GM1 and GM2-Gangliosidosis: Clinical Features, Neuroimaging Findings and Electroencephalography.

Abstract: Gangliosidosis is one of the hereditary metabolic diseases caused by the accumulation of Gangliosid in the central nervous system, leading to severe and progressive neurological deficits. Regarding phenotype, GM1 and GM2-Gangliosidosis are divided into Infantile, Juvenile, and Adult. Materials & Methods: In this study, thirty-seven patients with GM1 and GM2-Gangliosidosis were referred to the neurology department of Mofid Children's Hospital in Tehran, Iran, whose disease was confirmed from September 2019 to December 2021. This study assessed age, sex, and developmental status before the onset of the disease, clinical manifestations, brain imaging, and electroencephalography. Results: 97.20% of patients were the result of family marriage. Approximately 80% of juvenile patients were developmentally normal before the onset of the disease. Developmental delay was more common among infantile GM1-Gangliosidosis than infantile GM2-Gangliosidosis, but in total, more than 50% of GM1&GM2-Gangliosidosis patients had reached their developmental milestone before the onset of the disease. With the onset of disease symptoms, 100% of patients regressed in terms of movement, 97.20% of them mentally, and 75% of them had seizures during the disease. The most common clinical findings were cherry-red spot, Mongolian spot, macrocephaly, organomegaly, hyperacusis, and scoliosis. The most common brain imaging findings included bilateral thalamus involvement, brain atrophy, PVL, and delayed myelination. The most common finding in electroencephalography was background low voltage with abnormal sharp waves. Conclusion: This study concluded that most of the patients are the result of family marriage, and most of the juvenile patients are developmentally normal before the onset of the disease. In addition, more than 50% of infantile patients reach their developmental milestones before the onset of the disease. The most common clinical findings of these patients are seizures, cherry-red spot, macrocephaly, hyperacusis, Mongolian spot, and bilateral involvement of the thalamus.

Investigation of Parasitic Contaminations of Vegetables Sold in Markets in the City of Tabriz in 2014.

Parasitic diseases are among the most common problems in developing countries. Various parasitic forms such as cysts, larvae and eggs of the parasite are transmitted orally through the consumption of vegetables. So, the aim of this study was to evaluate parasitic contamination of fresh vegetables consumed in the city of Tabriz. This cross-sectional study was conducted for 3 months in 2014 in Tabriz. In this study, 1620 samples from 10 kinds of vegetables (leeks, basil, mint, chives, radishes, parsley, lettuce, watercress, tarragon and coriander) were collected from 54 vegetable shops. After the washing process, centrifugation, sediment preparation and staining, each sample was examined for parasitic contamination. The parasitic infection was observed in 316 (19.5%) which 141 samples (8.7%) was related to metazoan and 175 samples (10.8%) was related to protozoan. the highest contamination was observed in leeks and the lowest in radish. Most parasitic contaminations were related to Ascaris eggs and E. coli cyst and the lowest parasitic contaminations were related to Heterophyes eggs. It can be concluded that vegetables sold in Tabriz are potential sources of human contamination with parasites. It is suggested that the use of fertilizers of human source in agriculture should be avoided.