Pediatric macrophagic myofasciitis associated with motor delay.

BACKGROUND: Macrophagic myofasciitis (MMF) is a rare inflammatory myopathy characterized by accumulation of perifascicular macrophages without muscle fiber necrosis. Few sporadic pediatric cases have been described, and MMF is recognized as a possible reaction to intramuscular injections of aluminum-containing vaccines. The association of MMF and motor delay is unclear in the pediatric population. We report the clinical evaluation and follow-up of 4 young children with MMF and review of 4 cases previously reported of sporadic, pediatric MMF to better determine the possible association of sporadic MMF in children presenting with motor delay. PATIENTS AND METHODS: Described our 4 case reports in which we observed children presenting for evaluation of motor delay with unrevealing clinical and laboratory evaluations for common causes of motor delay and histopathological evaluations consistent with macrophagic myofasciitis. Muscle data was obtained by quadriceps muscle biopsy. RESULTS: Clinical presentations were similar in all children and were characterized by motor delay, hypotonia, and failure to thrive with an unrevealing evaluation for central nervous system disease, congenital, and mitochondrial myopathies. CONCLUSIONS: Our cases and those previously reported in the literature demonstrate MMF should be considered in the evaluation of children with failure to thrive, hypotonia, and muscle weakness, as clinical outcome appears to be favorable.

Predictors of noninvasive ventilation tolerance in patients with amyotrophic lateral sclerosis.

Noninvasive ventilation (NIV) appears to improve survival and quality of life in patients with amyotrophic lateral sclerosis (ALS), but little is known about predictors of NIV tolerance. NIV use was assessed and clinical predictors of tolerance were investigated, using predictive modeling, in ALS patients diagnosed and followed in our clinic until death over a 4-year time period. Patients were prescribed NIV based on current practice parameters when respiratory symptoms were present or forced vital capacity was less than 50%. We prescribed NIV in 52% (72) of patients. For those prescribed NIV, information regarding tolerance was available for 50 patients, with 72% (36) tolerant to its use. Tolerance was six times more likely in limb-onset than bulbar-onset ALS patients, with a trend toward reduced tolerance in those with lower forced vital capacity at NIV initiation. Age, gender, and duration of disease were not predictors of NIV tolerance. We conclude that a majority of ALS patients who are prescribed NIV can successfully become tolerant to its use.