident on DWIs, but it was faint on T2WIs. We concluded that DWIs is useful in early diagnosis of lateral medullary infarction presenting Wallenberg syndrome.

Aged↗
PubMed1998

[A case of clinically diagnosed Creutzfeldt-Jakob disease with serial MRI diffusion weighted images].

We reported MRI findings in a 49-year-old woman with clinically diagnosed Creutzfeldt-Jakob disease (CJD). She was admitted to our hospital because of confusion, which initially appeared 5 weeks prior and was rapidly worsened. Two weeks later, she developed myoclonic jerks in her extremities with periodic synchronous discharges on EEG. Six months later, she became mutic. Brain MRI at 3 weeks after the admission demonstrated high signal intensities in the bilateral caudate nuclei and putamina on T2 weighted images (T2WIs). Diffusion weighted images (DWIs) showed hyperintensities in the basal ganglia and in the parietal and occipital cortices. Five weeks later, the abnormal intensities in the basal ganglia were still observed on T2WIs but decreased on DWIs. Five months later, the increased signal intensities in the basal ganglia had disappeared both on T2WIs and DWIs, but new hyperintensities appeared in the insula and the temporal area on DWIs. We concluded that DWIs may be useful for the demonstrations of a lesion in the cerebral cortex and the spread of lesions.

Atrophy↗
PubMed1996

Elevated serum levels of manganese superoxide dismutase in polymyositis and dermatomyositis.

We studied serum concentrations of manganese superoxide dismutase (Mn SOD) and copper-zinc superoxide dismutase (Cu/Zn SOD) in 22 patients with polymyositis and dermatomyositis (PM/DM), 87 patients with four types of muscular dystrophy, 20 patients with amyotrophic lateral sclerosis, and 15 patients with collagen vascular diseases (CVD). Serum levels of Mn SOD were increased only in the patients with PM/DM and CVD, and the elevation was more prominent in those with PM/DM. Levels of Cu/Zn SOD were slightly elevated in some patients with PM/DM and Duchenne muscular dystrophy. In patients with PM/DM, the change in Mn SOD levels corresponded to disease activity as closely as or more closely than those of creatine kinase. The results indicate that serum Mn SOD may be a useful clinical marker for PM/DM.

Adolescent↗
PubMed1995

Clinico-pathophysiological features of acute autonomic and sensory neuropathy: a long-term follow-up study.

We evaluated the clinico-pathophysiological features of three patients with acute autonomic and sensory neuropathy (AASN) who were followed for over 3 years. Signs of an autonomic disturbance including vomiting, anhidrosis, urinary disturbances, orthostatic hypotension and reduced coefficient of variation of the R-R interval on electrocardiography gradually improved about 1 year after onset. However, all three exhibited severe generalized sensory impairment for all modalities with the development of persistent sensory ataxia. No sensory nerve action potentials could be elicited and no somatosensory evoked potentials could be obtained. Sural nerve biopsy revealed severe axonopathy. In two patients, a high-intensity area was observed in the posterior column of the spinal cord on T2*-weighted axial magnetic resonance images. The level of neuron-specific enolase in cerebrospinal fluid was markedly elevated in two patients, indicating spinal nerve root or sensory neuron damage. Motor nerve function was well preserved in all patients. Our findings suggests that the major lesion in patients with AASN, particularly those with a sensory deficit, is present in the dorsal root ganglion neurons, that is there is a ganglioneuronopathy.

Action Potentials↗
PubMed1995

Elevated cerebrospinal fluid levels of manganese superoxide dismutase in bacterial meningitis.

We examined the mechanism of increase of manganese superoxide dismutase (Mn SOD) in the cerebrospinal fluid (CSF) in bacterial meningitis (BM). The elevated levels of Mn SOD in the CSF in BM, measured with an enzyme immunoassay method, were more prominent than those in aseptic meningitis (AM) and encephalitis (EN). In AM and EN Mn SOD levels well correlated with levels of neuron-specific enolase and S-100b protein, which are markers of damages to nervous tissues, but did not with any of them in BM. CSF concentrations of tumor necrosis factor-alpha (TNF-alpha) and interleukin-1 alpha (IL-1 alpha) were higher in BM than in AM and EN. From the serial measurements in BM, the peak values of these cytokines chronologically preceded or corresponded to those of Mn SOD. Immunohistochemically, a large number of the glial cells were stained for Mn SOD in the cerebral cortex from a patient with BM. By contrast, in the normal cerebral cortex, the glial cells were negative for Mn SOD staining. These results suggest that the marked increase of Mn SOD in the CSF in BM may be related to the increase of such cytokines as TNF-alpha and IL-1 alpha and that these cytokines may play a role in the induction of Mn SOD in nervous tissues.

Adolescent↗
PubMed1995

Cerebrospinal fluid levels of S-100b protein and neuron-specific enolase in chronic inflammatory demyelinating polyneuropathy.

We measured the cerebrospinal fluid (CSF) concentrations of S-100b protein (S-100b) and neuron-specific enolase (NSE) using enzyme immunoassay methods in 15 patients with chronic inflammatory demyelinating polyneuropathy (CIDP), other three patients with chronic neuropathy with demyelination, eight patients with various axonal neuropathies (AN), and 46 controls, to investigate the clinical usefulness of the determination of these two specific proteins in these neuropathies. S-100b levels were elevated (> the mean +/- 2 SD levels of controls) in the majority of patients with clinically progressing CIDP (9/11), but not in the patients with AN (0/8). In parallel with the clinical improvement, S-100b levels were normalized in patients with CIDP (10/10), though total protein levels in the CSF still remained high in some of these patients (5/10). Elevation of NSE levels were seldom seen in clinically worsening patients with CIDP (1/11) or AN (1/8). Thus our results indicated that the level of S-100b in the CSF may be useful to assess the activity of actual disease process in CIDP.

Adolescent↗
PubMed1994

An immunohistochemical study of the neuronal expression of manganese superoxide dismutase in sporadic amyotrophic lateral sclerosis.

Neuronal expression of manganese superoxide dismutase (MnSOD) in sporadic amyotrophic lateral sclerosis (sALS) was investigated by an immunohistochemical method. The brains and spinal cords from 11 patients with sALS and 20 normal controls (NCs) were used, and the following four nuclei (three motor nuclei and one autonomic nucleus) were examined: the oculomotor nucleus; the hypoglossal nucleus; the cervical motor nucleus; and Onuf's nucleus. Serial sections were stained by the Klüver-Barrera (KB) method and with human-MnSOD-specific antibodies. We counted the total number of neurons visible after KB staining and the total number of positive neurons after immunostaining. The average total number of neurons after KB staining was similar in sALS patients and NCs in both the oculomotor nucleus and Onuf's nucleus, but the number in the hypoglossal and cervical motor nuclei was significantly lower in sALS. The ratio of MnSOD-positive neurons to total neurons visible after KB staining, calculated as an index of the expression of MnSOD, was significantly higher in the oculomotor nucleus and Onuf's nucleus, and lower in the hypoglossal nucleus in sALS patients than in NCs. In the cervical motor nucleus, the ratio in sALS patients did not differ from that in NCs. These results suggest that production of toxic superoxide radicals might be increased in sALS, and that neurons that successfully induce the expression of sufficient MnSOD can survive the disease process, while those failing to activate adequate expression of the enzyme succumb to the toxic effects of the radicals and die.

Adult↗
PubMed1994

Cerebrospinal fluid levels of superoxide dismutases in neurological diseases detected by sensitive enzyme immunoassays.

We measured cerebrospinal fluid (CSF) levels of Cu/Zn superoxide dismutase (Cu/Zn SOD) and Mn superoxide dismutase (Mn SOD) using enzyme immunoassays in 196 neurological patients and 44 controls. The mean Cu/Zn SOD level was 55.8 +/- 27.6 (SD) ng/ml and the Mn SOD, 8.0 +/- 2.5 ng/ml in the controls. Cu/Zn SOD or Mn SOD levels showed neither age-nor sex-related differences in the controls. Both SODs were markedly elevated in cerebrovascular diseases, bacterial meningitis and encephalitis. Mn SOD alone was significantly elevated in neurodegenerative diseases. We compared SODs with CSF levels of neuron-specific enolase (NSE) and S-100b protein (S-100b) in cerebral infarction and bacterial meningitis. Both SODs were correlated with NSE and S-100b in patients with cerebral infarction, but not in those with bacterial meningitis. This means that elevations of SODs in CSF may not only be due to leakage from damaged nervous tissues, but also to the induction of SOD in lesions. We conclude that the mean SOD levels were elevated in various neurological diseases, and their varied magnitudes may be associated with the underlying diseases.

Adolescent↗
PubMed1994

Induction of manganese superoxide dismutase by cytokines and lipopolysaccharide in cultured mouse astrocytes.

To determine whether cytokines or lipopolysaccharide (LPS) are involved in the induction of superoxide dismutase (SOD) in the nervous system, we examined the effects of these substances on the levels of SOD in cultured mouse astrocytes. Treatment of astrocytes with 10(2) to 10(4) U/ml tumor necrosis factor-alpha for 3 days increased the levels of Mn SOD in a dose- and time-dependent manner to as much as six times the level under nontreated conditions. Treatment with 1.0 microgram/ml LPS for 3 days elicited a fourfold increase in levels of Mn SOD, and the effect of LPS was also dose dependent. Furthermore, Mn SOD in astrocytes was induced by a 3-day exposure to interleukin-1 alpha at concentrations of 10(2) or 10(3) U/ml. However, these stimuli had no effect on levels of copper-zinc SOD (Cu/Zn SOD) in astrocytes. By contrast, interferon-gamma did not change the levels of either Mn or Cu/Zn SOD in the cells. The results indicate that the selective induction of Mn SOD by cytokines and LPS, which has been observed in nonnervous tissues, may also occur in nervous tissues. The induction of Mn SOD may represent a mechanism for protection of cells from oxidative stress.

Animals↗
PubMed1994

Tenascin in cerebrospinal fluid is a useful biomarker for the diagnosis of brain tumour.

Tenascin, an extracellular matrix glycoprotein, has been reported to be expressed predominantly on glioma tissue in the CNS, both in a cell associated and an excreted form. Recently, a highly sensitive sandwich type enzyme immunoassay for quantitative determination of tenascin was developed. In the present study, the amount of tenascin in CSF was measured. An increase of tenascin in CSF (> 100 ng/ml) was found in patients with an astrocytic tumour. The concentration was significantly higher (> 300 ng/ml) in high grade astrocytoma (anaplastic astrocytoma and glioblastoma) and a further increase (> 1000 ng/ml) was found in cases of CSF dissemination of high grade astrocytoma. On the other hand, tenascin concentrations were less than 100 ng/ml in non-astrocytic tumours and non-neoplastic neurological diseases, except meningeal dissemination of tumour cells, meningeal stimulation by infection, and subarachnoid haemorrhage. In cases of treated astrocytomas in remission, tenascin was negligible (< 100 ng/ml) in the CSF. The measurement of tenascin in CSF is useful for differential diagnosis of brain tumours and monitoring of astrocytic tumours.

Biomarkers, Tumor↗
PubMed1994

[The significance of elevated Mn SOD level in cerebrospinal fluid of patients with bacterial meningitis--its relation to cytokine].

We measured cerebrospinal fluid (CSF) levels of manganese superoxide dismutase (Mn SOD) using an enzyme immunoassay method in 19 patients with bacterial meningitis (BM), 33 with aseptic meningitis (AM) and 13 with encephalitis (EN), and examined the significance of their elevations, especially in BM. 1) In BM, the Mn SOD levels were obviously high, ranging from 10.4 to 1179.2 ng/ml. The mean level of Mn SOD was 234.6 +/- 306.7 (SD) ng/ml and 18 patients showed abnormal levels of Mn SOD (more than 13.1 ng/ml). On the other hand, in the remaining 2 diseases, the elevation of SOD levels was not remarkable: the mean levels of Mn SOD in AM and EN were 20.6 +/- 11.6 ng/ml and 41.9 +/- 23.6 ng/ml, respectively. 2) In AM and EN, Mn SOD levels well correlated with NSE or S-100b levels which are the markers of nervous tissue damages. But there was no correlation between the Mn SOD levels in BM and these markers. 3) In BM, there was a positive relationship between Mn SOD and total protein levels, but the disease days showing peak levels were different between them. In addition, Mn SOD levels showed no correlation with cell counts in CSF. 4) In BM, CSF levels of TNF-alpha and IL-1 alpha were remarkably high, whereas in AM and EN, the increases of these cytokines were not marked. And these cytokines in BM showed the peak values in the disease day before or when Mn SOD reached the peak levels.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1994

Prognostic value of cerebrospinal fluid neuron-specific enolase and S-100b protein in Guillain-Barré syndrome.

We measured the cerebrospinal fluid (CSF) concentrations of neuron-specific enolase (NSE) and S-100b protein (S-100b) using enzyme immunoassay methods, in 24 patients with Guillain-Barré syndrome and 46 controls, and examined their prognostic values. Sixteen of 24 patients showed elevated levels (> the mean + 2SD levels of controls) of NSE, S-100b, or both, and in those with higher NSE or S-100b levels there was a rather longer duration of disease, whereas 8 patients with the normal levels showed an early recovery. Further, NSE or S-100b levels were significantly correlated with months to recovery. Thus, NSE and S-100b in CSF may be useful markers for predicting the outcome of Guillain-Barré syndrome.

Adolescent↗
PubMed1993

Cerebrospinal fluid 28-kDa calbindin-D as a possible marker for Purkinje cell damage.

To examine the clinical value of 28-kDa calbindin-D (CaBP) in cerebrospinal fluid (CSF) as a marker for the damage to Purkinje cells, we measured CSF CaBP levels using an enzyme immunoassay method in 107 patients with cerebellar and other neurological diseases, and 26 controls. The mean CaBP level was markedly elevated in patients with cerebellar diseases, and the elevation of CaBP level was more frequent in the diseases involving Purkinje cells, such as multiple system atrophy (MSA) and subacute cerebellar degeneration in association with lung cancer. Further, in MSA patients, the CaBP levels decreased with duration of illness. The mean levels of CaBP were also elevated in some of the other diseases. We conclude that the elevations of CaBP levels are not specific for cerebellar diseases, but CSF CaBP may be a useful marker for examining the Purkinje cell involvement in cerebellar diseases.

Adolescent↗
PubMed1993

[Superoxide dismutase in cerebrospinal fluid in patients with neurological diseases].

To evaluate significance of superoxide dismutase in neurological diseases, we measured cerebrospinal fluid (CSF) levels of copper-zinc superoxide dismutase (Cu/Zn SOD) and manganese superoxide dismutase (Mn SOD) using enzyme immunoassay methods in 181 neurological patients and 43 controls. The mean level of Cu/Zn SOD in CSF of controls was 54.4 +/- 28.7 ng/ml, and that of Mn SOD 8.1 +/- 2.5 ng/ml, although other methods have reported that Mn SOD is undetectable in CSF. Cu/Zn SOD or Mn SOD showed no statistical difference in age or sex of the controls. The elevation of both SOD levels was marked in acute diseases such as cerebrovascular diseases (CVD), bacterial meningitis and encephalitis, but mild in aseptic meningitis. The elevation of Cu/Zn SOD level was more prominent than that of Mn SOD in CVD, whereas vice versa in bacterial meningitis and encephalitis. In neurodegenerative diseases and cervical spondylosis, only Mn SOD level was significantly elevated. To examine the source of CSF SOD, we compared it with CSF levels of neuron-specific enolase (NSE) and S-100b protein (S-100b) in cerebral infarction and bacterial meningitis. Both SOD levels were correlated with NSE and S-100b levels in patients with cerebral infarction, but in bacterial meningitis no significant relationship was found among SOD levels, NSE and S-100b levels. This means that elevations of SODs in CSF may be due to not only damage of the nervous tissues but also the other mechanisms, as induction of SOD in the lesions.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗