Citations

Thirty patients with apraxia of speech, 10 patients with aphasia and no apraxia of speech, and 30 normal subjects were tested by three oral sensory-perceptual measurements: oral form identification, two-point discrimination, and mandibular kinesthesia. The apraxia-of-speech group was significantly inferior to the aphasic and normal groups on all three oral sensory-perceptual tests. The normal and aphasic groups did not differ significantly from each other. Apraxia of speech and oral sensory-perceptual deficit were related in that the more severe the apraxia of speech, the more profound the oral sensory-perceptual deficit. Not all patients with apraxia of speech demonstrated impaired oral sensation and perception; however, higher cortical sensory dysfunction frequently accompanied apraxia of speech.

Rate and regularity of diadochokinetic syllable repetitions of /pΛ/, /tΛ/, and /kΛ/ in 30 normal subjects, 30 subjects with spastic dysarthria, and 30 subjects with ataxic dysarthria were measured by computer. Normal subjects had rates (syllables per second) of 6.4, 6.1, and 5.7; spastic dysarthric subjects, 4.6, 4.2, and 3.5; and ataxic dysarthric subjects, 3.8, 3.9, and 3.4 for /pΛ/, /tΛ/, and /kΛ/, respectively. Spastic and ataxic subjects were significantly slower and more variable than normal subjects and ataxic subjects were significantly more variable than spastic subjects. The significantly slower than normal rate of repetition in the ataxic subjects and the significantly more variable than normal rhythm of repetition in the spastic subjects were unexpected findings and are in contrast with results from perceptually based investigations of dysarthria. The study demonstrates that slowness of syllable repetition is not restricted to spastic dysarthria and that dysrhythmia of syllable repetition is not restricted to ataxic dysarthria, thus suggesting the need for additional quantitative measurements of acoustic features on which certain notions about the dysarthrias are currently based.

Laryngo-esophagectomy was performed with a colon transplant to replace the excised esophagus. The patient did not develop functional “colonic” speech but does function adequately using an artificial larynx. Cineradiologic evidence reveals that the transplanted segment of colon permits development of a pseudoglottis to produce sound. He was capable of injecting air and forming the pseudoglottis, but he interfered with the production of colonic voice by retropositioning his tongue to close the hypopharynx.

PurposeAdvanced systemic mastocytosis (SM), a fatal hematopoietic malignancy characterized by drug resistance, has no standard therapy. The effectiveness of allogeneic hematopoietic stem-cell transplantation (alloHCT) in SM remains unknown.Patients and MethodsIn a global effort to define the value of HCT in SM, 57 patients with the following subtypes of SM were evaluated: SM associated with clonal hematologic non–mast cell disorders (SM-AHNMD; n = 38), mast cell leukemia (MCL; n = 12), and aggressive SM (ASM; n = 7). Median age of patients was 46 years (range, 11 to 67 years). Donors were HLA-identical (n = 34), unrelated (n = 17), umbilical cord blood (n = 2), HLA-haploidentical (n = 1), or unknown (n = 3). Thirty-six patients received myeloablative conditioning (MAC), and 21 patients received reduced-intensity conditioning (RIC).ResultsResponses in SM were observed in 40 patients (70%), with complete remission in 16 patients (28%). Twelve patients (21%) had stable disease, and five patients (9%) had primary refractory disease. Overall survival (OS) at 3 years was 57% for all patients, 74% for patients with SM-AHNMD, 43% for those with ASM, and 17% for those with MCL. The strongest risk factor for poor OS was MCL. Survival was also lower in patients receiving RIC compared with MAC and in patients having progression compared with patients having stable disease or response.ConclusionAlloHCT was associated with long-term survival in patients with advanced SM. Although alloHCT may be considered as a viable and potentially curative therapeutic option for advanced SM in the meantime, given that this is a retrospective analysis with no control group, the definitive role of alloHCT will need to be determined by a prospective trial.

Background: Clinical decision support (CDS) for primary care has been shown to improve delivery of preventive services. However, there is little evidence for efficiency of physicians due to CDS assistance. In this article, we report a pilot study for measuring the impact of CDS on the time spent by physicians for deciding on preventive services and chronic disease management. Methods: We randomly selected 30 patients from a primary care practice, and assigned them to 10 physicians. The physicians were requested to perform chart review to decide on preventive services and chronic disease management for the assigned patients. The patients assignment was done in a randomized crossover design, such that each patient received 2 sets of recommendations—one from a physician with CDS assistance and the other from a different physician without CDS assistance. We compared the physician recommendations made using CDS assistance, with the recommendations made without CDS assistance. Results: The physicians required an average of 1 minute 44 seconds, when they were they had access to the decision support system and 5 minutes when they were unassisted. Hence the CDS assistance resulted in an estimated saving of 3 minutes 16 seconds (65%) of the physicians’ time, which was statistically significant ( P < .0001). There was no statistically significant difference in the number of recommendations. Conclusion: Our findings suggest that CDS assistance significantly reduced the time spent by physicians for deciding on preventive services and chronic disease management. The result needs to be confirmed by performing similar studies at other institutions.