Search PubMed⌕ Search

Biomedical subjects

Seiji Kikuchi

Publications and source records attributed to Seiji Kikuchi.

66 records · Page 4Linked to original sources
PubMed2002

Late onset ataxia phenotype in dentatorubro-pallidoluysian atrophy (DRPLA).

ABSTRACT We clinically and genetically studied three patients in a family with dentatorubro-pallidoluysian atrophy (DRPLA). The proband patient had 58/24 CAG repeat alleles of the DRPLA gene (normal < or = 34 repeats). Cerebellar ataxia first developed in the 6-7th decades and was the predominant feature for more than 10 years in all three, after which two of them manifested dementia and choreiform movements in the advanced stage. Atrophy of the cerebellum and brain stem an CT or MRI had suggested dominant spinocerebellar ataxia as a diagnosis in their ataxia-predominant stage, with a diagnosis of DRPLA being impossible based on the clinical findings alone. Our experience implies that DRPLA must be taken into account in the differential diagnosis of late onset ataxic disorders, since it can easily be overlooked.

Aged↗
PubMed2002

No association of vitamin D-binding protein gene polymorphisms in Japanese patients with MS.

Vitamin D-binding protein (DBP) is known to function as an immunomodulatory factor, as well as the main carrier of vitamin D. We analyzed the frequencies of two polymorphisms (codon 416 and codon 420) in the DBP gene through a case-control study involving 107 Japanese patients with multiple sclerosis (MS) and 109 healthy controls. None of these polymorphisms showed any association with the occurrence of MS. Furthermore, no association was observed between the DBP polymorphisms and the age at disease onset. These results suggest that DBP does not contribute to the development of MS in Japanese.

Adjuvants, Immunologic↗
PubMed2002

Estrogen receptor gene polymorphism and multiple sclerosis in Japanese patients: interaction with HLA-DRB1*1501 and disease modulation.

We investigated PvuII and XbaI polymorphism in the estrogen receptor gene (ERG) and HLA-DRB1*1501 positivity in 116 conventional multiple sclerosis (MS) patients and 101 healthy controls in a Japanese population. Logistic analysis revealed independent associations of [P] allele in the profiles for PvuII (p=0.0005, adjusted odds ratio (aOR)=3.17) and DRB1*1501 (p=0.0089, aOR=2.61) with conventional MS. Synergistic elevated risk of MS due to interaction between the [P] allele and HLA-DRB1*1501 allele was found among female patients (odds ratio=16.0; 95% CI=3.99-63.8, p<0.0001). The [P] allele-positive patients with disease duration of more than 5 years had a significantly higher progression index (PI) of disability (p=0.0230) and a worse ranked MS severity score (p=0.0152) than their non-[P] counterparts.

Adolescent↗
PubMed2002

Familial syringomyelia: the first Japanese case and review of the literature.

We reported on syringomyelia in a mother and her son. The mother was 74-year-old, who developed gait difficulty at the age of 54. The son, 47-year-old, developed the same symptoms at the age of 35. In the both cases, MRI revealed syringomyelia with Chiari malformation. Twenty-one families with syringomyelia have been reported. The existence of these families indicates that genetic factors may play in role to the pathogenesis of this disorder.

Aged↗
PubMed2002

Detection of N epsilon-(carboxymethyl)lysine (CML) and non-CML advanced glycation end-products in the anterior horn of amyotrophic lateral sclerosis spinal cord.

INTRODUCTION: The involvement of glycation in neurodegenerative diseases such as Alzheimer's disease, Parkinson's disease and amyotrophic lateral sclerosis (ALS) was recently indicated. We previously reported the existence of an Amadori product, 1-hexitol-lysine (1-HL), which is formed in the early glycation reaction, in axonal spheroids of the anterior horn of the ALS spinal cord. OBJECTIVE: The purpose of the present study was to confirm the occurrence of the later-stage glycation reaction that follows the early glycation reaction and leads to the formation of advanced glycation end products (AGEs). METHOD: We examined whether N(epsilon)-(carboxymethyl)lysine (CML) and non-CML AGE are present in ALS spinal cords. RESULTS: Immunohistochemical staining with anti-CML antibody revealed intense positivity in the cell bodies of the remaining atrophic motor neurons and in microglia. Microglia were also positive on staining with anti-non-CML antibody. Axonal spheroids were also positive on anti-non-CML-antibody staining. Vascular endothelial cells were slightly stained by both antibodies. CONCLUSIONS: The presence of non-CML AGE in the anterior horn of the ALS spinal cord indicates that the later stage of the glycation reaction is involved in the pathology of ALS. The presence of CML in the anterior horn was also confirmed, and this may reflect augmented oxidative stress.

Aged↗
PubMed2002

Intracranial hemorrhage in neuro-Behçet's syndrome.

OBJECTIVE: Most cerebrovascular disturbances in Behçet's syndrome are occlusive in nature, while hemorrhage is rare. In this paper, we report three cases of neuro-Behçet's syndrome presenting with intracerebral hemorrhaging, and discuss the possible causes as they relate to cyclosporine treatment. PATIENTS: Three cases of neuro-Behçet's syndrome presented with intracranial hemorrhage. One patient had been taking cyclosporine, and the other two patients had never taking cyclosporine. RESULTS: Together with previous reports, these cases suggest that there are two types of intracranial hemorrhage in neuro-Behçet's syndrome. One type occurs in the center of a lesion and during the acute phase of the disease, while the other occurs in the peripheral lesion and during the subacute phase. CONCLUSIONS: It appears that the intracranial hemorrhages in neuro-Behçet's syndrome can be divided into two groups. It is possible that the vascular pathologies caused by Behçet's syndrome and by cyclosporine conspire to induce CNS hemorrhaging in some cases.

Adult↗
PubMed2002

Cervical myelopathy due to a "tight dural canal in flexion" with a posterior epidural cavity.

A 41-year-old man noticed weakness and atrophy in his right hand and forearm resembling the non-progressive juvenile muscular atrophy of unilateral upper extremity (Hirayama's disease). MRI showed an abnormal cavity in the posterior epidural space which appeared on neck flexion communicating with the subarachnoid space in addition to the flattening of the lower cervical spinal cord on neck flexion. When evaluating atypical cases of Hirayama's disease, the pathomechanism demonstrated in the present case should be taken into consideration.

Adult↗
PubMed2002

Palmitate-induced apoptosis of microvascular endothelial cells and pericytes.

BACKGROUND: Recent observations in the EURODIAB Complications Study demonstrated that markers of insulin resistance are strong risk factors for retinopathy incidence in patients with diabetes. However, the molecular mechanism underlying this remains to be elucidated. In this study, we investigated the influence of palmitate, a major saturated free fatty acid in plasma, on the apoptotic cell death of cultured microvascular endothelial cells (EC) and retinal pericytes. MATERIALS AND METHODS: The intracellular formation of reactive oxygen species (ROS) was detected using the fluorescent probe CM-H(2)DCFDA. DNA synthesis was determined by measuring [(3) H]-thymidine incorporation into cells. DNA fragmentations of EC were quantitatively analyzed in an enzyme-linked immunosorbent assay, and DNA laddering was evaluated on agarose gel electrophoresis. RESULTS: Palmitate increased ROS generation in microvascular EC. Furthermore, palmitate significantly inhibited DNA synthesis and induced apoptotic cell death in EC, which were completely prevented by an antioxidant, N-acetylcysteine. Palmitate up-regulated pericyte mRNA levels of a receptor for advanced glycation end products (AGE), and thereby potentiated the apoptotic effects of AGE on pericytes. CONCLUSIONS: The results suggest that palmitate could induce apoptotic cell death in microvascular EC and pericytes through the overgeneration of intracellular ROS, and thus be involved in the development of diabetic retinopathy.

Apoptosis↗
PubMed2002

[A case of Ramsay Hunt syndrome initiated with hoarseness and dysphagia: consideration on spreading mechanisms of cranial neuropathy].

A 85-year-old woman was admitted to our hospital because of progressive hoarseness and dysphagia of two days' duration. Neurological examination on admission revealed right pharyngeal and vocal cord palsies. After admission, gradual swelling of her right ear was noted, and on day 6, vesicular eruptions in her right geniculate zone, the right VII and the VIIIth cranial nerve palsies were added. On the basis of these findings, she was diagnosed as Ramsay Hunt syndrome. Varicella zoster virus (VZV) infection was confirmed by the elevation of serum anti-VZV-antibody titer, and detection of VZV DNA from cerebrospinal fluid. Ramsay Hunt syndrome associated with multiple cranial neuropathy is not frequently reported. Reviewing Japanese literatures, we found that the IX and the Xth cranial nerves were most frequently affected, and the half of these cases were initiated with cranial neuropathy other than the VIIth. Additionally, spreading mechanisms of cranial neuropathy, and the early diagnostic problems of these conditions were discussed.

Aged↗
PubMedDate not supplied

Toxic advanced glycation end products (TAGE) theory in Alzheimer's disease.

Several epidemiological studies have reported moderately increased risks of Alzheimer's disease (AD) in diabetic patients compared with general population. In diabetes mellitus, the formation and accumulation of advanced glycation end products (AGEs) progress more rapidly. Recent understanding of this process has confirmed that interactions between AGEs and their receptor (RAGE) may play a role in the pathogenesis of diabetic complications and AD. The authors have recently found that glyceraldehyde-derived AGEs (AGE-2), which is predominantly the structure of toxic AGEs (TAGE), show significant toxicity on cortical neuronal cells and that the neurotoxic effect of diabetic serum is completely blocked by neutralizing antibody against the AGE-2 epitope. Moreover, in human AD brains, AGE-2 is distributed in the cytosol of neurons in the hippocampus and parahippocampal gyrus. These results suggest that TAGE is involved in the pathogenesis of AD as well as other age-related diseases. In this review, the authors discuss the molecular mechanisms of AD, especially focusing on TAGE-RAGE system.

Alzheimer Disease↗