VEMPs and Dysautonomia Assessment in Definite Cerebellar Ataxia, Neuropathy, Vestibular Areflexia Syndrome (CANVAS): a Case Series Study.

Cerebellar ataxia with neuropathy and vestibular areflexia syndrome (CANVAS) is a recently described slowly progressive ataxia with severe imbalance due to the compromise of three of the four sensory inputs for balance, leaving only vision unaffected. Bilateral vestibulopathy is present but saccular and utricular function, measured by vestibular evoked myogenic potentials (VEMPs), has not been widely studied in these patients. Dysautonomia has been reported but is not among the diagnostic criteria. We performed a database analysis to identify patients evaluated between 2003 and 2019 with probable diagnosis of CANVAS by using key words "bilateral vestibulopathy and/or cerebellar ataxia and/or sensory polyneuropathy." Five out of 842 met all conditions. Patients underwent neurological/neurootological exam, brain MRI, visually enhanced vestibulo-ocular reflex (VVOR) exam by high-speed video-oculography using video-Head Impulse Test (vHIT), VEMPs, neurophysiological studies, and genetic tests to exclude other causes of ataxia. Dysautonomia was addressed by the standardized survey of autonomic symptoms. All patients had clinically definite CANVAS as brain MRI showed vermal cerebellar atrophy, neurophysiological studies showed a sensory neuronopathy pattern (absent sensory action potentials), VVOR was abnormal bilaterally, and genetic tests ruled out other causes of ataxia including SCA 3 and Friedreich ataxia. Patients had at least 3 dysautonomic symptoms, including xerostomia/xerophthalmia (5/5). VEMP results varied among patients, ranging from normal to completely abnormal. We found inconsistent results with VEMPs. The utilization of VEMPs in more CANVAS cases will determine its utility in this syndrome. Dysautonomia may be included in the diagnostic criteria.

The influence of obesity on the assessment of carotid intima-media thickness.

BACKGROUND: The assessment of carotid intima-media thickness (CIMT) may improve cardiovascular risk prediction. The optimal protocol for CIMT measurement is unclear. CIMT may be measured in the common carotid artery (CCA), carotid bifurcation (CB), and internal carotid artery (ICA), but measurements from CB and ICA are more difficult to obtain. We studied the influence of body mass index (BMI) and atheroma plaques on the capacity to obtain CIMT measurements at different carotid sites. METHODS: Using an automatic system, CIMT was measured in 700 subjects aged 45-75, in the near and far walls of CCA, CB, and ICA bilaterally. The presence of atheroma plaques, BMI and vascular risk factors were recorded. RESULTS: CIMT measurements in CCA were possible in all except one subject. It was not possible to obtain CIMT measurements at CB or ICA in 24.1% of normal weight and 58.8% of obese subjects. The likelihood of obtaining CIMT measurement at all carotid sites decreased as the BMI increased. Atheroma plaques in a carotid segment did not preclude CIMT measurement at this site. CONCLUSIONS: CIMT measurements in distal carotid segments are more challenging in obese subjects. Measuring CIMT at CCA remains feasible in obese subjects and should be the primary endpoint in these subjects. Nevertheless, CB and ICA measurements, when feasible, would improve risk classification.

Dynamic posture assessment in patients with peripheral vestibulopathy.

OBJECTIVE: To assess the effects on balance of different strategies to create sensory conflict. MATERIAL AND METHODS: Two different systems of dynamic posturography were compared: computerized dynamic posturography and a static platform on which different visual and support conditions were used. The study was performed in 127 patients with dizziness of a peripheral vestibular origin. RESULTS: When patients stood on a foam support surface, the length and area of the center-of-pressure excursions increased. Interestingly, optokinetic stimulation evoked a longer length and a wider area of the center-of-pressure excursions in patients with a combined vestibular and visual deficit pattern than when they kept their eyes closed. This was opposite to the effect observed in patients with a pattern indicative of a vestibular deficit alone. CONCLUSION: The effects of visual stimulation on balance in patients with unilateral vestibulopathy reflect the type of sensory deficit, and can be considered to be specific to such a deficit.

The cost of stroke.

The control of health expenditure has become one of the main axes of health policy. Cost studies are an indispensable tool for determining the economic impact of disease and for assigning the material and human resources required for the prevention, diagnosis, treatment, and follow-up of patients with different diseases. Cost studies in stroke are necessary due to the increasing incidence of this disease which represents a significant cause of death and invalidity in adults and produces important hospital and social spending, and for which patients must be treated in specialized units (stroke units). The principal cost-determining factors in most studies are those generated by hospital admission (principally hospital stay). Other cost-determining factors include severity, stroke subtype or the fate of patients on discharge. Future health policies aimed at optimizing economic resources must be directed towards reducing hospital stay and minimizing patient disability.