may 2007

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3: Am J Phys Med Rehabil. 2007 May;86(5):349-55.

• Bach JR.

Department of Physical Medicine and Rehabilitation and Neurosciences, UMNDJ New Jersey Medical School, Newwark, New Jersey 07871, USA.

OBJECTIVES: To report intercurrent nonrespiratory complications of unprecedented survival for Werdnig-Hoffman disease (spinal muscular atrophy type 1 [SMA 1])\. DESIGN: A retrospective chart review and caregiver questionnaire for 103 consecutively referred SMA 1 patients for whom death was prevented during infancy\. RESULTS: Overall, 15 of 63 (23.8%) respondents had severe, symptomatic bradycardias\. Thirteen of 25 males had bilateral cryptorchidism, and two were unilateral\. Ten of 42 (24%) respondents had recurrent oral candidiasis, and eight (19.0%) had recurrent nonoral candidiasis\. All patients had hip dislocation/subluxation\. They had collapsing spines and scoliosis by 1 yr of age\. Ninety-six had indwelling gastrostomy/nasogastric tubes before 24 mos of age\. Twenty-six underwent fundoplication to decrease reflux\. Fifty-seven were on modified elemental diets with reported benefits\. About one half had early pubarche\. Three patients had episodes of acute pancreatitis\. One untreated patient died of candidal endocarditis at 4 mos of age, and a second child had mitral valve candidiasis on autopsy\. CONCLUSION: Prolonged survival of SMA 1 results in a high incidence of concomitant medical conditions that need to be addressed.

PMID: 17449979 [PubMed - indexed for MEDLINE]

2: J Rehabil Med. 2007 May;39(5):399-404.

• Laffont I,

• Dumas C,

• Pozzi D,

• Ruquet M,

• Tissier AC,

• Lofaso F,

• Dizien O.

Centre d'Innovations Technologiques, Hopital Raymond Poincare, Garches, France. isabelle.laffont@rpc.aphp.fr

OBJECTIVE: The aim of this study was to evaluate a speech synthesizer with respect to patterns of use and satisfaction, during a 2-month trial at home, and the usefulness of the word prediction function\. DESIGN: Prospective study\. PARTICIPANTS: Of the 24 patients with severe dysarthria recruited, 10 completed the study\. Five patients had cerebral palsy, 3 amyotrophic lateral sclerosis, one locked-in syndrome, and one anoxic brain damage\. Mean age was 32 (standard deviation 21) years (range 9-66 years)\. METHODS: Each participant received 10 hours of training with the device (Dialo) and then used it at home for 2 months\. The main outcome measures were: level of use recorded by the device, Quebec User Evaluation of Satisfaction with Assistive Technology (QUEST) satisfaction score (maximum = 5), and time needed to take dictations of standard-dictionary and personal-dictionary words with and without word prediction\. RESULTS: Level of use varied widely across participants\. Overall satisfaction at the end of the home trial was high, with a mean QUEST score of 3.4 (SD 1) and was related to the level of use of the device\. Level of satisfaction at the end of the training session could not predict the level of use at home\. No significant differences were found in dictation-taking times with and without word prediction\. However, 6 of the 10 patients took dictation faster with than without word prediction\. CONCLUSION: This study provides the first evidence supporting the benefits of a speech synthesizer used at home for several weeks\. Word prediction is useful for some patients even if increase in dictation speed did not reach significance.

PMID: 17549332 [PubMed - indexed for MEDLINE]