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Michiko Yamada,
Yasuyo Mimori,
Fumiyoshi Kasagi,
Takafumi Miyachi,
Tomohiko Ohshita,
Shinji Sudoh,
Junko Ikeda,
Kazuko Matsui, Shigenobu Nakamura,
Masayasu Matsumoto,
Saeko Fujiwara,
Hideo Sasaki
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ABSTRACT: To determine the age-, sex-, and subtype-specific incidence of dementia and to assess the effect of education level on the incidence in a Japanese population.
2,286 dementia-free subjects, aged > or =60 years, were followed for 5.9 years through biennial two-phase examinations.
206 cases of dementia were newly diagnosed based on DSM IV. The incidence per 1,000 person-years was 12.0 for men and 16.6 for women. Based on NINCDS-ADRDA criteria, 80 cases of probable Alzheimer disease (AD) and 50 cases of possible AD were diagnosed. Based on NINDS-AIREN criteria, 36 cases of probable vascular dementia (VaD) and 40 cases of possible VaD were diagnosed. Age and education showed the most statistically significant effects for all dementia. Probable AD showed the most remarkable increase with age and decreased with increasing education level (p = 0.001). Probable VaD showed significant effects of sex (p = 0.033) and sex-age interaction (p = 0.048), but not education (p = 0.26).
AD was the predominant type of dementia in this recent incidence study conducted in Japan, suggesting a reduction in VaD and an increase in AD. Age, sex, and education effects differed by dementia subtype.
Neuroepidemiology 02/2008; 30(3):152-60. · 2.31 Impact Factor
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ABSTRACT: We studied heterotopic abnormalities of the brain and spinal cord of a 48-year-old mentally retarded man with congenital myotonic dystrophy. He presented with a 2-year history of progressive muscular weakness in the trunk and he developed dyspnea on effort and orthopnea 4 months pro to death. He died of acute renal failure and severe congestive heart failure. The characteristic neuropathological finding was a number of heterotopic neurons in the cerebrum. In the molecular layer of the cerebral cortex, medium-sized neurons, pyramid-shaped large neurons, and horizontal cells were noted. Many neurons were also distributed sporadically in the subcortical white matter. In the spinal cord, abnormally situated neurons were present in be posterior and lateral columns near the gray matter. These findings suggest that intrauterine maturational disturbance occurs during the period of migration and organization of neurons in the brain and spinal cord.
Neuropathology 05/2007; 17(3):243 - 247. · 2.02 Impact Factor
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ABSTRACT: Progressive supranuclear palsy (PSP) is a progressive degenerative disorder characterized by neuronal loss, gliosis and abnormal fibril formation of abnormally phosphorylated tau protein in neurons and glia cells, but the cause is not clear at present. For the purpose of clarifying the pathological significance of accumulation of tau protein in neurons and oligodendroglia in PSP, we morphologically classified neurofibrillary tangles (NFT) and coiled bodies (CB) in oligodendroglia in three PSP brains into four stages, using double staining for immunohistochemistry with AT8 antibody and modified Gallyas-Braak (GB) staining. AT8-positive neurons without abnormal fibril structure with GB staining were classified as stage I, AT8-positive neurons containing a few fibril structures with GB staining were classified stage II, AT8-positive neurons containing mature fibril structures were classified as stage III, and AT8 negative neurons containing abnormal fibril structures stained only with GB staining were classified as stage IV (ghost tangles). These stages were also assessed for CB. Then we counted the number of cells of each stage in various brain regions to investigate the relationship of NFT and CB with neuronal loss and gliosis. The results showed that there were very few stage IV NFT and CB, which reflect cell death, but that stage III NFT and CB were abundant. Moreover, CB were abundant in regions with severe neuronal loss. These results suggest that appearance of CB is closely associated with degenerative regions.
Neuropathology 03/2006; 26(1):50-6. · 2.02 Impact Factor
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ABSTRACT: To quantitatively investigate simultaneous skin blood flow and sweating in a patient with complex regional pain syndrome.
Using one probe, skin blood flow and sweating were measured on the affected and non-affected sides at baseline and after activation of sympathetic system in a 47-year-old woman with complex regional pain syndrome type I of the left hand.
Basal sweating, sympathetic sweat response, basal blood flow, sympathetic flow response, and attrition rate of blood flow (the ratio of sympathetic flow response to basal blood flow) of the affected side were greater than those on the non-affected side.
Although sympathetic nerve activity (sympathetic flow response) appeared greater on one side, basal blood flow was also greater on that side. These contradictory results suggest that some factor or factors increase basal blood flow despite a higher sympathetic nerve tone on the side. Comparing basal blood flow on one side to the contralateral side does not necessarily indicate lesser vasoconstrictor nerve activity on that side. Advantages of simultaneous measurement of skin blood flow and sweating with a single probe may make the method useful.
Clinical Journal of Pain 02/2006; 22(1):109-10. · 2.81 Impact Factor
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ABSTRACT: To evaluate the electrophysiological findings of clinically unaffected cranial nerves (facial, accessory and hypoglossal nerves) in hereditary motor and sensory neuropathy (HMSN).
The conduction times of the facial, accessory, and hypoglossal nerves in 10 patients with HMSN type I (HMSN I), 2 patients with HMSN Type II (HMSN II), and 20 normal controls were determined. The extra- and intracranial segments of the cranial nerves were stimulated electrically and magnetically, respectively. The relationships between the conduction parameters of the cranial nerves and limb nerves were analyzed.
In patients with HMSN I, the conduction times of the distal and proximal segments were significantly prolonged in all 3 cranial nerves. A positive correlation was found between the conduction parameters of the cranial nerves and the limb nerves.
Electrophysiological involvement of the whole segment of the facial, accessory and hypoglossal nerves is common in patients with HMSN I without clinical signs of alterations. The degree of conduction slowing of the facial, accessory, and hypoglossal nerves paralleled that of limb nerves.
Clinical Neurophysiology 08/2004; 115(7):1689-96. · 3.41 Impact Factor
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ABSTRACT: Vasomotor function has often been evaluated with basal blood flow. Simultaneous measurement of skin blood flow and sweating using a single probe were provided as lesser basal blood flow on one side compared to the contralateral side in complex regional pain syndrome does not always indicate greater vasoconstrictor nerve activity.
The Pain Clinic 06/2004; 16(2):213-216.
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ABSTRACT: Asymptomatic microbleeds shown by T2*-weighted MR imaging are associated with small-artery diseases, especially with intracerebral hemorrhage. Few studies have focused on the prevalence of microbleeds in patients with recurrent stroke. We investigated frequency of microbleeds in patients with recurrent stroke and association of presence of microbleeds with a combination of stroke subtypes and severity of leukoaraiosis.
The study population consisted of 102 patients with primary stroke and 54 patients with recurrent stroke. Microbleeds were counted and classified by using T2*-weighted MR imaging with a 1.0-T system.
Patients with recurrent stroke showed a significantly higher prevalence of microbleeds (68.5%) than did patients with primary stroke (28.4%) (P <.0001). Among patients with recurrent stroke, the highest frequency of microbleeds occurred in those with intracerebral hemorrhage alone (92.3%), with the next highest frequency occurring in those with a combination of intracerebral hemorrhage and ischemic stroke (76.5%) and then those with ischemic stroke alone (50.0%) (P <.05). Leukoaraiosis was more severe in patients with recurrent stroke than in patients with primary stroke, and correlations between grade of microbleeds and severity of leukoaraiosis were found in patients with primary stroke (r = 0.367, P <.001) and in patients with recurrent stroke (r = 0.553, P <.0001). Logistic regression analysis identified recurrent stroke (odds ratio, 4.487; 95% confidence interval, 1.989-10.120) and leukoaraiosis (odds ratio, 5.079; 95% confidence interval, 2.125-12.143) as being significantly and independently associated with microbleeds.
Asymptomatic microbleeds are observed to occur frequently in patients with recurrent stroke, either hemorrhagic or ischemic stroke, and are closely associated with the severity of leukoaraiosis.
American Journal of Neuroradiology 06/2004; 25(5):714-9. · 2.93 Impact Factor
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ABSTRACT: Several studies have demonstrated abnormal MRI findings in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), especially hypertrophy and abnormal enhancement of spinal nerve roots, but there have been few reports on ultrasonographic findings of spinal nerve roots in CIDP. To determine whether ultrasonography (US) enables detection of hypertrophy of the cervical nerve roots, how frequently hypertrophy occurs in CIDP, and whether US findings correlate with any clinical and laboratory features, US of cervical nerve roots was performed using a 7.5-MHz linear-array transducer in 13 CIDP patients and 35 control subjects. A coronal oblique plane with a transducer placed on the lateral side of the neck was used to visualize the cervical nerve roots just after their point of exit from the cervical foramina, and their diameters were measured. US demonstrated hypertrophy of the cervical nerve roots in 9 (69%) of the 13 CIDP patients as compared with findings in control subjects. The degree of hypertrophy was significantly associated with the level of CSF protein (chi2=5.8, p<0.05, logistic simple regression analysis) but not with other clinical features. US is considered to be a useful method for evaluating cervical nerve root hypertrophy, which is frequently seen in patients with CIDP, particularly in patients with elevated level of CSF protein.
Journal of the Neurological Sciences 04/2004; 219(1-2):15-21. · 2.35 Impact Factor
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ABSTRACT: Various factors have been suggested to participate in Alzheimer's disease (AD) pathology, and some inflammatory cytokines may play an important role in the development of AD. Interferon-gamma (IFNG), an important pro-inflammatory cytokine, is encoded by a single gene mapped to chromosome 12, one of the candidate locus of AD. The first intron in the IFNG gene represents a CA repeat polymorphism that is possible to affect the IFNG secretion dose. We speculate that the polymorphism may have some roles on the inflammatory process and the pathologic change in AD, so we analyzed the IFNG gene polymorphism in 199 Japanese AD patients and 225 Japanese controls. There were no significant differences in allele frequency between the AD and control groups. We conclude that IFNG gene polymorphism is not associated with development of AD.
American Journal of Medical Genetics Part B Neuropsychiatric Genetics 02/2004; 124B(1):48-9. · 3.70 Impact Factor
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ABSTRACT: We elucidated the cause and clinical characteristics of malignant syndrome (MS) in patients with Parkinson's disease (PD), early-onset parkinsonism (EOP), and other neurological disorders.
Subjects were 260 patients with PD or EOP, and three patients with other neurological disorders associated with MS. We studied clinical symptoms before and after the onset of MS, and evaluated autonomic function particularly before the onset of MS.
The overall incidence of MS accompanying PD and EOP in our department was eight of 260 patients (3.1%). The incidence of MS in EOP was significantly higher than that in PD. All patients with MS showed comparatively longer duration of illness and advanced stage of PD and EOP. Factors triggering MS included reduced dosage or discontinuation of anti-parkinsonian drugs, reduction of oral intake, dehydration, infectious disease, postoperative state, and treatment with major tranquilizers. Although patients demonstrated marked autonomic symptoms at the onset of MS, in many cases autonomic dysfunction developed before the onset of MS. Even EOP patients, who usually demonstrated milder autonomic dysfunction, showed abnormalities in the correlation between circadian rhythm of blood pressure and pulse rate, and/or abnormal gastric emptying test, suggesting that autonomic dysfunction plays an important role in the cause of MS. Cooling the body, fluid replacement, resumption or increasing the dosage of anti-parkinsonian drugs and administration of dantrolene sodium overcame MS in all cases.
Autonomic dysfunction is related to the cause and clinical features of MS in PD, EOP and some other neurological disorders.
Parkinsonism & Related Disorders 05/2003; 9 Suppl 1:S15-23. · 3.80 Impact Factor
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ABSTRACT: Chronic inflammatory demyelinating polyneuropathy (CIDP) is a slowly progressive or recurrent neuropathy accompanied by infiltration of macrophages in the peripheral nerves. Macrophage colony-stimulating factor (M-CSF) and monocyte chemoattractant protein-1 (MCP-1) are a macrophage-related cytokine and chemokine, respectively. Although, intravenous immunoglobulin (IVIg) infusion therapy has been used for treating CIDP patients, not all CIDP patients have responded to IVIg infusion therapy. To determine the mechanisms of the action of IVIg, we examined serum M-CSF and MCP-1 levels during and after IVIg infusion therapy in 19 CIDP patients treated with IVIg (0.4 g/kg/day for 5 days). Ten of the 19 patients (52.6%) responded to IVIg therapy. Both M-CSF and MCP-1 concentrations in IVIg responders were significantly higher on day 1 postinfusion than those in nonresponders, but decreased to their pretreatment values on day 5 postinfusion. The results suggest that immunomodulation through M-CSF and MCP-1 is involved in the mechanisms underlying the effect of IVIg infusion therapy in CIDP patients.
Journal of the Neurological Sciences 05/2003; 208(1-2):43-50. · 2.35 Impact Factor
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Hideki Takubo,
Toshihide Harada,
Takao Hashimoto,
Yutaka Inaba,
Ichiro Kanazawa,
Sadako Kuno,
Yoshikuni Mizuno,
Eiji Mizuta,
Miho Murata,
Toshiharu Nagatsu, Shigenobu Nakamura,
Nobuo Yanagisawa,
Hirotaro Narabayashi
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ABSTRACT: We report the results of a collaborative study on malignant syndrome (MS) that developed in patients being treated with levodopa and other anti-parkinsonian drugs. We analyzed clinical features, laboratory findings, precipitating events, and risk factors for poor outcome. The study was conducted in five centers in Japan. Patients who developed MS between January 1991 and December 1997 were included. The enrollment criteria used were the same as those for neuroleptic MS proposed by Levenson et al. (1985).A total of 99 episodes were encountered in 93 patients (72 with Parkinson's disease and 21 with secondary parkinsonism); one patient had four recurrences of MS and three patients had two recurrences. High fever was the most frequent clinical manifestation of MS followed by worsening of parkinsonism, and then altered levels of consciousness. Serum creatine kinase was abnormally elevated in all the patients studied. Life-threatening complications were rhabdomyolysis, disseminated intravascular coagulation, and acute renal failure. The most frequent precipitating event was discontinuation or dose reduction of anti-parkinsonian drugs, particularly levodopa. No drug was the exception in the precipitation of MS. Intercurrent infection was the next most common precipitating event. MS developed without drug withdrawal or infection in some patients. In five patients, severe "wearing off" phenomenon was the only event preceding the onset of MS. Hot weather and dehydration appeared to be the cause in three patients. Among the total of 99 episodes, patients recovered to the pre-MS state following 68 episodes (68.7%); in the remaining 31.3%, patients failed to recover to their previous state. Older age, higher Hoehn and Yahr stage during the symptomatic phase of MS, higher akinesia score, and the absence of wearing off phenomenon prior to developing MS were associated with poor outcome. The most frequently used treatments of MS were intravenous fluid, levodopa, dantrolene sodium, and intragastric bromocriptine. Early introduction of treatment is important. Any elevation of body temperature during the course of anti-parkinsonian drug treatment should be considered as MS until proved otherwise.
Parkinsonism & Related Disorders 05/2003; 9 Suppl 1:S31-41. · 3.80 Impact Factor
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Shin-ichiro Ikebe,
Toshiaki Harada,
Takao Hashimoto,
Ichiro Kanazawa,
Sadako Kuno,
Yoshikuni Mizuno,
Eiji Mizuta,
Miho Murata,
Toshiharu Nagatsu, Shigenobu Nakamura,
Hideki Takubo,
Nobuo Yanagisawa,
Hirotaro Narabayashi
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ABSTRACT: We report a consensus statement of the collaborative research group on the prevention and treatment of malignant syndrome (MS) in Parkinson's disease. The syndrome is quite similar to neuroleptic MS. Although sudden withdrawal of levodopa was the most frequent cause, many other precipitating events were found such as intercurrent infections, dehydration, hot weather, discontinuation of other anti-parkinsonian drugs, and "wearing off" phenomenon. Awareness of this syndrome is most important for its early detection and the prompt commencement of treatment. MS should be suspected whenever the body temperature rises above 38 degrees C without an apparent cause. Treatment consists of ample intravenous fluid, cooling the body, anti-parkinsonian drugs (particularly levodopa and bromocriptine), dantrolene sodium, and antibiotics if infection is present. Rhabdomyolysis, disseminated intravascular coagulation, and acute renal failure constitute serious complications.
Parkinsonism & Related Disorders 05/2003; 9 Suppl 1:S47-9. · 3.80 Impact Factor
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Yuishin Izumi,
Hirofumi Maruyama,
Masaya Oda,
Hiroyuki Morino,
Takayuki Okada,
Hidefumi Ito,
Iwao Sasaki,
Hiroyasu Tanaka,
Osamu Komure,
Fukashi Udaka, Shigenobu Nakamura,
Hideshi Kawakami
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ABSTRACT: We analyzed the SCA8 CTA/CTG repeat in a large group of Japanese subjects. The frequency of large alleles (85-399 CTA/CTG repeats) was 1.9% in spinocerebellar ataxia (SCA), 0.4% in Parkinson disease, 0.3% in Alzheimer disease, and 0% in a healthy control group; the frequency was significantly higher in the group with SCA than in the control group. Homozygotes for large alleles were observed only in the group with SCA. In five patients with SCA from two families, a large SCA8 CTA/CTG repeat and a large SCA6 CAG repeat coexisted. Age at onset was correlated with SCA8 repeats rather than SCA6 repeats in these five patients. In one of these families, at least one patient showed only a large SCA8 CTA/CTG repeat allele, with no large SCA6 CAG repeat allele. We speculate that the presence of a large SCA8 CTA/CTG repeat allele influences the function of channels such as alpha(1A)-voltage-dependent calcium channel through changing or aberrant splicing, resulting in the development of cerebellar ataxia, especially in homozygous patients.
The American Journal of Human Genetics 04/2003; 72(3):704-9. · 10.60 Impact Factor
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ABSTRACT: To investigate a possible deficit of the voluntary movement mechanism within the central nervous system (CNS) in patients with myotonic dystrophy (MyD).
Movement-related cortical potentials preceding voluntary extension of the right middle and index fingers were studied in 9 patients with MyD and compared with those in 11 age-matched healthy subjects and 9 age-matched patients with other neuromuscular disorders (NMDs).
The amplitudes of Bereitschaftspotential was smaller in MyD patients than in age-matched controls and age-matched patients with other NMDs although there was no statistically significant difference. The amplitude of negative slope was significantly smaller in MyD patients than in age-matched controls and age-matched patients with other NMDs. Clinical findings such as age, disease duration, degree of motor impairment and cognitive function had no effect on the individual electrophysiological parameters.
The present results suggest that subclinical abnormalities exist in CNS function associated with motor preparation and execution, which is independent of muscle weakness.
Clinical Neurophysiology 02/2003; 114(1):99-106. · 3.41 Impact Factor
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ABSTRACT: To determine whether magnetization transfer imaging (MTI) demonstrates abnormalities in the brain structures of patients with multiple system atrophy (MSA), we examined 12 patients with clinically probable MSA and 11 control subjects. We calculated magnetization transfer ratios (MTRs) using region of interest analysis from MTI and assessed abnormal signal changes on T2-weighted images. MTRs of the base of the pons, middle cerebellar peduncle, putamen, and white matter of the precentral gyrus were significantly lower in the MSA patients than in the controls. Abnormal signal changes on T2-weighted images were observed in the base of the pons (n = 6), middle cerebellar peduncle (n = 7), and putamen (n = 7). MTRs of regions with abnormal signals were significantly lower than those of regions without abnormal signals and those in the controls. Even the MTRs of the regions without abnormal signals were lower than those in the controls. MTRs of the pyramidal tract, including white matter of the precentral gyrus, posterior limb of the internal capsule, cerebral peduncle, and base of the pons, were significantly lower in patients with pyramidal tract sign (n = 7) than in the controls. Patients with asymmetrical parkinsonism (n = 5) showed significantly lower MTRs in the putamen contralateral to the predominant side of parkinsonian symptoms than the ipsilateral side, although asymmetry of abnormal signal changes on T2-weighted images was not evident in more than half of those patients. This study showed that MTI demonstrates abnormalities in the brains of patients with MSA that seem to reflect underlying pathological changes and that the pathological changes detected by MTI seem to give rise to clinical symptoms. This study also showed that the abnormalities are detected more sensitively and over a larger area by MTI than by conventional magnetic resonance imaging.
NeuroImage 12/2002; 17(3):1572-8. · 5.89 Impact Factor
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ABSTRACT: To elucidate the functional properties of neurons in the human primary (SI) and ipsilateral and contralateral secondary (iSII or cSII) cortices in response to stimuli during finger movement.
We measured somatosensory evoked fields (SEFs) produced by electric stimuli delivered to the median nerve at 0.2 Hz in 6 healthy subjects.
The amplitudes of evoked fields from both iSII and cSII were gradually attenuated with time. Consecutive blocks of trials were obtained to assess the habituation of each evoked field. Complex finger movements with attention (gating session) increased the amplitude of evoked fields from the iSII cortices but reduced the amplitudes of evoked fields from the cSII cortices (P<0.01). In contrast, the amplitude of P30 m from the SI did not show habituation effects but decreased significantly in the gating session (P<0.01).
The enhanced iSII as well as suppressed cSII cortices during complex finger movements with attention are not only considered to be result of gating effect but also attention.
Clinical Neurophysiology 11/2002; 113(10):1573-8. · 3.41 Impact Factor
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Nihon Naika Gakkai Zasshi 10/2002; 91(9):2759-61.
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ABSTRACT: The authors describe a 16-year-old boy with severe muscular atrophy and signs of peripheral neuropathy compatible with Charcot-Marie-Tooth disease. Abnormalities in the cerebellum and central somatosensory pathway were also noted. Gene analysis revealed a novel gross insertion mutation in exon 2 of the connexin32 gene along with a 21-base pair duplication resulting in a seven-amino acid insertion in the first extracellular loop of the protein.
Neurology 10/2002; 59(6):923-6. · 8.31 Impact Factor
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ABSTRACT: We reported a 62-year-old man of late-onset familial amyloid polyneuropathy type I(transthyretin Met 30-associated familial amyloid polyneuropathy) from Ehime Prefecture. There was no family history related to endemic Japanese foci (Nagano and Kumamoto foci). He demonstrated paraesthesia in the legs and mild autonomic symptoms at the age of 52. These symptoms gradually developed. Analysis of the transthyretin gene from his leucocytes demonstrated he had Met 30 transthyretin mutation. Therefore, he was diagnosed with late-onset familial amyloid polyneuropathy type I(FAP 1). In some families, asymptomatic carriers with the mutant transthyretin gene were diagnosed. In early stage, this patient's polyneuropathy and autonomic nervous system dysfunction were less serious than those of FAP 1 patients from endemic Japanese foci. These symptoms of this patient was slowly progressive. He hoped liver transplantation (brain death or living-related) treatment if possible. Now he became 68-year-old and bed-ridden.
Nō to shinkei = Brain and nerve 08/2002; 54(7):615-9.
