There is an unmet need for objective disease-specific biomarkers in the heterogeneous autoimmune neuromuscular disorder myasthenia gravis (MG). This cross-sectional study identified a signature of 23 inflammatory serum proteins with proximity extension assay (PEA) that distinguishes acetylcholine receptor antibody seropositive (AChR+) MG patients from healthy controls (HCs). CCL28, TNFSF14, 4E-BP1, transforming growth factor alpha (TGF-α), and ST1A1 ranked top biomarkers. TGF-β1 and osteoprotegerin (OPG) differed between early- and late-onset MG, whereas CXCL10, TNFSF14, CCL11, interleukin-17C (IL-17C), and TGF-α differed significantly with immunosuppressive treatment. MG patients with moderate to high disease severity had lower uPA. Previously defined MG-associated microRNAs, miR-150-5p, miR-30e-5p, and miR-21-5p, correlated inversely with ST1A1 and TNFSF14. The presented inflammatory proteins that distinguish AChR+ MG are promising serum biomarkers for validation in prospective studies to allow for molecular signatures for patient subgroup stratification and monitoring of treatment response.

Introduction: Hypertrophic olivary degeneration (HOD) is a rare form of trans-synaptic degeneration involving the Guillain–Mollaret triangle, characterized by enlargement of the inferior olivary nucleus—unlike the atrophy typical of most neurodegenerative processes. It is usually associated with stroke, surgical injury, or demyelination, but rarely follows hemorrhage from a cavernous malformation (CM). This report presents a case of HOD secondary to a mesencephalic CM hemorrhage, with emphasis on imaging findings and diagnostic considerations. Case Description: A 55-year-old woman presented with acute-onset, right-sided facial, torso, and limb hypoesthesia, along with gait instability. Neurological examination revealed sensory impairment in the right maxillary (V2) and mandibular (V3) trigeminal territories, as well as diminished pain and temperature sensation throughout the right hemibody. MRI revealed a hemorrhage in the posterior mesencephalon near the left red nucleus, leading to the diagnosis of a CM with an associated venous angioma. She was managed conservatively and improved clinically. Six months later, MRI showed hypertrophy and T2/FLAIR hyperintensity of the left inferior olive, consistent with developing HOD. At 1.5 years follow-up, olivary enlargement had progressed—now consistent with stage 2 HOD—and a bilateral palatal tremor was observed, more pronounced on the right side. DTI revealed asymmetric volume loss in the left brainstem fiber pathways at the level of the medulla oblongata, confirming trans-synaptic degeneration. Conclusions: This case highlights HOD as a rare but important complication of mesencephalic CM hemorrhage. Recognition of its characteristic imaging features—olivary hypertrophy with persistent T2/FLAIR hyperintensity—is essential for accurate diagnosis. DTI supports the trans-synaptic mechanism, helping distinguish HOD from other pathologies and preventing unnecessary investigations.

Background: Myasthenia gravis (MG) is a rare autoimmune disorder that affects neuromuscular transmission, leading to muscle weakness. While most patients respond to standard treatments, approximately 15% remain refractory, prompting interest in alternative therapies. This study examined the use of rituximab in patients with refractory MG at a single centre in Latvia, intending to broaden discussions on treatment strategies. Materials and methods: The prospective cohort study was conducted at Pauls Stradiņš Clinical University Hospital from November 2022 to March 2024. Refractory MG was defined as failure to achieve adequate disease control despite standard immunosuppressive therapy, including patients with persistent symptoms, recurrent crises or intolerance to medications. Myasthenia Gravis Composite Score (MGCS), Myasthenia Gravis Activities of Daily Living and Myasthenia Gravis Quality of Life (scores at baseline and monthly for 6 months) were completed. Glucocorticosteroid doses were also recorded. Data were analysed using descriptive statistics and Wilcoxon signed-rank test, considering p<0.05 significant. Results: 98 patients with MG were evaluated to identify treatment-refractory cases. A total of nine patients with refractory MG (median disease duration 7 years, range 1–12) were included and received a single low dose of rituximab. Among the nine patients treated, seven were acetylcholine receptor-positive and two were muscle-specific kinase antibody-seropositive. 6 months after treatment, the median MGCS decreased from 11.5 (IQR 7.3–17.8) to 7.5 (IQR 3.8–8.0) (p=0.025). The median daily corticosteroid dose decreased from 15.0 mg (IQR 10.0–20.9) to 8.5 mg (IQR 5.0–12.5) (p=0.036). There were no exacerbations of MG or significant adverse reactions. Conclusions: Our findings suggest that rituximab may be a promising treatment option for refractory MG. The notable clinical benefits and safety profile make rituximab a valuable addition to this challenging autoimmune disorder treatment options. Further research is needed to refine patient selection and dosing for optimal treatment outcomes.