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1.
Adv Exp Med Biol ; 1378: 155-178, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-35902471

RESUMO

It has been well demonstrated that the cerebellum is associated with cognitive and affective processing as well as the traditionally conceptualized motor function. In the present chapter, we explore the behavioral and neurobiological implications of a common congenital cerebellar condition, Chiari malformation Type I, on cognitive and affective processing. We also emphasize the associations between Chiari-related chronic pain, cognitive dysfunction, and emotion dysregulation. Based on our review of the literature, we argue that chronic pain can account for a substantial amount of the cognitive dysfunction and emotion dysregulation in Chiari malformation Type I. Yet, there also exists aspects of Chiari-related cognitive dysfunction and emotion dysregulation that appear to be at least partially independent of chronic pain and more directly associated with abnormalities in cerebrospinal fluid flow dynamics and cerebro-cerebellar communication pathways.


Assuntos
Malformação de Arnold-Chiari , Dor Crônica , Disfunção Cognitiva , Malformação de Arnold-Chiari/complicações , Malformação de Arnold-Chiari/psicologia , Cerebelo , Dor Crônica/complicações , Disfunção Cognitiva/complicações , Emoções , Humanos , Imageamento por Ressonância Magnética
2.
J Neurosurg Pediatr ; 27(2): 139-144, 2020 Dec 04.
Artigo em Inglês | MEDLINE | ID: mdl-33276337

RESUMO

OBJECTIVE: The authors' previously published work validated the Chiari Health Index for Pediatrics (CHIP), a new instrument for measuring health-related quality of life (HRQOL) for pediatric Chiari malformation type I (CM-I) patients. In this study, the authors further evaluated the CHIP to assess HRQOL changes over time and correlate changes in HRQOL to changes in symptomatology and radiological factors in CM-I patients who undergo surgical intervention. Strong HRQOL evaluation instruments are currently lacking for pediatric CM-I patients, creating the need for a standardized HRQOL instrument for this patient population. This study serves as the first analysis of the CHIP instrument's effectiveness in measuring short-term HRQOL changes in pediatric CM-I patients and can be a useful tool in future CM-I HRQOL studies. METHODS: The authors evaluated prospectively collected CHIP scores and clinical factors of surgical intervention in patients younger than 18 years. To be included, patients completed a baseline CHIP captured during the preoperative visit, and at least 1 follow-up CHIP administered postoperatively. CHIP has 2 domains (physical and psychosocial) comprising 4 components, the 3 physical components of pain frequency, pain severity, and nonpain symptoms, and a single psychosocial component. Each CHIP category is scored on a scale, with 0 indicating absent and 1 indicating present, with higher scores indicating better HRQOL. Wilcoxon paired tests, Spearman correlations, and linear regression models were used to evaluate and correlate HRQOL, symptomatology, and radiographic factors. RESULTS: Sixty-three patients made up the analysis cohort (92% Caucasian, 52% female, mean age 11.8 years, average follow-up time 15.4 months). Dural augmentation was performed in 92% of patients. Of the 63 patients, 48 reported preoperative symptoms and 42 had a preoperative syrinx. From baseline, overall CHIP scores significantly improved over time (from 0.71 to 0.78, p < 0.001). Significant improvement in CHIP scores was seen in patients presenting at baseline with neck/back pain (p = 0.015) and headaches (p < 0.001) and in patients with extremity numbness trending at p = 0.064. Patients with syringomyelia were found to have improvement in CHIP scores over time (0.75 to 0.82, p < 0.001), as well as significant improvement in all 4 components. Additionally, improved CHIP scores were found to be significantly associated with age in patients with cervical (p = 0.009) or thoracic (p = 0.011) syrinxes. CONCLUSIONS: The study data show that the CHIP is an effective instrument for measuring HRQOL over time. Additionally, the CHIP was found to be significantly correlated to changes in symptomatology, a finding indicating that this instrument is a clinically valuable tool for the management of CM-I.


Assuntos
Malformação de Arnold-Chiari/psicologia , Malformação de Arnold-Chiari/cirurgia , Procedimentos Neurocirúrgicos/métodos , Procedimentos Neurocirúrgicos/psicologia , Adolescente , Criança , Feminino , Seguimentos , Nível de Saúde , Humanos , Masculino , Medição da Dor , Período Pós-Operatório , Valor Preditivo dos Testes , Qualidade de Vida , Estudos Retrospectivos , Resultado do Tratamento , Adulto Jovem
3.
World Neurosurg ; 143: 158-162, 2020 11.
Artigo em Inglês | MEDLINE | ID: mdl-32730962

RESUMO

BACKGROUND: While commonly seen in syndromic craniosynostosis, the incidence of Chiari malformation (CM) in nonsyndromic craniosynostosis has been reported at 5% and there is a lack of understanding of the pathophysiology and management of CM in this patient population. CASE DESCRIPTION: We present a 5-year-old male patient who underwent a sagittal craniosynostosis repair at the age of 5 months and returned at the age of 5 years with daily headaches associated with behavioral changes. He was found to have pan-synostoses and radiographic evidence of increased intracranial pressure, including a Chiari malformation. Neurologic and genetic workup was unremarkable. A cranial vault reconstruction was performed, and subsequent imaging demonstrated resolution of previously noted Chiari malformation. CONCLUSIONS: In our case, we provided a unique window into the underlying pathophysiology for CM in patients with concurrent nonsyndromic craniosynostosis that we hope will add to the current foundation of literature supporting the intricate relation between cranial vault compliance and Chiari malformation or hindbrain herniation. Furthermore, we provide insight into the management of acquired CM and support isolated cranial vault reconstruction in those who do not appear to have symptomatic suboccipital compression.


Assuntos
Malformação de Arnold-Chiari/cirurgia , Craniossinostoses/cirurgia , Procedimentos Neurocirúrgicos/métodos , Procedimentos de Cirurgia Plástica/métodos , Crânio/cirurgia , Malformação de Arnold-Chiari/etiologia , Malformação de Arnold-Chiari/psicologia , Transtornos do Comportamento Infantil/etiologia , Transtornos do Comportamento Infantil/psicologia , Pré-Escolar , Craniossinostoses/complicações , Craniossinostoses/psicologia , Cefaleia/etiologia , Humanos , Pressão Intracraniana , Imageamento por Ressonância Magnética , Masculino , Tomografia Computadorizada por Raios X , Resultado do Tratamento
4.
World Neurosurg ; 136: 462-469, 2020 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-32204298

RESUMO

Chiari malformation comprises a spectrum of congenital malformations characterized by a herniation of the cerebellar tonsils below the foramen magnum. Chiari malformation type I (CM-I) is the most prevalent subtype seen in clinical practice. This condition variably compresses the cerebellum and medulla-spinal cord junction secondary to malformation of the posterior fossa. Most neurologists and neurosurgeons recognize the sensorimotor and lower brainstem manifestations that result in the clinical picture of CM-I. The effects of CM-I on cognitive functioning, however, and their impact on neuropsychological performance are poorly understood, despite having long been recognized. This article reviews neuropsychological deficits demonstrated by individuals with CM-I, and explores cerebellocortical neuroanatomic pathways to provide possible rationale for the neurocognitive impairments present in affected individuals.


Assuntos
Malformação de Arnold-Chiari/diagnóstico por imagem , Malformação de Arnold-Chiari/cirurgia , Parafusos Ósseos , Procedimentos Neurocirúrgicos/métodos , Crânio/diagnóstico por imagem , Crânio/cirurgia , Adolescente , Adulto , Malformação de Arnold-Chiari/psicologia , Cadáver , Criança , Pré-Escolar , Estudos de Viabilidade , Feminino , Fixação Interna de Fraturas , Humanos , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Neuronavegação , Fusão Vertebral , Tomografia Computadorizada por Raios X , Resultado do Tratamento , Adulto Jovem
5.
Cerebellum ; 19(3): 392-400, 2020 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-32048182

RESUMO

Chiari malformation type I (CM-I) is a neurological disorder in which cerebellar tonsils are herniated through the foramen magnum into the spinal canal. A wide spectrum of cognitive deficits underlying this pathology has been reported, but the literature about social cognition is insufficient. Clinical research has pointed out the cerebellar role in Theory of Mind (ToM), indicating that there are several disorders with cerebellar pathology that reveal a poorer performance in social cognition tasks. The main purpose of this study is to compare the performance on ToM tasks between CM-I patients and healthy controls. The protocol includes Faux Pas test, Happé's Strange Stories test, Ice-Cream Van task, the FEEL test, and the Word Accentuation Test. In order to eliminate the possible influence of covariables, physical pain and anxious-depressive symptomatology have been controlled for. According to the results, CM-I patients performed worse than matched healthy controls on ToM tasks, except for facial emotion recognition. These differences remained even after controlling for the neuropsychiatric variables and physical pain. Thus, it can be suggested that patients with CM-I are impaired in their social skills related to their performance on ToM tasks. These findings can be considered to be a preliminary approach to the specific study of social cognition in relation to CM-I since it is similar to other cerebellar pathologies and to previous literature on the cerebellum's role in social cognition.


Assuntos
Malformação de Arnold-Chiari/diagnóstico , Malformação de Arnold-Chiari/psicologia , Desempenho Psicomotor/fisiologia , Cognição Social , Adulto , Feminino , Humanos , Masculino , Pessoa de Meia-Idade
6.
Cerebellum ; 19(1): 6-15, 2020 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-31286383

RESUMO

The involvement of the cerebellum in visuospatial abilities has been evidenced in numerous studies, based on the cerebellar-cortical circuitry. This domain has been evaluated in several patients with cerebellar disorders, but the assessment of visuospatial processing in Chiari malformation type I (CM-I) is scarce. The aim of this study is to analyze the visuospatial performance between CM-I adult patients and healthy controls. Participants have been tested using Block Design and Visual Puzzles subtests of the Wechsler Adult Intelligence Scale (WAIS), the Benton Judgment of Line Orientation test, and the Rey-Osterrieth Complex Figure test. The anxious-depressive symptomatology, the physical pain, and the premorbid intelligence have been controlled for, as well. The CM-I patients showed a significantly lower performance; however, after analyzing and controlling for the effect of clinical variables and psychopathological symptomatology, the main effect was maintained for visual puzzles and line orientation tasks. The findings suggest that CM-I patients show a poorer performance in tasks that require an exercise of perceptual reasoning without motor demand, accompanied by visualization and mental imagery of the stimuli. This study contributes towards the reinforcement of the evidence on the cognitive alterations associated to CM-I.


Assuntos
Malformação de Arnold-Chiari/diagnóstico por imagem , Malformação de Arnold-Chiari/psicologia , Desempenho Psicomotor/fisiologia , Percepção Espacial/fisiologia , Percepção Visual/fisiologia , Adulto , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estimulação Luminosa/métodos , Navegação Espacial/fisiologia
7.
Disabil Health J ; 12(4): 649-656, 2019 10.
Artigo em Inglês | MEDLINE | ID: mdl-31147250

RESUMO

BACKGROUND: 50% of patients with Chiari Malformation (CM) report a history of depression; however, rates of other psychological symptoms are unknown. Further, it is unclear whether surgical correction impacts pain, disability, and psychological symptoms. OBJECTIVE: /Hypothesis: We examined rates of symptoms in a nationwide sample of CM patients who had (n = 639) and had not (n = 551) undergone surgical correction. We hypothesized lower symptom severity in the latter group. METHODS: Participants completed assessments and submitted pre-surgical MRI scans online (n = 286). Informed by the Fear-Avoidance Model of pain, we controlled for psychological symptoms when assessing pain/disability, and pain/disability when assessing psychological symptoms. RESULTS: Overall, high rates of depression (44% moderate-severe) and anxiety (60% moderate-severe) were reported. Groups (surgery vs. no-surgery) did not differ in the proportion of patients meeting cutoff scores for current disability; however, the no-surgery group was more likely to meet cutoffs for anxiety (χ2 = 11.26, p < .05), stress (χ2 = 14.63, p < .01) and health anxiety (χ2 = 4.63, p < .05). The surgery group reported lower levels of continuous affective pain F(1, 1065) = 10.28, p < .001), anxiety F(1,1026) = 4.96, p < .05) and stress F(1, 978) = 5.67, p < .05) although effect sizes were small (η2s ranging from 0.010 to 0.006, Cohen's D ranging from 0.17 to 0.25). CONCLUSION: CM patients experience high rates of psychological symptomatology regardless of surgical status, suggesting that all CM patients may benefit from evidence-based interventions to address anxiety and depression.


Assuntos
Ansiedade/etiologia , Malformação de Arnold-Chiari/complicações , Depressão/etiologia , Pessoas com Deficiência/psicologia , Dor/etiologia , Estresse Psicológico/etiologia , Adolescente , Adulto , Idoso , Ansiedade/epidemiologia , Transtornos de Ansiedade/epidemiologia , Transtornos de Ansiedade/etiologia , Malformação de Arnold-Chiari/psicologia , Malformação de Arnold-Chiari/cirurgia , Depressão/epidemiologia , Transtorno Depressivo/epidemiologia , Transtorno Depressivo/etiologia , Medo , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Dor/epidemiologia , Dor/psicologia , Estresse Psicológico/epidemiologia , Adulto Jovem
8.
World Neurosurg ; 126: e641-e645, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-30831298

RESUMO

OBJECTIVE: Adults with Chiari malformation type 1 (CM1) often report cognitive impairment. This cross-sectional study investigates the cognitive and emotional functioning of a sample of adults with CM1 who presented for neurosurgical evaluation prior to intervention. METHODS: A total of 36 participants (18 patients with CM1 and 18 healthy control subjects) completed a comprehensive neurocognitive battery of tests. RESULTS: Demographic variables (sex, age, handedness, and education) were not statistically significant between the groups. Measures of gross cognition (Mini-Mental State Examination and Repeatable Battery for the Assessment of Neuropsychological Status) were statistically significant between the groups. On a more focused assessment of neurocognitive abilities, the CM1 group performed significantly worse on measures of learning, memory, fluency, and figural copy. A high rate of clinical depression was seen in the CM1 group; however, this did not correlate with cognitive performance. CONCLUSIONS: The CM1 group displayed subtle learning, semantic fluency, and complex construction difficulties compared with healthy control subjects. Although not correlated with cognition, adults with CM1 are at high risk for clinical depression.


Assuntos
Malformação de Arnold-Chiari/complicações , Disfunção Cognitiva/etiologia , Adulto , Malformação de Arnold-Chiari/psicologia , Disfunção Cognitiva/epidemiologia , Estudos Transversais , Depressão/epidemiologia , Depressão/etiologia , Feminino , Humanos , Masculino
9.
Soc Sci Med ; 231: 13-21, 2019 06.
Artigo em Inglês | MEDLINE | ID: mdl-29395547

RESUMO

Human geography's varied engagement with the brain has involved considerations of the way people know and respond to their environments, and their place-based experiences with emotions, mental illnesses and disorders, intellectual disabilities and particular neurological conditions. This paper argues however that this scholarship could be augmented by, and existing expertise be directed towards, considering physical brain abnormalities and injuries. As a case in point it considers the spatial experience of living with Type 1 Chiari Malformation. Through interviews with four sufferers, the research articulates three domains that they have had to re-negotiate - home space, social space and medical space - emphasizing supportive and challenging aspects of each, as well as meaningful and affective qualities to encounters. The paper concludes with some pointers towards the future study of physical brain abnormalities and injuries and the kinds of knowledge it might create to increase awareness and inform care.


Assuntos
Malformação de Arnold-Chiari/complicações , Malformação de Arnold-Chiari/psicologia , Geografia , Humanos , Entrevistas como Assunto/métodos , Limitação da Mobilidade , Pesquisa Qualitativa , Comportamento Social
10.
Neuropsychologia ; 121: 135-143, 2018 12.
Artigo em Inglês | MEDLINE | ID: mdl-30412712

RESUMO

BACKGROUND: Previous studies have suggested an association of Chiari Malformation type I (CM-I) and cognitive deficits. CM-I is a neurological disorder characterized by a descent of cerebellar tonsils into the foramen magnum, resulting in overcrowding of the upper cervical spine region. Posterior fossa decompression (PFD) is the surgical treatment of choice, however, the literature on the consequences for patients is mainly reduced to the assessment of physical symptoms. METHODS: Data from a neuropsychological assessment of 76 patients with CM-I, both with PFD (n = 37) and without PFD (n = 39) surgery, and 76 healthy controls, matched by gender, age and years of education are reported. RESULTS: CM-I patients show a generally lower cognitive performance in executive function, verbal fluency, spatial cognition, language (naming), verbal memory, processing speed, emotional facial recognition and theory of mind, compared to control group. The results are maintained even after statistically controlling for the influence of perceived physical pain and the presence of anxious-depressive symptomatology. Data also illustrate a similar cognitive profile between both groups with CM-I. CONCLUSION: These findings provide evidence of a deficient cognitive profile associated with CM-I, regardless of the PFD surgery. According to these results, both physical and cognitive consequences must be considered in the treatment of CM-I.


Assuntos
Malformação de Arnold-Chiari/psicologia , Malformação de Arnold-Chiari/cirurgia , Descompressão Cirúrgica , Processos Mentais , Estudos Transversais , Escolaridade , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Testes Neuropsicológicos , Resultado do Tratamento
11.
Psicothema (Oviedo) ; 30(3): 316-321, ago. 2018. tab
Artigo em Espanhol | IBECS | ID: ibc-175900

RESUMO

BACKGROUND: Over the last decade there has been growing evidence that neuropsychological deficits, principally in the executive functions, may be involved in the pathogenesis of Chiari Type I disease. The aim of this study is to compare changes in cognitive function in patients with Chiari Type I and healthy subjects. METHOD: The neuropsychological profile of these patients was compared with healthy controls. Three neuropsychological tests were administered to both Chiari Type I patients and healthy controls to assess the frontal executive functions of vigilance or selective attention, mental flexibility, planning and concept formation. RESULTS: The results suggest that Chiari Type I patients are affected in the processes of inhibition and self-control as well as in attention capacity and maintaining a course of thought and action. CONCLUSIONS: These results provide evidence of possible deficits or anomalies in the cognitive executive functions of patients with Chiari Type I


ANTECEDENTES: en la última década, existen evidencias crecientes de que déficits neuropsicológicos, esencialmente en funciones ejecutivas, pueden estar involucrados en la patogenia de la enfermedad de Chiari Tipo I. El objetivo del estudio es evaluar la influencia de anormalidades estructurales sobre las funciones neuropsicológicas, fundamentalmente ejecutivas, en pacientes con Chiari Tipo I. MÉTODO: para ello se comparó el perfil neuropsicológico de estos pacientes con controles sanos. Tanto a los pacientes Chiari Tipo I como a los controles sanos se les aplicó pruebas neuropsicológicas que valoraron funciones ejecutivas frontales de vigilancia o atención sostenida, flexibilidad mental, y planificación y formación de conceptos (Stroop, CPT, WCST). RESULTADOS: los resultados obtenidos sugieren una afectación de los pacientes Chiari Tipo I en los procesos de inhibición y autocontrol (Stroop) y en la capacidad atencional y en el mantenimiento del curso del pensamiento y la acción (WCST). CONCLUSIONES: estos resultados proporcionan evidencias de posibles déficits o anomalías en las funciones ejecutivas cognitivas, que permitirían diferenciar los pacientes con Chiari Tipo I


Assuntos
Humanos , Masculino , Feminino , Adulto , Pessoa de Meia-Idade , Idoso , Malformação de Arnold-Chiari/complicações , Malformação de Arnold-Chiari/psicologia , Cognição , Função Executiva , Transtornos Mentais/etiologia , Testes Neuropsicológicos
12.
Psicothema ; 30(3): 316-321, 2018 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-30009755

RESUMO

BACKGROUND: Over the last decade there has been growing evidence that neuropsychological deficits, principally in the executive functions, may be involved in the pathogenesis of Chiari Type I disease. The aim of this study is to compare changes in cognitive function in patients with Chiari Type I and healthy subjects. METHOD: The neuropsychological profile of these patients was compared with healthy controls. Three neuropsychological tests were administered to both Chiari Type I patients and healthy controls to assess the frontal executive functions of vigilance or selective attention, mental flexibility, planning and concept formation. RESULTS: The results suggest that Chiari Type I patients are affected in the processes of inhibition and self-control as well as in attention capacity and maintaining a course of thought and action. CONCLUSIONS: These results provide evidence of possible deficits or anomalies in the cognitive executive functions of patients with Chiari Type I.


Assuntos
Malformação de Arnold-Chiari/complicações , Malformação de Arnold-Chiari/psicologia , Cognição , Função Executiva , Transtornos Mentais/etiologia , Adulto , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Testes Neuropsicológicos
13.
Cerebellum ; 17(5): 564-574, 2018 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-29766459

RESUMO

Chiari Malformation type I (CM-I) is a neurological disorder characterized by a displacement of the cerebellar tonsils through the foramen magnum into the spinal canal. Most research has focused on physical symptomatology but few studies include neuropsychological examinations. Moreover, although current research highlights the involvement of the cerebellum on higher cognitive functions, little is known about cognitive consequences associated with CM-I. The aim of this study is to analyze cognitive functioning between 39 CM-I patients and 39 healthy controls, matched by gender, age and years of education. Participants have been examined on a large battery of neuropsychological tests, including executive functioning, verbal fluency, spatial cognition, language, verbal memory, processing speed, facial recognition and theory of mind. Results show a poorer performance of the clinical group compared to the control group, even after controlling the effect of physical pain and anxious-depressive symptomatology. The findings suggest the presence of a generalized cognitive deficit associated with CM-I, which makes it necessary to focus attention not only on physical consequences, but also on cognitive ones.


Assuntos
Malformação de Arnold-Chiari/psicologia , Processos Mentais , Disfunção Cognitiva/etiologia , Estudos Transversais , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Testes Neuropsicológicos
15.
Neuropsychol Rev ; 28(2): 176-187, 2018 06.
Artigo em Inglês | MEDLINE | ID: mdl-29468516

RESUMO

Displacement of the cerebellar tonsils in Chiari type I malformation (CMI) can affect functions controlled by the cerebellum and brainstem. While playing an integral role in the control of movement, the cerebellum also has widespread cortical connections, influencing a range of cognitive process. A systematic literature review was conducted to examine the relationship between cognition and CMI, assessing evidence for general or domain-specific cognitive change. The search protocol examined the AMED, CINAHL, Cochrane Library, EMBASE, MEDLINE, PsycINFO, and Scopus databases. Articles meeting the following criteria were included in this review (i) examined children or adults with a clinically defined diagnosis of CMI, (ii) assessed cognitive function with a prospective examination, (iii) included at least one standardized instrument designed to measure general or specific domains of cognitive function, and (iv) were published in English in a peer-reviewed journal. Twelve articles were identified, including 783 cases aged 3 months to 64 years. General cognition, processing speed, and learning and memory appeared less affected, while language deficits appeared to diminish with age. Executive dysfunction was the most commonly reported cognitive impairment, while attention and working memory, and visuospatial and perceptual skills also appeared vulnerable. Numerous methodological limitations were identified that should be considered in interpreting the impact of CMI and planning future investigations. Overall, there is currently insufficient evidence to describe a valid and reliable profile of cognitive impairment in CMI. Further research is required to confirm these preliminary psychometric results and integrate them with pathophysiological models.


Assuntos
Malformação de Arnold-Chiari/psicologia , Cognição , Disfunção Cognitiva/etiologia , Humanos
16.
Cerebellum ; 17(4): 404-418, 2018 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-29383659

RESUMO

Type I Chiari malformation (CMI) is a neurological condition in which the cerebellar tonsils descend into the cervical spinal subarachnoid space resulting in cervico-medullary compression. Early case-control investigations have indicated cognitive deficits in the areas of attention, memory, processing speed, and visuospatial function. The present study further examined cognitive and emotional processing deficits associated with CMI using a dual-task paradigm. Nineteen CMI patients were recruited during pre-surgical consultation and 19 matched control participants identified emotional expressions in separate single and asynchronous dual-task designs. To extend earlier behavioral studies of cognitive effects in CMI, we recorded event-related potentials (ERPs) in the dual-task design. Though response times were slower for CMI patients across the two tasks, behavioral and ERP analyses indicated that patients did not differ from matched controls in the ability to allocate attentional resources between the two tasks. P1 ERP component analyses provided no indication of an emotional arousal deficit in our CMI sample while P3 ERP component analyses suggested a CMI-related deficit in emotional regulation. P3 analysis also yielded evidence for a frontalization of neurophysiological activity in CMI patients. Pain and related depression and anxiety factors accounted for CMI deficits in single-task, but not dual-task, response times. Results are consistent with a dysfunctional fronto-parietal attentional network resulting from either the indirect effects of chronic pain or the direct effects of CMI pathophysiology stemming from cervico-medullary compression.


Assuntos
Malformação de Arnold-Chiari/fisiopatologia , Malformação de Arnold-Chiari/psicologia , Encéfalo/fisiopatologia , Cognição/fisiologia , Emoções/fisiologia , Reconhecimento Facial/fisiologia , Adulto , Atenção/fisiologia , Dor Crônica/fisiopatologia , Dor Crônica/psicologia , Eletroencefalografia , Potenciais Evocados , Feminino , Humanos , Masculino , Testes Neuropsicológicos , Percepção Social
17.
Artigo em Inglês | MEDLINE | ID: mdl-29173189

RESUMO

BACKGROUND: Introduction. Arnold Chiari Malformation (ACM) type I is a pathology whose symptomatology has repercussions for the quality of life of those affected by it. Quality-of-life measurement instruments can allow the severity of the impact of Chiari type I malformation on patients' lives to be monitored. The Chiari Symptom Profile (CSP) is a valid and reliable instrument designed for this purpose. The aim of the study was to adapt the CSP to Spanish and to explore the reliability and validity of this construct in the context of Spanish-speaking patients with ACM. METHODS: The English CSP instrument has a good internal validity and consistency. We used a standardized procedure for the linguistic validation of the translated scale. For the psychometric validation, we recruited 215 individuals with ACM and calculated the Cronbach's alpha for the sample. The construct was validated by analyzing the age, sex, and presence of syringomyelia, as well as by correlating the results with the sickness impact profile 30 (SIP-30) questionnaire, which can also evaluate quality of life in this type of patient. RESULTS: The Spanish version of the CSP has good internal consistency and validity (Cronbach's alpha of 0.90); age, sex, and the presence of syringomyelia does not significantly affect the quality of life of patients with ACM. There was a direct and significant correlation between the Spanish CSP and the validated SIP-30 questionnaire results (p < 0.05). Further analysis showed a positive correlation for the physical and psychological scopes of the CSP and SIP-30 questionnaires, but not for their functional and social scopes. CONCLUSION: This version of the CSP is a valid and reliable instrument for measuring quality of life in patients with ACM in the Spanish context.


Assuntos
Malformação de Arnold-Chiari/diagnóstico , Malformação de Arnold-Chiari/psicologia , Efeitos Psicossociais da Doença , Qualidade de Vida , Inquéritos e Questionários , Adulto , Malformação de Arnold-Chiari/epidemiologia , Feminino , Nível de Saúde , Humanos , Masculino , Pessoa de Meia-Idade , Valor Preditivo dos Testes , Psicometria , Reprodutibilidade dos Testes , Perfil de Impacto da Doença , Espanha/epidemiologia , Siringomielia/diagnóstico , Siringomielia/epidemiologia , Siringomielia/psicologia , Tradução
18.
Childs Nerv Syst ; 33(2): 313-320, 2017 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-27921214

RESUMO

INTRODUCTION: Chiari malformation type-1 (CM-1) may be treated by intradural (ID) or extradural (ED) posterior fossa decompression, although the optimal approach is debated. The Chiari Severity Index (CSI) is a pre-operative metric to predict patient-defined improvement after CM-1 surgery. In this study, we evaluate the results of ID versus ED decompression and assess the external validity of the CSI. METHODS: We performed a retrospective cohort study of pediatric CM-1 patients undergoing decompression at a single academic children's hospital. Characteristics of headache, syrinx, and myelopathy were collected to derive CSI grade. The primary outcome measure was pre-operative symptom resolution. The proportion of patients with favorable outcome was tabulated for each of the three CSI grades and compared to previously published results. RESULTS: From 2004 to 2014, 189 patients underwent ID (48%) or ED (52%) decompression at the Children's Hospital of Philadelphia (CHOP). Follow-up ranged from 1 to 75 months. Rates of symptom resolution (58-64%) and reoperation (8%) were similar regardless of surgical approach. Although proportions of favorable outcomes differed between the CHOP and Washington University (WU) cohorts, the difference was not related to CSI grade (p = 0.63). Furthermore, there was no difference in the proportion of favorable outcomes between the two cohorts regardless of ID (p = 0.26) or ED approach (p = 0.11). CONCLUSIONS: Equivalent rates of symptom resolution and reoperation following ID and ED decompression support the ED approach as a first-line surgical option for pediatric CM-1 patients. In addition, our findings provide preliminary evidence supporting the generalizability of the CSI and its use in future comparative trials.


Assuntos
Malformação de Arnold-Chiari/cirurgia , Descompressão Cirúrgica/métodos , Índice de Gravidade de Doença , Adolescente , Malformação de Arnold-Chiari/diagnóstico por imagem , Malformação de Arnold-Chiari/psicologia , Criança , Estudos de Coortes , Dura-Máter/cirurgia , Feminino , Humanos , Imageamento por Ressonância Magnética , Masculino , Qualidade de Vida , Reprodutibilidade dos Testes , Resultado do Tratamento
19.
Pediatr Neurosurg ; 51(5): 236-43, 2016.
Artigo em Inglês | MEDLINE | ID: mdl-27225878

RESUMO

BACKGROUND: Children with Chiari malformation type 1 (CM1) have increasingly presented to neurosurgery clinics. Limited research relating to the cognitive dysfunction experienced by this population has been completed. In adults, inhibition problems and executive dysfunction have been documented. METHODS: Seventy-seven parental reports of children with CM1 were included in the study. Parents completed questions on a scale rating daily executive functioning as well as reporting on common neurological symptoms. RESULTS: The sample consisted of 41 males and 36 females with a mean age of 133.57 ± 42.18 months. Thirty-eight subjects had had decompression surgery. The most common neurological symptoms included: headache (69%), a history of pain (31%) and gait disturbance (20%). One third of the sample demonstrated overall executive functioning impairment, with working memory elevations being most prevalent (44%). Depression, gender, age and decompression surgery were not related to executive dysfunction. CONCLUSIONS: The parental report of executive dysfunction in children with CM1 was higher than the standardized healthy sample. Metacognitive problems, especially working memory and initiation problems were most prevalent. A quick parental rating scale identifying children with executive dysfunction may be beneficial for neurosurgeons and assist with referrals for a more comprehensive neuropsychological assessment.


Assuntos
Malformação de Arnold-Chiari/epidemiologia , Malformação de Arnold-Chiari/psicologia , Transtornos Cognitivos/epidemiologia , Transtornos Cognitivos/psicologia , Função Executiva , Pais , Adolescente , Malformação de Arnold-Chiari/diagnóstico , Criança , Transtornos Cognitivos/diagnóstico , Feminino , Humanos , Masculino , Testes Neuropsicológicos , Inquéritos e Questionários
20.
Neurol Sci ; 36(9): 1617-24, 2015 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-25972139

RESUMO

Chiari malformation (CM) is a condition in which cerebellar tonsillar ectopia may manifest with various clinical presentations. This study reports from the only national, online patient registry available, the symptoms, comorbid neurocognitive and psychological conditions, and diagnostic experiences of patients living with CM type I (CM I). The current research is one component of a large investigation designed to collect information from individuals with CM through the online Conquer Chiari Patient Registry questionnaire. Analyses included descriptive statistics to study body system impact and patient diagnostic experiences. Participants were 768 individuals with CM I and were predominantly female (86.8 %) and Caucasian (93.8 %) with an average age of 35 years. Pain was the most frequently reported symptom (76.69 %) experienced prior to diagnosis with headaches implicated most often (73.44 %). Neurocognitive comorbidities included memory difficulties (43.88 %) and aphasia (43.75 %) and psychological disorders such as depression (31.77 %) and anxiety disorders (19.92 %) were reported. Average time to diagnosis from first physician visit to diagnosis was 3.43 years, and only 8.46 % of patients had previous awareness of CM. CM I diagnosis was found incidentally for 24.87 % of participants. Common misdiagnoses were classified as psychological (19.26 %) and neurological (19.26 %). Fear was the most frequent emotion elicited at the time of correct diagnosis (42.19 %). CM I can be a challenging condition for patients and physicians, during both the search for diagnosis and management of symptoms. Patient and physician education about CM I may permit early intervention and the prevention of further deterioration and patient suffering.


Assuntos
Malformação de Arnold-Chiari/diagnóstico , Malformação de Arnold-Chiari/fisiopatologia , Adulto , Malformação de Arnold-Chiari/epidemiologia , Malformação de Arnold-Chiari/psicologia , Bases de Dados Factuais , Feminino , Humanos , Masculino , Sistema de Registros , Autorrelato , Estados Unidos/epidemiologia , Adulto Jovem
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