Heakyung Kim

Prevention of Pulmonary Morbidity for Patients With Duchenne Muscular Dystrophy

STUDY OBJECTIVE To evaluate the effects of a new respiratory management protocol on respiratory morbidity and hospitalization rates for patients with Duchenne muscular dystrophy (DMD). DESIGN A retrospective cohort study. METHODS Using a protocol in which oxyhemoglobin desaturation was prevented or reversed by the use of noninvasive intermittent positive pressure ventilation (IPPV) and assisted coughing as needed, the hospitalization rates and days for 24 protocol DMD ventilator users were compared with those of 22 nonprotocol DMD tracheostomy IPPV users. RESULTS The 22 conventionally managed patients were hospitalized a mean of 72.2+/-112 days when undergoing tracheostomy. This included a 16.1+/-5.4-day period of translaryngeal intubation. The 24 protocol patients were hospitalized a mean of 6.0+/-2.4 days (p<0.005) when beginning ventilator use. Over their next 126.2 patient-years of ventilator use, the 24 protocol patients had significantly lower rates of hospitalization (p<0.008) and hospitalization days (p<0.005) than had the tracheostomy IPPV users over a 167.2 patient-year period. This is true although 14 of the 24 protocol patients went on to require 24-h noninvasive IPPV for 4.5+/-3.6 years. Five of the 14 have yet to be hospitalized. CONCLUSION The use of inspiratory and expiratory aids can prolong survival while significantly decreasing the pulmonary morbidity and hospitalization rates associated with conventional resort to tracheostomy IPPV.

The question of disproportionate impairments in visual and auditory information processing in multiple sclerosis.

The present study was conducted to investigate the hypothesis that subjects with MS have a disproportionate impairment in the phonological loop of working memory and whether such impairment is domain-specific. This was assessed by comparing performance in subjects with MS across both the visual (using the Paced Visual Serial Addition Test: PVSAT) and auditory (using the Paced Auditory Serial Addition Test: PASAT) domains. In addition, we assessed the role of processing speed in mediating performance deficits. The results showed that: (1) Although all subjects performed better on the PVSAT versus the PASAT, no disproportionate modality-specific differences were observed in the MS group relative to controls; and (2) the rate of decline across trials in the MS and healthy control groups did not differ on either the PVSAT or PASAT. Implications of the results are discussed within the context of a working memory model.

Efficacy of botulinum toxin A, serial casting, and combined treatment for spastic equinus: a retrospective analysis

Botulinum toxin type A (BTX-A), serial casting, and combined treatment for spastic equinus were compared by chart review. Fifty-five patients with diplegia (n=21), hemiplegia (n=17), quadriplegia (n=6), and other diagnoses (n=11) were reviewed. Thirty-one patients had Gross Motor Function Classification System (GMFCS) scores of I or II, 10 had a GMFCS score of III, and eight had GMFCS scores of IV or V (for six there were no available data). Mean age was 7 years 1 month (SD 4 y 8mo). The combined group showed a significantly greater increase in passive range of motion (ROM) of the ankle joint in comparison with BTX-A alone (p=0.0002), with a mean of 17 degrees (SD 11.2) versus 5 degrees (SD 6.7), and casting alone showed a significantly greater increase in comparison with BTX-A alone (p=0.0001), with a mean of 15 degrees (SD 4.5) versus 5 degrees (SD 6.7). With cast number controlled, change in ROM after casting with and without BTX-A was not significantly different (p=0.37). There was no significant difference between casting with or without BTX-A, and both improved ROM to a greater degree than BTX-A alone. Casting demonstrated a significantly more robust impact on range of motion than BTX-A alone.

Botulinum Toxin A Injection of Salivary Glands in Children with Drooling and Chronic Aspiration

PURPOSE To review outcomes of ultrasound (US)-guided percutaneous submandibular gland injection of botulinum toxin A (BTX-A) in the treatment of drooling and chronic aspiration. MATERIALS AND METHODS A 3-year retrospective review was performed of 220 US-guided salivary gland injections in 36 patients. There were 21 male patients and 15 female patients with an age range of 1.4 to 19.8 years (mean, 8.6 y) and a weight range of 7.8 to 73 kg (mean, 24.4 kg). The mean pretreatment analysis period was 48 months and the mean follow-up period was 21 months. The study group was divided into groups with anterior (n = 9) and posterior (n = 27) drooling, with those with both (n = 10) included in the posterior group. RESULTS All procedures were technically successful. Bilateral submandibular injections were performed in 34 procedures and bilateral submandibular and parotid injections were performed in 38 procedures. Of the 27 patients with posterior drooling, improvement occurred in 24 patients (88%), no improvement was seen in two (8%), and one (4%) was lost to follow-up. Of the nine patients with anterior drooling, six (66%) showed improvement, there was no response in two (22%), and one (12%) was lost to follow-up. The total number of hospitalizations for respiratory issues and presumed aspiration pneumonia decreased by 56.4% per year in the patients with posterior drooling. There was one procedure-related complication: an episode of self-limited oral bleeding. CONCLUSION Salivary gland BTX-A injection for salivary control shows promising results in decreasing saliva production and frequency of respiratory symptoms in children with drooling and chronic aspiration.

Pediatric Stroke Recovery: A Descriptive Analysis

OBJECTIVE To investigate recovery patterns and potential prognostic factors of pediatric stroke. DESIGN Retrospective study. SETTING Acute rehabilitation at a university-based childrens hospital. PARTICIPANTS Children (N=44; 25 boys, 19 girls; age range, 8mo-17y) with diagnosis of first-ever stroke. INTERVENTIONS Not applicable. MAIN OUTCOME MEASURES Functional outcomes at discharge and 1-year follow-up. Modified Brunnstrom stages, Gross Motor Function Classification System, activities of daily living (ADLs), swallowing, speech, and sphincter function were measured. RESULTS Recovery of swallowing function occurred earlier than other functions in the first 2 to 3 months poststroke. Less than half of the patients were able to use the affected arms and legs without assistive devices. Eleven of 32 patients who initially had poor body control became ambulatory without assistive devices. A total of 18 of 44 patients were able to walk without assistive devices. Bilateral hemisphere lesions and flaccid muscle tone of the affected extremity at stroke onset had a less favorable prognosis in terms of ambulation and ADLs. Hemorrhagic strokes without surgical complications had a better prognosis than nonhemorrhagic strokes. CONCLUSIONS Similar to the adult stroke population, most of the functional recovery in pediatric stroke occurs within the first 2 to 3 months after stroke, but the quality of functional recovery was better in the pediatric population. The lesion size of the stroke was found to be related to prognosis. Additional large cohort studies are suggested to understand the complex similarities and differences in recovery between pediatric and adult stroke.

Trampolines, children, and strokes.

Wechsler B, Kim H, Hunter J: Trampolines, children, and strokes. Am J Phys Med Rehabil 2001;80:608–613.Strokes in children related to sports injuries are rare, but pediatric trampoline injuries are dramatically increasing. Minor trauma to the vulnerable extracranial vertebral arteries as they travel superficially through the dorsum of the neck can begin a cascade of events that results in arterial dissection, thrombus formation, and embolization with cerebral infarction. We present the case of an 11-yr-old boy who developed left vertebral artery dissection subsequent to a trampoline injury.

Intrarater range of motion reliability in cerebral palsy: a comparison of assessment methods.

Purpose: To compare intrarater reliability of goniometry performed with and without an assistant and compare the reliability of the Staheli and Thomas tests of hip extension. Visual estimation was also evaluated as a method of range of motion assessment. Methods: Twenty-five children with cerebral palsy (50 legs) were evaluated in a blind fashion. Results: Interclass correlations (ICCs) ranged from 0.9701 to 0.9804 and from 0.9685 to 0.9822 for 1 and 2-person goniometry, respectively. Pearson product moment correlations of 0.8944 to 0.9553 for visual estimation were established. Staheli and Thomas test ICCs were 0.9793 and 0.9804, respectively. Conclusions: Goniometry with 1 and 2 assessors both produced ICCs in the excellent range as did the Staheli and Thomas test measurements of hip extension. The use of an assistant did not provide additional benefit. Visual estimation showed excellent correlation with goniometry.

Cardiovascular regulation in multiple sclerosis

Traditional assessments of autonomic nervous system function have depended on invasive and complex procedures. Vagal power, which is the respiratory component of heart rate variability (HRV) is an alternative and non-invasive measure for indexing autonomic nervous control of the heart In the current study, 18 multiple sclerosis (MS) and 20 healthy subjects matched with respect to age, education and intelligence served as subjects. The MS group showed significantly lower vagal power during natural and paced breathing than healthy subjects. Importantly, heart rate did not differ between the two groups. If MS patients exhibit abnormalities in mechanisms mediating cardiac parasympathetic control, the impact on quality of life and vulnerability to adverse cardiac events need to be further evaluated. The results of this study may have implications with respect to the feasibility of using HRV as both a diagnostic and prognostic tool for evaluating parasympathetic nervous system dysfunction and in providing valuable information for developing more effective treatment and rehabilitation strategies.

Central alveolar hypoventilation in neurosarcoidosis

Although the lung is almost always involved in sarcoidosis and respiratory failure is not uncommonly caused by intrinsic lung disease, chronic alveolar hypoventilation on the basis of neurosarcoidosis has not been previously described. This report describes the case of a 42-year-old woman with sarcoidosis and documented involvement of the lungs, muscle, peripheral nerves, and skin. She presented with ventilatory failure and a vital capacity of 700mL and required hospitalization for intubation and mechanical ventilation. After failing ventilator weaning attempts and requiring continuous ventilatory assistance, she was extubated to continuous use of noninvasive intermittent positive pressure ventilation (IPPV). She eventually weaned to nocturnal-only use of noninvasive IPPV. However, her PaCO2 remained elevated during daytime hours despite the fact that her vital capacity had returned to 3,600mL. She was not obese, and her oxyhemoglobin saturation (SaO2) was normal on room air. Subsequent attempts to discontinue nocturnal nasal IPPV resulted in marked nocturnal decreases in SaO2 and return of symptoms. This case illustrates successful noninvasive management of an apparently unique occurrence of central alveolar hypoventilation caused by neurosarcoidosis.

Pediatric rehabilitation: 1. Common medical conditions in children with disabilities.

This self‐directed learning module focuses on the physiatric management of the common morbidities associated with pediatric traumatic brain injury and cerebral palsy. It is part of the study guide on pediatric rehabilitation in the Self‐Directed Physiatric Education Program for practitioners and trainees in physical medicine and rehabilitation and pediatric medicine. The goal of this article is to enhance the learners knowledge regarding current physiatric management of complications related with pediatric traumatic brain injury and cerebral palsy.