Objectives: To evaluate potential relationship between qualitative CT features, quantitative texture analysis (QTA), histology, WHO staging, Masaoka classification and myasthenic syndrome in patients with thymic tumors.
Materials And Methods: Sixteen patients affected by histologically proven thymic tumors were retrospectively included in the study population. Clinical information, with special regard to myasthenic syndrome and serological positivity of anti-AchR antibodies, were recorded. Qualitative CT evaluation included the following parameters: (a) location; (b) tumor edges; (c) necrosis; (d) pleural effusion; (e) metastases; (f) chest wall infiltration; (g) tumor margins. QTA included evaluation of "Mean" (M), "Standard Deviation" (SD), "Kurtosis" (K), "Skewness" (S), "Entropy" (E), "Shape from Texture" (TX_sigma) and "average of positive pixels" (MPP). Pearson-Rho test was used to evaluate the relationship of continuous non-dichotomic parameters, whereas Mann-Whitney test was used for dichotomic parameters.
Results: Histological evaluation demonstrated thymoma in 12 cases and thymic carcinoma in 4 cases. Tumor necrosis was significantly correlated with QTA Mean (p = 0.0253), MPP (p = 0.0417), S (p = 0.0488) and K (p = 0.0178). WHO staging was correlated with Mean (p = 0.0193), SD (p = 0.0191) and MPP (p = 0.0195). Masaoka classification was correlated with Mean (p = 0.0322), MPP (p = 0.0315), skewness (p = 0.0433) and Kurtosis (p = 0.0083). Myasthenic syndrome was significantly associated with Mean (p = 0.0211) and MPP (p = 0.0261), whereas tumor size was correlated with Mean (p = 0.0241), entropy (p = 0.0177), MPP (p = 0.0468), skewness (p = 0.009) and Kurtosis (p = 0.006).
Conclusion: Our study demonstrates significant relationship between radiomics parameters, histology, grading and clinical manifestations of thymic tumors.
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http://dx.doi.org/10.1007/s11547-017-0845-4 | DOI Listing |
J Psychiatr Pract
January 2025
Department of Psychiatry and Neuropsychology, School for Mental Health and Neuroscience, Maastricht University Medical Centre, Maastricht University, Maastricht, The Netherlands.
Lambert-Eaton myasthenic syndrome (LEMS) is an autoimmune neuromuscular junction disorder characterized by proximal weakness, autonomic dysfunction, and areflexia associated with antibodies against voltage-gated calcium channels (VGCCs). Psychotic symptoms can occur in many autoimmune neurological disorders but they have rarely been observed in myasthenic syndromes. We report the case of a 21-year-old woman with primary autoimmune LEMS due to anti-VGCC antibodies subtype P/Q, who developed psychotic symptoms 3 years after the onset of motor symptoms.
View Article and Find Full Text PDFClin Dysmorphol
January 2025
Department of Medical Genetics, Kasturba Medical College, Manipal, Manipal Academy of Higher Education, Manipal, India.
Introduction: Agrin, encoded by AGRN, plays a vital role in the acetylcholine receptor clustering pathway, and any defects in this pathway are known to cause congenital myasthenic syndrome (CMS) 8 in early childhood with variable fatigable muscle weakness. The most severe or lethal form of CMS manifests as a fetal akinesia deformation sequence (FADS). To date, only one family has been reported with an association of null variants in AGRN and a lethal FADS.
View Article and Find Full Text PDFBMC Neurol
January 2025
Neuromuscular Neurology, Advocate Health, 1850 Dempster Street, Park Ridge, IL, 60068, USA.
This is an unusual case of voltage gated calcium channel (VGCC) antibodies leading to two distinct and chronologically separated neurologic syndromes without the presence of an underlying neoplasm. Lambert Eaton Myasthenic Syndrome (LEMS) presented five years prior to cerebellar ataxia. Both LEMS and cerebellar ataxia were responsive to treatment, but not the same therapy.
View Article and Find Full Text PDFJ Physiol
January 2025
Université Paris Cité, CNRS, ENS Paris Saclay, Centre Borelli UMR 9010, Paris, France.
Terminal Schwann cells (TSCs) are capable of regulating acetylcholine (ACh) release at the neuromuscular junction (NMJ). We have identified GABA as a gliotransmitter at mouse NMJs. When ACh activates α7 nicotinic ACh receptor (nAChRs) on TSCs, GABA is released and activates GABA receptors on the nerve terminal that subsequently reduce ACh release.
View Article and Find Full Text PDFFront Neurol
December 2024
Department of Internal Medicine, College of Medicine, King Saud University, Riyadh, Saudi Arabia.
Background: Lambert-Eaton myasthenic syndrome (LEMS) is an autoimmune disorder of the presynaptic neuromuscular junction associated with antibody mediated dysfunction of voltage-gated calcium channels (VGCCs). LEMS can exist as a paraneoplastic syndrome, paraneoplastic-LEMS (P-LEMS), when associated with tumors, most commonly, small cell lung carcinoma (SCLC) or as a non-paraneoplastic condition (NP-LEMS) when no malignancies are detected.
Methods: A retrospective chart review was conducted in 3 tertiary hospitals in Saudi Arabia for patients diagnosed with LEMS between January 2010 and January 2020.
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