RESUMO
A 44-year-old woman was admitted to our hospital due to dizziness and ataxia of the trunk and right upper limb. Brain MRI revealed an acute infarct lesion in the right posterior inferior cerebellar artery territory. In addition to the cognitive deterioration observed in the subacute phase, a change was noted in her food preference-from light-tasting, low-caloric Japanese cuisine, sugarless coffee, and hot drinks to strong-tasting, high-caloric Western cuisine, sugar-rich coffee, and iced drinks. Single-photon emission computed tomography showed hypoperfusion in the bilateral frontal lobes and right cerebellum. These cognitive and food preference-related changes were gradually restored over six months after the onset. The reduced cerebral blood flow in the bilateral frontal lobes also restored along with the clinical improvement, with the maximal changes in the bilateral subcallosal areas. This case suggests that changes in food preference can occur as a symptom of cerebellar infarction, possibly by the mechanism similar to cerebellar cognitive affective syndrome.
Assuntos
Isquemia Encefálica , Doenças Cerebelares , Humanos , Feminino , Adulto , Infarto Cerebral/complicações , Infarto Cerebral/diagnóstico por imagem , Preferências Alimentares , Café , Doenças Cerebelares/diagnóstico , Doenças Cerebelares/patologia , Isquemia Encefálica/patologia , Cerebelo/patologia , AçúcaresRESUMO
Cadmium (Cd) is one of the toxic environmental heavy metals that poses health hazard to animals due to its toxicity. Nano-Selenium (Nano-Se) is a Nano-composite form of Se, which has emerged as a promising therapeutic agent for its protective roles against heavy metals-induced toxicity. Heat shock proteins (HSPs) play a critical role in cellular homeostasis. However, the potential protective effects of Nano-Se against Cd-induced cerebellar toxicity remain to be illustrated. To investigate the toxic effects of Cd on chicken's cerebellum, and the protective effects of Nano-Se against Cd-induced cerebellar toxicity, a total of 80 male chicks were divided into four groups and treated as follows: (A) 0 mg/kg Cd, (B) 1 mg/kg Nano-Se (C) 140 mg/kg Cd + 1 mg/kg Nano-Se (D) 140 mg/kg Cd for 90 days. We tested heat shock protein pathway-related factors including heat shock factors (HSFs) HSF1, HSF2, HSF3 and heat shock proteins (HSPs) HSP10, HSP25, HSP27, HSP40, HSP60, HSP70 and HSP90 expressions. Histopathological results showed that Cd treatment caused degradation of Purkinje cells. In addition, HSFs and HSPs expression decreased significantly in the Cd group. Nano-Se co-treatment with Cd enhanced the expression of HSFs and HSPs. In summary, our findings explicated a potential protective effect of Nano-Se against Cd-induced cerebellar injury in chicken, suggesting that Nano-Se is a promising therapeutic agent for the treatment of Cd toxicity.
Assuntos
Cádmio/toxicidade , Doenças Cerebelares/tratamento farmacológico , Proteínas de Choque Térmico/metabolismo , Nanocompostos/química , Fármacos Neuroprotetores/uso terapêutico , Selênio/uso terapêutico , Animais , Doenças Cerebelares/induzido quimicamente , Doenças Cerebelares/patologia , Galinhas , Masculino , Fármacos Neuroprotetores/química , Células de Purkinje/efeitos dos fármacos , Células de Purkinje/patologia , Selênio/químicaRESUMO
Vigabatrin is the drug of choice in resistant epilepsy and infantile spasms. Ataxia, tremors, and abnormal gait have been frequently reported following its use indicating cerebellar involvement. This study aimed, for the first time, to investigate the involvement of necroptosis and apoptosis in the VG-induced cerebellar cell loss and the possible protective role of combined omega-3 and vitamin B12 supplementation. Fifty Sprague-Dawley adult male rats (160-200 g) were divided into equal five groups: the control group received normal saline, VG200 and VG400 groups received VG (200 mg or 400 mg/kg, respectively), VG200 + OB and VG400 + OB groups received combined VG (200 mg or 400 mg/kg, respectively), vitamin B12 (1 mg/kg), and omega-3 (1 g/kg). All medications were given daily by gavage for four weeks. Histopathological changes were examined in H&E and luxol fast blue (LFB) stained sections. Immunohistochemical staining for caspase-3 and receptor-interacting serine/threonine-protein kinase-1 (RIPK1) as well as quantitative real-time polymerase chain reaction (qRT-PCR) for myelin basic protein (MBP), caspase-3, and receptor-interacting serine/threonine-protein kinase-3 (RIPK3) genes were performed. VG caused a decrease in the granular layer thickness and Purkinje cell number, vacuolations, demyelination, suppression of MBP gene expression, and induction of caspases-3, RIPK1, and RIPK3 in a dose-related manner. Combined supplementation with B12 and omega-3 improved the cerebellar histology, increased MBP, and decreased apoptotic and necroptotic markers. In conclusion, VG-induced neuronal cell loss is dose-dependent and related to both apoptosis and necroptosis. This could either be ameliorated (in low-dose VG) or reduced (in high-dose VG) by combined supplementation with B12 and omega-3.
Assuntos
Anticonvulsivantes/efeitos adversos , Caspase 3/metabolismo , Doenças Cerebelares/induzido quimicamente , Proteína Serina-Treonina Quinases de Interação com Receptores/metabolismo , Vigabatrina/efeitos adversos , Animais , Apoptose , Caspase 3/genética , Doenças Cerebelares/tratamento farmacológico , Doenças Cerebelares/metabolismo , Doenças Cerebelares/patologia , Relação Dose-Resposta a Droga , Ácidos Graxos Ômega-3/administração & dosagem , Regulação da Expressão Gênica/fisiologia , Masculino , Proteína Básica da Mielina/genética , Necroptose , RNA Mensageiro/genética , Ratos , Ratos Sprague-Dawley , Reação em Cadeia da Polimerase em Tempo Real , Proteína Serina-Treonina Quinases de Interação com Receptores/genética , Vitamina B 12/administração & dosagemAssuntos
Edema Encefálico/induzido quimicamente , Doenças Cerebelares/induzido quimicamente , Suplementos Nutricionais/efeitos adversos , Síndromes Neurotóxicas/etiologia , Ataxia/induzido quimicamente , Edema Encefálico/diagnóstico por imagem , Edema Encefálico/patologia , Ataxia Cerebelar/induzido quimicamente , Doenças Cerebelares/diagnóstico por imagem , Doenças Cerebelares/patologia , Diabetes Mellitus Tipo 2/complicações , Disartria/induzido quimicamente , Humanos , Cirrose Hepática/complicações , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Síndromes Neurotóxicas/diagnóstico por imagem , Síndromes Neurotóxicas/patologia , Hepatopatia Gordurosa não Alcoólica/complicações , Nistagmo Patológico/induzido quimicamente , Vertigem/induzido quimicamenteRESUMO
A widely held view suggests that homocysteine (Hcy) can contribute to neurodegeneration through promotion of oxidative stress. There is evidence that homocysteine is toxic to cerebellar Purkinje neurons in vitro; however, in vivo action of Hcy on Purkinje cell has not been investigated so far. Thus, this study was designed to evaluate the Hcy effects on neonatal rat cerebellum and cerebellar oxidative stress. We also evaluated the folic acid effects on biochemical alterations elicited by hyperhomocysteinemia (hHcy) in the cerebellum. Group I received normal saline, group II received Hcy subcutaneously twice a day at 8-h intervals (0.3-0.6 µmol/g body weight), group III received Hcy + folic acid (0.011 µmol/g body weight), and group IV received folic acid on postnatal day (PD) 4 until 25. On day 25, superoxide dismutase (SOD) and glutathione peroxidase (GPx) activities in the cerebellum and motor cortex were assayed. Malondialdehyde (MDA) levels were also evaluated as a marker of lipid peroxidation. Rotarod and locomotor activity tests were performed in PD 25-27. Our results indicated that administration of Hcy increased plasma, cortical, and cerebellar total Hcy levels; reduced GPx activity; and induced lipid peroxidation in the cerebellum. Hcy impaired performance on the rotarod in rats. However, treatment with folic acid significantly attenuated motor coordination impairment, GPx activity reduction, the lipid peroxidation process, and significantly reduced plasma total Hcy levels. Histological analysis indicated that Hcy could decrease Purkinje cell count and folic acid prevented this toxic effect. We conclude that Hcy can induce neurotoxicity and folic acid has neuroprotective effects against cerebellar Hcy toxicity.
Assuntos
Cerebelo/efeitos dos fármacos , Cerebelo/crescimento & desenvolvimento , Ácido Fólico/farmacologia , Homocisteína/efeitos adversos , Fármacos Neuroprotetores/farmacologia , Animais , Antioxidantes/farmacologia , Doenças Cerebelares/induzido quimicamente , Doenças Cerebelares/tratamento farmacológico , Doenças Cerebelares/metabolismo , Doenças Cerebelares/patologia , Cerebelo/metabolismo , Cerebelo/patologia , Ácido Fólico/sangue , Homocisteína/sangue , Peroxidação de Lipídeos/efeitos dos fármacos , Peroxidação de Lipídeos/fisiologia , Masculino , Córtex Motor/efeitos dos fármacos , Córtex Motor/crescimento & desenvolvimento , Córtex Motor/metabolismo , Córtex Motor/patologia , Doenças Neurodegenerativas/induzido quimicamente , Doenças Neurodegenerativas/tratamento farmacológico , Doenças Neurodegenerativas/metabolismo , Doenças Neurodegenerativas/patologia , Fármacos Neuroprotetores/sangue , Estresse Oxidativo/efeitos dos fármacos , Estresse Oxidativo/fisiologia , Distribuição Aleatória , Ratos WistarRESUMO
ETHNOPHARMACOLOGICAL RELEVANCE: The development of compounds able to improve metabolic syndrome and mitigate complications caused by inappropriate glycemic control in type 1 diabetes mellitus is challenging. The medicinal plant with established hypoglycemic properties Garcinia kola Heckel might have the potential to mitigate diabetes mellitus metabolic syndrome and complications. AIM OF THE STUDY: We have investigated the neuroprotective properties of a suspension of G. kola seeds in long-term type 1 diabetes mellitus rat model. MATERIALS AND METHODS: Wistar rats, made diabetic by single injection of streptozotocin were monitored for 8 months. Then, they were administered with distilled water or G. kola oral aqueous suspension daily for 30 days. Body weight and glycemia were determined before and after treatment. After sacrifice, cerebella were dissected out and processed for stereological quantification of Purkinje cells. Histopathological and immunohistochemical analyses of markers of neuroinflammation and neurodegeneration were performed. RESULTS: Purkinje cell counts were significantly increased, and histopathological signs of apoptosis and neuroinflammation decreased, in diabetic animals treated with G. kola compared to diabetic rats given distilled water. Glycemia was also markedly improved and body weight restored to non-diabetic control values, following G. kola treatment. CONCLUSIONS: These results suggest that G. kola treatment improved the general condition of long-term diabetic rats and protected Purkinje cells partly by improving the systemic glycemia and mitigating neuroinflammation.
Assuntos
Doenças Cerebelares/prevenção & controle , Cerebelo/efeitos dos fármacos , Diabetes Mellitus Experimental/tratamento farmacológico , Diabetes Mellitus Tipo 1/tratamento farmacológico , Neuropatias Diabéticas/prevenção & controle , Garcinia kola/química , Hipoglicemiantes/farmacologia , Degeneração Neural , Fármacos Neuroprotetores/farmacologia , Preparações de Plantas/farmacologia , Animais , Apoptose/efeitos dos fármacos , Glicemia/efeitos dos fármacos , Glicemia/metabolismo , Doenças Cerebelares/sangue , Doenças Cerebelares/etiologia , Doenças Cerebelares/patologia , Cerebelo/metabolismo , Cerebelo/patologia , Diabetes Mellitus Experimental/sangue , Diabetes Mellitus Experimental/induzido quimicamente , Diabetes Mellitus Tipo 1/sangue , Diabetes Mellitus Tipo 1/induzido quimicamente , Neuropatias Diabéticas/sangue , Neuropatias Diabéticas/etiologia , Neuropatias Diabéticas/patologia , Hipoglicemiantes/isolamento & purificação , Neuroimunomodulação/efeitos dos fármacos , Fármacos Neuroprotetores/isolamento & purificação , Fitoterapia , Preparações de Plantas/isolamento & purificação , Plantas Medicinais , Células de Purkinje/efeitos dos fármacos , Células de Purkinje/metabolismo , Células de Purkinje/patologia , Ratos Wistar , Estreptozocina , Fatores de Tempo , Fator de Necrose Tumoral alfa/metabolismo , Receptor fas/metabolismoRESUMO
Intoxication with Solanum bonariense in cattle causes cerebellar cortical degeneration with perikaryal vacuolation, axonal swelling, and death primarily of Purkinje cells, with accumulation of electron-dense residual storage bodies in membrane-bound vesicles. The pathogenesis of this disease is not fully understood. Previously, we proposed that inhibition of protein synthesis in Purkinje cells among other altered metabolic pathways could lead to cytoskeletal alterations, subsequently altering cell-specific axonal transport. In the present study, immunohistochemical and histochemical methods were used to identify neuronal cytoskeletal alterations and axonal loss, demyelination, and astrogliosis in the cerebellum of intoxicated bovines. Samples of cerebellum from 3 natural and 4 experimental cases and 2 control bovines were studied. Immunoreactivity against neurofilament (NF)-200KDa confirmed marked loss of Purkinje neurons, and phospho-NF protein, ß-tubulin, and affinity reaction against phalloidin revealed an altered perikaryal distribution of neuronal cytoskeletal proteins in the remaining Purkinje cells in intoxicated cattle. Reactive astrogliosis in every layer of the cerebellar cortex was also observed with anti-glial fibrillary acidic protein immunohistochemistry. In affected cattle, demyelination and axonal loss in the cerebellar white matter, as well as basket cell loss were demonstrated with Klüver-Barrera and Bielschowsky stains, respectively. Based on these results, we propose that neuronal cytoskeletal alterations with subsequent interference of the axonal transport in Purkinje cells may play a relevant role in the pathogenesis of this neurodegenerative disorder, and also that demyelination and axonal loss in the cerebellar white matter, as well as astrogliosis in the gray matter, likely occur secondarily to Purkinje cell degeneration and death.
Assuntos
Doenças dos Bovinos/patologia , Doenças Cerebelares/veterinária , Doenças Neurodegenerativas/veterinária , Plantas Tóxicas , Solanum/toxicidade , Animais , Estudos de Casos e Controles , Bovinos , Doenças Cerebelares/patologia , Feminino , Proteína Glial Fibrilar Ácida/imunologia , Imuno-Histoquímica/veterinária , Masculino , Doenças Neurodegenerativas/patologia , Células de Purkinje/patologiaRESUMO
OBJECTIVES: CNS dengue infection is a rare condition and the pattern of brain involvement has not been well described. We report the MR imaging (MRI) features in eight cases of dengue encephalitis. MATERIALS AND METHODS: We retrospectively searched cases of dengue encephalitis in which imaging was performed. Eight cases (three men, five women; age range: 8-42 years) diagnosed with dengue encephalitis were included in the study. MR studies were performed on 3-T and 1.5-T MR clinical systems. Two neuroradiologists retrospectively reviewed the MR images and analysed the type of lesions, as well as their distribution and imaging features. RESULTS: All eight cases exhibited MRI abnormalities and the cerebellum was involved in all cases. In addition, MRI signal changes were also noted in the brainstem, thalamus, basal ganglia, internal capsule, insula, mesial temporal lobe, and cortical and cerebral white matter. Areas of susceptibility, diffusion restriction, and patchy post-contrast enhancement were the salient imaging features in our cohort of cases. CONCLUSION: A pattern of symmetrical cerebellar involvement and presence of microbleeds/haemorrhage may serve as a useful imaging marker and may help in the diagnosis of dengue encephalitis.
Assuntos
Doenças Cerebelares/patologia , Dengue/patologia , Encefalite por Arbovirus/patologia , Adolescente , Adulto , Gânglios da Base/patologia , Encéfalo/patologia , Tronco Encefálico/patologia , Cerebelo/patologia , Córtex Cerebral/patologia , Hemorragia Cerebral/patologia , Criança , Feminino , Humanos , Cápsula Interna/patologia , Imageamento por Ressonância Magnética , Masculino , Estudos Retrospectivos , Tálamo/patologia , Tomografia Computadorizada por Raios X , Adulto JovemRESUMO
We live in a dynamic and changing environment, which necessitates that we adapt to and efficiently respond to changes of stimulus form ('what') and stimulus occurrence ('when'). Consequently, behaviour is optimal when we can anticipate both the 'what' and 'when' dimensions of a stimulus. For example, to perceive a temporally expected stimulus, a listener needs to establish a fairly precise internal representation of its external temporal structure, a function ascribed to classical sensorimotor areas such as the cerebellum. Here we investigated how patients with cerebellar lesions and healthy matched controls exploit temporal regularity during auditory deviance processing. We expected modulations of the N2b and P3b components of the event-related potential in response to deviant tones, and also a stronger P3b response when deviant tones are embedded in temporally regular compared to irregular tone sequences. We further tested to what degree structural damage to the cerebellar temporal processing system affects the N2b and P3b responses associated with voluntary attention to change detection and the predictive adaptation of a mental model of the environment, respectively. Results revealed that healthy controls and cerebellar patients display an increased N2b response to deviant tones independent of temporal context. However, while healthy controls showed the expected enhanced P3b response to deviant tones in temporally regular sequences, the P3b response in cerebellar patients was significantly smaller in these sequences. The current data provide evidence that structural damage to the cerebellum affects the predictive adaptation to the temporal structure of events and the updating of a mental model of the environment under voluntary attention.
Assuntos
Atenção , Doenças Cerebelares/fisiopatologia , Discriminação Psicológica/fisiologia , Modelos Psicológicos , Reconhecimento Fisiológico de Modelo/fisiologia , Estimulação Acústica , Adulto , Análise de Variância , Doenças Cerebelares/patologia , Eletroencefalografia , Potenciais Evocados/fisiologia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Fatores de TempoRESUMO
Hypomagnesaemia is common among hospitalised patients and is often under-recognised. Chronic alcohol abuse and alcohol withdrawal are known causes for severe hypomagnesaemia. Hypomagnesaemia can present with cardiac arrhythmias, seizures and other neurological symptoms, among which ataxia. We present a 57-year-old man with a history of chronic alcohol abuse who developed a subacute cerebellar syndrome with hypertension after alcohol withdrawal. A severe hypomagnesaemia of 0.19 mmol/L (normal values 0.70-1.10) was found. MRI showed diffuse, T2 hyperintense lesions in and swelling of the cerebellum. Symptoms, hypertension and MRI abnormalities significantly improved rapidly after intravenous magnesium supplementation. Hypomagnesaemia can cause a subacute, cerebellar syndrome and hypertension. Symptoms, hypertension and MRI abnormalities can be reversed with rapid magnesium supplementation. MRI abnormalities are similar to those caused by vascular endothelial dysregulation seen in posterior reversible encephalopathy syndrome (PRES). A similar case was recently described. We confirm that magnesium is likely to be involved in the pathophysiology of PRES.
Assuntos
Doenças Cerebelares/etiologia , Deficiência de Magnésio/complicações , Doenças Cerebelares/patologia , Cerebelo/patologia , Seguimentos , Humanos , Magnésio/administração & dosagem , Deficiência de Magnésio/sangue , Deficiência de Magnésio/patologia , Deficiência de Magnésio/terapia , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Resultado do TratamentoRESUMO
The present work reports cerebellar degeneration in cattle associated with the ingestion of Solanum subinerme in northern Brazil. The main clinical signs were periodic crises with loss of balance, falls, opisthotonus, and nystagmus. The histological lesions consisted of diffuse vacuolation of the perikaryon of the Purkinje neurons, followed by the loss of these cells and their substitution by Bergman glia. It is concluded that S. subinerme is another species of Solanum that causes cerebellar degeneration in cattle.
Assuntos
Doenças dos Bovinos/patologia , Córtex Cerebelar/patologia , Doenças Cerebelares/induzido quimicamente , Doenças Cerebelares/veterinária , Doenças Neurodegenerativas/induzido quimicamente , Doenças Neurodegenerativas/veterinária , Intoxicação por Plantas/patologia , Intoxicação por Plantas/veterinária , Solanum/toxicidade , Animais , Comportamento Animal , Brasil , Bovinos , Doenças Cerebelares/patologia , Feminino , Masculino , Doenças Neurodegenerativas/patologia , Células de Purkinje/patologiaRESUMO
In the context of spina bifida, hydrocephalus is usually caused by crowding of the posterior fossa with obstruction to cerebrospinal fluid flow from the forth ventricle, and less often by malformation of the cerebral aqueduct. Enlargement of the cerebral ventricles causes gradual destruction of periventricular white matter axons. Motor, sensory, visual, and memory systems may be disturbed through involvement of the long projection axons, periventricular structures including the corpus callosum, and the fimbria-fornix pathway. Secondary changes occur in neuronal cell bodies and synapses, but there is minimal death of neurons. The clinical syndrome of hydrocephalic brain dysfunction is thus due to subcortical disconnection. Some of the brain dysfunction is reversible by shunting, probably through restoration of cerebral blood flow and normalization of the extracellular environment. However, destroyed axons cannot be restored.
Assuntos
Encéfalo/patologia , Encéfalo/fisiopatologia , Hidrocefalia/patologia , Hidrocefalia/fisiopatologia , Doenças Cerebelares/epidemiologia , Doenças Cerebelares/patologia , Doenças Cerebelares/fisiopatologia , Ventrículos Cerebrais/patologia , Comorbidade , Humanos , Hidrocefalia/etiologia , Hipotálamo/fisiopatologia , Degeneração Neural/patologia , Rede Nervosa/fisiopatologia , Disrafismo Espinal/complicações , Disrafismo Espinal/patologia , Disrafismo Espinal/fisiopatologiaRESUMO
The cerebellum may monitor motor commands and through internal feedback correct for anticipated errors. Saccades provide a test of this idea because these movements are completed too quickly for sensory feedback to be useful. Earlier, we reported that motor commands that accelerate the eyes toward a constant amplitude target showed variability. Here, we demonstrate that this variability is not random noise, but is due to the cognitive state of the subject. Healthy people showed within-saccade compensation for this variability with commands that arrived later in the same saccade. However, in people with cerebellar damage, the same variability resulted in dysmetria. This ability to correct for variability in the motor commands that initiated a saccade was a predictor of each subject's ability to learn from endpoint errors. In a paradigm in which a target on the horizontal meridian jumped vertically during the saccade (resulting in an endpoint error), the adaptive response exhibited two timescales: a fast timescale that learned quickly from endpoint error but had poor retention, and a slow timescale that learned slowly but had strong retention. With cortical cerebellar damage, the fast timescale of adaptation was effectively absent, but the slow timescale was less impaired. Therefore, the cerebellum corrects for variability in the motor commands that initiate saccades within the same movement via an adaptive response that not only exhibits strong sensitivity to previous endpoint errors, but also rapid forgetting.
Assuntos
Adaptação Fisiológica/fisiologia , Cerebelo/fisiologia , Desempenho Psicomotor/fisiologia , Movimentos Sacádicos/fisiologia , Adulto , Idoso , Análise de Variância , Biorretroalimentação Psicológica , Fenômenos Biomecânicos , Canais de Cálcio/genética , Estudos de Casos e Controles , Doenças Cerebelares/genética , Doenças Cerebelares/patologia , Doenças Cerebelares/fisiopatologia , Movimentos Oculares/fisiologia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Ruído , Tempo de Reação/fisiologia , Estatística como Assunto , Análise e Desempenho de Tarefas , Fatores de TempoRESUMO
In the last two decades, a growing body of research showing cerebellar involvement in an increasing number of nonmotor tasks and systems has prompted an expansion of speculations concerning the function of the cerebellum. Here, we tested the predictions of a hypothesis positing cerebellar involvement in sensory data acquisition. Specifically, we examined the effect of global cerebellar degeneration on primary auditory sensory function by means of a pitch discrimination task. The just noticeable difference in pitch between two tones was measured in 15 healthy controls and in 15 high functioning patients afflicted with varying degrees of global cerebellar degeneration caused by hereditary, idiopathic, paraneoplastic, or postinfectious pancerebellitis. Participants also performed an auditory detection task assessing sustained attention, a test of verbal auditory working memory, and an audiometric test. Patient pitch discrimination thresholds were on average five and a half times those of controls and were proportional to the degree of cerebellar ataxia assessed independently. Patients and controls showed normal hearing thresholds and similar performance in control tasks in sustained attention and verbal auditory working memory. These results suggest there is an effect of cerebellar degeneration on primary auditory function. The findings are consistent with other recent demonstrations of cerebellar-related sensory impairments, and with robust cerebellar auditorily evoked activity, confirmed by quantitative meta-analysis, across a range of functional neuroimaging studies dissociated from attention, motor, affective, and cognitive variables. The data are interpreted in the context of a sensory hypothesis of cerebellar function.
Assuntos
Doenças Auditivas Centrais/fisiopatologia , Transtornos da Percepção Auditiva/fisiopatologia , Doenças Cerebelares/fisiopatologia , Cerebelo/fisiopatologia , Discriminação da Altura Tonal/fisiologia , Estimulação Acústica , Adulto , Idoso , Atrofia/complicações , Atrofia/patologia , Atrofia/fisiopatologia , Audiometria , Doenças Auditivas Centrais/etiologia , Doenças Auditivas Centrais/patologia , Vias Auditivas/patologia , Vias Auditivas/fisiopatologia , Transtornos da Percepção Auditiva/etiologia , Transtornos da Percepção Auditiva/patologia , Limiar Auditivo/fisiologia , Doenças Cerebelares/complicações , Doenças Cerebelares/patologia , Cerebelo/patologia , Avaliação da Deficiência , Feminino , Humanos , Masculino , Memória de Curto Prazo/fisiologia , Pessoa de Meia-Idade , Testes Neuropsicológicos , Degenerações Espinocerebelares/complicações , Degenerações Espinocerebelares/patologia , Degenerações Espinocerebelares/fisiopatologiaAssuntos
Transtornos da Articulação/patologia , Doenças Cerebelares/patologia , Infarto da Artéria Cerebral Média/patologia , Hemorragias Intracranianas/patologia , Remissão Espontânea , Anticoagulantes/efeitos adversos , Transtornos da Articulação/etiologia , Transtornos da Articulação/fisiopatologia , Doenças Cerebelares/induzido quimicamente , Doenças Cerebelares/fisiopatologia , Cerebelo/irrigação sanguínea , Cerebelo/patologia , Cerebelo/fisiopatologia , Feminino , Lobo Frontal/irrigação sanguínea , Lobo Frontal/patologia , Lobo Frontal/fisiopatologia , Lateralidade Funcional/fisiologia , Humanos , Infarto da Artéria Cerebral Média/complicações , Infarto da Artéria Cerebral Média/fisiopatologia , Hemorragias Intracranianas/fisiopatologia , Pessoa de Meia-Idade , Vias Neurais/patologia , Vias Neurais/fisiopatologia , Plasticidade Neuronal/fisiologia , Lobo Parietal/irrigação sanguínea , Lobo Parietal/patologia , Lobo Parietal/fisiopatologia , Recuperação de Função Fisiológica/fisiologia , Fala/fisiologia , Comportamento Verbal/fisiologia , Varfarina/efeitos adversosRESUMO
Cystathionine beta-synthase-deficient mice (Cbs(-/-)) exhibit several pathophysiological features similar to hyperhomocysteinemic patients, including endothelial dysfunction and hepatic steatosis. Heterozygous mutants (Cbs(+/-)) on the C57BL/6J background are extensively analyzed in laboratories worldwide; however, detailed analyses of Cbs(-/-) have been hampered by the fact that they rarely survive past the weaning age probably due to severe hepatic dysfunction. We backcrossed the mutants with four inbred strains (C57BL/6J(Jcl), BALB/cA, C3H/HeJ and DBA/2J) for seven generations, and compared Cbs(-/-) phenotypes among the different genetic backgrounds. Although Cbs(-/-) on all backgrounds were hyperhomocysteinemic/hypermethioninemic and suffered from lipidosis/hepatic steatosis at 2 weeks of age, >30% of C3H/HeJ-Cbs(-/-) survived over 8 weeks whereas none of DBA/2J-Cbs(-/-) survived beyond 5 weeks. At 2 weeks, serum levels of total homocysteine and triglyceride were lowest in C3H/HeJ-Cbs(-/-). Adult C3H/HeJ-Cbs(-/-) survivors showed hyperhomocysteinemia but escaped hypermethioninemia, lipidosis and hepatic steatosis. They appeared normal in general behavioral tests but showed cerebellar malformation and impaired learning ability in the passive avoidance step-through test, and required sufficient dietary supplementation of cyst(e)ine for survival, demonstrating the essential roles of cystathionine beta-synthase in the central nervous system function and cysteine biosynthesis. Our C3H/HeJ-Cbs(-/-) mice could be useful tools for investigating clinical symptoms such as mental retardation and thromboembolism that are found in homocysteinemic patients.
Assuntos
Cistationina beta-Sintase/genética , Cistationina beta-Sintase/metabolismo , Modelos Animais de Doenças , Hiper-Homocisteinemia/enzimologia , Hiper-Homocisteinemia/genética , Aminoácidos/sangue , Animais , Comportamento Animal , Doenças Cerebelares/enzimologia , Doenças Cerebelares/genética , Doenças Cerebelares/patologia , Doenças Cerebelares/fisiopatologia , Cisteína/metabolismo , Feminino , Humanos , Hiper-Homocisteinemia/patologia , Hiper-Homocisteinemia/fisiopatologia , Estimativa de Kaplan-Meier , Lipídeo A/sangue , Masculino , Camundongos , Camundongos Endogâmicos , Camundongos Knockout , Fosfatidilcolina-Esterol O-Aciltransferase/sangue , Especificidade da EspécieRESUMO
BACKGROUND: Vestibular evoked myogenic potentials (VEMPs) provide assessment of vestibular function. They consist in picking up compound muscle action potentials in the sternocleidomastoid (SCM) muscles in response to auditory stimulation of the vestibulum. VEMP testing has found application mainly in peripheral vestibular disorders, whereas reports about VEMPs in central vestibular lesions are rather scarce. AIMS OF THE STUDY: Based on the physiological connections between the cerebellum and the vestibular nuclei, we investigated the influence on VEMPs of cerebellar and lower-brainstem strokes. We examined whether or not this method may be suitable as a clinical tool for the evaluation of the extent of cerebellar strokes. PATIENTS AND METHODS: Nineteen patients with cerebellar ischemic stroke and 15 patients with lower-brainstem ischemic stroke (11 in the pons, four in the medulla) were included. The latencies and amplitudes of P13 and N23 in both groups of patients were compared with those obtained in a control group of 53 normal individuals. RESULTS: VEMP responses were obtained in all patients and controls. At the group level, mean peak latencies and amplitudes, and the number of subjects with significantly deviant values did not differ between patients and controls. There were no latency or amplitude differences ipsilaterally or contralaterally to the lesion. At the individual level, there was no correlation between laterality of lesion and that of P13 or N23 abnormalities in patients with cerebellar strokes; however, there were two patients (one pontine, one medullar stroke) who presented P13 and N23 latency abnormalities ipsilaterally to the lesion. CONCLUSION: Cerebellar strokes do not influence VEMPs. Moreover, despite previous reports, we were unable to find at a group level any statistically significant VEMP changes in patients with lower-brainstem strokes as compared with controls. Therefore, VEMPs do not appear a suitable tool for assessment of brainstem integrity in patients with posterior fossa strokes. However, they could constitute a sensitive method for documentation of involvement of the central vestibular pathways in patients with brainstem stroke.
Assuntos
Infartos do Tronco Encefálico/diagnóstico , Doenças Cerebelares/diagnóstico , Potenciais Evocados/fisiologia , Músculo Esquelético/fisiopatologia , Acidente Vascular Cerebral/diagnóstico , Vestíbulo do Labirinto/fisiologia , Estimulação Acústica , Infartos do Tronco Encefálico/patologia , Infartos do Tronco Encefálico/fisiopatologia , Doenças Cerebelares/patologia , Doenças Cerebelares/fisiopatologia , Fossa Craniana Posterior/patologia , Feminino , Humanos , Imageamento por Ressonância Magnética , Masculino , Bulbo/patologia , Pessoa de Meia-Idade , Ponte/patologia , Acidente Vascular Cerebral/patologia , Acidente Vascular Cerebral/fisiopatologiaRESUMO
Cattle in western Uruguay that were eating Solanum bonariense developed periodic episodes of ataxia, hypermetria, hyperesthesia, head and thoracic limb extension, opisthotonus, nystagmus, and falling to the side or backward. Similar clinical signs were experimentally reproduced in cattle by administration of S. bonariense via rumen cannula at a dose of 1,024 g/kg body mass. No significant gross lesions were observed in field cases or experimentally induced cases. Spontaneous and induced histologic lesions were similar and included vacuolation, degeneration, and loss of Purkinje cells. Axonal spheroids, microcavitations, and other changes of wallerian-type degeneration in cerebellar white matter were also observed. Ultrastructural changes included increased number of electron-dense residual storage bodies in membrane-bound vesicles in affected Purkinje cells, and similar vesicles and mitochondria in axonal spheroids. No histologic lesions were detected in the other examined tissues. The Purkinje-cell swelling and vacuolation with subsequent cerebellar degeneration are suggestive of Purkinje-cell specific toxin that produces abnormal lysosome function and cell specific axonal transport. This is the first report of S. bonariense toxicity.
Assuntos
Encéfalo/patologia , Doenças dos Bovinos/etiologia , Doenças Cerebelares/veterinária , Doenças Neurodegenerativas/veterinária , Plantas Tóxicas , Solanum , Fosfatase Alcalina/sangue , Animais , Aspartato Aminotransferases/sangue , Bovinos , Doenças dos Bovinos/enzimologia , Doenças dos Bovinos/patologia , Doenças Cerebelares/enzimologia , Doenças Cerebelares/etiologia , Doenças Cerebelares/patologia , Feminino , Histocitoquímica/veterinária , Masculino , Microscopia Eletrônica de Transmissão/veterinária , Doenças Neurodegenerativas/enzimologia , Doenças Neurodegenerativas/etiologia , Doenças Neurodegenerativas/patologia , Células de Purkinje/patologia , Células de Purkinje/ultraestrutura , gama-Glutamiltransferase/sangueRESUMO
We report unusual findings on MR imaging in a 62-year-old woman with Wernicke's encephalopathy (WE). Initial fluid-attenuated inversion recovery (FLAIR) and diffusion weighted MR imaging (DW-MRI) showed hyperintense lesions in the cerebellum and medial thalami, with a decreased apparent diffusion coefficient (ADC) in the cerebellum (reduced by 45%). After thiamine supplementation, the T2 and diffusion hyperintensities disappeared. However, clinical examination at three months showed persistent cerebellar impairment. The importance of the ADC values should be further investigated.
Assuntos
Doenças Cerebelares/patologia , Imagem de Difusão por Ressonância Magnética , Encefalopatia de Wernicke/patologia , Doenças Cerebelares/tratamento farmacológico , Feminino , Humanos , Pessoa de Meia-Idade , Tiamina/uso terapêutico , Encefalopatia de Wernicke/tratamento farmacológicoRESUMO
OBJECT: Thalamic neurons firing at frequencies synchronous with tremor are thought to play a critical role in the generation and maintenance of tremor. The authors studied the incidence and locations of neurons with tremor-related activity (TRA) in the thalamus of patients with varied pathological conditions-including Parkinson disease (PD), essential tremor (ET), multiple sclerosis (MS), and cerebellar disorders--to determine whether known differences in the effectiveness of thalamic stereotactic procedures for these tremors could be correlated to differences in the incidence or locations of TRA cells. METHODS: Seventy-five operations were performed in 61 patients during which 686 TRA cells were recorded from 440 microelectrode trajectories in the thalamus. The locations of the TRA cells in relation to electrophysiologically defined thalamic nuclei and the commissural coordinates were compared among patient groups. The authors found that TRA cells are present in patients with each of these disorders and that these cells populate several nuclei in the ventral lateral tier of the thalamus. There were no large differences in the locations of TRA cells among the different diagnostic classes, although there was a difference in the incidence of TRA cells in patients with PD, who had greater than 3.8 times more cells per thalamic trajectory than patients with ET and approximately five times more cells than patients with MS or cerebellar disorders. CONCLUSIONS: There was an increased incidence of TRA in the thalamus of patients with PD. The location of thalamic TRA cells in patients with basal ganglia and other tremor disorders was similar.