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1.
Reumatol. clín. (Barc.) ; 19(3): 168-170, Mar. 2023. ilus
Artículo en Inglés | IBECS | ID: ibc-217291

RESUMEN

The chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) and autoimmune/inflammatory syndrome induced by adjuvants (ASIA) were described a decade ago. There are a limited number of reports and these diseases are still underdiagnosed.We presented a 35 years-old patient with clinical manifestations of cerebellum affection and enhancement in the MRI, with the influenza vaccine as the only triggering factor.Infectious diseases, malignancy, and additional systemic involvement were ruled out; therefore, on suspicion of CLIPPERS syndrome, the patient received corticosteroid therapy with an adequate response.The knowledge of CLIPPERS syndrome as an unusual ASIA presentation and high response to the corticosteroids may lead to a timely diagnosis, appropriate treatment, and follow up with better outcomes for patients.(AU)


La inflamación linfocítica crónica con realce perivascular pontino que responde a los esteroides (CLIPPERS) y el síndrome autoinmune/inflamatorio inducido por adyuvantes (ASIA) se describieron hace una década. Hay un número limitado de reportes y estas enfermedades aún están infra diagnosticadas.Presentamos un paciente de 35 años con cuadro clínico de afectación del cerebelo y realce en la RM, con la vacuna anti-influenza como único factor desencadenante.Se descartaron enfermedades infecciosas, malignidad y afectación sistémica, por lo tanto, ante sospecha de síndrome de CLIPPERS, recibió tratamiento con corticoesteroides con una respuesta adecuada.El conocimiento del síndrome de CLIPPERS como una presentación inusual de ASIA y su alta respuesta a los corticosteroides puede conducir a un diagnóstico oportuno, un tratamiento adecuado y un seguimiento con mejores resultados para los pacientes.(AU)


Asunto(s)
Humanos , Masculino , Adulto , Enfermedades del Sistema Nervioso , Vacunación/efectos adversos , Vacunas contra la Influenza/efectos adversos , Corticoesteroides , Cerebelo/lesiones , Reumatología , Enfermedades Reumáticas
2.
Sci Rep ; 12(1): 143, 2022 01 07.
Artículo en Inglés | MEDLINE | ID: mdl-34996954

RESUMEN

Blast exposure can injure brain by multiple mechanisms, and injury attributable to direct effects of the blast wave itself have been difficult to distinguish from that caused by rapid head displacement and other secondary processes. To resolve this issue, we used a rat model of blast exposure in which head movement was either strictly prevented or permitted in the lateral plane. Blast was found to produce axonal injury even with strict prevention of head movement. This axonal injury was restricted to the cerebellum, with the exception of injury in visual tracts secondary to ocular trauma. The cerebellar axonal injury was increased in rats in which blast-induced head movement was permitted, but the pattern of injury was unchanged. These findings support the contentions that blast per se, independent of head movement, is sufficient to induce axonal injury, and that axons in cerebellar white matter are particularly vulnerable to direct blast-induced injury.


Asunto(s)
Axones/patología , Traumatismos por Explosión/patología , Lesiones Traumáticas del Encéfalo/patología , Cerebelo/patología , Degeneración Nerviosa , Sustancia Blanca/patología , Animales , Axones/metabolismo , Biomarcadores/metabolismo , Traumatismos por Explosión/metabolismo , Lesiones Traumáticas del Encéfalo/metabolismo , Cerebelo/lesiones , Cerebelo/metabolismo , Modelos Animales de Enfermedad , Movimientos de la Cabeza , Masculino , Nervio Óptico/metabolismo , Nervio Óptico/patología , Traumatismos del Nervio Óptico/metabolismo , Traumatismos del Nervio Óptico/patología , Ratas Long-Evans , Vías Visuales/lesiones , Vías Visuales/metabolismo , Vías Visuales/patología , Sustancia Blanca/lesiones , Sustancia Blanca/metabolismo
3.
J Neurosci ; 41(42): 8725-8741, 2021 10 20.
Artículo en Inglés | MEDLINE | ID: mdl-34462307

RESUMEN

Galanin, one of the most inducible neuropeptides, is widely present in developing brains, and its expression is altered by pathologic events (e.g., epilepsy, ischemia, and axotomy). The roles of galanin in brain development under both normal and pathologic conditions have been hypothesized, but the question of how galanin is involved in fetal and early postnatal brain development remains largely unanswered. In this study, using granule cell migration in the cerebellum of early postnatal mice (both sexes) as a model system, we examined the role of galanin in neuronal cell migration during normal development and after brain injury. Here we show that, during normal development, endogenous galanin participates in accelerating granule cell migration via altering the Ca2+ and cAMP signaling pathways. Upon brain injury induced by the application of cold insults, galanin levels decrease at the lesion sites, but increase in the surroundings of lesion sites. Granule cells exhibit the following corresponding changes in migration: (1) slowing down migration at the lesion sites; and (2) accelerating migration in the surroundings of lesion sites. Experimental manipulations of galanin signaling reduce the lesion site-specific changes in granule cell migration, indicating that galanin plays a role in such deficits in neuronal cell migration. The present study suggests that manipulating galanin signaling may be a potential therapeutic target for acutely injured brains during development.SIGNIFICANCE STATEMENT Deficits in neuronal cell migration caused by brain injury result in abnormal development of cortical layers, but the underlying mechanisms remain to be determined. Here, we report that on brain injury, endogenous levels of galanin, a neuropeptide, are altered in a lesion site-specific manner, decreasing at the lesion sites but increasing in the surroundings of lesion sites. The changes in galanin levels positively correlate with the migration rate of immature neurons. Manipulations of galanin signaling ameliorate the effects of injury on neuronal migration and cortical layer development. These results shed a light on galanin as a potential therapeutic target for acutely injured brains during development.


Asunto(s)
Lesiones Encefálicas/metabolismo , Encéfalo/crecimiento & desarrollo , Encéfalo/metabolismo , Movimiento Celular/fisiología , Cerebelo/metabolismo , Galanina/metabolismo , Animales , Animales Recién Nacidos , Lesiones Encefálicas/patología , Células Cultivadas , Cerebelo/lesiones , Cerebelo/patología , Relación Dosis-Respuesta a Droga , Femenino , Masculino , Ratones
4.
Medicine (Baltimore) ; 100(48): e28024, 2021 Dec 03.
Artículo en Inglés | MEDLINE | ID: mdl-35049215

RESUMEN

INTRODUCTION: The cortico-ponto-cerebellar tract (CPCT) is involved in coordination of movement; injury of the CPCT can therefore be accompanied by ataxia. In this study, using diffusion tensor tractography (DTT), we investigated injury of the CPCT in patients with mild traumatic brain injury (TBI). METHODS: We recruited 45 consecutive patients with ataxia following mild TBI and 20 normal control subjects. The score of assessment and rating of ataxia (SARA) was used to evaluate of ataxia. The patients were classified into 2 groups based on the SARA; patient group A had with post-traumatic ataxia and patient group B had without post-traumatic ataxia. The fractional anisotropy (FA) value and fiber number (FN) of the CPCT was measured. RESULTS: Significant differences were observed in the FA and FN values of the CPCT between patient group A and the control group and between patient groups A and B (P < .05). In addition, a significant difference was observed in the FA value only of the CPCT between patient group B and the control group (P < .05). However, no significant difference was observed in the FN value of the CPCT between patient group B and the control group (P > .05). CONCLUSION: By using DTT, injury of the CPCT was demonstrated in patients who showed ataxia following mild TBI. These results suggest that DTT would be useful for evaluation of the CPCT in patients with ataxia after mTBI because mTBI usually does not show any abnormalities on conventional brain MRI.


Asunto(s)
Ataxia/etiología , Conmoción Encefálica/complicaciones , Cerebelo/lesiones , Adulto , Anciano , Conmoción Encefálica/diagnóstico por imagen , Cerebelo/diagnóstico por imagen , Imagen de Difusión Tensora , Femenino , Humanos , Masculino , Persona de Mediana Edad
5.
Neurosurg Rev ; 44(1): 351-361, 2021 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-31758338

RESUMEN

This study aimed to compare the surgical outcomes and morbidities of retrosigmoid and translabyrinthine approaches for large vestibular schwannoma (VS), with a focus on cerebellar injury and morbidities. Eighty-six consecutive patients with large VS, with a maximal extrameatal diameter > 3.0 cm, were reviewed between August 2010 and September 2018. The surgical outcomes, operating time, volume change of perioperative cerebellar edema, and inpatient rehabilitation related to cerebellar morbidities were compared between the two approaches. In total, 53 and 33 patients underwent the retrosigmoid and translabyrinthine approaches, respectively. The median follow-up time was 34.5 months. Surgical outcomes, including the extent of resection, tumor recurrence, and facial nerve preservation, showed no significant differences between the two groups. Patients who underwent the retrosigmoid approach showed a marginal trend for postoperative lower cranial nerve (LCN) dysfunction (P = 0.068). Although the approaching procedure time was longer in the translabyrinthine group, the tumor resection time was significantly longer in the retrosigmoid group (P = 0.001). The median change in the volume of the perioperative cerebellar edema was significantly larger in the retrosigmoid group (P < 0.001) and significantly related to the retrosigmoid approach, solid VS, and tumor resection time. Most cerebellar and LCN deficits were transient; however, the patients in the retrosigmoid group underwent inpatient rehabilitation more than those in the translabyrinthine group (P = 0.018). Both surgical approaches show equivalent surgical outcomes. Notably, the translabyrinthine approach for large VS has advantages in that it reduces cerebellar injury and related morbidities.


Asunto(s)
Cerebelo/lesiones , Oído Interno/cirugía , Recurrencia Local de Neoplasia/cirugía , Neuroma Acústico/cirugía , Procedimientos Neuroquirúrgicos/métodos , Complicaciones Posoperatorias/etiología , Adulto , Cerebelo/diagnóstico por imagen , Oído Interno/diagnóstico por imagen , Femenino , Estudios de Seguimiento , Humanos , Masculino , Persona de Mediana Edad , Morbilidad , Recurrencia Local de Neoplasia/diagnóstico por imagen , Neuroma Acústico/diagnóstico por imagen , Complicaciones Posoperatorias/diagnóstico por imagen , Estudios Retrospectivos
6.
J Neurosci Res ; 99(2): 446-454, 2021 02.
Artículo en Inglés | MEDLINE | ID: mdl-33089563

RESUMEN

Soccer, as a contact sport, exposes players to repetitive head impacts, especially through heading the ball. The question of a long-term brain cumulative effect remains. Our objective was to determine whether exposure to head impacts over one soccer season was associated with changes in functional brain connectivity at rest, using magnetic resonance imaging (MRI). In this prospective cohort study, 10 semi-professional men soccer players, aged 18-25 years, and 20 age-matched men athletes without a concussion history and who do not practice any contact sport were recruited in Bordeaux (France). Exposure to head impacts per soccer player during competitive games over one season was measured using video analysis. Resting-state functional magnetic resonance imaging data were acquired for both groups at two times, before and after the season. With a seed-based analysis, resting-state networks that have been intimately associated with aspects of cognitive functioning were investigated. The results showed a mean head impacts of 42 (±33) per soccer player over the season, mainly intentional head-to-ball impacts and no concussion. No head impact was found among the other athletes. The number of head impacts between the two MRI acquisitions before and after the season was associated with increased connectivity within the default mode network and the cortico-cerebellar network. In conclusion, our findings suggest that the brain functioning changes over one soccer season in association with exposure to repetitive head impacts.


Asunto(s)
Lesiones Traumáticas del Encéfalo/patología , Conectoma , Red en Modo Predeterminado/diagnóstico por imagen , Traumatismos Cerrados de la Cabeza/complicaciones , Fútbol/lesiones , Adolescente , Adulto , Conmoción Encefálica/diagnóstico por imagen , Conmoción Encefálica/etiología , Conmoción Encefálica/patología , Lesiones Traumáticas del Encéfalo/diagnóstico por imagen , Lesiones Traumáticas del Encéfalo/etiología , Cerebelo/diagnóstico por imagen , Cerebelo/lesiones , Cerebelo/patología , Francia , Traumatismos Cerrados de la Cabeza/epidemiología , Humanos , Imagen por Resonancia Magnética , Masculino , Estudios Prospectivos , Recurrencia , Descanso , Adulto Joven
7.
J Child Neurol ; 36(6): 482-490, 2021 05.
Artículo en Inglés | MEDLINE | ID: mdl-33356784

RESUMEN

OBJECTIVE: Acute brain injury is a frequent perinatal neurologic complication that can involve the cerebellum. Although short-term outcomes of infants with neonatal cerebellar injury are well described, neurologic sequelae in older children are underreported. Here, we describe epilepsy-related outcomes in young children who suffered from neonatal cerebellar injuries. METHODS: In-house automated software identified patients with neonatal brain injuries who were evaluated at our institution both as neonates (≤28 days) and as children (≥1 year). Neonatal hospital course, neuroimaging, and outcomes related to epilepsy were reviewed from the medical record. Patients were stratified into 2 groups based on neonatal brain injuries: those with cerebellar injury and those without cerebellar involvement. RESULTS: Of the 282 neonates followed through childhood over the decade-long study period, 33 (12%) experienced neonatal brain injury. All 33 cases involved supratentorial injury, and 5 (15%) also included cerebellar injury. The development of epilepsy was significantly less likely in the group with cerebellar involvement (40%) compared to that with cerebellar sparing (82%; P = 0.043). In some cases, children with cerebellum-sparing injuries required admission for seizure control and developed drug-resistant epilepsy as well as status epilepticus. These outcomes occurred less frequently in the group with cerebellar involvement. CONCLUSIONS: Epilepsy-related sequelae may occur less frequently when the cerebellum is involved in neonatal brain injury. Larger prospective studies are needed to clarify how cerebellocortical networks impact functional brain connectivity and epilepsy longitudinally.


Asunto(s)
Lesiones Encefálicas/complicaciones , Epilepsia/complicaciones , Cerebelo/diagnóstico por imagen , Cerebelo/lesiones , Femenino , Humanos , Lactante , Recién Nacido , Imagen por Resonancia Magnética/métodos , Masculino , Neuroimagen/métodos , Estudios Retrospectivos
8.
Radiología (Madr., Ed. impr.) ; 62(4): 298-305, jul.-ago. 2020. ilus, tab
Artículo en Español | IBECS | ID: ibc-194247

RESUMEN

OBJETIVOS: Describir los patrones normales de activación en el cerebelo de funciones específicas cerebrales (motor, lenguaje, memoria) y su relación topográfica en la corteza cerebelosa utilizando resonancia magnética funcional. MATERIALES Y MÉTODOS: Se evaluaron 25 sujetos sanos, 8 mujeres y 17 hombres de entre 16 y 64 años, 23 diestros y 2 zurdos, mediante resonancia magnética funcional basada en tareas de lenguaje, memoria, motor y visual, en equipo de resonancia de 1.5 Teslas. RESULTADOS: Se caracterizó la actividad funcional en el cerebelo asociada a los componentes motores, de lenguaje y memoria, describiendo la relación con las regiones topográficas, así como su relación funcional con áreas corticales cerebrales. CONCLUSIONES: El conocimiento sobre los patrones de normalidad de las características morfológicas y del comportamiento funcional en el cerebelo, así como su relación con el cerebro, es importante para el radiólogo y médico clínico en la evaluación del cerebelo y sus posibles condiciones patológicas


OBJECTIVES: To describe the normal patterns of cerebellar activation in specific cerebral functions (motor, language, memory) and their topographical correlations in the cerebellar cortex on functional magnetic resonance imaging. MATERIALS AND METHODS: We evaluated 25 healthy subjects (8 women and 17 men; 23 right-handed and 2 left-handed; age range, 16-64 years), who did language, memory, and motor tasks while undergoing 1.5T functional magnetic resonance imaging. RESULTS: We assessed functional activity of the cerebellum associated with motor, language, and memory components, describing their relations with topographical regions of the cerebellum and their functional relations with areas in the cerebral cortex. CONCLUSIONS: Knowledge of the normal patterns of morphological characteristics and functional behavior in the cerebellum as well as their relations with the brain is important for radiologists and clinicians evaluating the cerebellum and possible pathological conditions that affect it


Asunto(s)
Humanos , Masculino , Femenino , Adolescente , Adulto Joven , Adulto , Persona de Mediana Edad , Cerebelo/diagnóstico por imagen , Imagen por Resonancia Magnética , Habla/fisiología , Memoria/fisiología , Neuroimagen Funcional , Lateralidad Funcional , Cerebelo/lesiones
9.
AJNR Am J Neuroradiol ; 41(8): E61, 2020 08.
Artículo en Inglés | MEDLINE | ID: mdl-32586961
10.
Rev. bras. neurol ; 55(3): 33-37, jul.-set. 2019. ilus, tab
Artículo en Inglés | LILACS | ID: biblio-1022916

RESUMEN

Luigi Luciani (1840-1919) was an illustrious Italian citizen and physiologist whose research scope covered mainly cardiovascular subjects, the nervous system, and fasting. He published in 1891 a modern landmark of the study of cerebellar physiology - "Il cervelletto: nuovistudi di normal and pathología physiology" / "The cerebellum: new studies on normal and pathological physiology." In his experiment, a dog survived after cerebellectomy, reporting a triad of symptoms (asthenia, atonia, and astasia). In this way, the eminent neurophysiologist improved the operative technique and sterile processes to redirect the issue of cerebellar symptoms. Luciani died at age 78, a hundred years ago, and left mainly the understanding of the role of the cerebellum in regulating postural tone and muscle strength, which represented a step forward in understanding cerebellar motor physiology. In recent decades, cognitive / affective function has been added to the cerebellar motor, and there has also been a better understanding of cerebellar circuits.


Luigi Luciani (1840-1919) foi um ilustre cidadão e fisiologista italiano, cujo escopo de pesquisa abrangia principalmente assuntos cardiovasculares, sistema nervoso e jejum. Ele publicou em 1891 um marco moderno do estudo da fisiologia do cerebelo - "Il cervelletto: nuovistudi di fisiologia normale and patologica" / "O cerebelo: novos estudos sobre fisiologia normal e patológica". Em seu experimento, um cão sobreviveu após a cerebelectomia, com o relatório de uma tríade de sintomas (astenia, atonia e astasia). Dessa maneira, o eminente neurofisiologista aprimorou a técnica operatória e os processos estéreis para redirecionar a questão dos sintomas cerebelares. Luciani morreu aos 78 anos, cem anos atrás, e deixou principalmente a compreensão do papel do cerebelo na regulação do tônus postural e da força muscular, o que representou um passo adiante na compreensão da fisiologia motora cerebelar. Nas últimas décadas, a função cognitivo / afetiva foi adicionada à motora cerebelar e, também, houve uma melhor compreensão dos circuitos do cerebelo.


Asunto(s)
Humanos , Historia del Siglo XIX , Historia del Siglo XX , Neurociencias/historia , Cerebelo/fisiología , Neurofisiología/historia , Cerebelo/lesiones , Italia
11.
Dev Biol ; 455(2): 458-472, 2019 11 15.
Artículo en Inglés | MEDLINE | ID: mdl-31376393

RESUMEN

The cerebellum undergoes major rapid growth during the third trimester and early neonatal stage in humans, making it vulnerable to injuries in pre-term babies. Experiments in mice have revealed a remarkable ability of the neonatal cerebellum to recover from injuries around birth. In particular, recovery following irradiation-induced ablation of granule cell precursors (GCPs) involves adaptive reprogramming of Nestin-expressing glial progenitors (NEPs). Sonic hedgehog signaling is required for the initial step in NEP reprogramming; however, the full spectrum of developmental signaling pathways that promote NEP-driven regeneration is not known. Since the growth regulatory Hippo pathway has been implicated in the repair of several tissue types, we tested whether Hippo signaling is involved in regeneration of the cerebellum. Using mouse models, we found that the Hippo pathway transcriptional co-activator YAP1 (Yes-associated protein 1) but not TAZ (transcriptional coactivator with PDZ binding motif, or WWTR1) is required in NEPs for full recovery of cerebellar growth following irradiation one day after birth. Although Yap1 plays only a minor role during normal development in differentiation of NEPs or GCPs, the size of the cerebellum, and in particular the internal granule cell layer produced by GCPs, is significantly reduced in Yap1 mutants after irradiation, and the organization of Purkinje cells and Bergmann glial fibers is disrupted. The initial proliferative response of Yap1 mutant NEPs to irradiation is normal and the cells migrate to the GCP niche, but subsequently there is increased cell death of GCPs and altered migration of granule cells, possibly due to defects in Bergmann glia. Moreover, loss of Taz along with Yap1 in NEPs does not abrogate regeneration or alter development of the cerebellum. Our study provides new insights into the molecular signaling underlying postnatal cerebellar development and regeneration.


Asunto(s)
Proteínas Adaptadoras Transductoras de Señales/fisiología , Proteínas de Ciclo Celular/fisiología , Cerebelo/citología , Células-Madre Neurales/fisiología , Aciltransferasas , Proteínas Adaptadoras Transductoras de Señales/genética , Animales , Encéfalo/efectos de la radiación , Lesiones Encefálicas , Regeneración Cerebral/fisiología , Proteínas de Ciclo Celular/genética , Muerte Celular , Movimiento Celular , Cerebelo/crecimiento & desarrollo , Cerebelo/lesiones , Eliminación de Gen , Ratones , Nestina , Neuroglía/metabolismo , Transducción de Señal , Factores de Transcripción/genética , Factores de Transcripción/metabolismo , Proteínas Señalizadoras YAP
12.
Nat Commun ; 10(1): 3128, 2019 07 16.
Artículo en Inglés | MEDLINE | ID: mdl-31311934

RESUMEN

To select actions based on sensory evidence, animals must create and manipulate representations of stimulus information in memory. Here we report that during accumulation of somatosensory evidence, optogenetic manipulation of cerebellar Purkinje cells reduces the accuracy of subsequent memory-guided decisions and causes mice to downweight prior information. Behavioral deficits are consistent with the addition of noise and leak to the evidence accumulation process. We conclude that the cerebellum can influence the accurate maintenance of working memory.


Asunto(s)
Cerebelo/fisiología , Toma de Decisiones/fisiología , Memoria a Corto Plazo/fisiología , Animales , Conducta Animal/fisiología , Cerebelo/citología , Cerebelo/lesiones , Craneotomía , Femenino , Masculino , Ratones , Modelos Animales , Optogenética , Estimulación Luminosa , Células de Purkinje/fisiología
13.
Eur J Paediatr Neurol ; 23(4): 604-608, 2019 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-31147107

RESUMEN

Pediatric abusive head trauma (AHT) or non accidental head trauma (NAHT) is a major cause of death from trauma in children under 2 years of age. Main etiological factor for non accidental head trauma is shaking a baby, causing brain injury by rotational head acceleration and deceleration. The consequent brain damage as shown by magnetic resonance imaging (MRI) is subdural haemorrhage and to a lesser extent parenchymal injuries of variable severity. Involvement of the cerebellum has very rarely been described. We report the clinical history and the development of cerebral magnetic resonance imaging findings in two children with serious brain injury following probable shaking who presented the typical "triad" with subdural haematoma, retinal haemorrhage and encephalopathy. We want to draw attention to cerebellar involvement characterized by cortico-subcortical signal alterations most prominent on T2w images following diffusion changes during the acute period. We discuss cerebellar involvement as a sign of higher severity of AHT which is probably underrecognized.


Asunto(s)
Lesiones Encefálicas/patología , Cerebelo/lesiones , Cerebelo/patología , Maltrato a los Niños , Traumatismos Craneocerebrales/patología , Lesiones Encefálicas/diagnóstico por imagen , Cerebelo/diagnóstico por imagen , Maltrato a los Niños/diagnóstico , Traumatismos Craneocerebrales/diagnóstico por imagen , Femenino , Humanos , Lactante , Imagen por Resonancia Magnética/métodos , Masculino
14.
Neurochem Res ; 44(2): 374-387, 2019 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-30460640

RESUMEN

VGF nerve growth factor inducible (VGF) is a neuropeptide precursor induced by brain-derived neurotrophic factor and nerve growth factor. VGF is increased in the prefrontal cortex and cerebrospinal fluid in schizophrenia patients. In our previous study, VGF-overexpressing mice exhibited schizophrenia-like behaviors and smaller brain weights. Brain developmental abnormality is one cause of mental illness. Research on brain development is important for discovery of pathogenesis of mental disorders. In the present study, we investigated the role of VGF on cerebellar development. We performed a histological analysis with cerebellar sections of adult and postnatal day 3 mice by Nissl staining. To investigate cerebellar development, we performed immunostaining with antibodies of immature and mature granule cell markers. To understand the mechanism underlying these histological changes, we examined MAPK, Wnt, and sonic hedgehog signaling by Western blot. Finally, we performed rotarod and footprint tests using adult mice to investigate motor function. VGF-overexpressing adult mice exhibited smaller cerebellar sagittal section area. In postnatal day 3 mice, a cerebellar sagittal section area reduction of the whole cerebellum and external granule layer and a decrease in the number of mature granule cells were found in VGF-overexpressing mice. Additionally, the number of proliferative granule cell precursors was lower in VGF-overexpressing mice. Phosphorylation of Trk and Erk1 were increased in the cerebellum of postnatal day 3 VGF-overexpressing mice. Adult VGF-overexpressing mice exhibited motor disability. All together, these findings implicate VGF in the development of cerebellar granule cells via promoting MAPK signaling and motor function in the adult stage.


Asunto(s)
Cerebelo/metabolismo , Regulación del Desarrollo de la Expresión Génica/inmunología , Neuronas/metabolismo , Neuropéptidos/metabolismo , Células de Purkinje/metabolismo , Animales , Encéfalo/metabolismo , Proliferación Celular/fisiología , Cerebelo/lesiones , Ratones , Factores de Crecimiento Nervioso
15.
Sleep Med ; 52: 134-137, 2018 12.
Artículo en Inglés | MEDLINE | ID: mdl-30321820

RESUMEN

BACKGROUND: REM sleep (REMS) is considered vital for supporting well-being and normal cognition. However, it remains unclear if and how decreases in REMS impair cognitive abilities. Rare case studies of patients with REMS abolishment due to pontine lesions remain sporadic, and formal evaluation of cognitive status is lacking. In 1984, Lavie and colleagues described the case of Y.C. - a man with a pontine lesion and near-total absence of REMS who led a normal life. Here, we set out to re-evaluate this individual's REMS status 30 years after the original report, and formally assess his cognitive abilities. METHODS: Four whole-night polysomnographic sleep recordings were conducted to evaluate sleep architecture. Sleep scoring was performed according to the American Academy of Sleep Medicine (AASM) guidelines. Cranial Computed Tomography (CT) imaging was performed, as well as formal neuropsychological testing to evaluate cognitive functions. RESULTS: Y.C. averaged 4.5% of sleep time in REMS, corresponding to the 0.055 percentile of normal values for his age. Furthermore, residual REMS episodes were short and only occurred towards the end of the night. CT imaging revealed damage and metallic fragments in pons, cerebellum, and thalamus. Neuropsychological evaluation demonstrated average to high-average cognitive skills, normal memory, and motor difficulties including speech and left hand dyspraxia. CONCLUSIONS: To our knowledge, this is the only case where REMS loss resulting from pontine lesion was re-evaluated after many years. We find a near-total absence of REMS with no signs of significant compensation throughout adult life, along with normal cognitive status. The results provide a unique perspective on the ongoing debate regarding the functional role of REMS in supporting cognition.


Asunto(s)
Cognición/fisiología , Pruebas Neuropsicológicas/estadística & datos numéricos , Puente/lesiones , Sueño REM/fisiología , Cerebelo/lesiones , Humanos , Masculino , Persona de Mediana Edad , Polisomnografía/métodos , Tomografía Computarizada por Rayos X/métodos
16.
Medicina (B Aires) ; 78(5): 364-367, 2018.
Artículo en Español | MEDLINE | ID: mdl-30285930

RESUMEN

We present two cases of lateral medullary stroke in subjects with extracranial trajectory of the postero-inferior cerebellar artery. Case 1: a 21-year-old male who presented ataxia and right dysmetria after cervical trauma in a rugby match. Case 2: 56-year-old woman, who started with vertigo and left hemiparesis after intense physical effort. In both cases, the angiographic studies showed an extracranial trajectory of the posterior inferior cerebellar artery. This vessel rarely originates below the foramen magnum, in close relationship with the first three cervical vertebrae and the atlanto-axial joint. At this level, it is exposed to mechanical damage causing dissection, such as direct trauma, abrupt cervical manipulation or prolonged cephalic extension. Therefore, this association should be considered in patients with stroke of the lateral region of the bulb and extracranial trajectory of the posterior-inferior cerebellar artery.


Asunto(s)
Disección Aórtica/complicaciones , Cerebelo/irrigación sanguínea , Síndrome Medular Lateral/etiología , Arteria Cerebral Posterior/lesiones , Disección Aórtica/diagnóstico por imagen , Disección Aórtica/patología , Cerebelo/diagnóstico por imagen , Cerebelo/lesiones , Cerebelo/patología , Angiografía Cerebral/métodos , Femenino , Humanos , Síndrome Medular Lateral/diagnóstico por imagen , Síndrome Medular Lateral/patología , Imagen por Resonancia Magnética/métodos , Masculino , Persona de Mediana Edad , Arteria Cerebral Posterior/diagnóstico por imagen , Arteria Cerebral Posterior/patología , Adulto Joven
17.
Medicina (B.Aires) ; 78(5): 364-367, oct. 2018. ilus
Artículo en Español | LILACS | ID: biblio-976126

RESUMEN

Presentamos dos casos de accidente cerebrovascular en sujetos con trayecto extracraneal de la arteria cerebeloso póstero-inferior. Caso 1: varón de 21 años, quien presentó ataxia y dismetría derecha luego de un traumatismo cervical en un partido de rugby. Caso 2: mujer de 56 años, quien inició con vértigo y hemiparesia izquierda luego de esfuerzo físico intenso. En ambos casos, los estudios angiográficos mostraron un trayecto extracraneal de la arteria cerebelosa póstero-inferior. Este vaso raramente se origina por debajo del foramen magno, en relación cercana con las primeras tres vértebras cervicales y la articulación atlanto-axial. En este nivel, está expuesta a daño mecánico causante de disección, como por ejemplo trauma directo, manipulación cervical abrupta o extensión cefálica prolongada. Por lo tanto, en pacientes con accidente cerebrovascular de región lateral de bulbo y trayecto extracraneal de la arteria cerebelosa póstero-inferior se debería considerar esta asociación.


We present two cases of lateral medullary stroke in subjects with extracranial trajectory of the postero-inferior cerebellar artery. Case 1: a 21-year-old male who presented ataxia and right dysmetria after cervical trauma in a rugby match. Case 2: 56-year-old woman, who started with vertigo and left hemiparesis after intense physical effort. In both cases, the angiographic studies showed an extracranial trajectory of the posterior inferior cerebellar artery. This vessel rarely originates below the foramen magnum, in close relationship with the first three cervical vertebrae and the atlanto-axial joint. At this level, it is exposed to mechanical damage causing dissection, such as direct trauma, abrupt cervical manipulation or prolonged cephalic extension. Therefore, this association should be considered in patients with stroke of the lateral region of the bulb and extracranial trajectory of the posterior-inferior cerebellar artery.


Asunto(s)
Humanos , Masculino , Femenino , Persona de Mediana Edad , Síndrome Medular Lateral/etiología , Cerebelo/irrigación sanguínea , Arteria Cerebral Posterior/lesiones , Disección Aórtica/complicaciones , Síndrome Medular Lateral/patología , Síndrome Medular Lateral/diagnóstico por imagen , Imagen por Resonancia Magnética/métodos , Angiografía Cerebral/métodos , Cerebelo/lesiones , Cerebelo/patología , Cerebelo/diagnóstico por imagen , Arteria Cerebral Posterior/patología , Arteria Cerebral Posterior/diagnóstico por imagen , Disección Aórtica/patología , Disección Aórtica/diagnóstico por imagen
18.
Elife ; 72018 08 09.
Artículo en Inglés | MEDLINE | ID: mdl-30091706

RESUMEN

Outside of the neurogenic niches of the brain, postmitotic neurons have not been found to undergo efficient regeneration. We demonstrate that mouse Purkinje cells (PCs), which are born at midgestation and are crucial for development and function of cerebellar circuits, are rapidly and fully regenerated following their ablation at birth. New PCs are produced from immature FOXP2+ Purkinje cell precursors (iPCs) that are able to enter the cell cycle and support normal cerebellum development. The number of iPCs and their regenerative capacity, however, diminish soon after birth and consequently PCs are poorly replenished when ablated at postnatal day five. Nevertheless, the PC-depleted cerebella reach a normal size by increasing cell size, but scaling of neuron types is disrupted and cerebellar function is impaired. Our findings provide a new paradigm in the field of neuron regeneration by identifying a population of immature neurons that buffers against perinatal brain injury in a stage-dependent process.


Asunto(s)
Proliferación Celular , Cerebelo/crecimiento & desarrollo , Cerebelo/lesiones , Células de Purkinje/fisiología , Regeneración , Células Madre/fisiología , Factores de Edad , Animales , Ratones
19.
Brain Dev ; 40(9): 743-752, 2018 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-29776704

RESUMEN

PURPOSE: Cerebellar injury is well established as an important finding in preterm infants with cerebral palsy (CP). In this study, we investigated associations between injury to the cerebellar peduncles and motor impairments in preterm infants using quantitative tractography at term-equivalent age, which represents an early phase before the onset of motor impairments. METHODS: We studied 64 preterm infants who were born at <33 weeks gestational age. These infants were divided into three groups: CP, Non-CP (defined as infants with periventricular leukomalacia but having normal motor function), and a Normal group. Diffusion tensor imaging was performed at term-equivalent age and motor function was assessed no earlier than a corrected age of 2 years. Using tractography, we measured fractional anisotropy (FA) and apparent diffusion coefficient (ADC) of the superior cerebellar peduncles (SCP) and middle cerebellar peduncles (MCP), as well as the motor/sensory tracts. RESULTS: The infants in the CP group had significantly lower FA of the SCP and sensory tract than those in the other groups. There was no significant difference in FA and ADC of the motor tract among the three groups. Severity of CP had a significant correlation with FA of the MCP, but not with the FA of other white matter tracts. CONCLUSION: Our results suggested that the infants with CP had injuries of the ascending tracts (e.g. the SCP and sensory tract), and that additional MCP injury might increase the severity of CP. Quantitative tractography assessment at term-equivalent age may be useful for screening preterm infants for prediction of future motor impairments.


Asunto(s)
Cerebelo/diagnóstico por imagen , Cerebelo/lesiones , Recien Nacido Prematuro , Trastornos del Movimiento/diagnóstico por imagen , Parálisis Cerebral/diagnóstico por imagen , Imagen de Difusión Tensora , Femenino , Humanos , Recién Nacido , Imagen por Resonancia Magnética , Masculino , Pronóstico
20.
Pediatr Res ; 83(5): 1004-1010, 2018 05.
Artículo en Inglés | MEDLINE | ID: mdl-29360805

RESUMEN

BackgroundPreterm birth is associated with an increased risk of cerebellar injury. The aim of this study was to assess the impact of cerebellar hemorrhages (CBH) on cerebral white matter microstructural tissue organization and cerebellar volume at term-equivalent age (TEA) in extremely preterm infants. Furthermore, we aimed to evaluate the association between CBH and neurodevelopmental outcome in late infancy.MethodsA total of 24 preterm infants with punctate CBH were included and each matched to two preterm control infants. T1-, T2-weighted images and diffusion-weighted imaging were acquired on a 3T magnetic resonance imaging (MRI) system. Regions of interest were drawn on a population-specific neonatal template and automatically registered to individual fractional anisotropy (FA) maps. Brain volumes were automatically computed. Neurodevelopmental outcome was assessed using the Bayley scales of Infant and Toddler Development at 2 years of corrected age.ResultsCBHs were not significantly related to FA in the posterior limb of the internal capsule and corpus callosum or to cerebellar volume. Infants with CBH did not have poorer neurodevelopmental outcome compared with control infants.ConclusionThese findings suggest that the impact of mild CBH on early macroscale brain development may be limited. Future studies are needed to assess the effects of CBH on long-term neurodevelopment.


Asunto(s)
Mapeo Encefálico/métodos , Encéfalo/diagnóstico por imagen , Cerebelo/diagnóstico por imagen , Cerebelo/lesiones , Sustancia Blanca/diagnóstico por imagen , Sustancia Blanca/patología , Anisotropía , Estudios de Casos y Controles , Preescolar , Estudios de Cohortes , Imagen de Difusión por Resonancia Magnética , Femenino , Edad Gestacional , Humanos , Procesamiento de Imagen Asistido por Computador/métodos , Lactante , Recién Nacido , Recien Nacido Prematuro , Imagen por Resonancia Magnética , Masculino , Trastornos del Neurodesarrollo , Reconocimiento de Normas Patrones Automatizadas , Riesgo
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