Ultrasound pupillometry for the detection of a relative afferent pupillary defect (RAPD): Systematic evaluation in patients with optic neuritis and comparison with infrared video pupillometry.

Purpose: A relative afferent pupillary defect (RAPD) is a characteristic clinical sign of optic neuritis (ON). Here, we systematically evaluated ultrasound pupillometry (UP) for the detection of an RAPD in patients with ON, including a comparison with infrared video pupillometry (IVP), the gold standard for objective pupillometry.

Materials And Methods: We enrolled 40 patients with acute (n = 9) or past (n = 31) ON (ON+), 31 patients with multiple sclerosis (MS) without prior ON, and 50 healthy controls (HC) in a cross-sectional observational study.

Successful plasmapheresis and immunoglobulin treatment for severe lipid storage myopathy: Doing the right thing for the wrong reason.

Three consecutive skeletal muscle biopsies during a several months time-frame, showing different degrees of neutral lipid storage. This is highlighted by Oil-red-O stains (D, E, F) and electron microscopy (G, H, I). Note the impact on mitochondrial morphology with so called 'parking lots (K, L).

The Impact of Patient Support Programs on Adherence to Disease-Modifying Therapies of Patients with Relapsing-Remitting Multiple Sclerosis in Germany: A Non-Interventional, Prospective Study.

Introduction: Disease-modifying therapies (DMTs) in multiple sclerosis (MS) are chronic therapies, and patients are likely to face challenges in adhering to DMT dosing regimens over time. DMT manufacturers offer patient support programs (PSPs) to increase adherence. PSPs are managed offerings typically encompassing nurse services, phone services, online resources, or mobile offerings.

Lumbar puncture rapidly improves olfaction in patients with idiopathic intracranial hypertension: A cohort study.

Background: A lumbar puncture constitutes an important diagnostic procedure in the evaluation of idiopathic intracranial hypertension. Chronic overflow of cerebrospinal fluid into the sheaths of the olfactory nerves appears to be related to olfactory impairment in these patients. Here, we asked whether cerebrospinal fluid drainage in idiopathic intracranial hypertension patients improves olfactory function.

Olfactory and Gustatory Dysfunction in Patients With Autoimmune Encephalitis.

To test the hypothesis that olfactory (OF) and gustatory function (GF) is disturbed in patients with autoimmune encephalitides (AE). The orthonasal OF was tested in 32 patients with AE and 32 age- and sex-matched healthy controls (HC) with the standardized Threshold Discrimination Identification (TDI) score. This validated olfactory testing method yields individual scores for olfactory threshold (T), odor discrimination (D), and identification (I), along with a composite TDI score.

Objective assessment of a relative afferent pupillary defect by B-mode ultrasound.

Purpose: To evaluate B-mode ultrasound as a novel method for objective and quantitative assessment of a relative afferent pupillary defect (RAPD) in a prospective case-control study.

Methods: Seventeen patients with unilateral optic neuropathy and a clinically detectable RAPD and 17 age and sex matched healthy controls were examined with B-mode ultrasound using an Esaote-Mylab25 system according to current guidelines for orbital insonation. The swinging flashlight test was performed during ultrasound assessment with a standardized light stimulus using a penlight.

7 Tesla MRI of Balo's concentric sclerosis versus multiple sclerosis lesions.

Background: Baló's concentric sclerosis (BCS) is a rare condition characterized by concentrically layered white matter lesions. While its pathogenesis is unknown, hypoxia-induced tissue injury and chemotactic stimuli have been proposed as potential causes of BCS lesion formation. BCS has been suggested to be a variant of multiple sclerosis (MS).

Tryptophan immunoadsorption during pregnancy and breastfeeding in patients with acute relapse of multiple sclerosis and neuromyelitis optica.

Background: Up to every fourth woman with multiple sclerosis (MS) or neuromyelitis optica spectrum disorder (NMOSD) suffers a clinically relevant relapse during pregnancy. High doses of steroids bear some serious risks, especially within the first trimester of pregnancy. Immunoadsorption (IA) is an effective and more selective treatment option in disabling MS relapse than plasma exchange.

Fulminant rebound of relapsing-remitting multiple sclerosis after discontinuation of dimethyl fumarate: A case report.

Background: Rebound phenomena after discontinuation of different treatments for relapsing-remitting multiple sclerosis (RRMS) have previously been described. Systematic database research in PubMed did not show any report with relapse directly associated with dimethyl fumarate (DMF) cessation.

Case Presentation: Here, we report on a 38-year-old Caucasian male patient suffering from a relatively mild course of RRMS who developed a fulminant clinical rebound 2 months after discontinuation of DMF therapy.

Neurofascin (NF)155- and NF186-Specific T Cell Response in a Patient Developing a Central Pontocerebellar Demyelination after 10 Years of CIDP.

Background: Information and pathobiological understanding about central demyelinating manifestation in patients, who primarily suffer from chronic inflammatory demyelinating polyneuropathy (CIDP), are scarce.

Methods: IFN-γ-response as well as antibodies against the (para)nodal antigens neurofascin (NF)155 and NF 186 had been tested by Elispot assay and ELISA before clinical manifestation and at follow-up.

Case Description And Results: The patient described here developed a subacute brainstem syndrome more than 10 years after diagnosis of CIDP under low-dose maintenance treatment of intravenous immunoglobulins (IVIG).

B-mode ultrasound assessment of pupillary function: Feasibility, reliability and normal values.

Purpose: To evaluate B-mode ultrasound as a novel method for the examination of pupillary function and to provide normal values for the pupillary reflex as assessed by B-mode ultrasound.

Methods: 100 subjects (49 female, 51 male, mean [range] age 51 [18-80 years]) with no history of ophthalmologic disease, no clinically detectable pupillary defects, and corrected visual acuity ≥ 0.8 were included in this prospective observational study.

High prevalence of neuronal surface autoantibodies associated with cognitive deficits in cancer patients.

The recent discovery of neuronal cell-surface antibodies profoundly expanded the clinical spectrum of paraneoplastic neurological syndromes. Many of these syndromes are associated with impaired cognitive function, a clinical symptom that is of increasing concern in cancer patients. However, the frequency of these antibodies in cancer patients and their relation to clinical syndromes is currently unknown.

Olfactory dysfunction in patients with primary progressive MS.

Objective: We tested the hypothesis that olfactory function is more impaired in patients with primary progressive MS (PPMS) than that in relapsing-remitting MS (RRMS).

Methods: Standardized olfactory testing was performed in 32 patients with PPMS, 32 patients with RRMS, and 32 healthy controls (HCs). Patients with olfactory dysfunction due to an alternative primary etiology were excluded.

A prospective study comparing tryptophan immunoadsorption with therapeutic plasma exchange for the treatment of chronic inflammatory demyelinating polyneuropathy.

Background: Chronic inflammatory demyelinating polyneuropathy (CIDP) is a rare paralyzing inflammatory neuropathy with probably autoimmune origin. While plasma exchange (PE) constitutes a first-line treatment option for CIDP, there is only little known about the efficacy and safety of immunoadsorption (IA), a more selective apheresis procedure with assumed better tolerability.

Methods: In this prospective-randomized pilot trial, patients were randomly assigned to receive 6 sessions of PE (n = 10) or IA (n = 10) treating equal plasma volumes.

Identification of neovascularization by contrast-enhanced ultrasound to detect unstable carotid stenosis.

Background: Plaque neovascularization accompanies local inflammation and critically contributes to plaque instability. Correct identification of intraplaque neovascularization by contrast-enhanced ultrasound (CEUS) may provide an additional risk marker in carotid stenosis. This pilot study investigates the correlation between histological evaluation of carotid plaque specimens and pre-surgery CEUS to identify neovascularization.

Does IIH Alter Brain Microstructures? - A DTI-Based Approach.

Introduction: To investigate the correlation of microstructural parameters with CSF pressure and macroscopic changes assessed by diffusion tensor imaging (DTI) in patients with idiopathic intracranial hypertension (IIH).

Methods: Twenty-three patients with IIH as well as age-, sex-, and body mass index (BMI)-matched controls underwent high resolution MR imaging of the optic nerve sheaths (ONS), pituitary gland, and ventricles. For DTI data a voxelwise permutation analysis was performed for the whole brain and ROI analysis was performed for the optic nerve and optic radiation.

Longitudinal Testing of Olfactory and Gustatory Function in Patients with Multiple Sclerosis.

Background: The aim of the study was to investigate changes of the olfactory and gustatory capacity in patients with multiple sclerosis (MS).

Methodology: 20 MS patients were tested longitudinally for 3 years after initial testing. The Threshold Discrimination Identification test (TDI) was used for subjective olfactometry.

Immunoadsorption or plasma exchange in the treatment of autoimmune encephalitis: a pilot study.

Therapeutic apheresis has emerged as a major treatment option for autoantibody-associated inflammatory diseases of the nervous system. This includes patients with autoimmune encephalitides caused by antibodies against neuronal proteins. Plasma exchange (PE) and immunoadsorption (IA) constitute two possibilities to eliminate pathogenic antibodies from patients' plasma, but their efficacy and safety has not been prospectively assessed in larger patient groups of autoimmune encephalitides.

Human cerebrospinal fluid monoclonal N-methyl-D-aspartate receptor autoantibodies are sufficient for encephalitis pathogenesis.

SEE ZEKERIDOU AND LENNON DOI101093/AWW213 FOR A SCIENTIFIC COMMENTARY ON THIS ARTICLE: Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is a recently discovered autoimmune syndrome associated with psychosis, dyskinesias, and seizures. Little is known about the cerebrospinal fluid autoantibody repertoire. Antibodies against the NR1 subunit of the NMDAR are thought to be pathogenic; however, direct proof is lacking as previous experiments could not distinguish the contribution of further anti-neuronal antibodies.

MRI phase changes in multiple sclerosis vs neuromyelitis optica lesions at 7T.

Objective: To characterize paramagnetic MRI phase signal abnormalities in neuromyelitis optica spectrum disorder (NMOSD) vs multiple sclerosis (MS) lesions in a cross-sectional study.

Methods: Ten patients with NMOSD and 10 patients with relapsing-remitting MS underwent 7-tesla brain MRI including supratentorial T2*-weighted imaging and supratentorial susceptibility weighted imaging. Next, we analyzed intra- and perilesional paramagnetic phase changes on susceptibility weighted imaging filtered magnetic resonance phase images.

Escalation Therapy of Steroid Refractory Multiple Sclerosis Relapse with Tryptophan Immunoadsorption - Observational Multicenter Study with 147 Patients.

Background: A marked proportion of multiple sclerosis (MS) relapses is followed by incomplete recovery. Our aim was to considerably increase the evidence of the clinical use of immunoadsorption (IA) as escalation therapy for patients with MS relapse.

Methods: A retrospective multicenter study was performed in MS patients with steroid refractory relapse who were treated with tryptophan IA.

Neuromyelitis optica does not impact periventricular venous density versus healthy controls: a 7.0 Tesla MRI clinical study.

Objective: To quantify the periventricular venous density in neuromyelitis optica spectrum disease (NMOSD) in comparison to that in patients with multiple sclerosis (MS) and healthy control subjects.

Materials And Methods: Sixteen patients with NMOSD, 16 patients with MS and 16 healthy control subjects underwent 7.0-Tesla (7T) MRI.