RESUMO
AIMS: Axonal aggregates of phosphorylated (p-) transactive response DNA-binding protein 43 kDa (TDP-43) in sporadic amyotrophic lateral sclerosis (sALS) were examined in relation to propagation of the protein in the nervous system. METHODS: Brains and spinal cords of Japanese patients with sALS and control subjects were examined immunohistochemically using formalin-fixed paraffin-embedded specimens with special reference to the topographical distribution, microscopic features, presynaptic aggregates, and correlation between the aggregates in axons and the clinical course. RESULTS: (i) Aggregates of p-TDP-43 were frequently present in axons of the hypoglossal and facial nerve fibres and the spinal anterior horn cells. (ii) Aggregates of p-TDP-43 in the axons showed two characteristic microscopic features - dash-like granuloreticular aggregates (GRAs) and massive aggregates (MAs). (iii) MAs were surrounded by p-neurofilaments, but p-neurofilament immunnoreactivity decreased at the inside of axons with GRAs. (iv) Patients showing MAs and GRAs had a relatively shorter clinical course than patients without the aggregates. (v) Some neurones in the red nucleus in patients were surrounded by synapses containing p- and p-independent (i)-TDP-43, and almost all neurones had lost their nuclear TDP-43 immunoreactivity; 17% of those neurones in the red nucleus also had TDP-43-immunopositive neuronal cytoplasmic inclusions, but no postsynaptic p-TDP-43 deposition was evident. CONCLUSIONS: There are two types of axonal p-TDP-43 aggregates, MAs and GRAs, located predominantly in the facial and hypoglossal nuclei and anterior horn cells. These aggregates may influence the function of neurones, and presynaptic aggregates of the protein induce loss of p-i-TDP-43 in the nuclei of postsynaptic neurones.
Assuntos
Esclerose Lateral Amiotrófica/patologia , Axônios/patologia , Proteínas de Ligação a DNA/metabolismo , Corpos de Inclusão/patologia , Idoso , Idoso de 80 Anos ou mais , Esclerose Lateral Amiotrófica/metabolismo , Povo Asiático , Axônios/metabolismo , Encéfalo/metabolismo , Encéfalo/patologia , Feminino , Humanos , Corpos de Inclusão/metabolismo , Masculino , Pessoa de Meia-Idade , Medula Espinal/metabolismo , Medula Espinal/patologiaRESUMO
A specific isoform of apolipoprotein E has been associated with the accelerated rate of disease expression of sporadic Alzheimer's disease (AD) and late-onset familial AD (FAD). An earlier age at onset has also been demonstrated in familial AD patients with mutations in the amyloid precursor protein (APP) gene (APP717 and APP670/671)13 carrying the APOE epsilon-4 allele compared to those who do not, but not in familial AD patients with APP692 or 693 mutations, or in chromosome 14-linked familial AD patients. Hypothesizing that receptors for apoE-containing lipoproteins act as a potential risk factor for AD, we performed an association study using a polymorphic triplet (CGG) repeat in the gene for the VLDL receptor (VLDL-R), a receptor for apoE-containing lipoproteins. The frequency of the 5-repeat allele was significantly higher in all of the Japanese sporadic AD patients (P < 0.02) than in the Japanese controls. Moreover, the odds ratio was significantly increased in the AD patients homozygous for the 5-repeat allele (OR = 2.1, 95% CI = [1.1-4.2]). Multiple logistic regression analysis reveals that the relative risk conferred by the presence of two copies of the 5-repeat allele and at least one copy of the APOE epsilon-4 allele is 8.7 (95% CI = [2.9-25.8]). Our results suggest that the VLDL-R gene is a susceptibility gene for AD.
Assuntos
Doença de Alzheimer/genética , Cromossomos Humanos Par 14 , Receptores de LDL/genética , Sequências Repetitivas de Ácido Nucleico , Alelos , Doença de Alzheimer/epidemiologia , Apolipoproteínas E/genética , Sequência de Bases , Córtex Cerebral/metabolismo , Primers do DNA , Humanos , Japão , Dados de Sequência Molecular , Razão de Chances , Reação em Cadeia da Polimerase , Polimorfismo Genético , Valores de Referência , Análise de Regressão , Fatores de RiscoRESUMO
Thermoelectric effects have been applied to power generators and temperature sensors that convert waste heat into electricity. The effects, however, have been limited to electrons to occur, and inevitably disappear at low temperatures due to electronic entropy quenching. Here, we report thermoelectric generation caused by nuclear spins in a solid: nuclear-spin Seebeck effect. The sample is a magnetically ordered material MnCO3 having a large nuclear spin (I = 5/2) of 55Mn nuclei and strong hyperfine coupling, with a Pt contact. In the system, we observe low-temperature thermoelectric signals down to 100 mK due to nuclear-spin excitation. Our theoretical calculation in which interfacial Korringa process is taken into consideration quantitatively reproduces the results. The nuclear thermoelectric effect demonstrated here offers a way for exploring thermoelectric science and technologies at ultralow temperatures.
RESUMO
AIMS: Sporadic amyotrophic lateral sclerosis (ALS) is a progressive and invariably fatal disease involving the upper and lower motor neurones of adult humans. Among the neuropathological features of the disease, abnormalities in the protein-synthesizing system in motor neurones of the brainstem and spinal cord, such as a decrease of cytoplasmic RNA and rough endoplasmic reticulum (rER) (chromatolysis), defective editing of the Q/R site of the glutamate receptor subunit GluR2 mRNA, fragmentation of the Golgi apparatus and accumulation of ubiquitinated inclusions and abnormal TdP-43 protein have been reported to be essential for the degeneration. In relation to these features, although the possibility of ER stress has been reported in motor neurones of the brainstem and spinal cord of ALS patients, the rER itself has not been a main target of ultrastructural investigation. METHODS: The present study examined the rER, ultrastructurally and quantitatively in the spinal anterior horn cells (AHCs) of 21 Japanese patients with sporadic ALS and eight Japanese control subjects. RESULTS AND CONCLUSIONS: It was found that: (i) the rER cisternae in AHCs showing central chromatolysis were fragmented, but retained their width and had normally attached ribosomes, and (ii) the rER cisternae in shrunken AHCs were irregularly distended with detachment of the ribosomes, thus suggesting that (iii) ribosomal detachment was related to rER distention.
Assuntos
Esclerose Lateral Amiotrófica/patologia , Células do Corno Anterior/patologia , Retículo Endoplasmático Rugoso/ultraestrutura , Adulto , Idoso , Idoso de 80 Anos ou mais , Análise de Variância , Feminino , Humanos , Imuno-Histoquímica , Masculino , Pessoa de Meia-Idade , Ribossomos/ultraestrutura , Medula Espinal/patologiaRESUMO
We investigated, at both light and ultrastructural levels, the fate of swollen astrocytes and remodeling of neurites connected to disseminated, dying neurons in the ischemic neocortical penumbra. Specimens from left cerebral cortex were cut coronally at the infundibulum and observed by light and electron microscopy. We measured synapses and spines, and the thickness of neuritic trunks in the neuropil on electron microscopy photos. We also determined percent volume of axon terminals and spines by Weibel's point-counting method. Astrocytic swelling gradually subsided from day 4 after the ischemic insult, with increases in cytoplasmic glial fibrils and GFAP-positive astrocytes. Disseminated dying electron-dense neurons were fragmented by invading astrocytic cell processes and accumulated as granular pieces. The number of synapses and spines and total percent volume of axon terminals and spines decreased with an increasing sparsity of synaptic vesicles until day 4. One to 12 weeks after the ischemic insult, these values increased to or exceeded control values, and sprouting and increased synaptic vesicles were seen. Axons that had been attached to the dying neurons appeared to have shifted their connections to the spines and the neurites of the surviving neurons, increasing their thickness. Astrocytic restitution and neuronal remodeling processes started at 4 days continuing until 12 weeks after ischemic insult.
Assuntos
Astrócitos/ultraestrutura , Axônios/ultraestrutura , Edema Encefálico/patologia , Isquemia Encefálica/patologia , Córtex Cerebral/ultraestrutura , Neuritos/ultraestrutura , Traumatismo por Reperfusão/patologia , Animais , Apoptose , Edema Encefálico/etiologia , Isquemia Encefálica/complicações , Células Cultivadas , Córtex Cerebral/irrigação sanguínea , Gerbillinae , Regeneração Nervosa , Neurônios/ultraestrutura , Traumatismo por Reperfusão/complicaçõesRESUMO
Disease-specific findings in the substantia nigra were examined in cases of progressive supranuclear palsy (PSP), corticobasal degeneration (CBD), and parkinsonism-dementia complex of Guam (PDC); diseases in which the patients exhibit dementia and parkinsonism, with neurofibrillary tangles (NFTs) and glial tangles composed of hyperphosphorylated tau. Loss of pigmented neurons was extremely severe in these 3 diseases, and decrease of the nonpigmented neurons was severe in PSP and CBD. On the other hand, in PDC the decrease of the nonpigmented neurons was different in each patient. Topographically, in PSP the nonpigmented neurons were particularly depleted in the ventral part and relative preservation of the pigmented neurons was observed in the medial part at the level examined. Many NFTs were observed in PDC. Although the number of NFTs was small, many pretangles were seen in the neurons in CBD. Granular and hazy astrocytic inclusions were identified exclusively in PDC. Numerous argyrophilic neuropile threads were identified in CBD and PSP, but these were few in PDC. Many foamy spheroid bodies as well as coiled bodies were observed in PSP and CBD, but only a few were observed in PDC. In conclusion, PDC is a disease that is distinctly different from PSP and CBD. It is possible to differentiate between PSP and CBD by the occurrence of many pretangles in CBD, but some similarities between these 2 diseases indicate the existence of common pathological mechanisms.
Assuntos
Doenças dos Gânglios da Base/patologia , Demência/patologia , Doenças Neurodegenerativas/patologia , Substância Negra/patologia , Adulto , Idoso , Idoso de 80 Anos ou mais , Contagem de Células , Feminino , Guam/etnologia , Humanos , Japão/etnologia , Masculino , Pessoa de Meia-Idade , Emaranhados Neurofibrilares/patologia , Neurônios/patologia , Doença de Parkinson/patologia , Paralisia Supranuclear Progressiva/patologia , SíndromeRESUMO
We investigated by immunohistochemistry the deposition of alpha-synuclein in the brains of deceased patients with the parkinsonism-dementia complex (PDC) of Guam. Five of 13 PDC brains showed numerous alpha-synuclein positive neuronal inclusions and abnormal neurites, chiefly in the amygdala. Similar alpha-synuclein positive lesions were observed, although to a lesser extent, in the entorhinal cortex and the dorsal vagal nucleus. No alpha-synuclein positive inclusions were observed in motor cortex or locus coeruleus, and only a small number of positive inclusions were found in the Sommer's sector, temporal cortex, or substantia nigra. Some of the alpha-synuclein positive inclusions were reminiscent of cortical Lewy bodies (LB), but many of those in the amygdala coexisted with tau-positive pretangles and/or neurofibrillary tangles (NFT) within the same neurons. In these neurons, tau-positive shells encapsulated alpha-synuclein positive central cores or irregularly shaped alpha-synuclein-positive deposition intermingled with pretangles/NFT. Thus, the present study suggests that a common mechanism may govern aggregation of alpha-synuclein and tau in the amygdala, and that aggregation of alpha-synuclein may play some role in the neurodegenerative process of a tauopathy (i.e. PDC) in which Abeta deposition is virtually absent.
Assuntos
Tonsila do Cerebelo/patologia , Esclerose Lateral Amiotrófica/patologia , Demência/patologia , Corpos de Inclusão/química , Proteínas do Tecido Nervoso/análise , Transtornos Parkinsonianos/patologia , Adulto , Idoso , Tonsila do Cerebelo/química , Imunofluorescência , Guam , Humanos , Corpos de Inclusão/patologia , Pessoa de Meia-Idade , Placa Amiloide/química , Placa Amiloide/patologia , Sinucleínas , alfa-Sinucleína , Proteínas tau/análiseRESUMO
We examined 50 patients with parkinsonism-dementia complex of Guam (Guam PDC), 10 Guamanian patients with amyotrophic lateral sclerosis (ALS), 5 patients with combined PDC and ALS (PDC-ALS), and 20 non-PDC non-ALS Guamanians, who had been autopsied between 1979 and 1982, paying special attention to glial inclusions. Gallyas-positive and tau-immunopositive intracytoplasmic inclusions were observed in many of the glial cells, in addition to extensive neurofibrillary tangles (NFTs) in the brains of Guam PDC and PDC-ALS patients. Granular hazy inclusions were seen in the astrocytes, and some crescent/coiled inclusions were observed in the oligodendroglia. Many granular hazy inclusions were observed in the amygdaloid nucleus, inferior olivary nucleus, and lateral funiculus of the spinal cord. The crescent/coiled inclusions were observed predominantly in the anterior nucleus of the thalamus, motor cortex, midbrain tegmentum, pyramids of the medulla oblongata, and lateral funiculus of the spinal cord. The granular hazy inclusions have never been reported previously, and the topographic distribution of the crescent/coiled inclusions in Guam PDC and PDC-ALS differs from those reported previously in other NFT-forming diseases. These findings indicate that Guam PDC and PDC-ALS involve not only neurons but also glia, and that their morphological and topographic differences from other NFT-forming diseases may provide further insights into their distinct etiopathogenesis, and thus prove useful for diagnosis.
Assuntos
Esclerose Lateral Amiotrófica/patologia , Demência/patologia , Neuroglia/patologia , Doença de Parkinson/patologia , Adulto , Idoso , Idoso de 80 Anos ou mais , Feminino , Guam , Humanos , Masculino , Pessoa de Meia-IdadeRESUMO
Neurodegenerative diseases are characterized by neuronal degeneration of specific neurons, e.g., degeneration of motoneurons in amyotrophic lateral sclerosis. As an approach to understand molecular mechanisms of neuronal degeneration of human spinal cord motoneurons in various motor neuron diseases, we have constructed a human spinal cord cDNA library and developed a strategy for isolating spinal cord-specific genes by subtractive cloning. We constructed human spinal cord and brain cDNA libraries from postmortem human spinal cord and brain. To isolate human spinal cord-specific cDNAs, a spinal cord-enriched [32P]cDNA probe was generated by the phenol emulsion reassociation technique. Forty-eight cDNA clones out of 10,000 colonies gave strong signals with the subtracted probe, and individual spinal cord cDNA clones were isolated. Northern blotting analysis confirmed that two spinal cord cDNA clones are, in fact, more abundant in spinal cord compared to brain.
Assuntos
Clonagem Molecular/métodos , DNA/genética , Biblioteca Gênica , Neurônios Motores , Doenças Neuromusculares/genética , Poli A/genética , RNA Mensageiro/genética , Medula Espinal/química , Northern Blotting , Química Encefálica , Sondas de DNA , Regulação da Expressão Gênica , Humanos , Especificidade de Órgãos , Poli A/isolamento & purificação , RNA Mensageiro/isolamento & purificaçãoRESUMO
A Leu148Phe substitution of the ornithine transcarbamylase (OTC) gene was identified in a 2-year-old girl with OTC deficiency (14% of control). Her two elder sisters died in childhood of hyperammonemia, and the patient also died of OTC deficiency. Enzyme activity in Cos1 cells transfected by the mutant cDNA was undetectable, thereby indicating a definite pathogenic mutation. Familial gene analysis showed that the mother had wild-type OTC alleles on both X-chromosomes and the father was a mosaic for the mutant allele in his lymphocytes and spermatozoa. This clinical case shows that a somatic and germline mosaicism for a single-gene disorder led to an unusual pattern of X-linked inheritance in the family, and all three daughters in the family died of OTC deficiency. The possibility that inherited factors will lead to skewed X-inactivation needs to be considered.
Assuntos
Ligação Genética , Mosaicismo/genética , Doença da Deficiência de Ornitina Carbomoiltransferase , Cromossomo X , Evolução Fatal , Feminino , Heterozigoto , Humanos , Lactente , Ornitina Carbamoiltransferase/genética , Linhagem , Mutação Puntual , Reação em Cadeia da Polimerase , Análise de Sequência de DNARESUMO
We analyzed neurological data, including DQ or IQ, EEG, and CT scan, in 49 patients with urea cycle enzymopathies, all of whom were included in a retrospective survey from 1978-1988 in Japan. We classified 3 groups depending on age-at-onset: group 1 (0-28 days, N = 11), group 2 (29 days-5 years, N = 31), and group 3 (greater than 5 years, N = 7). The least DQ or IQ score and the highest CT score, representing the most severe brain damage was found in group 1, and the highest DQ or IQ and the least CT score was found in group 3. Intermediate scores of both parameters were found in group 2. There was a negative correlation between these 2 parameters (r = -0.82, P less than 0.01). Abnormal EEG during the attack-free period was predominantly observed in patients with CT abnormalities compared to those with a normal CT scan (P less than 0.01). Approximately 40% of the patients, mostly in groups 2 and 3 (92.8%) had normal findings in all 3 parameters. Thus, the magnitude of developmental abnormalities is clearly related to the degree of brain damage and to the age-at-onset of these diseases.
Assuntos
Erros Inatos do Metabolismo/complicações , Doenças do Sistema Nervoso/etiologia , Ureia/metabolismo , Encéfalo/diagnóstico por imagem , Pré-Escolar , Eletroencefalografia , Enzimas/deficiência , Humanos , Lactente , Recém-Nascido , Inteligência , Erros Inatos do Metabolismo/enzimologia , Doenças do Sistema Nervoso/diagnóstico , Doenças do Sistema Nervoso/enzimologia , Estudos Retrospectivos , Tomografia Computadorizada por Raios XRESUMO
In a retrospective survey done from 1978-1988 in Japan, 32 male patients with ornithine transcarbamylase (OTC) deficiency were identified. We classified a neonatal and 2 late-onset groups, depending on clinical manifestations and the age at onset; group 1 (0-28 days; N = 10), group 2 (29 days-5 years; N = 13), and group 3 (greater than 5 years; N = 9). Compared to findings in the group 2 patients, there was a higher rate of mortality and a higher incidence of mental retardation in association with a great decrease in enzyme activity in group 1. In group 3, the mortality rate and enzyme activities were similar to those in group 1. However, patients in this group were asymptomatic prior to the first episode. Enzyme activities were measured mostly in autopsy samples. The serum citrulline levels (enzyme product) were highest in this group. Thus, the mutant enzymes were apparently labile with greater activities in vivo than in vitro. Treatments, including a protein-restricted diet, arginine supplementation, and sodium benzoate administration, resulted in a favorable prognosis for survivors with partial enzyme deficiency. We wish to emphasize that the incidence of late onset of this disease is higher than heretofore considered.
Assuntos
Doença da Deficiência de Ornitina Carbomoiltransferase , Ureia/metabolismo , Pré-Escolar , Humanos , Lactente , Recém-Nascido , Japão/epidemiologia , Masculino , Mortalidade , Ornitina Carbamoiltransferase/genética , Estudos Retrospectivos , Inquéritos e QuestionáriosRESUMO
A case of a severe and fatal form of arthrogryposis multiplex congenita with a full necropsy examination is presented in which the central nervous system and many muscles of the four extremities were examined histologically. The most striking feature was a great reduction in the muscular tissue of the limbs with a marked increase in the adipose tissue. The muscular changes were thought to be caused by neurogenic atrophy, and microscopy of the spinal cord revealed developmental abnormalities, including degenerative changes of the anterior horn cells. Neonatal and fatal cases of arthrogryposis multiplex congenita reported in the literature are briefly reviewed, and the characteristics of the present case and its relation to other congenital neuromuscular disorders are discussed.
Assuntos
Artrogripose/diagnóstico , Doenças do Prematuro/diagnóstico , Tecido Adiposo/patologia , Artrogripose/congênito , Artrogripose/mortalidade , Encéfalo/patologia , Feminino , Humanos , Recém-Nascido , Doenças do Prematuro/mortalidade , Músculos/patologia , Medula Espinal/patologiaRESUMO
We studied histopathologic findings from the retina and optic nerve of a patient with the late-infantile type of galactosialidosis and related them to clinical features of the condition. Markedly fewer ganglion cells were evident histopathologically using light microscopy. Results of histochemical studies demonstrated abnormal accumulation of lipid and proteinaceous material in the residual swollen ganglion cells. Marked loss of myelinated nerve fibers and thickening of the pial septum were also observed in the optic nerve. Both retinal ganglion cells and amacrine cells had intracytoplasmic inclusion bodies, but none were found in the optic nerve. These findings suggested that optic atrophy was induced by axonal wallerian degeneration secondary to retinal ganglion cell death. Although the fundus showed advanced optic nerve atrophy, a cherry red spot was not evident, possibly because of the marked decrease in ganglion cells in this case.
Assuntos
Gangliosidoses/patologia , Doenças do Nervo Óptico/patologia , Nervo Óptico/ultraestrutura , Retina/ultraestrutura , Doenças Retinianas/patologia , Adolescente , Histocitoquímica , Humanos , Masculino , Pessoa de Meia-Idade , Células Ganglionares da Retina/ultraestruturaRESUMO
An acid sialidase [EC 3.2.1.18], partially purified from human placenta by Con A-Sepharose adsorption and p-aminophenyl thio-beta-D-galactoside-CH-Sepharose (PATG-Sepharose) affinity chromatographies, was activated by incubation at 37 degrees C. This activation showed both time and temperature dependencies, with the most effective activation observed at 37 degrees C in the pH range between 4.3 and 5.2. The influence of various protease inhibitors on its activation was investigated. Among the protease inhibitors tested, amastatin, an inhibitor of aminopeptidase A, significantly inhibited activation. The partially purified enzyme preparation contained aminopeptidase activity, which was inhibited by amastatin. Zinc ions inhibited either the activation of sialidase or the aminopeptidase activity in the enzyme preparation. These results suggest the possibility of participation of aminopeptidase function in the activation process of sialidase.
Assuntos
Lisossomos/enzimologia , Neuraminidase/química , Aminopeptidases/metabolismo , Animais , Bovinos , Galinhas , Ativação Enzimática/efeitos dos fármacos , Glicoproteínas/análise , Humanos , Concentração de Íons de Hidrogênio , Fígado/enzimologia , Metais/farmacologia , Neuraminidase/isolamento & purificação , Inibidores de Proteases/farmacologia , Coelhos , RatosRESUMO
We examined neuroprotective effects of an adenoviral vector encoding glial cell line-derived neurotrophic factor (AxCAhGDNF) on the lesioned adult rat facial motoneurons. After facial nerve avulsion, animals locally injected into the facial canal with AxCALacZ (adenovirus encoding beta-galactosidase gene) or AxCAhGDNF showed expression of beta-galactosidase activity or intense immunolabeling for GDNF in lesioned facial motoneurons, respectively. The treatment with AxCAhGDNF after avulsion significantly prevented the loss of lesioned facial motoneurons, ameliorated choline acetyltransferase immunoreactivity, and suppressed the activity of nitric oxide synthase in these neurons. These results indicate that the adenovirus-mediated gene transfer of GDNF may prevent the degeneration of motoneurons in adult humans with peripheral nerve injury and motor neuron diseases.
Assuntos
Adenoviridae/genética , Nervo Facial/fisiopatologia , Técnicas de Transferência de Genes , Vetores Genéticos , Neurônios Motores/fisiologia , Fatores de Crescimento Neural , Proteínas do Tecido Nervoso/genética , Animais , Sobrevivência Celular/fisiologia , Colina O-Acetiltransferase/metabolismo , Nervo Facial/patologia , Traumatismos do Nervo Facial/patologia , Traumatismos do Nervo Facial/fisiopatologia , Fator Neurotrófico Derivado de Linhagem de Célula Glial , Masculino , Neurônios Motores/enzimologia , Óxido Nítrico Sintase/metabolismo , Óxido Nítrico Sintase Tipo I , Ratos , Ratos Endogâmicos F344RESUMO
In order to evaluate the quantitative changes in the neostriatum in progressive supranuclear palsy (PSP), sections of the caudate head (CN) and putamen (PT) from 4 PSP patients were stained with Klüver-Barrera, and the cell body and nuclear area of the neurons were measured by a digitizer. Obtained results were compared to those of 6 age-matched control and 4 Alzheimer's disease and senile dementia of Alzheimer type (AD/SDAT) subjects which were previously reported. The number of large neurons (nuclear area greater than 101 microns 2) in PSP was about 40% (P less than 0.01) and 30% (P less than 0.01) of that of the controls in CN and PT, respectively. In contrast, the number of small neurons in PSP (nuclear area less than 100 microns 2) was well preserved. The values were quite similar to those in AD/SDAT. The implications and possible significance are discussed.
Assuntos
Núcleo Caudado/patologia , Neurônios/citologia , Putamen/patologia , Paralisia Supranuclear Progressiva/patologia , Idoso , Humanos , Pessoa de Meia-Idade , Valores de Referência , Coloração e RotulagemRESUMO
In order to evaluate the quantitative changes in the neostriatum of Alzheimer type (SDAT), sections of the caudate head (CN) and putamen (PT) from 4 AD/SDAT and 6 age-matched control cases were stained with Klüver-Barrera, and the cell body and nuclear areas of the neurons were measured by a digitizer. This study revealed a significant decrease in the number of large neurons (nuclear area; greater than 101 micron 2) and good preservation of the number of small neurons (nuclear area; less than 100 micron 2) in CN and PT of AD/SDAT.
Assuntos
Doença de Alzheimer/patologia , Núcleo Caudado/patologia , Demência/patologia , Neurônios/patologia , Putamen/patologia , Idoso , Contagem de Células , Humanos , Pessoa de Meia-Idade , Neurônios/classificaçãoRESUMO
A quantitative investigation was performed on the large neurons in the neostriatum and basal nucleus of Meynert (bnM) in patients with Alzheimer's disease (AD) and progressive supranuclear palsy (PSP). The degree of decrease of the large neurons in the neostriatum was quite similar to that in the bnM; these decreases were significantly correlative in AD, but not in PSP. These findings indicate that the large neurons in the neostriatum and bnM, which are considered to be cholinergic and to exclusively possess nerve growth factor receptors in the cerebrum, degenerate simultaneously in an equal ratio in AD.