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Clinical and biochemical evaluation of the effectiveness of therapy for hereditary motor neuronal neuropathy in children
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| Author:
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BERDIBAEVA SAODAT ULUGBEKOVNA, MADJIDOVA YAKUTKHON NABIEVNA
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| Abstract:
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Hereditary motosensory neuropathies or Charcot-Marie-Tooth disease is an extensive heterogeneous group of diseases characterized by lesions of peripheral nerves, leading to early disability and a decrease in the quality of life, the diagnosis of which largely depends on medical genetic counseling and prenatal DNA diagnostics.
The aim of the study. To assess the effect of some trace elements (calcium, magnesium, phosphorus) on the possibility of hereditary motor-sensory neuropathies in children.
Materials and research methods. To achieve this goal, the results of treatment of 100 patients with various types of peripheral neuropathies were analyzed. The main group was divided into 2 subgroups: 65 children with hereditary motosensory neuropathy and 35 children with peripheral neuroinfections. The age of the sick children ranged from 5 to 14 years. The average age was 9.0 ± 4.5 years. The observation groups were comparable in terms of gender and age characteristics and the number of people (p=0.05).
Research results. The total number of patients with proximal weakness of the arms and legs with hereditary motosensory neuropathies is 8 (12.3%), in patients with neuroinfection - 17 (48.57%). The total number of patients with distal weakness of the arms and legs in patients with hereditary motosensory neuropathies - The total number of patients with distal weakness of the arms and legs - 57 (87.7%), in patients with neuroinfection - 18 (51.43%). The clinical picture in all patients was dominated by polyneurotic sensory disorders (97.6 ± 2.4%). According to the types of sensory impairment, painful hyperesthesia was more common (71.4 ± 7.0%), hypoesthesia was detected in 28.6 ± 7.0% of children. Paresthesias were observed in 50 ± 7.7% of cases. Tactile sensitivity on the plantar surface of the feet was reduced in 40.5 ± 7.6% of children. Changes in the reflex sphere were more common at the level of the lower extremities (76.2 ± 6.6%): hyporeflexia - in 84.3 ± 6.4% of cases, areflexia - 12.3 ± 3.6%, hyperreflexia - 3, 4 ± 0.6% of cases. Since hereditary motor-sensory neuropathies have a length-dependent pattern, most of them have sensitive ataxia.
Conclusions: Against the background of pathogenetic therapy and physiotherapeutic rehabilitation measures, it is possible to limit the severity of hereditary motorsensory neuropathy, which is especially important in pediatric neurology.
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| Keyword:
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hereditary motor-sensory neuropathy; neuroinfection; scale GMFCS; FMS scale; biochemical methods; trace elements; tau protein
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| EOI:
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| DOI:
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https://doi.org/10.31838/ijpr/2021.13.01.369
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| Download:
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