Neuromuscular Diseases In The Pediatric Intensive Care Unit: 11 Years Of Experience From A Tertiary Children's Hospital

Objective: We present the clinical spectrum of neuromuscular diseases (NMD) that were admitted to pediatric intensive care unit of a tertiary children's hospital for 11 years.

Methods: The data of cases including age, gender, diagnosis, PRISM scores, age at hospitalization, cause of admission to pediatric intensive care unit, clinical course, complications and clinical discharge status were retrospectively analyzed.

Results:A Neuromuscular disorder was detected in 43 of the 1411 patients admitted within the study period and accounted for approximately 3% of pediatric intensive care unit admissions. The origin of neuromuscular disorder was genetic (n= 35, 74.8%), acquired (n=6, 13.8%) and metabolic (n=2, 4.6%), respectively. The diagnoses included spinal muscular atrophy type 1 (n=12, 27.9%), DMD-BMD (n=7, 16.2%), congenital myopathy (n=6, 13.9%), congenital muscular dystrophy (n=5, 11.6%), Guillain-Barre Syndrome and its variants (n=2, 4.6%), spinal muscular atrophy associated with respiratory distress type 1 (n=2, 2.3%), critical illness neuropathy (n=2, 4.6%), acute flaccid myelitis (n=2, 4.6%), congenital myasthenic syndrome (n=1, 2.3%), peripheral neuropathy associated with disorder of riboflavin transporter (n=1, 2.3%), juvenile amyotrophic lateral sclerosis (n=1, 2.3%), stress-induced childhood-onset neurodegeneration with ataxia and variable seizures (n=1, 2.3%), and metabolic myopathy (n=1, 2.3%). Respiratory complications (n=31, 72%) were the most common causes of admission to the pediatric intensive care unit. Mortality rate was 6.9% (n=3).

Conclusion: Accurate diagnosis of neuromuscular diseases and knowledge of causes requiring admission to pediatric intensive care units is crucial for management of these cases.
Keywords: Neuromuscular disease, pediatric intensive care unit