Task-Specific and General Cognitive Effects in Chiari Malformation Type I

Abstract

ObjectiveOur objective was to use episodic memory and executive function tests to determine whether or not Chiari Malformation Type I (CM) patients experience cognitive dysfunction.BackgroundCM is a neurological syndrome in which the cerebellum descends into the cervical spine causing neural compression, severe headaches, neck pain, and number of other physical symptoms. While primarily a disorder of the cervico-medullary junction, both clinicians and researchers have suspected deficits in higher-level cognitive function.Design and MethodsWe tested 24 CM patients who had undergone decompression neurosurgery and 24 age- and education-matched controls on measures of immediate and delayed episodic memory, as well as three measures of executive function.ResultsThe CM group showed performance decrements relative to the controls in response inhibition (Stroop interference), working memory computational speed (Ospan), and processing speed (automated digit symbol substitution task), but group differences in recall did not reach statistical significance. After statistical control for depression and anxiety scores, the group effects for working memory and processing speed were eliminated, but not for response inhibition. This response inhibition difference was not due to overall general slowing for the CM group, either, because when controls' data were transformed using the linear function fit to all of the reaction time tasks, the interaction with group remained statistically significant. Furthermore, there was a multivariate group effect for all of the response time measures and immediate and delayed recall after statistical control of depression and anxiety scores.ConclusionThese results suggest that CM patients with decompression surgery exhibit cognitive dysfunction compared to age- and education-matched controls. While some of these results may be related to anxiety and depression (likely proxies for chronic pain), response inhibition effects, in particular, as well as a general cognitive deficit persisted even after control for anxiety and decompression.

Parent-Reported Executive Dysfunction in Children and Adolescents with Chiari Malformation Type 1

“…The authors hypothesized that CM1 patients may have abnormal cerebellar white matter development and myelin integrity, leading to cognitive impairments. More recently, Allen et al [15] compared 24 adults with CM1 who had undergone decompression surgery with 24 age- and education-matched controls in several tasks of memory and executive function. Participants with CM1 performed significantly worse, when compared to control subjects, on tasks involving response inhibition, even after controlling for depression and anxiety.…”

Bibliographic review on the cognitive symptomatology of type I Chiari malformation

“…La muestra total incluyó un total de 862 sujetos, divididos en dos grupos con edades entre los 5 y 74 años. El grupo clínico se compuso por 799 participantes diagnosticados con MC-I y el grupo control por 63 controles sanos, emparejados por edad y años de educación (Allen et al, 2014;García et al, 2018). En este sentido, cabe destacar que varios estudios se caracterizaron por la ausencia de grupo control Novegno et al, 2008Lacy et al, 2011 y que un estudio contribuyó con 638 casos de pacientes diagnosticados con MC-I.…”

“…Por otro lado, cabe mencionar que 664 pacientes diagnosticados con MC-I se habían sometido a cirugía de descomprensión (Allen et al, 2014;Allen et al, 2017;Riva et al, 2011). En contraposición, 49 sujetos no fueron sometidos a intervención quirúrgica (García et al, 2018;Novegno et al, 2008).…”

“…La MC-I se correlaciona con sintomatología ansioso-depresiva y de dolor (Allen et al, 2014;Allen et al, 2017;García et al, 2018). García et al (2018) mayormente encontraron déficits en tareas que involucran una red compleja de estructuras cerebrales en las que el lóbulo frontal desempeña un papel fundamental junto con las conexiones que tiene con el cerebelo como, por ejemplo, la tarea de Historias de Happe, utilizada para evaluar la teoría de la mente.…”

Prevalence of Psychiatric Diagnoses in Pediatric Chiari Malformation Type 1

“…Moreover, the hypothesis that decompressive Chiari surgery may cause or exacerbate comorbid psychiatric illness is of surgical interest. In an adult sample, Allen et al [30] suggested that neurocognitive symptoms might be attributed to surgical insult associated with decompression injury. However, psychiatric indicators such as depression or anxiety were not noted.…”