Childhood Neuromuscular Disorders

@#Infantile and childhood neuromuscular disorders are a significant cause of motor delays in childhood. Neuromuscular disorders may present either with hypotonia and weakness in early infancy or falls and difficulties in walking later in childhood. The first goal in approaching a patient with suspected muscle disease is to ascertain the correct site of the lesion, followed by the cause of the lesion. Extraordinary breakthroughs in the area of genetic testing have resulted in a decrease in reliance on muscle biopsies and neurophysiological testing. The importance of recognising such disorders is because viable treatment options are now available for the treatment of affected children. Early recognition allows patients to receive therapy at a stage of the disease that will give the best long-term outcomes. Even in the absence of definitive treatment, supportive management and preventive care have revolutionized the care of neuromuscular disorders with longer quality life spans in a good majority of patients.

Enterovirus infections in Singaporean children: an assessment of neurological manifestations and clinical outcomes

<p><b>INTRODUCTION</b>Enterovirus infections in childhood can be associated with significant neurological morbidity. This study aimed to describe the prevalence and range of neurological manifestations, determine the clinical characteristics and assess differences in clinical outcomes for Singaporean children diagnosed with enterovirus infections.</p><p><b>METHODS</b>In this single-centre, case-control study, clinical data was collected retrospectively from patients admitted to National University Hospital, Singapore, from August 2007 to October 2011 and diagnosed with enterovirus infection, based on the enterovirus polymerase chain reaction test, or cultures from throat and rectal swabs or cerebrospinal fluid samples. The occurrence of neurological manifestations was reviewed and clinical outcomes were assessed.</p><p><b>RESULTS</b>A total of 48 patients (age range: six days-17.8 years) were included in the study. Neurological manifestations were seen in 75.0% of patients, 63.9% of whom presented with aseptic meningitis. Other neurological manifestations included encephalitis, acute cerebellitis, transverse myelitis and autonomic dysfunction. The incidence of neurological manifestations was significantly higher in patients aged > 1 year as compared to younger patients (p = 0.043). In patients without neurological manifestations, a significantly higher proportion presented with hand, foot and mouth disease and poor feeding. Long-term neurological sequelae were seen in 16.7% of patients with neurological manifestations.</p><p><b>CONCLUSION</b>A wide spectrum of neurological manifestations resulting in a relatively low incidence of long-term neurological sequelae was observed in our study of Singaporean children with enterovirus infections. As some of these neurological morbidities were severe, careful evaluation of children with neurological involvement is therefore necessary.</p>