Electrophysiological Findings of Children Presented with Neuromuscular Disorder

Mustafa Mahbub1, Selina Hosna Banu2, AZM Moshiul Azam3, S. Chandra Majumder4, Naila Zaman Khan5

Background: Neuromuscular disease includes disorders of spinal motor neuron,
spinal nerves and muscle and can be acquired or hereditary. Neurophysiological
investigations like EMG and NCV are important tools for diagnosis.
Objective : To distinguish between the disorders of anterior horn cells, peripheral
nerves and muscles and to relate neurophysiological abnormalities to the clinical
Method : Thirty (37) children with neuromuscular disorders were seen from Feb.
2010 to Feb. 2015. Consanguinity, history of perinatal insult and important clinical
feature were recorded. Serum Creatinine Phosphokinase (CPK), Electromyography
(EMG), Nerve Conduction Velocity (NCV) suggested for all and muscle biopsy for
selected cases. EMG & NCV was done in the Neurophysiology Lab of Bangladesh
Protibondhi Foundation by trained personnel.
Result : EMG and NCV was done in all 37 children. According to the findings of
EMG 13 (35%) were suggestive of myogenic disorder, 15 (40%) were neurogenic
disorder, 1 (3%) was mixed neurogenic disorder and 8 (22%) was normal. Clinical
diagnosis of 37 children was, Spinal Muscular Atrophy (SMA) 12 (32%), Muscular
Dystrophy in 8 (22%) cases. Myopathy in 7 (20%), Hereditary Motor & Sensory
Neuropathy (HMSN) in 5 (14%) and Paraplegia in 3 (8%). Of the 16 children with
clinically myogenic disorders, 13(81%) cases were compatible with electrophysiological
findings. Of the 20 with neurogenic disorders, 15 (75%) were compatible with the
electrophysiological findings.
Conclusion : In most of the cases EMG and NCV findings were in favor of the
clinical diagnosis It is necessary to supplement clinical examination by the study of
electrical activity in nerve and muscle to validate the diagnosis and for future reference
during follow up visits..
Key words: Neuromuscular disorders; Myogenic; Neurogenic; EMG; NCV


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