Pilot Single-Blind Trial of AbobotulinumtoxinA in Oromandibular Dystonia.

Oromandibular dystonia (OMD) causes involuntary movements of masticatory and lingual muscles impairing eating, speaking, and swallowing. Treatment options are limited. The objective of this study was to determine the safety and efficacy of abobotulinumtoxinA (aboBoNTA) in OMD. A dose-finding study (phase 1) followed by a single session, prospective, single-blind trial (phase 2) was carried out. OMD subjects were evaluated at baseline, 6 and 12 weeks. Muscles injected were tailored to individual symptoms using EMG guidance, but the aboBoNTA dose for each muscle was pre-specified based on phase 1 results. Evaluations were Global Dystonia Rating Scale (GDS), Unified Dystonia Rating Scale (UDRS), Clinical Global Impression (CGI) improvement and severity, and quality of life (OMDQ-25). Adverse events were monitored. The lowest dosage in phase 1 resulted in adverse effects in two of three patients and thus was used in phase 2. In phase 2, adverse effects were observed in 50% of subjects including dysphagia, voice change, and soft palate weakness. Most were mild. Significant improvement was seen in quality of life (OMDQ-25), speech (BFMq21), and change in GDS, UDRS, CGI severity assessed by the unblinded investigator, but not in blinded video ratings. We conclude that aboBoNTA therapy in this study was associated with improved quality of life and was generally well tolerated in OMD, but occurrence of dysphagia dictated the importance of using low genioglossus dosing. Face to face assessment appears to be more sensitive than video assessment for change in OMD severity. Consideration of the disability in OMD places constraints on traditional placebo-control trial design. Development of novel trial designs is warranted.

The "shirt collar sign" of cervical dystonia.

The diagnosis of cervical dystonia (CD) is clinical. We describe a physical examination observation that has been noted in CD patients. There is a tendency for their shirt collars to be shifted to one side. We validated this apparently consistent finding by having blinded evaluators rating the symmetry of the shirt collars in CD and non-cervical dystonia control subjects. A high correlation was found between the physical finding which we call "shirt collar sign" and the diagnosis. "Shirt collar sign" may be a helpful sign in diagnosing CD.

Case report of optic atrophy in Dentatorubropallidoluysian Atrophy (DRPLA).

BACKGROUND: Dentatorubropallidoluysian atrophy (DRPLA) is a rare autosomal dominant neurodegenerative disease that is associated with numerous movement disorders. Ocular problems also occur with DRPLA with reports of corneal endothelial degeneration in some patients living with the disease. We report a new visual problem associated with DRPLA, optic atrophy. CASE PRESENTATION: A 47 year-old man presented complaining of progressive visual loss associated with optic atrophy on ophthalmological evaluation. He gradually developed a progressive ataxia with dystonia. Brain MRI revealed a diffuse leukoencephalopathy. Genetic analysis revealed 62 CAG repeats in one allele of the DRPLA gene and he was diagnosed with DRPLA. CONCLUSION: Optic atrophy should be included in the clinical spectrum of DRPLA.

Variation in academic medical centers' coding practices for postoperative respiratory complications: implications for the AHRQ postoperative respiratory failure Patient Safety Indicator.

BACKGROUND: The Agency for Healthcare Research and Quality Patient Safety Indicator (PSI) 11 uses International Classification of Disease, 9th Clinical Modification diagnosis code 518.81 ("Acute respiratory failure")-but not the closely related alternative, 518.5 ("Pulmonary insufficiency after trauma and surgery")-to detect cases of postoperative respiratory failure. We sought to determine whether hospitals vary in the use of 518.81 versus 518.5 and whether such variation correlates with coder beliefs. STUDY DESIGN: We conducted a cross-sectional analysis of administrative data from July 2009 through June 2010 for UHC (formerly University HealthSystem Consortium)-affiliated centers to assess the use of diagnosis codes 518.81 and 518.5 in PSI 11-eligible cases. We also surveyed coders at these centers to evaluate whether variation in the use of 518.81 versus 518.5 might be linked to coder beliefs. We asked survey respondents which diagnosis they would use for 2 ambiguous cases of postoperative pulmonary complications and how much they agreed with 6 statements about the coding process. RESULTS: UHC-affiliated centers demonstrated wide variation in the use of 518.81 and 518.5, ranging from 0 to 26 cases and 0 to 56 cases/1000 PSI 11-eligible hospitalizations, respectively. Of 56 survey respondents, 64% chose 518.81 and 30% chose 518.5 for a clinical scenario involving postoperative respiratory failure, but these responses were not associated with actual coding of 518.81 or 518.5 at the center level. Sixty-two percent of respondents agreed that they are constrained by the words that physicians use. Their self-reported likelihood of querying physicians to clarify the diagnosis was significantly associated with coding of 518.5 at the center level. CONCLUSIONS: The extent to which diagnosis code 518.81 is used relative to 518.5 varies considerably across centers, based on local coding practice, the specific wording of physician documentation, and coder-physician communication. To standardize the coding of postoperative respiratory failure, the 518.81 and 518.5 codes have recently been revised to make the available options clearer and mutually exclusive, which may improve the capacity of PSI 11 to discriminate true differences in quality of care.

Detection of postoperative respiratory failure: how predictive is the Agency for Healthcare Research and Quality's Patient Safety Indicator?

BACKGROUND: Patient Safety Indicator (PSI) 11, or postoperative respiratory failure, was developed by the US Agency for Healthcare Research and Quality to detect incident cases of respiratory failure after elective operations through use of ICD-9-CM diagnosis and procedure codes. We sought to determine the positive predictive value (PPV) of this indicator. STUDY DESIGN: We conducted a retrospective cross-sectional study, sampling consecutive cases that met PSI 11 criteria from 18 geographically diverse academic medical centers on or before June 30, 2007. Trained abstractors from each center reviewed medical records using a standard instrument. We assessed the PPV of the indicator (with 95% CI adjusted for clustering within centers) and conducted descriptive analyses of the cases. RESULTS: Of 609 cases that met PSI 11 criteria, 551 (90.5%; 95% CI, 86.5-94.4%) satisfied the technical criteria of the indicator and 507 (83.2%; 95% CI, 77.2-89.3%) represented true cases of postoperative respiratory failure from a clinical standpoint. The most frequent reasons for being falsely positive were nonelective hospitalization, prolonged intubation for airway protection, and insufficient evidence to support a diagnosis of acute respiratory failure. Fifty percent of true-positive cases involved substantial baseline comorbidities, and 23% resulted in death. CONCLUSIONS: Although PSI 11 predicts true postoperative respiratory failure with relatively high frequency, the indicator does not limit detection to preventable cases. The PPV of PSI 11 might be increased by excluding cases with a principal diagnosis suggestive of a nonelective hospitalization and those with head or neck procedures. Removing the diagnosis code criterion from the indicator might also increase PPV, but would decrease the number of true positive cases detected by 20%.

Improving outcomes: focus on workplace issues.

PURPOSE OF REVIEW: Staff satisfaction has not traditionally been included as an intensive care unit quality indicator. The process of providing intensive care may profoundly affect clinicians. Dysfunctional encounters with coworkers and ethical burdens may extract a considerable personal toll and affect work attitudes and performance. RECENT FINDINGS: Mounting evidence indicates that psychosocial tensions, burnout and ethical stress are common and serious problems in the intensive care unit. These experiences impact negatively on job satisfaction, turnover, workplace disruption and patient care. Addressing workplace issues will help improve quality of care. SUMMARY: Two common sources of staff dissatisfaction are examined. Improving staff satisfaction can improve unit performance, and serve to attract and retain quality clinicians.

Anemia in the long-term ventilator-dependent patient with respiratory failure.

Anemia occurs in virtually all critically ill patients receiving long-term mechanical ventilation and has been associated with increased mortality and poor outcomes. Allogeneic RBC transfusions are routinely administered to critically ill anemic patients, especially during lengthy stays in ICUs or in long-term acute care facilities. Although RBC transfusions are a physiologically rational approach to raising hemoglobin levels, they may increase the risk of complications and have been associated with higher mortality in critically ill patients. Treatment with epoetin alfa, an erythropoiesis-stimulating agent, as a means of reducing transfusion requirements has been studied in the critically ill and in patients receiving long-term mechanical ventilation. Promising results have been reported, including a potential survival benefit, although larger and more definitive studies are needed in order to establish whether raising hemoglobin levels affects clinical outcomes in patients receiving mechanical ventilation.

Acute lung injury induced by arsenic trioxide in a patient with refractory myelodysplastic syndrome.

Arsenic trioxide has been used successfully for the treatment of refractory acute promyelocytic leukemia and has shown promise in the treatment of myelodysplastic syndrome (MDS), although it is not a labeled indication. Retinoic acid syndrome is manifested by fever, dyspnea, peripheral edema, pulmonary infiltrates, and pleural and/or pericardial effusions and is typically seen in conjunction with all- trans retinoic acid therapy of acute promyelocytic leukemia. We report a case of acute lung injury and a retinoic acid syndrome-like illness in a patient who received arsenic for MDS. To our knowledge, this is the first such report, and clinicians should be aware of this potentially life-threatening complication of arsenic trioxide treatment in patients with MDS.

Patient safety: a tale of two systems.

Patient safety remains a major issue for health care delivery systems and the American public. One community hospital has kept pace with this issue using traditional quality management approaches. A national multihospital health care system has leveraged its electronic medical record system to facilitate data collection and analysis of events impacting patient safety. The article describes both approaches for addressing the complex issues surrounding patient safety.