Dominik Straumann

Susac syndrome is an extremely rare autoimmune disease which is characterized by encephalopathy, cochleo-vestibular damage resulting in sensorineural hearing loss, and branch retinal artery occlusion (BRAO) leading to visual disturbances. The number of reported cases worldwide since Susac’s first report in 1979 on two patients is about 500. Long-term data on cognitive and audiometric course in Susac syndrome are scarce. We present a case of a 20-year-old male diagnosed with Susac syndrome in 2017, with cognitive and audiometric assessments conducted over spans of 50 and 71 months after initial diagnosis, in conjunction with functional MRI analysis of expressive speech lateralization. At the initial examination, the patient exhibited significant impairments in attention, processing speed, executive function, memory, and visuoconstructional abilities, together with left–right discrimination difficulties. Following monthly cyclophosphamide treatment, rehabilitation, and time, cognitive performance improved substantially across domains, and high-frequency sensorineural hearing loss in the left ear improved toward normal limits. On fMRI, sentence generation showed a leftward bias in the posterior perisylvian cortex whereas word generation was essentially bilateral, indicating that speech-production related lateralization was preserved but not uniformly left-dominant despite extensive callosal lesions. This longitudinal, multimodal case underscores the potential benefits of early diagnosis and treatment and the apparent preservation of speech-production lateralization despite callosal injury.

Background/Objectives: Dizziness is highly prevalent in patients with concussion, with a significant proportion of these patients experiencing visually induced dizziness (VID) in complex visual environments. As current diagnoses rely primarily on subjective questionnaires, establishing objective diagnostic frameworks remains a challenge. This study aimed to quantify responses to optokinetic stimulation to explore potential clinical subtypes based on objective postural and subjective symptom measures. Methods: Concussed patients and healthy controls underwent optokinetic stimulation, with patients stratified into High and Low VVAS groups by the median Visual Vertigo Analogue Scale (VVAS) score. Induced postural metrics and symptoms were statistically compared and modelled to establish a framework for subtype classification of VID. Results: Patients with High VVAS exhibited significantly greater postural instability and higher symptom scores during optokinetic stimulation than healthy controls. While mean induced postural performance and dizziness in patients with Low VVAS were comparable to healthy controls, this patient cohort demonstrated increased heterogeneity in individual responses. Regression modelling successfully distinguished patients with High VVAS from healthy controls, achieving strong diagnostic accuracy for the postural (AUC = 0.815) and symptom-based (AUC = 0.958) models. Using this diagnostic framework, distinct postural and visuoperceptual subtypes were identified within the visual–vestibular mismatch cohort. Conclusions: This study provides a robust framework for post-concussive VID by combining objective postural metrics with subjective induced symptom scales. This classification identifies distinct clinical subtypes and characterises specific manifestations to facilitate tailored rehabilitation programmes. Future research should investigate whether these findings can guide targeted and individualised programmes to optimise rehabilitation.

Background/Objectives: The auditory middle-latency responses (AMLRs) assess central sensory processing beyond the brainstem and serve as a measure of sensory gating. They have clinical relevance in the diagnosis of neurological conditions. In this study, magnitude and habituation of the AMLRs were tested for sensitivity and specificity in classifying dizzy patients with vestibular migraine (VM) and post-concussive syndrome. Methods: Twenty-three healthy individuals, 12 concussion and 26 VM patients were recruited. AMLR were recorded performing five blocks of 200 binaural click-stimulations at 60 dB sensation level with a repetition rate of 6.1 Hz. Reduction in P0, Na and Pa magnitudes between blocks was measured. Group classifications were performed through logistic and multiple regression. Results: Among healthy subjects, a consistent P0 and Na habituation can be observed. Concussed subjects show control-like Na habituation, despite a lower magnitude, while P0 habituation was negligible. VM patients showed poor habituation for all waves. Regression analyses suggest that P0 and Na better distinguish healthy subjects from neurological patients, whereas Pa best distinguishes concussion from VM patients. Conclusions: The results support that AMLR habituation can contribute to unraveling different mechanisms of dizziness due to concussion compared to VM, providing insights that can complement routine diagnostic assessments.

Transcatheter aortic valve implantation (TAVI) is an alternative to open heart surgery in the treatment of symptomatic aortic valve stenosis, which is often the treatment of choice in elderly and frail patients. It carries a risk of embolic complications in the whole cerebral vascular bed, which includes the retinal vasculature. The main objective was the evaluation of retinal emboli visible on optical coherence tomography angiography (OCTA) following TAVI. This is a prospective, single center, observational study enrolling consecutive patients over two years. Patients were assessed pre- and post-TAVI. Twenty-eight patients were included in the final analysis, 82.1% were male, median age was 79.5 (range 52–88), median BCVA was 82.5 letters (range 75–93). Eight patients (28.6%) presented new capillary dropout lesions in their post-TAVI OCTA scans. There was no statistically significant change in BCVA. Quantitative analysis of macular or peripapillary OCTA parameters did not show any statistically significant difference in pre- and post-intervention. In conclusion, capillary dropout lesions could frequently be found in patients after TAVI. Quantitative measurements of macular and peripapillary flow remained stable, possibly indicating effective ocular blood flow regulation within the range of left ventricular ejection fraction in our cohort.

Downbeat nystagmus (DBN) observed in head-hanging positions, may be of central or peripheral origin. Central DBN in head-hanging positions is mostly due to a disorder of the vestibulo-cerebellum, whereas peripheral DBN is usually attributed to canalolithiasis of an anterior semicircular canal. Here, we describe an atypical case of a patient who, after head trauma, experienced severe and stereotypic vertigo attacks after being placed in various head-hanging positions. Vertigo lasted 10–15 s and was always associated with a robust DBN. The provocation of transient vertigo and DBN, which both showed no decrease upon repetition of maneuvers, depended on the yaw orientation relative to the trunk and the angle of backward pitch. On a motorized, multi-axis turntable, we identified the two-dimensional Helmholtz coordinates of head positions at which vertigo and DBN occurred (y-axis: horizontal, space-fixed; z-axis: vertical, and head-fixed; x-axis: torsional, head-fixed, and unchanged). This two-dimensional area of DBN-associated head positions did not change when whole-body rotations took different paths (e.g., by forwarding pitch) or were executed with different velocities. Moreover, the intensity of DBN was also independent of whole-body rotation paths and velocities. So far, therapeutic approaches with repeated liberation maneuvers and cranial vibrations were not successful. We speculate that vertigo and DBN in this patient are due to macular damage, possibly an unstable otolithic membrane that, in specific orientations relative to gravity, slips into a position causing paroxysmal stimulation or inhibition of macular hair cells.

Head injuries can result in substantially different outcomes, ranging from no detectable effect to transient functional impairments to life‐threatening structural lesions. In high‐level international football (soccer) tournaments, on average, one head injury occurs in every third match. Making the diagnosis and determining the severity of a head injury immediately on‐pitch or off‐field is a major challenge for team physicians, especially because clinical signs of a brain injury can develop over several minutes, hours, or even days after the injury. A standardized approach is useful to support team physicians in their decision whether the player should be allowed to continue to play or should be removed from play after head injury. A systematic, football‐specific procedure for examination and management during the first 72 hours after head injuries and a graduated Return‐to‐Football program for high‐level players have been developed by an international group of experts based on current national and international guidelines for the management of acute head injuries. The procedure includes seven stages from the initial on‐pitch examination to the graduated Return‐to‐Football program. Details of the assessments and the consequences of different outcomes are described for each stage. Criteria for emergency management (red flags), removal from play (orange flags), and referral to specialists for further diagnosis and treatment (persistent orange flags) are provided. The guidelines for return to sport after concussion‐type head injury are specified for football. Thus, the present paper presents a comprehensive procedure for team physicians after a head injury in high‐level football.