Characterization of the thymus in Lrp4 myasthenia gravis: Four cases

Myasthenia gravis (MG) is an autoimmune disease of the neuromuscular junction. Most patients have pathogenic autoantibodies against the acetylcholine receptor (AChR). In the last years a novel subpopulation of MG patients has been described that harbors antibodies against low-density lipoprotein receptor-related protein 4 (Lrp4), another postsynaptic neuromuscular antigen. In early-onset AChR MG (EOMG), the thymus plays an important role in immunopathogenesis, and early thymectomy is beneficial. It is still unknown if the thymus plays any role in Lrp4-MG. In this pilot study, we compared thymus samples from four patients with Lrp4-MG (one pre-treated with immunosuppressive drugs), four non-MG controls and five EOMG patients (not pretreated with immunosuppressive drugs). Immunohistochemistry of the Lrp4-MG thymi revealed normal architecture, with normal numbers and distribution of B-cells, lymphoid follicles and Hassall's corpuscles. Primary CD23⁺ lymphoid follicles were similarly infrequent in Lrp4-MG and control thymic sections. In none of the control or Lrp4-MG thymi did we find secondary follicles with CD10⁺ germinal centers. These were evident in 2 of the 5 EOMG thymi, where primary lymphoid follicles were also more frequent on average, thus showing considerable heterogeneity between patients. Even if characteristic pathological thymic changes were not observed in the Lrp4 subgroup, we cannot exclude a role for the thymus in Lrp4-MG pathogenesis, since one Lrp4-MG patient went into clinical remission after thymectomy alone (at one year follow-up) and one more improved after thymectomy in combination with immunosuppressive therapy.     

Psychiatrie und gerichtliche Psychologie

International Journal of Legal Medicine -     

The role of linguistic context in deriving word meanings in individuals with Down Syndrome

Deriving the meaning of unknown words from context and its relationship to text comprehension was investigated in 24 individuals with Down syndrome and in 24 typically developing children matched for the Peabody Picture Vocabulary Test (PPVT) score.The study consisted of three phases. Unknown words were identified during the first phase (PPVT). Those words were presented embedded in brief linguistic contexts during the second phase. Recognition (maintenance) of word meanings was verified in the third and final phase. Both groups of participants recognized the meanings of a noteworthy number of words in contexts and a high percentage of these was maintained when they were presented at a later date without the support of context. Over and above group differences and basic linguistic skills, text comprehension seems to predict the ability to use context. Context provides the semantic information necessary to extract word meaning by activating relevant world knowledge.     

Nutritional rickets

Nutritional rickets was diagnosed in 18 infants aged eight to 24 months. Clinical features included progressive leg bowing, poor linear growth, a diet deficient in vitamin D, seizures, and abnormal serum calcium, phosphate and alkaline phosphatase levels. Wrist radiographs and serum alkaline phosphatase levels were the most useful confirmatory tests. Breast milk may not contain enough vitamin D to protect infants, particularly dark-skinned children and those living in cloudy, northern U.S. cities, from rickets after six months of age. As breast feeding becomes more widely practiced, care is required to ensure that infants at high risk for rickets receive appropriate vitamin D supplementation.     

Diagnosis and treatment of a carcinoid tumor using iodine-131 meta-iodobenzylguanidine

Scintigraphy using I-131 meta-iodobenzylguanidine has been introduced as an effective method to detect pheochromocytomas and neuroblastomas, and the radiopharmaceutical also is applied in therapy of these tumors. The authors present a case of a metastatic gastric carcinoid tumor, another neural crest tumor, concentrating I-131 MIBG, which was documented by conventional scintigraphy and SPECT in correlation with CT scans and colloid scintigrams of the liver. Two therapeutic attempts in this patient, using I-131 MIBG, are described. The metabolic basis of this phenomenon is discussed, and the importance of I-131 MIBG imaging in the detection of neural crest tumors is underlined.     

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Acute und chronische Infectionskrankheiten

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