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1.
Z Gerontol Geriatr ; 54(2): 141-145, 2021 Mar.
Article in German | MEDLINE | ID: mdl-33624143

ABSTRACT

BACKGROUND: Following the COVID-19 pandemic residents of nursing homes, their visitors and families as well as employees are faced with new challenges. Protective measures have a strong impact on the well-being of all these groups of persons. MATERIAL AND METHODS: A systematic search was carried out for studies investigating the psychosocial consequences of the COVID-19 pandemic for residents, their visitors and families as well as nursing home staff. Results were analyzed by narrative synthesis. RESULTS: A total of 756 studies were screened and 15 studies were included. These studies were conducted between February and June 2020 with participants from 14 countries. Participants reported loneliness, grief and depressive symptoms among residents. Some gave an account of fear as a reaction of residents to social distancing. Residents with cognitive impairment suffered more although there are conflicting reports. The well-being of visitors and friends was compromised and their feeling of loneliness increased. Nursing home personnel reported fear of getting infected and of infecting residents or their own families. Infected workers in the USA expressed anger about a lack of protection. Furthermore, an increase in workload was reported. CONCLUSION: Studies conducted during the first months of the pandemic reported negative consequences for the psychosocial well-being of residents, their visitors and nursing home staff. Individual needs for future support of these groups are distinct and need further evaluation during the on-going pandemic.


Subject(s)
COVID-19 , Nursing Staff , Humans , Nursing Homes , Pandemics , SARS-CoV-2
2.
Niger J Clin Pract ; 21(9): 1132-1138, 2018 Sep.
Article in English | MEDLINE | ID: mdl-30156197

ABSTRACT

OBJECTIVE: The objective of this study was to determine the ora-dental health and its relation with medication and dental fear in a group of Turkish children with attention-deficit hyperactivity disorder (ADHD). SUBJECTS AND METHODS: The levels of dental fear of children were determined with The Dental Subscale of Children's Fear Survey Schedule (CFSS-DS). The oral and dental health evaluation was performed. This study included a total of 117 children aged between 6 and 15 years and they were examined under two groups as "ADHD" (n = 59) and "Control" (n = 58). Ora-dental health variables were compared between the groups and were also analyzed in accordance with dental fear and medication. RESULTS: ADHD children and the control group exhibited similar CFSS-DS scores (15-32). No significant differences existed in df(t)/df(s), DMF(T)/DMF(S), d/D values, and presence of the white spot lesions. ADHD children's Mutans streptococci and Lactobacillus quantities were found significantly higher than the control group. The incidence of parafunctional habits of the ADHD children was also found high. CONCLUSIONS: ADHD children that were medicated exhibited similar dental caries prevalence and periodontal health status. Although ADHD group had similar dental-periodontal health status and dental fear level with the control group and using ADHD medicines did not make a significant effect on the ora-dental health parameters, the patients should be carefully followed up because they were categorized in high caries risk groups.


Subject(s)
Attention Deficit Disorder with Hyperactivity/psychology , Dental Anxiety/epidemiology , Dental Caries/epidemiology , Oral Health , Adolescent , Attention Deficit Disorder with Hyperactivity/complications , Case-Control Studies , Child , Cross-Sectional Studies , DMF Index , Dental Anxiety/psychology , Dental Caries/complications , Female , Health Status , Humans , Incidence , Male , Oral Hygiene , Prevalence , Turkey/epidemiology
3.
J Phys Condens Matter ; 30(36): 365502, 2018 Sep 12.
Article in English | MEDLINE | ID: mdl-30051888

ABSTRACT

We characterize the confinement of massless Dirac electrons under axially symmetric magnetic fields in graphene, including zero energy modes and higher energy levels. In particular, we analyze in detail the Aharonov-Casher theorem, on the existence of zero modes produced by magnetic fields with finite flux in two dimensions. We apply techniques of supersymmetric quantum mechanics to determine the confined states by means of the quantum number j associated to isospin and angular momentum. We focus on magnetic fields, regular at the origin, whose asymptotic behaviour is [Formula: see text], with α a real number. A confinement of infinite zero-energy modes and excited states is possible as long as [Formula: see text]. When [Formula: see text] the quantum dot is able to trap an infinite number of zero modes but no excited states, while for [Formula: see text] only a finite number of zero modes are confined.

4.
Biotech Histochem ; 91(3): 195-203, 2016.
Article in English | MEDLINE | ID: mdl-26960158

ABSTRACT

We investigated the antioxidant and anti-inflammatory effects of propolis on bleomycin induced lung fibrosis and compared these effects to prednisolone treatment. Forty rats were divided into four groups of ten: group 1 was treated with intratracheal infusion of 0.2 ml physiological saline followed by daily treatment with 0.5 ml physiological saline for 20 days. In the remaining groups (groups 2 - 4), 5 mg/kg bleomycin was given via the trachea. Rats in group 2 were given 0.5 ml physiological saline. Rats in group 3 were treated with 100 mg/kg propolis, and 10 mg/kg prednisolone was given to rats in group 4. The treatments for all groups were continued for 20 days. On postoperative day 21, blood and lung samples were taken for biochemistry, histopathology and electron microscopy evaluation. We compared oxidative stress parameters and found lower malondialdehyde and myeloperoxidase levels, and higher total sulfhydryl levels and catalase activities for the bleomycin + propolis group than for the bleomycin and bleomycin + prednisolone groups. The highest mean fibrosis score was detected in the bleomycin group. Although the mean fibrosis scores of the bleomycin + propolis and bleomycin + prednisolone groups were not significantly different, electron microscopy revealed that propolis diminished bleomycin induced lung fibrosis more effectively than prednisolone. The effects of propolis might be due to its potent antioxidant and anti-inflammatory properties.


Subject(s)
Bleomycin , Lung/drug effects , Lung/ultrastructure , Oxidative Stress/drug effects , Propolis/pharmacology , Pulmonary Fibrosis/chemically induced , Animals , Anti-Infective Agents/pharmacology , Antioxidants/pharmacology , Lung/pathology , Microscopy, Electron, Transmission , Pulmonary Fibrosis/pathology , Pulmonary Fibrosis/prevention & control , Rats
5.
J Phys Condens Matter ; 27(28): 285501, 2015 Jul 22.
Article in English | MEDLINE | ID: mdl-26102328

ABSTRACT

The aim of this article was to study the degeneracy of the energy spectrum in a nanotube under a transverse magnetic field. The massless Dirac-Weyl equation has been used to describe the low energy states of this system. The particular case of a singular magnetic field approximated by Dirac delta distributions is considered. It is shown that, under general symmetry conditions, there is a double degeneracy corresponding to periodic solutions with null axial momentum k(z)=0. Also, there may be a kind of sporadic degeneracy for non-vanishing values of k(z), which are explicitly computed in the present example. The proof of these properties is obtained by means of the supersymmetric structure of the Dirac-Weyl Hamiltonian.

6.
BMJ Open ; 3(10): e003603, 2013 Oct 31.
Article in English | MEDLINE | ID: mdl-24176796

ABSTRACT

OBJECTIVES: Muscle volume in patients with muscle disease is an index of disease progression. The aim of this study was to demonstrate a new method of muscle volumetry using CT of thigh muscles. DESIGN: Observational study. PARTICIPANTS: (1) For muscle volumetry using CT and dual-energy X-ray absorptiometry (DXA), 13 patients with muscle disease participated. (2) For prospective CT volumetry, 12 patients participated over 4 years. PRIMARY AND SECONDARY OUTCOME MEASURES: To establish the new CT volumetry, the results of which were correlated with the muscle mass calculated from DXA (primary outcome). To show the decrease in muscle volume using our method over 4 years (secondary outcome) METHODS: (1) Helical CT imaging of the thigh was performed. CT images were analysed by applying estimated functions, and the accumulation of outcomes resulted in muscle volumes. We refer to this method as 'net muscle volumetry'. Simultaneously, DXA was performed in these patients, and the muscle mass of the thigh was calculated with vendor-provided software. (2) To evaluate longitudinal changes in muscle volume, net muscle volumetry at the 14 cm section of the middle part of the thigh was performed repeatedly over a 4-year period. RESULTS: (1) Volumes of the thigh muscle on one side were calculated to be 300-3400 cm(3) using CT. Muscle mass of the thigh was calculated from DXA to be 1100-5000 g. These results correspond closely, with a Pearson correlation coefficient of 0.993. (2) Thigh net muscle volumes of seven patients with gait disturbance decreased over the 4-year study period (p<0.01). CONCLUSIONS: A method of measuring net muscle volume using CT, which was validated by muscle mass calculated from DXA, was developed. Decrements in net muscle volume over 4 years support the reliability of this method. This less arbitrary method is suitable for assessment of muscle volume in patients with muscular dystrophy.

7.
J Phys Condens Matter ; 25(16): 165301, 2013 Apr 24.
Article in English | MEDLINE | ID: mdl-23553418

ABSTRACT

Methods of supersymmetric quantum mechanics are used to obtain analytical solutions for massless Dirac electrons in spherical molecules, including fullerenes, in the presence of magnetic fields. The solutions for Dirac massive charges are also obtained via the solutions of the Dirac-Weyl equation.

9.
Bratisl Lek Listy ; 113(12): 692-7, 2012.
Article in English | MEDLINE | ID: mdl-23173625

ABSTRACT

AIM: Our goal was to determine the effects of a diosmine-hesperidine combination on wound healing in a rat model of colonic anastomosis. MATERIALS AND METHODS: In this study, 20 Wistar Albino female rats were randomized into four experimental groups containing five rats in each group. A segment of 1 cm of colon was excised 4 cm proximally to the peritoneal reflection in all rats without carrying out any mechanical or antibacterial bowel preparation. Colonic anastomosis was performed with interrupted, inverting sutures of 6/0 polypropylene. Beginning from the first postoperative day, the rats in Groups II and IV received 100 mg/kg per day of diosmine-hesperidine via orogastic route by 4F fine feeding catheter. RESULTS: A significant difference was detected between groups in terms of their hydroxyproline levels (p<0.05); the hydroxyproline level of Group I was significantly lower than that of the other groups while no significant difference was noted between Groups II and III. CONCLUSION: The administration of diosmine-hesperidine increased the amount of collagen and bursting pressures at the anastomotic site and thus had favorable influences on the healing of colonic anastomosis (Tab. 1, Fig. 3, Ref. 33).


Subject(s)
Anastomosis, Surgical , Colon/surgery , Diosmin/administration & dosage , Hesperidin/administration & dosage , Wound Healing/drug effects , Animals , Colon/metabolism , Drug Combinations , Female , Hydroxyproline/metabolism , Rats , Rats, Wistar , Tensile Strength , Wound Healing/physiology
10.
J Phys Condens Matter ; 21(45): 455305, 2009 Nov 11.
Article in English | MEDLINE | ID: mdl-21694010

ABSTRACT

Exact analytical solutions for the bound states of a graphene Dirac electron in various magnetic fields with translational symmetry are obtained. In order to solve the time-independent Dirac-Weyl equation the factorization method used in supersymmetric quantum mechanics is adapted to this problem. The behavior of the discrete spectrum, probability and current densities are discussed.

11.
Tech Coloproctol ; 10(3): 208-14, 2006 Oct.
Article in English | MEDLINE | ID: mdl-16969615

ABSTRACT

BACKGROUND: Leakage from colonic anastomoses leads to mortality and morbidity. Fibrin adhesives can be used to increase the strength of the anastomosis. In this study, we evaluated the early effects of fibrin sealant and hyaluronic acid-carboxymethylcellulose on colonic anastomosis in rats. METHODS: Anastomoses were made in the descending colon of 38 female Wistar-Albino rats, in three groups: control group (n = 12), group 1 treated with hyaluronic acid-carboxymethylcellulose (n = 16), and group 2 treated with fibrin sealant (n = 10). After 72 hours, adhesion scores, bursting pressure, rupture strength and histopathologic healing scores were evaluated. RESULTS: Due to postoperative mortality, we evaluated 10, 10 and 9 rats in the control group and in groups 1 and 2, respectively. Of these, we excluded 4, 5 and 4 rats that had macroperforations at autopsy. In the remaining rats, bursting pressure (123.2 +/- 14.8 mmHg) and rupture strength (400 +/- 16 mg) in the fibrin sealant group were significantly greater than in the two other groups ( CONTROL: 68.0 +/- 10.6 p = 0.006 and 325 +/- 52 p = 0.009; Group 1: 74.0 +/- 9.8 p = 0.03, 330 +/- 27 p = 0.016). However, we did not observe any significant difference between adhesion scores (2.5 +/- 0.6, 2.0 +/- 0.7, 2.0 +/- 0.7, p = 0.343). CONCLUSIONS: In this experimental study, fibrin sealant increased bursting pressure and rupture strength of colonic anastomoses while hyaluronic acid-carboxymethylcellulose had no effects in rats, but both of them showed no effect on adhesion scores. In order to use fibrin sealant to decrease the rate of early leakages from colonic anastomoses, further studies have to be performed.


Subject(s)
Colon/surgery , Fibrin Tissue Adhesive/therapeutic use , Tissue Adhesives/therapeutic use , Anastomosis, Surgical , Animals , Carboxymethylcellulose Sodium/therapeutic use , Female , Hyaluronic Acid/therapeutic use , Pressure , Rats , Rats, Wistar , Tissue Adhesions/etiology
13.
Acta Neuropathol ; 100(5): 521-7, 2000 Nov.
Article in English | MEDLINE | ID: mdl-11045674

ABSTRACT

Polymyositis (PM) is a cell-mediated autoimmune disease. Perforin (PF), Fas ligand (FasL) and TNF-alpha are considered to be important factors in cytotoxic T lymphocyte-mediated cell injury, and several studies have established a role of lymphotoxin (LT) in T helper type 1 (Th1)-induced cell-mediated autoimmune diseases. In the present study, to determine how LT, PF and FasL are involved in the pathogenesis of PM, we used immunohistochemical staining (IHC), reverse transcription polymerase chain reaction (RT-PCR), and in situ hybridization (ISH) on muscle specimens from patients with PM, amyotrophic lateral sclerosis (ALS), myotonic dystrophy (MyD) and controls (NC). There were many mononuclear cells (MNCs) immunoreactive for LT and some for PF and FasL within the fasciculus in PM muscles. On the other hand, only few or no LT-, PF- and FasL-positive cells were detected in MyD, ALS and NC muscles. The results of mRNA expression of these three molecules with RT-PCR were consistent with those using IHC methods. The number of MNCs positive for LT with ISH was far higher in PM compared to MyD, ALS and NC (P < 0.05 or 0.01). The MNCs located in the connective tissue or in the vicinity of necrotizing or non-necrotizing muscles were mainly LT mRNA and CD4 positive, while MNCs invading the non-necrotic fibers were mainly LT mRNA and CD8 positive. Our results indicated that the expression of LT was up-regulated in PM, and LT plays an important role in muscle injury and orchestrating the inflammatory reaction in PM.


Subject(s)
Lymphotoxin-alpha/physiology , Polymyositis/etiology , Aged , Amyotrophic Lateral Sclerosis/metabolism , Fas Ligand Protein , Female , Humans , Immunohistochemistry , In Situ Hybridization , Lymphotoxin-alpha/genetics , Lymphotoxin-alpha/metabolism , Male , Membrane Glycoproteins/genetics , Membrane Glycoproteins/metabolism , Middle Aged , Monocytes/metabolism , Muscle, Skeletal/metabolism , Myotonic Dystrophy/metabolism , Perforin , Pore Forming Cytotoxic Proteins , RNA, Messenger/metabolism , Reference Values , Reverse Transcriptase Polymerase Chain Reaction
14.
Muscle Nerve ; 23(9): 1421-6, 2000 Sep.
Article in English | MEDLINE | ID: mdl-10951446

ABSTRACT

In myotonic dystrophy (DM), the expansion of CTG triplet repeats in the 3'-untranslated region of DM-protein kinase (DMPK) is a causal gene mutation. However, the pathogenic molecular mechanism of CTG repeat expansion for DM phenotypic expression is unclear. To investigate this issue, we examined the influence of CTG repeat expansion on the expression levels of DMPK gene and 3'-flanking DM locus-associated homeodomain protein (DMAHP)/SIX5 gene in the muscles of DM patients. We isolated RNA from muscle tissues of six DM patients and six controls, and performed a competitive reverse transcriptional polymerase chain reaction (RT-PCR) assay. The total mRNA level of DMAHP/SIX5 was significantly lower in DM than in controls, but the DMPK mRNA level was unchanged. Our results suggest that CTG repeat expansion influences the expression of genes other than DMPK to cause the DM phenotype.


Subject(s)
Homeodomain Proteins/metabolism , Muscle, Skeletal/metabolism , Myotonic Dystrophy/metabolism , Adult , Female , Homeodomain Proteins/genetics , Humans , Male , Middle Aged , RNA, Messenger/metabolism , Reverse Transcriptase Polymerase Chain Reaction/methods
15.
Acta Neuropathol ; 99(5): 585-8, 2000 May.
Article in English | MEDLINE | ID: mdl-10805105

ABSTRACT

We evaluated the expression of tumor necrosis factor-alpha (TNF-alpha) mRNA in muscle biopsy specimens from patients with polymyositis (PM) and dermatomyositis (DM) to clarify its role in the pathogenesis of PM and DM. We performed non-radioactive in situ hybridization studies for TNF-alpha combined with immunohistochemistry for cell type-specific markers on muscles from ten PM and five DM patients. TNF-alpha-positive infiltrating cells present in the endomysium and perimysium were found in all PM and DM muscles. The frequency of TNF-alpha-positive cells against total infiltrating cells was similar among PM and DM (27.1 +/- 7.4% in PM and 28.5 +/- 13.6% in DM). However, TNF-alpha/CD8-positive lymphocytes and TNF-alpha-positive macrophages invading the non-necrotic muscle fiber were observed only in PM but not in DM. TNF-alpha was more highly expressed in PM and DM than was previously thought, and it was suggested that TNF-alpha plays a role in muscle fiber degeneration in PM.


Subject(s)
Dermatomyositis/pathology , Muscle, Skeletal/pathology , Tumor Necrosis Factor-alpha/genetics , Aged , Biopsy , Dermatomyositis/physiopathology , Female , Gene Expression , Humans , In Situ Hybridization , Male , Middle Aged , Muscle Fibers, Skeletal/pathology , Muscle, Skeletal/physiology , RNA, Messenger/analysis
16.
Muscle Nerve ; 23(3): 385-92, 2000 Mar.
Article in English | MEDLINE | ID: mdl-10679715

ABSTRACT

Polymyositis (PM) is an autoimmune inflammatory muscle disease of unknown cause in which cellular immunity is thought to play an important pathogenic role. Class II major histocompatibility complex (class II MHC: human leukocyte antigen (HLA)-DR operates as a cofactor of antigen presentation in immunological responses. There has been a major debate over whether muscle fibers themselves synthesize and express HLA-DR molecules and play a role in antigen presentation in PM pathogenesis. In this study, we demonstrated that most muscle fibers from patients with PM synthesized and expressed HLA-DR molecules on their surface. Human leukocyte antigen-DR expression was highly specific to PM. In addition, class II transactivator (CIITA), human leukocyte antigen DM (HLA-DM), and invariant chain (Ii), which are indispensable for expression of mature HLA-DR molecules and for antigen processing and presentation, were co-expressed. One of the cytokines that could induce this expression is interferon-gamma (IFN-gamma), released by activated lymphocytes. Our results indicate that in PM muscle fibers synthesize and express HLA-DR molecules and may contribute to the inflammatory responses together with lymphocytes.


Subject(s)
HLA-DR Antigens/genetics , Muscle Fibers, Skeletal/chemistry , Polymyositis/immunology , Adult , Aged , Antibodies , Biopsy , DNA Primers , DNA, Complementary , Female , Gene Expression/immunology , HLA-DR Antigens/analysis , HLA-DR Antigens/immunology , Humans , Immunoenzyme Techniques , Lymphocytes/immunology , Male , Middle Aged , Muscle Fibers, Skeletal/immunology , Muscle Fibers, Skeletal/pathology , Muscle, Skeletal/chemistry , Muscle, Skeletal/immunology , Muscle, Skeletal/pathology , Polymyositis/pathology , RNA, Messenger/analysis , Reverse Transcriptase Polymerase Chain Reaction
17.
Rinsho Shinkeigaku ; 40(7): 741-5, 2000 Jul.
Article in Japanese | MEDLINE | ID: mdl-11186916

ABSTRACT

A putative new type of familial parkinsonism with peculiar putaminal changes in MRI was reported. The pedigree was cousin marriage, and three out of four siblings developed parkinsonism in their 2nd or 3rd decade. Their clinical signs were saccadic eye movement, dysarthria, rigidity, bradykinesia and postural instability. These symptoms partially responded to levodopa therapy and showed mild progression. There was no diurnal fluctuation of the symptoms or alleviation after sleep. Lack of Parkin gene mutation and normal beta-galactosidase activities was observed. The cranial MRI study disclosed putaminal increased signal intensities in T2-weighted and proton density images. The severity of these finding correlated with the severity of the symptoms. Familial parkinsonism with MRI findings similar to this pedigree has not been reported in the literature. It is suggested that the present pedigree could be classified as a new subgroup of familial parkinsonism.


Subject(s)
Magnetic Resonance Imaging , Parkinsonian Disorders/diagnosis , Putamen/pathology , Female , Humans , Male , Middle Aged , Nuclear Family , Parkinsonian Disorders/genetics , Parkinsonian Disorders/pathology , Pedigree , Severity of Illness Index
18.
No To Shinkei ; 51(7): 621-6, 1999 Jul.
Article in Japanese | MEDLINE | ID: mdl-10457950

ABSTRACT

We evaluated cranial CT findings of 160 patients with various type of progressive muscular dystrophy (PMD). Significant brain atrophy was observed in 21 out of 63 cases of Duchenne muscular dystrophy (DMD), 7 out of 15 Becker muscular dystrophy (BMD), no case of 2 female dystrophinopathy (F-dyst), 11 out of 21 limb-girdle muscular dystrophy (LG), all cases of 10 Fukuyama type congenital muscular dystrophy (FCMD), 2 out of 5 fascioscapulohumeral muscular dystrophy (FSH), and 32 out of 44 myotonic dystrophy (MyD). Genetical degenerative process and vascular insufficiency seemed to cause brain atrophy in these disease. The intracranial calcification was observed in one DMD, one LG and seven MyD. One LG patient showed focal atrophy in left temporal lobe, and one MyD demonstrated right temporal meningioma. The trace of cerebral vascular accident was disclosed in eleven patients with PMD (1 DMD, 2 BMD, 1 F-dyst, 2 LG, 5 MyD). In these cases, 2 patients had dilated cardiomyopathy, 6 patients with decreased left ventricular ejection fraction, 3 with atrial fibrillation, 1 with cardiac arrest followed by pacemaker instillation, 1 with Adam-Stokes attack, and 3 with 1 degree AV-block. Diffuse low density in the white matter was seen in a patient with F-dyst, a FCMD patient, and 8 MyD patients. Cardiac emobolism, severe arrythmia, cardiogenic shock and hemodynamic disorder were seemed to cause cerebral vascular disease in PMD.


Subject(s)
Brain/diagnostic imaging , Cerebrovascular Disorders/etiology , Heart Diseases/etiology , Muscular Dystrophies/diagnostic imaging , Adult , Cardiomyopathy, Dilated/etiology , Female , Humans , Male , Middle Aged , Muscular Dystrophies/complications , Radiography
19.
Rinsho Shinkeigaku ; 39(2-3): 356-9, 1999.
Article in Japanese | MEDLINE | ID: mdl-10391081

ABSTRACT

A patient with polymyositis manifesting severe myocardial damage and conduction block is described. A 57-year-old man presented dysarthria, dysphagia, proximal-dominant muscle weakness and wasting of the extremities. Muscle biopsy revealed degeneration and regeneration of muscle fibers and infiltration of mononuclear cells. After admission, muscle weakness rapidly progressed and mechanical ventilation was needed for respiratory failure. Simultaneously, cardiac symptom developed and resulted in bradycardia and trifascicular conduction block, which required a pacemaker. Echocardiogram revealed diffuse hypokinesia, ventricular enlargement and thickened wall. Marked elevations of serum CK-MB, cardiac myosin light chain I and cardiac troponin T were observed. High dose administration of methylprednisolone resulted in improvement of muscular and cardiac symptoms, and prevented complete heart block. Immediate and high dose of steroid therapy was considered to be effective for severe myocarditis in polymyositis.


Subject(s)
Cardiomyopathies/etiology , Heart Block/etiology , Polymyositis/complications , Cardiomyopathies/drug therapy , Heart Block/therapy , Humans , Male , Methylprednisolone/administration & dosage , Middle Aged , Pacemaker, Artificial , Polymyositis/drug therapy
20.
Rinsho Shinkeigaku ; 39(11): 1118-24, 1999 Nov.
Article in Japanese | MEDLINE | ID: mdl-10689933

ABSTRACT

The patient was 68 year-old woman with an 8-year history of parkinsonism which was followed by psychiatric symptoms and neurogenic amyotrophy 5 years after the onset. She had a family history of parkinsonism associated with dementia in all of her three siblings. They grew up in Hobara village, a focus of amyotrophic lateral sclerosis (ALS) in the Kii Peninsula of Japan, in their childhood. Their parents were neither consanguineous nor natives of the Kii Peninsula. The brain weight was 1,040 g, and there were mild frontal lobe atrophy, moderate atrophy of pes hippocampi, decoloration of the substantia nigra and locus ceruleus, and anterior spinal root atrophy. The microscopic examinations revealed degeneration of CA 1 portion of the hippocampus to parahippocampal gyrus, substantia nigra, locus ceruleus and spinal anterior horn with Bunina body. The spinal pyramidal tracts also degenerated mildly. The neurofibrillary tangles (NFT) were observed in the cerebral cortex, especially in the cortices through hippocampus to lateral occipitotemporal gyri, basal nucleus of Meynert, basal ganglia, thalamus, substantia nigra and widespread regions of the central nervous system through the brainstem to the spinal cord including the nucleus of Onufrowicz. In spite of a few amount of the senile plaques in the cerebral cortex and Lewy bodies in the substantia nigra and locus ceruleus, abundant NFT distributed mainly in the third layer of the cerebral cortex, which is the characteristic finding of amyotrophic lateral sclerosis/Parkinsonism-dementia Complex (ALS/PDC) in the island of Guam. Thus this was verified ALS/PDC case outside the Guam island. The high incidence of neurological disease in her siblings and their history of living in the focus region in childhood suggest the genetic factor of ALS/PDC which is sensitive to certain environmental agents in the early stage of the life.


Subject(s)
Amyotrophic Lateral Sclerosis/genetics , Dementia/genetics , Parkinsonian Disorders/genetics , Aged , Amyotrophic Lateral Sclerosis/complications , Amyotrophic Lateral Sclerosis/pathology , Central Nervous System/pathology , Dementia/complications , Dementia/pathology , Environment , Female , Genetic Predisposition to Disease , Humans , Japan , Male , Neurofibrillary Tangles/pathology , Parkinsonian Disorders/complications , Parkinsonian Disorders/pathology
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