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Biomedical subjects

I Akiguchi

Publications and source records attributed to I Akiguchi.

At least 109 records · Page 6Linked to original sources
PubMed1997

[Relapsing polychondritis with mental disorders: a case report].

A 60-year-old man developed disorientation, euphoria, hyperactive behavior, mental confusion, and a moderate decrease of cognition during the course of relapsing polychondritis with bilateral auricular chondritis, sensorineural hearing loss, episcleritis and nasal chondritis. An electroencephalogram showed diffuse slow wave abnormality. The CSF analysis revealed pleocytosis and increased protein. MRI with contrast showed enhancement and edema in bilateral medial temporal regions. These abnormal findings improved markedly with the treatment of corticosteroids. This is the first Japanese case report of relapsing polychondritis presenting as mental disorders.

Anti-Inflammatory Agents↗
PubMed1997

[White matter lesions after occlusion of the bilateral carotid arteries in the rat--temporal profile of cerebral blood flow (CBF), oligodendroglia and myelin].

In the present investigation, we examined cerebral blood flow (CBF), numerical density of oligodendroglia and extent of white matter lesions after bilateral ligation of common carotid arteries in Wistar rats. Doppler flow-meter revealed a reduction of CBF to 30-40% of that before operation after 1 and 3 days, however recovered to 50-60% after 7 and 14 days. Semiquantitative evaluation with immunohistochemistry for transferrin showed a numerical decrease of oligodendroglia in the medial corpus callosum after 14 and 30 days. Tissue rare-faction promptly occurred in the optic nerve and optic tract after 3 days, whereas it was delayed to 7 days after operation and increased in intensity gradually in the other white matter regions. These results indicate that bilateral occlusion of common carotid arteries in Wistar rats elicits a chronic cerebral hypoperfusion, which cumulatively leads to delayed appearance of white matter lesions.

Animals↗
PubMed1997

[Bilateral vertebral artery occlusion].

We studied 9 patients with bilateral vertebral artery occlusion (BVAO). BVAO was confirmed using angiography in order to clarify its clinical feature, mechanism, and long term prognosis. Three patients showed bilateral intra-cranial occlusion, 3 bilateral extra-cranial occlusion, and 3 intra and extra-cranial occlusion. The basilar artery was fed by the posterior communicating artery in 8 out of 9 patients. In one of the 8, reconstitution of the thyrocervical artery was seen. We divided the patients into 4 groups according to MRI findings, as follows: Group 1 with no abnormal finding on MRI (N = 2); Group 2 with deep pontine infarcts and non-territorial small cerebellar infarcts (N = 2); Group 3 with extended pontine infarcts (N = 3); and Group 4 with cerebellar cortical artery infarcts, deep pontine infarcts, and non-territorial small cerebellar infarcts (N = 2). Transient episodes were seen in all patients, 8 patients out of 9 had vertigo/dizziness, 3 tinnitus, 2 diplopia, 2 headache, 2 numbness, and 1 hearing disturbance. These episodes preceded a final attack or complete stroke 2 days to 5 months, and those who had a longer period of episodes in the preceding term tended to have less severe deficits. Six of the patients had vertebro-basiler symptoms after being in the upright position, including all the patients in Groups 2 and 4, which had cerebellar border zone/terminal zone infarcts. These results indicate that the hemodynamic mechanism plays an important role in BVAO. The prognosis was not always grave. Four of the patients could walk independently, 2 could walk with a cane, and 3 were bed ridden (2 of which died). Long-term follow-up data (a mean of 5 years) were obtained in all patients. In the patients who could walk, one had asymptomatic cerebellar infarcts, and one had TIAs frequently. Patients with BVAO often also have TIAs and/or preceding episode and show cerebellar border zone/terminal zone infarcts. This research strongly suggests that hemodynamic mechanism might play an important role in BVAO, and that paying attention to border zone infarction in MRI and transient episodes can lead to earlier diagnosis and treatment.

Activities of Daily Living↗
PubMed1997

[Detection of mit DNA point mutations by mutation-specific PCR].

Growing understanding in recent years about the prevalence and diversity of mitochondrial DNA mutations in human diseases has prompted a need for a simple screening method. We designed multiplex mutation-specific PCR, in which mutant DNAs were selectively amplified, for the 3243 (A to G) and 8344 (A to G) mutations. This method was simple and sensitive. We detected bands corresponding to the mutations in 1 and 2% mixtures of the 3243 and 8344 mutations respectively. Mutation-specific PCR is useful for detecting mutant mitochondrial DNA in peripheral blood, where its ratio to wild type DNA is sometimes too small to be detected by conventional assay, and for large scale screening to uncover a hitherto unknown relationship between mitochondrial DNA mutations and certain diseases.

DNA Mutational Analysis↗
PubMed1997

[Bilateral caudate head infarcts].

We reported a 67-year-old woman with bilateral caudate head infarcts. She developed sudden mutism followed by abulia. She was admitted to our hospital 2 months after ictus for further examination. She showed prominent abulia and was inactive, slow and apathetic. Spontaneous activity and speech, immediate response to queries, spontaneous word recall and attention and persistence to complex programs were disturbed. Apparent motor disturbance, gait disturbance, motor aphasia, apraxia and remote memory disturbance were not identified. She seemed to be depressed but not sad. Brain CT and MRI revealed bilateral caudate head hemorrhagic infarcts including bilateral anterior internal capsules, in which the left lesion was more extensive than right one and involved the part of the left putamen. These infarct locations were thought to be supplied by the area around the medial striate artery including Heubner's arteries and the A1 perforator. Digital subtraction angiography showed asymptomatic right internal carotid artery occlusion. She bad had hypertension, diabetes mellitus and atrial fibrillation and also had a left atrium with a large diameter. The infarcts were thought to be caused by cardioembolic occlusion to the distal portion of the left internal carotid artery. Although some variations of vasculature at the anterior communicating artery might contribute to bilateral medial striate artery infarcts, we could not demonstrate such abnormalities by angiography. Bilateral caudate head infarcts involving the anterior internal capsule may cause prominent abulia. The patient did not improve by drug and rehabilitation therapy and died suddenly a year after discharge.

Aged↗
PubMed1996

Pancreastatin-like immunoreactivity in globular dystrophic neurites of senile plaques in brains of patients with Alzheimer's disease.

The immunohistochemical localization of pancreastatin (PST) was examined in brains of Alzheimer's disease (AD) and control cases using three different antisera to PST, and was compared with the staining for chromogranin A (CgA), the precursor of PST. In control brains, CgA-like immunoreactivity was observed in the cytoplasm and fibers of certain neuronal populations, which were not immunostained with any of the PST antisera. In AD brains, dystrophic neurites of globular shape located in senile plaques were immunostained with each of the PST antisera, as well as with the CgA antibody. PST-positive and CgA-positive dystrophic neurites showed similar profiles. The present study indicates that CgA is probably cleaved to produce PST in some globular dystrophic neurites in senile plaques.

Aged↗
PubMed1996

Midkine-like immunoreactivity in extracellular neurofibrillary tangles in brains of patients with parkinsonism-dementia complex of Guam.

Midkine (MK) has been shown to be present in amyloid deposits in Alzheimer's disease (AD). In the present study, expression of midkine was examined immunohistochemically in brains of patients with the parkinsonism-dementia complex of Guam, lytico bodig disease (LB), using an affinity purified antibody to midkine. Positive staining was identified on some extracellular neurofibrillary tangles. The relative prevalence was somewhat less than for beta-amyloid protein (Abeta)-positive extracellular tangles. The results demonstrate that expression of MK and Abeta is not an exclusive feature of amyloid deposits in Alzheimer brain, but is also found in LB brain.

Antibody Specificity↗
PubMed1996

Glial expression of cytokines in the brains of cerebrovascular disease patients.

We examined the immunohistochemical localization of the proinflammatory cytokines tumor necrosis factor-alpha, lymphotoxin and interferon-gamma in 22 autopsy brains of patients with either cerebrovascular disease (CVD) or other neurological diseases as well as 2 non-neurological control brains. These cytokines were coexpressed mostly in the microglia/macrophages and in a few astroglia in the brains with acute cerebral infarction and cerebral hemorrhage. In cases with cerebral infarction, they were observed as early as 33 h after the onset of the illness and persisted for up to 40 days after the onset. In one patient with cerebral hemorrhage who survived for 4 h, the cytokine-immunoreactive glial cells were confined to the margins of the hematoma. In contrast, the cytokine-immunoreactive glia were distributed diffusely in one patient with cerebral hemorrhage who died 12 days after the onset of the illness. Labeling for these cytokines was weak in the glial cells of control brains and those with neurodegenerative diseases, including Alzheimer's disease, Parkinson's disease and multiple system atrophy, in so far as there were no concomitant acute CVD foci. The present results indicate that proinflammatory cytokines are up-regulated in the brains of patients with acute stroke, and suggest an early inflammatory response in human CVD.

Aged↗
PubMed1996

A case of primary progressive aphasia with abnormally ubiquitinated neurites in the cerebral cortex.

We report the histopathological and immunohistochemical findings in a patient with primary progressive aphasia and abnormally ubiquitinated neurites in the cerebral cortex. Neuropathological examination showed severe neuronal loss and astrocytosis with a spongy change in the frontal cortex and neostriatum. Immunohistochemistry for ubiquitin antibody showed many immunoreactive dystrophic neurites in the superficial layer of the affected cortices and putamen. Those neurites were neither argentophilic nor stained with other antibodies against neurofilament, tau, or microtubule-associated protein-2. There were no neuropathological changes characteristic of Alzheimer's disease, Pick's disease, or Creutzfeldt-Jakob disease. Immunoelectron microscopy using anti-ubiquitin antibody showed inclusions in the dendrites, consisting mainly of granular and filamentous material. These pathological features, unusual in primary progressive aphasia, indicate the neuropathological heterogeneity of this disease condition.

Aphasia, Primary Progressive↗
PubMed1996

Peripheral neuropathy in late-onset Krabbe's disease: histochemical and ultrastructural findings.

We performed histochemical and ultrastructural studies on a sural nerve biopsy specimen obtained from a 31-year-old man with late-onset Krabbe's disease. Myelin sheaths were uniformly thin for the fiber diameter, with a moderate reduction in the myelinated fiber population. Despite a few small onion-bulb formations, quantitative studies demonstrated uniformly thin myelin sheaths and less variability in internodal lengths. This suggests that the main pathophysiology of the peripheral neuropathy observed in late-onset Krabbe's disease is hypomyelination rather than segmental demyelination as in infantile Krabbe's disease. Ultrastructurally, curvilinear lamellar cytoplasmic inclusions were observed in Schwann cells and fibroblasts.

Adult↗
PubMed1996

Synaptophysin expression in the anterior horn of Werdnig-Hoffmann disease.

This report concerns the study of synaptophysin (SP) expression in the anterior horn in four cases of Werdnig-Hoffmann disease (WHD). All patients had distinct anterior horn cell degeneration, and died before the age of one year. Normal spinal cords from five age-matched children served as controls. Five cases of sporadic amyotrophic lateral sclerosis (S-ALS), three cases of lower motor neuron disease (L-MND), three cases of peripheral neuropathy with axonal reaction, and six adult cases with normal spinal cords were included for comparison. Immunohistochemical techniques were used throughout. The results show that normal spinal cords of children have similar SP immunoreactivity patterns as those of normal adults. We also found that despite relatively preserved or slightly increased SP immunoreactivity on the surface of the cell body and proximal processes of the remaining neurons, there was a diffuse decrease of immunoreaction product deposits in the anterior horn neuropil of the WHD cases. The ballooned neurons in the anterior horns of patients with WHD, S-ALS, L-MND, and axonal reaction had few SP immunoreactive dots or granules around the cell bodies and proximal processes. The perikarya of some ballooned neurons of the children with WHD was diffusely stained for SP. There was no SP immunoreactive structures within the empty cell beds of these patients. The observed decrease in SP expression around ballooned neurons in these disorders is indicative of a disconnection of presynaptic terminals of afferent fibers from the proximal portion of the swollen degenerated anterior horn cells.

Anterior Horn Cells↗
PubMed1996

Immunohistochemical localization of brain-derived neurotrophic factor in adult rat brain.

To investigate the role of brain-derived neurotrophic factor in the central nervous system, we produced an anti-peptide antibody that specifically recognized brain-derived neurotrophic factor and performed immunohistochemistry for brain-derived neurotrophic factor-like immunoreactivity in normal adult rat brain. A synthetic peptide (EKVPVSKGQL), derived from mature brain-derived neurotrophic factor, was conjugated to bovine thyroglobulin at a ratio of 1:3 and used as an immunogen to produce a high-titre anti-brain-derived neurotrophic factor polyclonal antibody in Japanese white rabbits. Dot blotting demonstrated that the antiserum could detect 3.91 pmol of synthetic peptide, and Western blotting showed that the antiserum recognized one band with a molecular weight consistent with that of brain-derived neurotrophic factor. In immunohistochemistry, brain-derived neurotrophic factor-like immunoreactivity was widespread in adult rat brain, including cerebral cortex, hippocampus, basal forebrain, striatum, hypothalamus, brainstem and cerebellum. Not only neuronal somata but also nerve fibres showed positive staining. Our data suggest that brain-derived neurotrophic factor is transported through axons in a subpopulation of neurons in adult rat brain, and that brain-derived neurotrophic factor influences a great variety of neurons and acts as a neurotrophic factor in the central nervous system.

Animals↗
PubMed1996

Supplementary motor area seizure resembling sleep disorder.

Two patients presented with a complaint of frequent sudden arousals during sleep followed by tachypnea and palpitation associated with stiffness in the upper extremities in one case and by elevation of the left lower limb in the other. All night video-electroencephalogram (EEG) polysomnography (VPSG) confirmed the diagnosis of seizure arising from the supplementary motor area (SMA seizure) in both cases. Carbamazepine (CBZ) produced remarkable improvement both in clinical seizures and in their subjective sleep quality. Repeated polysomnography after treatment showed a clear improvement in sleep architecture with higher percentages of slow wave sleep. SMA seizure could disturb nocturnal sleep and is one of the important differential diagnoses for a patient complaining of frequent arousals associated with motor disturbance during sleep.

Adult↗
PubMed1996

Bromocriptine therapy in early-stage Parkinson's disease.

We evaluated bromocriptine monotherapy during the early stage of Parkinson's disease (Hoehn and Yahr stage I or II). Of the 120 patients registered as participants in this trial, 96 were studied. The follow-up period was 48 weeks. For the first 24 weeks they were all placed on bromocriptine with or without anticholinergic drugs; for the second 24 weeks, 75 of them were still kept on the same medication, with 21 requiring additional levodopa. Clinically, the former group showed a good response to the bromocriptine monotherapy, while the latter responded poorly to it (requiring in addition levodopa). We think that Parkinson's disease encompasses a heterogeneous group of patients of whom about 75% are well treated by bromocriptine alone at the early stages of the illness.

Antiparkinson Agents↗
PubMed1996

Alterations of the blood-brain barrier and glial cells in white-matter lesions in cerebrovascular and Alzheimer's disease patients.

BACKGROUND AND PURPOSE: The underlying cause of white-matter lesions, which are frequent findings in cerebrovascular disease (CVD) and Alzheimer's disease (AD), remains uncertain. We performed immunohistochemical analysis of serum protein extravasation to investigate the function of the blood-brain barrier in white-matter lesions. METHODS: White-matter lesions were estimated by use of Kluver-Barrera staining in patients diagnosed clinicopathologically as having ischemic CVD (n = 14) and AD (n = 12) and from nonneurological control subjects (n = 6). Axonal damages were investigated by use of immunohistochemistry for amyloid protein precursor. Alteration of the blood-brain barrier was examined with fibrinogen and immunoglobulins used as markers. The numbers of HLA-DR-positive microglia and glial fibrillary acidic protein-positive astroglia were examined comparatively. RESULTS: White-matter lesions were graded as normal (grade 0) in 14 of the 32 cases (44%), slight (grade I) in 10 cases (31%), moderate (grade II) in 6 cases (19%), and severe (grade III) in 2 cases (6%). Amyloid precursor protein was accumulated most frequently in grade II white-matter lesions. Immunohistochemistry for serum proteins labeled astroglial cell bodies and their processes, which seemed to have sequestered extravasated proteins. The groups with detectable white-matter lesions had significantly higher grading scores for fibrinogen and immunoglobulins than the control group (P < .05). Although the higher scores for serum protein extravasation were statistically significant in ischemic CVD cases (P < .05), there was no significant increase in AD cases. Activated microglia and astroglia were more numerous in the groups with white-matter lesions in both ischemic CVD and AD cases, although this increase in the number of astroglia was not evident in regions with clasmatodendrosis. CONCLUSIONS: Dysfunction of the blood-brain barrier is more prominent in white-matter lesions seen in ischemic CVD than in AD and may have a role in the pathogenesis of cerebrovascular white-matter lesions.

Aged↗
PubMed1996

Large neurons in the tuberomammillary nucleus in patients with Parkinson's disease and multiple system atrophy.

To investigate whether the histaminergic neurons degenerate in Parkinson's disease (PD) and multiple system atrophy (MSA), we studied the number of large-sized neurons in the tuberomammillary nucleus in patients with PD, patients with MSA, and age-matched controls. The number of large-sized neurons in the tuberomammillary nucleus in PD patients was not altered compared with controls, and Lewy bodies were rarely present in the tuberomammillary nucleus. In contrast, the number of large-sized neurons in the tuberomammillary nucleus in MSA patients was significantly decreased compared with controls. Thus, the central histaminergic neurons are affected in MSA and preserved in PD.

Aged↗
PubMed1996

[Familial Binswanger-type encephalopathy with Sneddon syndrome].

We reported a family with early onset cerebrovascular disease. Patient 1 (a 36-year-old man) demonstrated a combination of livedo reticularis and cerebral infarction as previously described as Sneddon syndrome. He also showed transient focal neurologic symptoms and mild dementia. Patient 2 (an elder sister of Patient 1) was suffering from migraine. Their father and paternal uncle died of cerebral infarction, which had developed in their thirties or forties. Patients 1 and 2 showed MRI findings compatible with encephalopathy with Binswanger-type. Contrary to the previous reports on Binswanger-type encephalopathy, both of these patients demonstrated decreased levels of fibrinogen as well as those of factor V, together with negative antiphospholipid antibody. Thus, juvenile onset, autosomal dominant inheritance, the diversity of clinical findings and the coagulopathy in this family were characteristic features. The level of thrombin-antithrombin III complex (TAT) was markedly increased in Patient 1. Treatment with antithrombin (argatroban 20mg i.v. everyday for 28 days) not only reduced the level of TAT but also improved the livedo reticularis and neurological findings. Although gene analysis has not been performed yet on this family, this condition is similar to cerebral autosomal dominant arteriopathy with subcortical infarct and leukoencephalopathy (CADASIL), which involve juvenile cerebral infarction and dementia as well as migraine.

Adult↗