Apheresis treatment in autoimmune neurological diseases: Predictors of good clinical outcome and success of follow-up therapy with B-cell depletion.

OBJECTIVE: Apheresis treatment (AT) is an established standard of treatment in various neurological autoimmune diseases. Since not all patients equally benefit from AT, we saw the need to investigate the effect of different clinical, paraclinical and technical-apparative factors on the clinical outcome. Additionally, we wanted to find out whether patients who improved due to AT continue to be clinically stable under B-cell depletion (BCD). METHODS: We screened all patients (n = 358) with neurological diseases who received AT at the Medical center of the University of the Saarland in the past 20 years. Different factors (e.g., age, sex, duration until onset of AT, type of AT, number of cycles, csf parameters) were analyzed retrospectively. Clinical disability was measured using the modified Rankin scale (mRS), visual acuity and the Expanded Disability Status Scale (EDSS). RESULTS: 335 patients, categorized into 11 different autoimmune diagnosis groups, received a total of 2669 treatment cycles and showed a statistically significant improvement in mRS with AT (p < 0.001). Patients in American Society for Apheresis (ASFA) categories I (p = 0.013) and II (p = 0.035) showed a significantly greater benefit under AT than those in category III. The clinical outcome was better with shorter duration until AT onset, more cycles of AT, and more plasma volume exchanged and the presence of an autoimmune antibody. Patients who initially profited had a significantly more stable course of the disease after 1-Year-BCD (p = 0.039). DISCUSSION: In the present study, we were able to identify various significant factors influencing the outcome of patients due to AT. Furthermore, we could show that patients with a response to AT can benefit from BCD follow-up therapy.

B-mode transorbital ultrasonography for the diagnosis of idiopathic intracranial hypertension: an updated systematic review and meta-analysis.

OBJECTIVES: This systematic review and meta-analysis aimed to evaluate the role of B-mode transorbital ultrasonography (TOS) for the diagnosis of idiopathic intracranial hypertension (IIH) in adults. METHODS: MEDLINE, EMBASE, and Cochrane Central Register of Controlled Trials (CENTRAL) (1966-May 2022) were searched to identify studies reporting ultrasonographic data about the optic nerve sheath diameter (ONSD) and optic disc elevation (ODE) in adults with IIH compared to subjects without IIH. The quality of the included studies was evaluated by the Newcastle-Ottawa Quality. RESULTS: Fifteen studies were included (total of 439 patients). The values of ODE ranged from 0.6 to 1.3 mm in patients with IIH. The values of ONSD ranged from 4.7 to 6.8 mm in IIH patients and from 3.9 to 5.7 mm in controls. In IIH patients, the ONSD was significantly higher compared to controls (standardized mean difference: 2.5 mm, 95% confidence interval (CI): 1.6-3.4 mm). Nine studies provided data about the presence of papilledema and the pooled prevalence was 95% (95% CI, 92-97%). CONCLUSIONS: In adults, the thickness of ONSD and the entity of ODE were significantly associated with IIH. B-mode TOS enables to noninvasively detect increased ICP and should be performed, potentially routinely, in any patient with suspected IIH.

Development of a Deep Learning-Based System for Optic Nerve Characterization in Transorbital Ultrasound Images on a Multicenter Data Set.

OBJECTIVE: Characterization of the optic nerve through measurement of optic nerve diameter (OND) and optic nerve sheath diameter (ONSD) using transorbital sonography (TOS) has proven to be a useful tool for the evaluation of intracranial pressure (ICP) and multiple neurological conditions. We describe a deep learning-based system for automatic characterization of the optic nerve from B-mode TOS images by automatic measurement of the OND and ONSD. In addition, we determine how the signal-to-noise ratio in two different areas of the image influences system performance. METHODS: A UNet was trained as the segmentation model. The training was performed on a multidevice, multicenter data set of 464 TOS images from 110 subjects. Fivefold cross-validation was performed, and the training process was repeated eight times. The final prediction was made as an ensemble of the predictions of the eight single models. Automatic OND and ONSD measurements were compared with the manual measurements taken by an expert with a graphical user interface that mimics a clinical setting. RESULTS: A Dice score of 0.719 ± 0.139 was obtained on the whole data set merging the test folds. Pearson's correlation was 0.69 for both OND and ONSD parameters. The signal-to-noise ratio was found to influence segmentation performance, but no clear correlation with diameter measurement performance was determined. CONCLUSION: The developed system has a good correlation with manual measurements, proving that it is feasible to create a model capable of automatically analyzing TOS images from multiple devices. The promising results encourage further definition of a standard protocol for the automatization of the OND and ONSD measurement process using deep learning-based methods. The image data and the manual measurements used in this work will be available at 10.17632/kw8gvp8m8x.1.

Transorbital sonography and MRI reliability to assess optic nerve sheath diameter in idiopathic intracranial hypertension.

BACKGROUND AND PURPOSE: The purpose of this study was to evaluate the performance of magnetic resonance imaging (MRI) in measuring the optic nerve sheath diameter (ONSD) compared to the established method transorbital sonography (TOS) in patients with idiopathic intracranial hypertension (IIH). METHODS: Twenty-three patients with IIH were prospectively included applying IIH diagnostic criteria. All patients received a lumbar puncture with assessment of the cerebrospinal fluid (CSF) opening pressure to assure the IIH diagnosis. Measurement of ONSD was performed 3 mm posterior to inner sclera surface in B-TOS by an expert examiner, while three independent neuroradiologists took measurements in axial T-weighted MRI examinations. The sella turcica with the pituitary gland (and potential presence of an empty sella) and the trigeminal cavity were also assessed on sagittal and transversal T1-weighted MRI images by one independent neuroradiologist. RESULTS: The means of ONSD between ultrasound and MRI measurements were 6.3 mm (standard deviation [SD] = 0.6 mm) and 6.2 mm (SD = 0.8 mm). The interrater reliability between three neuroradiologists showed a high interclass correlation coefficient (ICC) (confidence interval: .573 < ICC < .8; p < .001). In patients with an empty sella, the ONSD evaluated by MRI was 6.6 mm, while measuring 6.1 mm in patients without empty sella. No correlation between CSF opening pressure and ONSD was found. CONCLUSIONS: MRI can reliably measure ONSD and yields similar results compared to TOS in patients with IIH. Moreover, patients with empty sella showed significantly larger ONSD than patients without empty sella.

Striatal dopamine transporters and cognitive function in Parkinson's disease.

BACKGROUND: Idiopathic Parkinson's disease (PD) is characterized by clinical motor symptoms including hypokinesia, rigidity and tremor. In addition to the movement disorder, cognitive deficits are commonly described. In the present study, we applied FP-CIT SPECT to investigate the impact of nigrostriatal dopaminergic degeneration on cognitive function in PD patients. METHODS: Fifty-four PD patients underwent [123I]FP-CIT SPECT and CERAD (Consortium to Establish a Registry for Alzheimer's Disease) testing. FP-CIT SPECT visualized the density of presynaptic dopamine transporters in both striata, each subdivided into a limbic, executive and sensorimotor subregion according to the atlas of Tziortzi et al (Cereb Cortex 24, 2014, 1165). CERAD testing quantified cognitive function. RESULTS: In the CERAD testing, PD patients exhibited deficits in the domains of semantic memory, attention, visuospatial function, non-verbal memory and executive function. After correction for multiple testing, the performance of the subtests Figure Recall and Trail-Making Test A correlated significantly with FP-CIT uptake into the ipsilateral executive subregion. The performance of the subtest Figure Saving correlated significantly with FP-CIT uptake into the contralateral executive subregion. CONCLUSIONS: The significant correlation between cognitive function and density of nigrostriatal dopamine transporters, as assessed by FP-CIT SPECT, indicate that striatal dopaminergic pathways-primarily the executive striatal subregion-are relevant to cognitive processing in PD.