Participación del cerebelo en la regulación del afecto, la emoción y la conducta

Goñi, Pilar Hernáez; Ustárroz, Javier Tirapu; Fernández, M. Dolores Iglesias; Lario, Pilar Luna

doi:10.33588/rn.5110.2010394

Cited by 7 publications

(5 citation statements)

References 69 publications

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“…In the present work, we found a direct correlation between the performance in the Go/NoGo task and vermal lobule VI, indicating that good accuracy in this task correlates with preserved volume in vermal lobule VI. It is important to emphasize that in healthy subjects, Habas and colleagues (2009) indicated that the vermis and paravermal lobules (i.e., lobule VI) act within a network involved in the regulation of executive decisions, such as working memory, attention, emotion evaluation, and response selection (van Harskamp et al, 2005;Habas et al, 2009;Hernáez-Goñi et al, 2010).…”

Section: Discussionmentioning

confidence: 99%

The cerebellar topography of attention sub-components in spinocerebellar ataxia type 2

Lupo

Olivito

Iacobacci

et al. 2018

Cortex

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Spinocerebellar ataxia type 2 (SCA2) is an autosomal dominant neurodegenerative disease characterized by a progressive cerebellar syndrome and multiple-domain cognitive impairments. The cerebellum is known to contribute to distinct functional networks related to higher-level functions. The aims of the present study were to investigate the different sub-components of attention and to analyse possible correlations between attention deficits and specific cerebellar regions in SCA2 patients. To this purpose, 11 SCA2 patients underwent an exhaustive attention battery that evaluated several attention sub-components. The SCA2 group performed below the normal range in tasks assessing selective attention, divided attention, and sustained attention, obtaining negative Z-scores. These results were confirmed by non-parametric Mann-Whitney U tests that showed significant differences between SCA2 and control subjects in the same sub-components of the attention battery, allowing us to speculate on cerebellar involvement when a high cognitive demand is required (i.e., multisensory integration, sequencing, prediction of events, and inhibition of inappropriate response behaviours). The voxel-based morphometry analysis showed a pattern of significantly reduced grey matter volume in specific cerebellar lobules. In particular, the SCA2 patients showed significant grey matter loss in bilateral regions of the anterior cerebellar hemisphere (IV) and in the posterior lobe (VI-IX) and posterior vermis (VI-IX). Statistical analysis found significant correlations between grey matter reductions in the VIIb/VIIIa cerebellar lobules and impairments in Sustained and Divided Attention tasks and between grey matter reduction in the vermal VI lobule and impairment in the Go/NoGo task. For the first time, the study demonstrated the involvement of specific cerebellar lobules in different sub-components of the attention domain, giving further support to the inclusion of the cerebellum within the attention network.

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Section: Discussionmentioning

confidence: 99%

The cerebellar topography of attention sub-components in spinocerebellar ataxia type 2

Lupo

Olivito

Iacobacci

et al. 2018

Cortex

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“…Typically, deterioration in these both regions is observed in SCA2 patients ( 13 , 71 , 72 ) and may be associated with motor alterations and lack of coordination clearly manifested by the participants and reflected in the SARA. Nevertheless, besides the motor aspects, the vermis plays an important role in regulating emotional expressions and inferring others’ emotions ( 73 ) through a network involving other cerebellar regions, such as the flocculonodular lobe, the fastigial nucleus, and, the globose nucleus, as well as subcortical limbic regions ( 74 , 75 ). The role of the vermis in SCA2 should be studied in a more accurate manner in future research since its damage is associated with the cerebellar cognitive affective syndrome and with emotional fragility and alterations in facial recognition in several SCA types ( 25 , 29 – 31 ).…”

Section: Discussionmentioning

confidence: 99%

Social and Cultural Elements Associated with Neurocognitive Dysfunctions in Spinocerebellar Ataxia Type 2 Patients

et al. 2015

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Spinocerebellar Ataxia Type 2 (SCA2) is a rare genetic disorder producing cerebellar degeneration and affecting motor abilities. Neuroimaging studies also show neurodegeneration in subcortical and cortical regions related to emotional and social processes. From social neuroscience, it is suggested that motor and social abilities can be influenced by particular cultural dynamics so, culture is fundamental to understand the effect of brain-related alterations. Here, we present the first analysis about the cultural elements related to the SCA2 disorder in 15 patients previously evaluated with neuroimaging and psychometric instruments, and their nuclear relationships distributed in six geographical and cultural regions in Mexico. Ethnographic records and photographic and video archives about the quotidian participant’s routine were obtained from the patients, their relatives and their caregivers. The information was categorized and interpreted taking into consideration cultural issues and patients’ medical files. Our analyses suggest that most of the participants do not understand the nature of the disease and this misunderstanding favors magic and non-medical explanations. Patients’ testimonies suggest a decrease in pain perception as well as motor alterations that may be related to interoceptive dysfunctions. Relatives’ testimonies indicate patients’ lack of social and emotional interests that may be related to frontal, temporal, and cerebellar degeneration. In general, participants use their religious beliefs to deal with the disease and only a few of them trust the health system. Patients and their families are either openly rejected and ignored, tolerated or even helped by their community accordingly to different regional traits. We propose that ethnography can provide social representations to understand the patients’ alterations, to formulate neurobiological hypotheses, to develop neurocognitive interventions, and to improve the medical approach to the disease.

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“…Otro aspecto importante es la presencia de alteraciones en la morfología cerebelosa, con ausencia de alteraciones cerebrales. Ello pone de manifiesto la importancia del cerebelo en las funciones cognitivas y de inteligencia [16,17].…”

Section: Discussionunclassified

Síndrome de Joubert: a propósito de cuatro hermanos adultos afectados

Angemi¹,

Zuccotti²

2012

RevNeurol

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syndrome is a rare, autosomal recessive genetic transmission illness, with ten associated genes discovered at the time. They code for primary ciliary proteins; that is why Joubert syndrome is considered a 'ciliopathie'. The primary cilia are involved in cell proliferation and neuronal migration in cerebellum and axonal brain, being essential for their proper development. The first description was made in 1969 by Marie Joubert and colleagues. They reported four cases with partial or total agenesis of the cerebellar vermis, apnea-hyperpnea neonatal episodic, abnormal eye movements, ataxia and mental retardation. One of them also showed occipital meningoencephalocele. Case reports. Four adult individuals affected by the disease are described. All of them biological siblings, within 24-35 years old, and presenting the 'sign of the molar' midbrain in their clinical and neuroimaging. It is an entity characteristic, with the image formed by agenesis or hypoplasia of the cerebellar vermis, superior cerebellar peduncle narrow, flatten, thickened and elongated with a lack of decussation and deep interpeduncular fossa at the level of the isthmus and upper bridges. Conclusions. This study shows the need for early disease diagnosis to ensure proper monitoring, therapeutic approach and family genetic counseling, as well as the role of the cerebellum in cognitive functions and intelligence development.

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Participación del cerebelo en la regulación del afecto, la emoción y la conducta

Cited by 7 publications

References 69 publications

The cerebellar topography of attention sub-components in spinocerebellar ataxia type 2

The cerebellar topography of attention sub-components in spinocerebellar ataxia type 2

Social and Cultural Elements Associated with Neurocognitive Dysfunctions in Spinocerebellar Ataxia Type 2 Patients

Síndrome de Joubert: a propósito de cuatro hermanos adultos afectados

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