Cerebellar Syndrome Research Articles

Cerebellar Transcranial Direct Current Stimulation in the Cerebellar Cognitive Affective Syndrome: A Randomized, Double-Blind, Sham-Controlled Trial.

The cerebellar cognitive affective syndrome (CCAS) encompasses cognitive and affective symptoms in patients with cerebellar disorders, for which no proven treatment is available. Our primary objective was to study the effect of cerebellar anodal transcranial direct current stimulation (tDCS) on cognitive performance in CCAS patients. Secondary effects on ataxia severity, mood, and quality of life were explored. We performed a randomized, double-blind, sham-controlled trial. Thirty-five patients with CCAS were included and received 10 sessions of 20 minutes sham (n = 17) or real (n = 18) tDCS, with a current of 2 mA. Cognitive performance was assessed using executive function subtests of the computerized Test of Attentional Performance (TAP), with the composite as primary endpoint. Secondary outcomes were ataxia severity, mood, and quality of life. Outcomes were evaluated 1, 3, 6, and 12 months post-intervention. Cerebellar tDCS was well tolerated and no serious adverse events related to the intervention occurred. No significant tDCS effect was found on cognitive performance. Improvement on the TAP was observed in the sham group 1 month post-treatment (estimate = -0.248, 95% CI, -0.49 to -0.01), but not clinically relevant. A positive tDCS effect was observed for ataxia severity 1 month post-treatment (estimate = -0.985, 95% CI, -1.94 to -0.03). Ten sessions of 20 minutes cerebellar anodal tDCS did not prove efficacious for CCAS-related cognitive impairment, but a significant positive effect of tDCS was found for ataxia severity, aligning with previous findings indicative of tDCS as a therapeutic neuromodulation tool in cerebellar disorders. © 2024 The Author(s). Movement Disorders published by Wiley Periodicals LLC on behalf of International Parkinson and Movement Disorder Society.

Paraneoplastic Neurologic Syndromes Associated With Merkel Cell Carcinoma.

To define the clinical and immunologic profile of patients with paraneoplastic neurologic syndromes (PNSs) associated with Merkel cell carcinoma (MCC). Retrospective analysis was conducted on patients with suspected MCC-related PNS assessed at the French Reference Center, and cases were identified by a systematic review of the literature (MEDLINE, Embase) following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. A total of 17 patients were identified in our center and 30 in the systematic review, resulting in an overall cohort of 47 patients. The median age was 65 years (range 41-90), and 30 of 46 (65%) were men. Lambert-Eaton myasthenic syndrome (LEMS) (14/47, 29%), rapidly progressive cerebellar syndrome (11/47, 23%), and encephalomyelitis (EM) (8/47, 17%) were the most common associated clinical phenotypes. The most frequently associated neural antibodies (Abs) were voltage-gated calcium channel (VGCC)-Abs (14/45, 31%), followed by Hu-Abs (8/45, 17%) and neurofilament (NF)-Abs (8/45, 17%). Patients with NF-Abs only exhibited CNS disorders (8/8, 100%) and often had antibodies against >1 NF subunit (6/8, 75%). At onset, 26 of 43 patients (60%) had no identifiable primary skin tumor but had lymph node metastasis; these patients were more frequently men (21/26, 80%, vs 7/17, 41%; p = 0.007), had more frequently VGCC-Abs (12/26, 46%, vs 2/17, 11%, p = 0.02) predominantly among those with LEMS, and presented reduced mortality than patients with a known primary tumor (5/25, 20%, vs 8/15, 53%; p = 0.02). MCC-related PNSs present as a heterogeneous clinical spectrum including central and/or peripheral nervous system disorders such as LEMS, RCPS, and EM, mainly associated with VGCC-Abs, NF-Abs, and Hu-Abs. NF-Abs were only seen among patients with CNS disorders. At onset, the absence of a primary skin tumor but presence of lymph node metastasis is frequently observed, and this particular clinical presentation is linked to reduced mortality, highlighting distinctive clinical and immunologic features of MCC-related PNS.

Primary and metastatic cerebral Ewing’s sarcoma: A case report about a rare entity and literature review

Background: Ewing’s sarcoma (ES) is a rare malignant tumor primarily affecting young individuals, with cranial localization being particularly uncommon. While intracranial metastatic ES is infrequent, only four cases of intracranial metastatic ES are reported in the literature; it presents unique diagnostic and therapeutic challenges. Case Description: We present a distinctive case of ES to delineate its clinical, radiological, and histopathological characteristics. Our patient, a 33-year-old, manifested symptoms of intracranial hypertension and gait disturbance. Neurological examination revealed a static and kinetic cerebellar syndrome. Imaging studies and stereotactic biopsy confirmed the diagnosis of primary and metastatic cerebral ES. The treatment regimen encompassed chemotherapy and radiation therapy. Conclusion: Our case underscores the importance of considering ES in the differential diagnosis of dural-based lesions exhibiting cystic components and heterogeneous contrast enhancement, particularly in young individuals. Early recognition and intervention hold promise for optimizing patient outcomes.

Seminoma Resulting in Rapidly Progressive Cerebellar Syndrome with Associated Dual Positive Kelch-like Protein 11 (KLHL11) and Leucine Zipper 4 (LUZP4)–immunoglobulin G (IgG) Antibodies

Seminoma Resulting in Rapidly Progressive Cerebellar Syndrome with Associated Dual Positive Kelch-like Protein 11 (KLHL11) and Leucine Zipper 4 (LUZP4)–immunoglobulin G (IgG) Antibodies

Current Overview of Spinocerebellar Ataxia Type 7 in Mexican Population: Challenges in Specialized Care for a Rare Disease.

Spinocerebellar ataxia type 7 (SCA7) is a rare genetic disease characterized by progressive cerebellar syndrome and macular degeneration. In a previous study, we clinically and genetically characterized a group of Mexican patients, which represented one of the largest cohorts of SCA7 patients worldwide and demonstrated that all patients had a unique genetic origin. Our laboratory developed a program for the diagnosis, medical care, and long-term follow-up of these patients living in Veracruz State, and in this report, we present an update to this research, covering 2013 to 2024. So far, we identified 172 SCA7 carriers, with a few cases outside Veracruz, and our data support that the length of the CAG repeat tract mainly determines disease severity and life expectancy, and accordingly, we define three different phenotypes, early-onset (EO), classical-onset (CO), and late-onset (LO), with EO patients showing the lowest life expectancy. Furthermore, we found that parental transmission of mutant alleles leads to increased CAG repeat instability, compared to maternal ones. Interestingly, a haplotype analysis revealed that patients outside Veracruz may have different genetic origins. In conclusion, longitudinal observations of SCA7 patients provide insight into the natural history of SCA7 and help to design strategies for diagnosis, genetic counseling, physical rehabilitation, and therapeutic alternatives.

Age-Related Features of Clinical and Neurological Characteristics and Pathogenetic Therapy in Patients with Multiple Sclerosis

INTRODUCTION: Multiple sclerosis (MS) is a neurological demyelinating disease of the central nervous system manifested by pronounced heterogeneous symptoms. Peculiarities of the onset, progression rate, and severity of syndromes are different in different age groups. An increase in the frequency of exacerbations, high degree of disability of patients in different age groups evidence insufficient effectiveness of the conducted pathogenetic therapy. Due to a wide choice of multiple sclerosis disease modifying drugs (MSDMDs), it is important to study the clinical and neurological characteristics of patients with MS in different age groups with the aim of further developing an individual approach to treatment. AIM: To study clinical and neurological features and effectiveness of pathogenetic therapy of MS in patients of different age groups. MATERIALS AND METHODS: A prospective analysis of the data of neurological examination and results of magnetic resonance imaging of 100 patients of different age groups with relapsing-remitting MS, who received outpatient treatment in Kursk Regional Multidisciplinary Clinical Hospital, was conducted in the period of stable remission. RESULTS: The leading syndromes in patients with relapsing-remitting course of MS were cerebellar (78%) and pyramidal (70%) syndromes, besides, dysfunction of the cranial nerves (71%) was also observed. A quantitative parameter of explicitness of pelvic disorder syndrome predominated in middle-aged and elderly patients compared to young ones (p 0.001). Cognitive disorders were more often (14%) recorded in middle-aged and elderly patients (p 0.001). The use of glatiramer acetate permitted to reduce the degree of disability in young subjects (p 0.05); in patients of the middle age group, the most effective was a timely transition from interferon beta to ocrelizumab (p 0.05). The use of first-line MSDMDs (interferon-beta) in elderly patients demonstrated low effectiveness (p 0.05). CONCLUSION: In middle-aged and elderly patients with equal timing of MS onset, the neurological deficit is more expressed than in young ones. First-line pathogenetic therapy, in particular, glatiramer acetate, is more effective in young patients, while patients of middle age are recommended an early transition to second-line MSDMDs.

Proton pump inhibitor-induced hypomagnesemia, a rare cause of reversible delirium: A case report with literature review.

Hypomagnesemia is associated with multiple electrolyte disturbances such as hypokalemia, hypocalcemia and hypoparathyroidism. Proton pump inhibitors (PPIs) are widely used in gastrointestinal disorders and are generally considered safe by clinicians. However, it is unusual side effect of hypomagnesemia is potentially under-recognized. Delirium is usually thought to be a clue of cerebrovascular disease, and the association between delirium and hypomagnesemia is unexpected. We describe a patient used PPI with hypomagnesemia showed normal parathyroid hormone (PTH) despite hypocalcemia and reversible delirium. To enhance clinicians' vigilance, we performed a literature review on cerebellar syndromes due to hypomagnesemia. A 74-year-old woman was admitted to our hospital with intermittent nausea, vomiting, hand tremors, and delirium. Laboratory analysis showed hypokalemia, hypomagnesemia, and normal parathyroid hormone despite hypocalcemia, physical examination showed horizontal nystagmus and the brain MRI was negative. Surprising, detailed medical history revealed that the etiology was the usage of omeprazole. Omeprazole was discontinued and oral supplementation with magnesium, calcium, and potassium was administered. Delirium quickly disappeared and the serum potassium, magnesium, and calcium levels gradually normalized; at discharge, nystagmus gradually disappeared, and plasma electrolyte levels were stable at follow-up. Hypomagnesemia is associated with a variety of neurological symptoms up to life-threatening conditions if left untreated; as Mg is not present in routine electrolyte panels, hypoparathyroidism, hypokalemia, and delirium may be a clue, and physicians must be alert to consider PPI as a potential cause of unexplained hypomagnesemia, and timely treatment to avoid sequelae.

B-076 Detection of KLHL11 Autoantibodies Using a New Cell-based Indirect Immunofluorescence Assay

Abstract Background Autoantibodies against kelch-like protein 11 (KLHL11) were first described in 2019 as markers of paraneoplastic encephalitis. Early diagnosis and treatment are imperative to improve prognosis and outcome of affected patients. Rat brain immunohistochemistry was reported to be not useful in routine screening for KLHL11 antibodies. This study evaluates the performance of a new recombinant cell-based assay for the standardized detection of KLHL11-specific IgG and reports the characteristics of the examined patients. Methods Serum (n=9), CSF (n=5) and/or plasmapheresis (n=1) samples from 10 patients with clinical presentations compatible with anti-KLHL11 encephalitis as well as sera from 100 healthy blood donors were analyzed using a prototype indirect immunofluorescence assay (IFA; EUROIMMUN) based on recombinant HEK293 cells expressing human KLHL11. Results KLHL11 autoantibodies were detected at high titers in all samples (serum >1:1,000; CSF >1:320; plasma >1:100,000) from all (10/10) patients but in none (0/100) of the blood donors, indicating 100% sensitivity and specificity. Anti-KLHL11-positive patients had a median age of 52 years (range 27-71) and 80% were male. The most common reported clinical presentations were cerebellar syndrome (ataxia), brainstem diencephalic encephalitis, rhombencephalitis and limbic encephalitis. Testicular or ovarian tumors were found in 87.5% (7/8) of the cases with available results from malignancy screening. Conclusions The new cell-based IFA enables the sensitive and specific detection of KLHL11 autoantibodies, thus supporting the diagnosis of patients with predominantly paraneoplastic brainstem cerebellar syndrome. Future studies will evaluate assay performance in larger patient cohorts to address the reported association of KLHL11 autoimmunity with a wider spectrum of syndromes and tumors.

Acute Cerebellitis Following COVID-19: Alarming Clinical Presentation Challenged by Normal Paraclinical Findings.

We report the case of an acute cerebellitis following COVID-19 in 32-year-old man who presented with a life-threatening critical cerebellar syndrome contrasting with normal paraclinical findings. Despite this fulminant critical presentation, the patient fully recovered in 37days after early treatment with high-dose steroids and intravenous immunoglobulins. This case highlights the need for clinicians to be aware of acute cerebellitis following COVID-19, despite normal laboratory, imaging and electroencephalography findings and the importance to start appropriate treatment as soon as possible.

CACNA1G Causes Dominantly Inherited Myoclonus-Ataxia with Intellectual Disability: A Case Report.

Spinocerebellar ataxias (SCAs) are characterized by substantial phenotypic variability. Among them, SCA42 is a rare non-expansion entity presenting with slowly progressive cerebellar syndrome but whose clinical spectrum may be also wider. A 53-year-old male presented with progressive myoclonus-ataxia and intellectual disability. Genetic screening revealed a novel c.3835G > A (p. Asp1279Asn) variant in the CACNA1G gene. SCA42 is a rare non-expansion SCA caused by mutations in CACNA1G on chromosome 17q21, encoding the Ca(V)3.1, a low-threshold voltage-gated T-type calcium channel. The novel variant we identified is potentially involved in channel activity. This case expands the knowledge regarding CACNA1G-associated phenotype and highlights the importance of genetic screening in myoclonus-ataxia disorders.

Predicting cerebellar mutism syndrome in children using lesion map combined with clinical features.

This study aimed to develop a predictive model for cerebellar mutism syndrome (CMS) in pediatric patients with posterior fossa tumors, integrating lesion-symptom mapping (LSM) data with clinical factors, and to assess the model's performance. A cohort of pediatric patients diagnosed with posterior fossa tumors and undergoing surgery at Beijing Children's Hospital from July 2013 to December 2023 was analyzed. Clinical variables gender, age at surgery, tumor characteristics, hydrocephalus, surgical route and pathology were collected. LSM was used to link tumor locations with CMS outcomes. Lasso regression and logistic regression were employed for feature selection and model construction, respectively. Model performance was assessed using area under the curve (AUC) and accuracy metrics. The study included 197 patients in total, with CMS rates consistent across training, validation, and prospective groups. Significant associations were found between CMS and gender, tumor type, hydrocephalus, paraventricular edema, surgical route, and pathology. A predictive model combining voxel location data from LSM with clinical factors achieved high predictive performance (C-index: training 0.956, validation 0.933, prospective 0.892). Gender, pathology, and voxel location were identified as key predictors for CMS. The study established an effective predictive model for CMS in pediatric posterior fossa tumor patients, leveraging LSM data and clinical factors. The model's accuracy and robustness suggest its potential utility in clinical practice for early CMS risk assessment and intervention planning.

Cerebellar cognitive affective syndrome and vertebrobasilar ischemia. From cerebello-cerebral diaschisis to "dysmetria of thought"

Cerebellum, along with it' s role in coordinating motor functions, exercises a significant regulatory influence in fields of cognitive and affective functions. Therefore, studying the effect of cerebrovascular atherosclerotic pathology on mood and cognition should not be limited to stenotic dysfunctions of carotid arteries, but also extend its methodological framework to the consideration of the integrity of vertebrobasilar system (VBS), cerebellar perfusion and posterior cerebral circulation in general, as it has not been yet sufficiently addressed whether VBS insufficiency is associated with deterioration of patients' mental and emotional status and quality of life (QoL). Vertebrobasilar circulatory dysfunction has been pointed out, since decades, as a cause of progressive memory impairment and dementia, due to multiple infarcts in cerebral areas which are topographically critical for mental and emotional functions. Indicative of the pathophysiological and anatomic-functional association of VBS with these neuro-psychiatric domains are cerebellar cognitive-affective syndrome (CCAS) and crossed cerebello-cerebral diaschisis (CCCD). Mental and psychiatric components of CCAS, along with ataxic motor disability, constitute the conceptual hypothesis of "dysmetry of thought", while diagnostic significance of mental dysfunctions and psychopathological manifestations, in terms of symptoms preceding motor impairments that ascribe cerebellar malfunction in the epicenter of their pathophysiology, such as cerebellar ataxias, in which, early recognition of CCAS may facilitate therapeutic interventions aimed at improving QoL, reveal that cerebellar pathology, either of degenerative etiology or vascular substrate on the ground of vertebrobasilar insufficiency (VBI) or other surgical conditions of the posterior fossa, is associated with deterioration of patients' QoL which is related to significant impairments in their cognitive functions with (co)manifested emotional disorders. Studies in animal models also support these conclusions. Since VBI is responsible for a wide range of psychiatric and neurological symptoms, new findings concurred with current indications advocating that, without consideration of VBS disorders, it is impossible to clarify the connection of cerebral perfusion dysfunctions to neurocognitive deficits. The inclusion of cerebellar perfusion disorders in scientific research and clinical approaches to cognitive and affective disorders that may occur in patients with cerebrovascular lesions constitutes a paradigm of best clinical practices implementation and interdisciplinary convergence of neurosciences and vascular medicine.

Thorough perioperative and anesthetic considerations for cerebellar syndrome induced by posterior fossa tumors

Posterior fossa tumors are relatively rare pathologies, predominantly observed in pediatric populations. Nevertheless, there have been documented cases in adults, where these tumors are often associated with a higher prevalence of vascular lesions compared to those found in children. Given the inherent high mortality risk associated with this condition, meticulous anesthetic management is paramount. A comprehensive evaluation of patient-specific factors, including existing comorbidities, hemodynamic stability, and neurological status, is essential. Moreover, perioperative considerations such as patient positioning, the choice of anesthetic technique, and the duration of the surgical procedure must be carefully planned to optimize outcomes. This article presents a case review of a young adult patient with no significant medical history who was diagnosed with a posterior fossa tumor. The discussion encompasses the anatomical considerations of this pathology, critical preoperative assessments, anesthetic strategies tailored to this surgical context, and an analysis of the patient’s postoperative course.

Decoding Multiple Antibody Positivity: Lessons from Paraneoplastic Sensory Ataxia.

Paraneoplastic neurologic syndromes are cancer-associated, immune-mediated neurologic manifestations that may involve any part of the nervous system. They usually present with characteristic neurologic features and should be considered in high-risk phenotypes such as limbic encephalitis, encephalomyelitis, rapidly progressive cerebellar syndrome, opsoclonus-myoclonus, sensory neuronopathy, enteric neuropathy, and Lambert-Eaton myasthenic syndrome. The diagnosis is made by antibody positivity in the serum or cerebrospinal fluid, in the presence of an appropriate clinical phenotype. Findings on antibody testing by immunoblot should always be verified by immunofluorescence. We report a rare case of sensory neuronopathy with triple paraneoplastic antibody positivity (anti-Hu, anti-collapsing response-mediator protein 5, and anti-amphiphysin) on immunoblot but only anti-Hu positivity on immunofluorescence. The presence of lower facial dyskinesias should raise the possibility of an immune-mediated neurologic syndrome in the appropriate clinical context.

Association of Neurofibromatosis Type 1 and Multiple Sclerosis: A Case Report

Neurofibromatosis type 1 (NF1) is an autosomal dominant neurocutaneous disorder, while multiple sclerosis (MS) is a prevalent chronic inflammatory demyelinating disease of the central nervous system. The coexistence of these two conditions is rare. Thus, patients with NF1 may exhibit signal abnormalities on brain imaging, referred to as focal areas of signal intensity (FASI), which can mimic lesions seen in MS and complicate the differential diagnosis. We present the case of a female patient suffering from progressive paraparesis, accompanied by urinary disorders and coordination disorders of the upper limbs. Neurological examination revealed a pyramidal syndrome and a cerebellar syndrome, with an Expanded Disability Status Scale (EDSS) score of 7.5. Additionally, the skin examination showed multiple café-au-lait spots larger than 15 mm and numerous diffuse neurofibromas. Brain magnetic resonance imaging showed white matter lesions typical of MS, and isoelectrofocalization of cerebrospinal fluid proteins revealed positive oligoclonal bands. The diagnosis was primary progressive multiple sclerosis associated with neurofibromatosis type 1. The aim of this case report is to document the association of these two conditions and discuss the challenges in differential diagnosis and treatment, as early diagnosis of MS is crucial to prevent further disability in patients with NF1.

Natural history of non-polyglutamine CACNA1A disease in Austria.

Non-polyglutamine CACNA1A variants underlie an extremely variable phenotypic spectrum encompassing developmental delay, hemiplegic migraine, epilepsy, psychiatric symptoms, episodic and chronic cerebellar signs. We provide our experience with the long-term follow-up of CACNA1A patients and their response to interval therapy. Patients with genetically confirmed non-polyglutamine CACNA1A disease were prospectively followed at the Center for Rare Movement Disorders of the Medical University of Innsbruck from 2004 to 2024. We recruited 41 subjects with non-polyglutamine CACNA1A disease, of which 38 (93%) familial cases. The mean age at the first examination was 35 ± 22years. Disease onset was in the childhood/adolescence in 31/41 patients (76%). Developmental delay and episodic symptoms were the first disease manifestation in 9/41 (22%) and 32/41 (78%) patients respectively. Chronic neurological signs encompassed a cerebellar syndrome in 35/41 (85%), which showed almost no progression during the observation period, as well as cognitive deficits in 9/20 (45%, MOCA test score < 26), psychiatric and behavioral symptoms in 11/41(27%). Seizures occurred in two patients concomitant to severe hemiplegic migraine. At the last visit, 27/41 patients (66%) required an interval prophylaxis (including acetazolamide, flunarizine, 4-aminopyridine, topiramate), which was efficacious in reducing the frequency and severity of episodic symptoms in all cases. In one patient in his 70ies with progressively therapy resistant hemiplegic migraine, treatment with the anti-CGRP antibody galcanezumab successfully reduced the frequency of migraine days from 4 to 1/month. Non-polyglutamine CACNA1A disease show an evolving age-dependent presentation. Interval prophylaxis is effective in reducing the burden of episodic symptoms.

Whole genome joint analysis reveals ATM:C.1564_1565del variant segregating with Ataxia-Telangiectasia and breast cancer

ATM gene is implicated in the development of breast cancer in the heterozygous state, and Ataxia-telangiectasia (A-T) in a homozygous or compound heterozygous state. Ataxia-telangiectasia (A-T) is a rare cerebellar ataxia syndrome presenting with progressive neurologic impairment, telangiectasia, and an increased risk of leukemia and lymphoma.Although the role of ATM, separately, in association with A-T and breast cancer is well documented, there is a limited number of studies investigating ATM variants when segregating with both phenotypes in the same family. Here, using joint analysis and whole genome sequencing, we investigated ATM c.1564_1565del in a family with one homozygous member presenting with A-T (OMIM # 208900) and three heterozygous members, of whom one had breast cancer (OMIM #114480). To our knowledge, this is the first study of ATM c.1564_1565del segregation with both A-T and breast cancer phenotypes within the same kindred. This study highlights the need for a comprehensive genomic approach in the appropriate cancer risk management of heterozygote carriers of ATM in families with A-T.

A Comparative Study on Cognitive Assessment in Cerebellar and Supratentorial Stroke.

This study aims to understand the cognitive profiles of cerebellar infarction patients and compare them to those with supratentorial infarctions, particularly frontal infarctions. This current study also aims to find reliable assessment tools for detecting cognitive impairment in cerebellar infarction patients. A total of fifty cerebellar infarction patients, sixty supratentorial infarction patients, and thirty-nine healthy controls were recruited. The Mini-Mental State Examination (MMSE), Montreal Cognitive Assessment (MoCA), Addenbrooke's Cognitive Examination III (ACE-III), and Cerebellar Cognitive Affective Syndrome scale (CCAS-s) were used to assess global cognitive function. An extensive neuropsychological assessment battery was also tested to evaluate the characteristics of each cognitive domain. To assess the features of cognitive function, a comprehensive neuropsychological evaluation tool was also utilized. The cerebral infarction patients demonstrated cognitive impairment comparable to those with frontal infarcts, notably characterized by disturbance in attention and executive function. However, the degree of cognitive impairment was comparatively milder in cerebellar infarction patients. Furthermore, the patients in the cerebellar group had worse scores in the ACE-III and CCAS-s compared to healthy controls. The two assessments also demonstrated a significant area under the curve values, indicating their effectiveness in distinguishing cognitive impairment in cerebellar infarctions. In conclusion, cognitive impairment in a cerebellar infarction resembles frontal lobe dysfunction but is generally mild. It can be accurately assessed using the ACE-III and CCAS-s scales.

Reliability and validity study of the Chinese version of the Cerebellar Cognitive Affective Syndrome Scale in patients with cerebellar injury.

To preliminarily investigate the reliability and validity of the Chinese version of the Cerebellar Cognitive Affective Syndrome Scale (CCAS scale) in the cerebellar injury population. In this study, 40 patients with cerebellar injury and 39 normal individuals hospitalized in a stroke center were assessed using the Chinese version of the CCAS scale A, MMSE, and PHQ2, and the results were analyzed using content validity, structural validity, internal consistency, inter- rater agreement, and test-retest reliability. The correlation coefficients of semantic fluency, phonemic fluency, category switching, digit span forward, digit span backward, cube, verbal recall, similarities and Go No-Go subscores in the Chinese version of the CCAS scale A were 0.586-0.831 (P ≤ 0.05) with the total score, but there was no significant correlation between the affect and the total score (P = 0.110). The total cognitive score of the Chinese version of the CCAS scale A was correlated with the (r = 0.807, P ≤ 0.01), and the total score of the Chinese version of the CCAS scale A affect was correlated with the total score of PHQ2 (r = 0.884, P ≤ 0.01). The 2 factors were extracted using principal component analysis, and the cumulative variance contribution rate was 59.633%. The factor loadings of each of the corresponding factors were > 0.5, indicating good structural validity of the Chinese version of the CCAS scale A. Cronbach α = 0.827 indicated good internal consistency, and inter-rater reliability (ICC > 0.95) and test-retest reliability (ICC = 0.717-0.895)indicated that the Chinese version of the CCAS scale A had good inter-rater reliability and test-retest reliability. The Chinese version of the CCAS scale A has good reliability and validity in the cerebellar injury population and is useful for screening cerebellar cognitive-emotional syndrome.

Cognitive impairment in young adults following cerebellar stroke: Prevalence and longitudinal course

IntroductionCognitive impairment is a well-known result of a stroke, but for cerebellar stroke in young patients detailed knowledge on the nature and extent of cognitive deficits is limited. This study examined the prevalence and course of cognitive impairment in a large cohort of patients with cerebellar stroke. MethodsSixty young (18–49 years) cerebellar stroke patients completed extensive neuropsychological assessments in the subacute (<9 months post-stroke) and/or chronic phase (≥9 months post-stroke). Performance and course were assessed using standardized scores and Reliable Change Index analyses. Associations between cognitive deficits and lesion locations were explored using subtraction analyses, and associations with subjective cognitive complaints and fatigue were examined. ResultsSixty patients (52% male) were included with a mean age at event of 43.1 years. Cognitive impairment was observed in 60.3% of patients in the subacute phase and 51.2% during the chronic phase. Deficits were most frequent for visuo-spatial skills and executive functioning (42.5–54.6%). Both improvement and decline were observed over time, in 17.9% and 41.0% of participants, respectively. Cognitive deficits seem to be associated with lesions in certain cerebellar regions, however, no distinct correlation was found for a specific subregion. Subjective cognitive complaints were present in the majority of participants (61–80.5%) and positively correlated with fatigue in both phases (ρ = −.661 and ρ = −.757, p < .001, respectively). DiscussionCognitive impairment in cerebellar stroke patients is common, with deficits most pronounced for visuo-spatial skills and executive functioning, as in line with the Cerebellar Cognitive Affective Syndrome. The course of cognitive performance was heterogenous, with cognitive decline despite the fact that no recurrent strokes occurred. No clear association between lesion location and cognitive deficits was observed. Subjective cognitive complaints and fatigue were prevalent and positively correlated. Clinicians could use this information to actively screen for and better inform patients about possible cognitive sequalae.