الفهرس 
Duchenne and Becker Muscular DystrophiesOnly 14 pages are availabe for public view
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Abstract
A neuromuscular disorder is defined as any disorder affecting the peripheral nervous system. Pediatric neuromuscular disorders are highly versatile and include disorders of motor neuron, nerve root, peripheral nerve, neuromuscular junction, and muscle. The diagnostic process of Pediatric neuromuscular disorders is usually complex and requires a combination of electrophysiology, serology, genetic testing, and neuromuscular imaging in addition to muscle biopsy in myopathic diseases.
Neuromuscular ultrasound is an attractive complementary tool to electrodiagnostic tests and can provide valuable information that cannot be obtained by the electrodiagnostic tests alone. Ultrasound can localize the lesion in traumatic nerve injuries and focal neuropathies, detect an underlying structural cause of neuropathies, identify nerve tumors, and be used as a rapid screening tool to differentiate between nerve and muscle disorders. It was reported that muscle size and cross-sectional area can be used as an index to determine the disruption in muscle architecture caused by neuromuscular diseases in children.
DMD is the most frequent and best studied early-onset muscular dystrophy. It was described as pseudohypertrophic muscular dystrophy by Gaetano Conte (1836) and Meryon (1851). It was named after Duchenne who discussed and wrote about it in 1868. DMD begins in early childhood and runs a relatively rapid, progressive course. The incidence is about 1 in 3500 live male births.
GBS is an umbrella term describing a heterogeneous group of related disorders. Common pathogenesis of these disorders is mirrored by several shared clinical features, including history of antecedent infection, monophasic disease course and symmetrical cranial or limb weakness. In the last hundred years, some landmark observations have been made in the understanding and therapy of GBS.
In the present study we aimed to evaluate the diagnostic accuracy and prognostic value of ultrasonography in various neuromuscular pathologies in relation to standard investigations.
This study was performed between September 2020 and June 2022 in the Pediatric Neurology unit, Children hospital, at Ain Shams University. 64 children were included. They were stratified into 3 groups: All children with acute weakness or inability to walk admitted to the hospital with symptom onset within 3 weeks, as confirmed by clinical, laboratory, electrodiagnostic examinations and GBS diagnostic criteria used for classification. GBS subtypes were classified as AIDP or axonal GBS, according to the electrophysiological criteria. Age and sex matched healthy children for children with GBS were enrolled to obtain normal values of nerve cross sectional area. They were recruited from General Pediatrics clinic. Children with DMD (who are ambulatory) following at the pediatric neurology clinic.
Twenty children with acute flaccid weakness were included, all were diagnosed as acute Guillain Barre syndrome and 24 boys with Duchenne muscular dystrophy presented with chronic weakness.