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ObjectiveFunctional dizziness syndromes are among the most common diagnoses made in patients with chronic dizziness, but their underlying neural characteristics are largely unknown. The aim of this neuroimaging study was to analyze the disease‐specific brain changes in patients with phobic postural vertigo (PPV).MethodsWe measured brain morphology, task response, and functional connectivity in 44 patients with PPV and 44 healthy controls.ResultsThe analyses revealed a relative structural increase in regions of the prefrontal cortex and the associated thalamic projection zones as well as in the primary motor cortex. Morphological increases in the ventrolateral prefrontal cortex positively correlated with disease duration, whereas increases in dorsolateral, medial, and ventromedial prefrontal areas positively correlated with the Beck depression index. Visual motion stimulation caused an increased task‐dependent activity in the subgenual anterior cingulum and a significantly longer duration of the motion aftereffect in the patients. Task‐based functional connectivity analyses revealed aberrant involvement of interoceptive, fear generalization, and orbitofrontal networks.InterpretationOur findings agree with some of the typical characteristics of functional dizziness syndromes, for example, excessive self‐awareness, anxious appraisal, and obsessive controlling of posture. This first evidence indicates that the disease‐specific mechanisms underlying PPV are related to networks involved in mood regulation, fear generalization, interoception, and cognitive control. They do not seem to be the result of aberrant processing in cortical visual, visual motion, or vestibular regions.

No existing medication has yet been shown to convincingly improve cerebellar ataxia. Therefore, the identification of new drugs for its symptomatic treatment is desirable. The objective of this case series was to evaluate the efficacy of treatment of cerebellar ataxia with the amino acid acetyl-dl-leucine (Tanganil). Thirteen patients (eight males, median age 51 years) with degenerative cerebellar ataxia of different etiologies (SCA1/2, ADCA, AOA, SAOA) were treated with acetyl-dl-leucine (5 g/day) without titration for 1 week. Motor function was evaluated by changes in the Scale for the Rating and Assessment of Ataxia (SARA) and in the Spinocerebellar Ataxia Functional Index (SCAFI) during treatment compared to a baseline examination. Quality of life (EuroQol-5D-3L) and side effects were also assessed. Mean total SARA decreased remarkably (p = 0.002) from a baseline of 16.1 ± 7.1 to 12.8 ± 6.8 (mean ± SD) on medication. There were also significant improvements in sub-scores for gait (p = 0.022), speech (p = 0.007), finger-chase (p = 0.042), nose-finger-test (p = 0.035), rapid-alternating-movements (p = 0.002) and heel-to-shin (p = 0.018). Furthermore, patients showed better performance in the SCAFI consisting of the 8-m-walking-time (8 MW, p = 0.003), 9-Hole-Peg-Test of the dominant hand (9HPTD, p = 0.011) and the PATA rate (p = 0.005). Quality of life increased during treatment (p = 0.003). No side effects were reported. In conclusion, acetyl-dl-leucine significantly improved ataxic symptoms without side effects and therefore showed a good risk–benefit profile. These findings need to be confirmed in placebo-controlled trials.

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BackgroundMaking the correct diagnosis of patients presenting with vertigo and dizziness in clinical practice is often challenging.ObjectiveIn this study we examined the performance of the iPad based program medx in the prediction of different clinical vertigo and dizziness diagnoses and as a diagnostic tool to distinguish between them.Patients and methodsThe data collection was done in the outpatient clinic of the German Center of Vertigo and Balance Disorders. The “gold standard diagnosis” was defined as the clinical diagnosis of the specialist during the visit of the patient based on standardized history and clinical examination. Another independent and blinded physician finalized each patient’s case in the constellatory diagnostic system of medx based on an algorithm using all available clinical information. These diagnoses were compared to the “gold standard” by retrospective review of the charts of the patients. The accuracy provided by medx was defined as the number of correctly classified diagnoses. In addition, the probability of being test positive when a disease was present (sensitivity), of being test negative when a disease was absent (specificity), of having the disease when the test is positive (positive predictive value) and of not having the disease when the test is negative (negative predictive value) for the most common diagnoses were reported. Sixteen possible different vertigo and dizziness diagnoses could be provided by medx.ResultsA total of 610 patients (mean age 58.1 ± 16.3 years, 51.2% female) were included. The accuracy for the most common diagnoses was between 82.1 and 96.6% with a sensitivity of 40 to 80.5% and a specificity of more than 80%. When analyzing the quality of medx in a multiclass problem for the six most common clinical diagnoses, the sensitivity, specificity, positive and negative predictive values were as follows: Bilateral vestibulopathy (81.6, 97.1, 71.1, and 97.5%), Menière’s disease (77.8, 97.6, 87.0, and 95.3%), benign paroxysmal positional vertigo (61.7, 98.3, 86.6, and 93.4%), downbeat nystagmus syndrome (69.6, 97.7, 71.1, and 97.5%), vestibular migraine (34.7, 97.8, 76.1, and 88.3%), and phobic postural vertigo (80.5, 82.5, 52.5, and 94.6%).ConclusionThis study demonstrates that medx is a new and easy approach to screen for different diagnoses. With the high specificity and negative predictive value, the system helps to rule out differential diagnoses and can therefore also lead to a cost reduction in the health care system. However, the sensitivity was unexpectedly low, especially for vestibular migraine. All in all, this device can only be a complementary tool, in particular for non-experts in the field.