Proprioceptive and tactile processing in individuals with Friedreich ataxia: an fMRI study.

Objective: Friedreich ataxia (FA) neuropathology affects dorsal root ganglia, posterior columns in the spinal cord, the spinocerebellar tracts, and cerebellar dentate nuclei. The impact of the somatosensory system on ataxic symptoms remains debated. This study aims to better evaluate the contribution of somatosensory processing to ataxia clinical severity by simultaneously investigating passive movement and tactile pneumatic stimulation in individuals with FA. Methods: Twenty patients with FA and 20 healthy participants were included. All subjects underwent two 6 min block-design functional magnetic resonance imaging (fMRI) paradigms consisting of twelve 30 s alternating blocks (10 brain volumes per block, 120 brain volumes per paradigm) of a tactile oddball paradigm and a passive movement paradigm. Spearman rank correlation tests were used for correlations between BOLD levels and ataxia severity. Results: The passive movement paradigm led to the lower activation of primary (cSI) and secondary somatosensory cortices (cSII) in FA compared with healthy subjects (respectively 1.1 ± 0.78 vs. 0.61 ± 1.02, p = 0.04, and 0.69 ± 0.5 vs. 0.3 ± 0.41, p = 0.005). In the tactile paradigm, there was no significant difference between cSI and cSII activation levels in healthy controls and FA (respectively 0.88 ± 0.73 vs. 1.14 ± 0.99, p = 0.33, and 0.54 ± 0.37 vs. 0.55 ± 0.54, p = 0.93). Correlation analysis showed a significant correlation between cSI activation levels in the tactile paradigm and the clinical severity (R = 0.481, p = 0.032). Interpretation: Our study captured the difference between tactile and proprioceptive impairments in FA using somatosensory fMRI paradigms. The lack of correlation between the proprioceptive paradigm and ataxia clinical parameters supports a low contribution of afferent ataxia to FA clinical severity.

Cognitive impairment in essential tremor assessed by the cerebellar cognitive affective syndrome scale.

Background: Essential tremor (ET) is a movement disorder characterized by cerebellar neurodegenerative changes. ET is also associated with non-motor symptoms including cognitive impairment. The neuropsychologic profile of a patient with ET could relate to cerebellar cognitive affective syndrome (CCAS). Objective: This study aimed to assess the prevalence of cognitive impairment in patients with ET and identify whether the cognitive impairment in ET corresponds to a CCAS. Methods: Cognitive functions were evaluated with the CCAS-Scale (CCAS-S) in 20 patients with ET and 20 controls matched for age, sex, and level of education. The results of the CCAS-S were compared between patients and controls. The underlying determinant of CCAS inpatients with ET was identified through the correlation between the results of the CCAS-S and age at onset of symptoms, disease duration, and the Essential Tremor Rating Assessment Scale (TETRAS). Results: On a group level, ET patients performed significantly worse than matched controls. In total, 13 individuals with ET had a definite CCAS (CCAS-S failed items ≥ 3). ASO and TETRAS scores significantly correlated with CCAS-S performances in ET patients. Conclusion: CCAS is highly prevalent in patients with ET which supports the cerebellar pathophysiology of associated cognitive impairment and supports a more systematic use of the CCAS-S to cognitively assessed patients with ET.

Anodal Cerebellar Transcranial Direct Current Stimulation Reduces Motor and Cognitive Symptoms in Friedreich's Ataxia: A Randomized, Sham-Controlled Trial.

BACKGROUND: Friedreich Ataxia is the most common recessive ataxia with only one therapeutic drug approved solely in the United States. OBJECTIVE: The aim of this work was to investigate whether anodal cerebellar transcranial direct current stimulation (ctDCS) reduces ataxic and cognitive symptoms in individuals with Friedreich's ataxia (FRDA) and to assess the effects of ctDCS on the activity of the secondary somatosensory (SII) cortex. METHODS: We performed a single-blind, randomized, sham-controlled, crossover trial with anodal ctDCS (5 days/week for 1 week, 20 min/day, density current: 0.057 mA/cm2 ) in 24 patients with FRDA. Each patient underwent a clinical evaluation (Scale for the Assessment and Rating of Ataxia, composite cerebellar functional severity score, cerebellar cognitive affective syndrome scale) before and after anodal and sham ctDCS. Activity of the SII cortex contralateral to a tactile oddball stimulation of the right index finger was evaluated with brain functional magnetic resonance imaging at baseline and after anodal/sham ctDCS. RESULTS: Anodal ctDCS led to a significant improvement in the Scale for the Assessment and Rating of Ataxia (-6.5%) and in the cerebellar cognitive affective syndrome scale (+11%) compared with sham ctDCS. It also led to a significant reduction in functional magnetic resonance imaging signal at the SII cortex contralateral to tactile stimulation (-26%) compared with sham ctDCS. CONCLUSIONS: One week of treatment with anodal ctDCS reduces motor and cognitive symptoms in individuals with FRDA, likely by restoring the neocortical inhibition normally exerted by cerebellar structures. This study provides class I evidence that ctDCS stimulation is effective and safe in FRDA. © 2023 International Parkinson and Movement Disorder Society.

Intracranial Vessel Wall MRI in Cryptogenic Stroke and Intracranial Vasculitis.

INTRODUCTION: Intracranial vasculopathies easily elude classic stroke work-up. We aim in this work to show that vessel wall-MRI could prove an efficient alternative to digital subtraction angiography for the diagnosis of intracranial vasculopathies by identifying intracranial arterial vessel walls anomalies and contrast enhancement, suggestive of angiitis of the central nervous system. MATERIALS AND METHODS: Clinical and imaging characteristics of stroke patients diagnosed with primary angiitis of the central nervous system based on vessel wall-MRI were retrospectively reviewed and the clinical and imaging features of angiitis associated with intracranial vessel walls anomalies and contrast enhancement detailed. RESULTS: Twenty patients were included (mean age was 59 years old). All patients were admitted for focal neurological deficits of sudden onset that were recurrent in 13 subjects. Cognitive impairment, headache and seizures occurred in, respectively, 13, 5, and 2 patients. Cerebrospinal fluid analysis was abnormal in 15 patients. In MRI, FLAIR sequences showed ischemic infarcts in 20 patients and DWI showed acute infarct in 15 patients. Digital subtraction angiography was performed in 11 patients and disclosed proximal and distal multifocal stenosis in 10 patients along distal irregularities in different vascular territories in 7. For all of our patients, VW-MRI revealed a concentric contrast enhancement of arterial walls, localized in multiple vascular territories, suggesting angiitis. Abnormalities on digital subtraction angiography and/or MR-Angiography, and vessel wall-MRI were consistent in all patients. CONCLUSIONS: This report underlies the added value of vessel wall-MRI to the diagnosis of underlying intracranial vasculopathy, particularly primary angiitis of the central nervous system, without the use of invasive endovascular techniques and the yield of vessel wall-MRI in the work-up of cryptogenic stroke.

Are Intensive Cares Worthwhile for Breast Cancer Patients: The Experience of an Oncological ICU.

PURPOSE: One among seven women will present with breast cancer for which major therapeutic advances led to a significant increase in survival and cure rates. During or after cancer treatment, severe complications may occur requiring admission in intensive care unit (ICU). Intensivists could be reluctant for accepting cancer patients in the ICU, and there are very few data about causes of admission and prognosis of patients with breast cancer admitted in the ICU for an acute complication. Our study seeks to determine, in a population of patients with breast cancer, the main causes for ICU admission and the predictors of death during hospital stay and prognostic factors for survival after hospital discharge. METHODS: This retrospective study includes all unplanned ICU admissions of patients with breast cancer in a cancer hospital from January 1, 2009 to December 31, 2014. To search for predictive factors of death during hospitalization, Mann-Whitney or Fisher Exact (or chi-square) tests were used for continuous variables or categorical variables, respectively. A logistic regression model was applied for multivariate analysis. Multivariate analysis of prognostic factors for survival after hospital discharge was performed with a Cox's proportional hazards model. RESULTS: Of 1586 ICU admissions during the study period, 282 (18%) concerned breast cancer of which 175 met the inclusion criteria. The main causes of admission were of cardiovascular (26%), respiratory (19%), neurologic (19%), or infectious (14%) origin. ICU death rate was 15% and, overall, 28% of the patients died during hospitalization. The median survival time after hospitalization was 12.8 months (95% CI: 8.2-20.7). Independent predictors of death during hospitalization were the sequential organ failure assessment (SOFA) score (OR 1.36, 95% CI 1.15-1.60), high GPT values (OR 3.70, 95% CI: 1.52-9.03), and cardiovascular disease (OR 0.23, 95% CI: 0.06-0.86). Independent predictors of death after hospital discharge were metastatic disease (HR 7.90, 95% CI 3.69-16.92), high GOT value (HR 3.22 95% CI: 1.93-5.36), simplified acute physiology score (SAPS) (HR 1.95 95% CI: 1.21-3.16), and therapeutic limitations during the first 24 h after ICU admission (HR 8.52 95% CI: 3.66-19.87). CONCLUSION: Independent predictors of death during hospitalization were related to the acute complications (SOFA score, GPT level and cardiovascular-related admission) while cancer parameters retained their prognostic significance for survival after hospital discharge (metastatic disease, therapeutic limitations).