Ferdinando Rossi Lecture: the Cerebellar Cognitive Affective Syndrome —Implications and Future Directions
AbstractThe notion that the cerebellum is devoted exclusively to motor control has been replaced by a more sophisticated understanding of its role in neurological function, one that includes cognition and emotion. Early clinical reports, as well as physiological and behavioral studies in animal models, raised the possibility of a nonmotor role for the cerebellum. Anatomical studies demonstrate cerebellar connectivity with the distributed neural circuits linked with autonomic, sensorimotor, vestibular, associative and limbic/paralimbic brain areas. Identification of the cerebellar cognitive affective syndrome in adults and ...
Source: The Cerebellum - August 10, 2022 Category: Neurology Source Type: research

Altered Effective Connectivity Among the Cerebellum and Cerebrum in Patients with Major Depressive Disorder Using Multisite Resting-State fMRI
AbstractMajor depressive disorder (MDD) is a serious and widespread psychiatric disorder. Previous studies mainly focused on cerebrum functional connectivity, and the sample size was relatively small. However, functional connectivity is undirected. And, there is increasing evidence that the cerebellum is also involved in emotion and cognitive processing and makes outstanding contributions to the symptomology and pathology of depression. Therefore, we used a large sample size of resting-state functional magnetic resonance imaging (rs-fMRI) data to investigate the altered effective connectivity (EC) among the cerebellum and ...
Source: The Cerebellum - August 6, 2022 Category: Neurology Source Type: research

Respective Involvement of the Right Cerebellar Crus I and II in Syntactic and Semantic Processing for Comprehension of Language
AbstractThe right posterolateral portions of the cerebellum (crus-I/II) are involved in language processing. However, their functional role in language remains unknown. The cerebellum is hypothesized to acquire an internal model that is a functional copy of mental representations in the cerebrum and to contribute to cognitive function. In this research, based on the cerebellar internal model hypothesis, we conducted task-based and resting-state functional magnetic resonance imaging (fMRI) experiments to investigate the role of the cerebellum in the syntactic and semantic aspects of comprehension of sentences. In a syntacti...
Source: The Cerebellum - August 4, 2022 Category: Neurology Source Type: research

Correction to: Episodic Ataxia Type 1: Natural History and Effect on Quality of Life
(Source: The Cerebellum)
Source: The Cerebellum - July 29, 2022 Category: Neurology Source Type: research

Ocular Dysmetria, Flutter, and Opsoclonus: Refining the Oculomotor Deficits in Cerebellar Patients
(Source: The Cerebellum)
Source: The Cerebellum - July 26, 2022 Category: Neurology Source Type: research

The Discovery of Anti-Yo (Anti-PCA1) Antibody in Patients with Paraneoplastic Cerebellar Degeneration: Opening a Window into Autoimmune Neurological Disease
AbstractPrior to 1982, ovarian and certain other cancers were known to have a rare complication of progressive cerebellar ataxia, a disorder characterized pathologically by severe —often total—obliteration of cerebellar Purkinje cells. However, the cause of cerebellar injury in these patients was unknown. In that year, we began studies in which sera from individuals with this disorder were reacted with frozen sections of human cerebellum. These studies revealed that patie nts with ovarian cancer and cerebellar degeneration had high titers of antibodies directed against cytoplasmic antigens of Purkinje cells and deep ce...
Source: The Cerebellum - July 26, 2022 Category: Neurology Source Type: research

Male Predisposition in Cerebellar Mutism Syndrome: a Cohort Study
AbstractThe aim of this study was to explore the association between sex and cerebellar mutism syndrome and to examine other potential risk factors. This ambispective cohort study examined 218 pediatric patients (132 boys) with a posterior fossa tumor who underwent tumor resection from July 2013 to March 2021. The patients ’ demographics and tumor characteristics were examined and statistically analyzed to explore the associations among the variables. Multivariable and subgroup analyses were conducted to validate the independent risk factors for cerebellar mutism syndrome (CMS). The male and female patients did not diffe...
Source: The Cerebellum - July 23, 2022 Category: Neurology Source Type: research

The Cerebellar Gene Database: a Collective Database of Genes Critical for Cerebellar Development
This report presents the first comprehensive database that specifically compiles genes critical for cerebellar development and function. The Cerebellar Gene Database details genes that, when perturbed in mouse models, result in a cerebellar phenotype according to available data from both Mouse Genome Informatics and PubMed, as well as references to the corresponding studies for further examination. This database also offers a compilation of human genetic disorders with a cerebellar phenotype and their associated gene information from the Online Mendelian Inheritance in Man (OMIM) database. By comparing and contrasting the ...
Source: The Cerebellum - July 20, 2022 Category: Neurology Source Type: research

24S-Hydroxycholesterol and Cerebellar Degeneration: Insights from SCA2
(Source: The Cerebellum)
Source: The Cerebellum - July 18, 2022 Category: Neurology Source Type: research

Cerebellar Dysfunction as a Source of Dystonic Phenotypes in Mice
AbstractThere is now a substantial amount of compelling evidence demonstrating that the cerebellum may be a central locus in dystonia pathogenesis. Studies using spontaneous genetic mutations in rats and mice, engineered genetic alleles in mice, shRNA knockdown in mice, and conditional genetic silencing of fast neurotransmission in mice have all uncovered a common set of behavioral and electrophysiological defects that point to cerebellar cortical and cerebellar nuclei dysfunction as a source of dystonic phenotypes. Here, we revisit thePtf1aCre/+;Vglut2flox/flox mutant mouse to define fundamental phenotypes and measures th...
Source: The Cerebellum - July 12, 2022 Category: Neurology Source Type: research

Nothnagel Syndrome
Abstract  The internist Hermann Nothnagel (1841 –1905) took a special interest in the cerebellum. In an early experimental study on rabbits conducted in 1876, he demonstrated the involvement of the vermis in the pathophysiology of motor ataxia. Between 1879 and 1889, he reported four cases of tectal tumors that clinically manifested with bilate ral ophthalmoplegia and unilateral gait ataxia, culminating in theCerebellar Classic highlighted here. Nothnagel attributed this clinical syndrome to lesions of the colliculi ( “quadrigeminal bodies”) and compression of the nuclei of the third cranial nerves, but also left op...
Source: The Cerebellum - July 11, 2022 Category: Neurology Source Type: research

An Exploratory Survey on the Care for Ataxic Patients in the American Continents and the Caribbean
AbstractLittle is known about access of rare disease carriers to health care. To increase this knowledge, the Pan American Hereditary Ataxia Network (PAHAN) conducted an exploratory survey about care for hereditary ataxias in American continents and the Caribbean. A questionnaire was sent to health professionals about the hereditary ataxias identified; access to care; and local teaching and research. The number of ataxics under current care per 100,000 inhabitants was subtracted from the expected overall prevalence of 6/100,000, to estimate the prevalence of uncovered ataxic patients. Local Human Development Indexes (HDI) ...
Source: The Cerebellum - July 7, 2022 Category: Neurology Source Type: research

Emotional and Spontaneous Locomotor Behaviors Related to cerebellar Daidzein-dependent TrkB Expression Changes in Obese Hamsters
AbstractCurrent evidence supports the beneficial role of phytoestrogens in metabolic diseases, but their influences on spontaneous motor and anxiety behaviors plus neuroprotective effects have still not been completely elucidated. With the present study, neuro-behavioral activities were correlated to daidzein (DZ)-dependent expression changes of a high affinity catalytic receptor for several neurotrophins, and namely tropomyosin-related kinase B receptor (TrkB) in the cerebellar cortex of high-fat diet (HFD) hamsters (Mesocricetus auratus). Indeed, these changes appear to be tightly linked to altered plasma lipid profiles ...
Source: The Cerebellum - July 6, 2022 Category: Neurology Source Type: research

Correction to: The Strange Case of the Multiple MRI Phenotypes of RFC1 Mutation
(Source: The Cerebellum)
Source: The Cerebellum - July 5, 2022 Category: Neurology Source Type: research

The Cerebellar Cortex Receives Orofacial Proprioceptive Signals from the Supratrigeminal Nucleus via the Mossy Fiber Pathway in Rats
AbstractProprioceptive sensory information from muscle spindles is essential for the regulation of motor functions. However, little is known about the motor control regions in the cerebellar cortex that receive proprioceptive signals from muscle spindles distributed throughout the body, including the orofacial muscles. Therefore, in this study, we investigated the pattern of projections in the rat cerebellar cortex derived from the supratrigeminal nucleus (Su5), which conveys orofacial proprioceptive information from jaw-closing muscle spindles (JCMSs). Injections of an anterograde tracer into the Su5 revealed that many bi...
Source: The Cerebellum - July 4, 2022 Category: Neurology Source Type: research