Clusters of cognitive impairment among different phenotypes of myotonic dystrophy type 1 and type 2

Neuropsychological examinations in myotonic dystrophy (DM) patients show a great variability of results from a condition of intellectual disability to the subtle cognitive impairments. It is unclear if different clusters of neuropsychological deficits appear in different phenotypes of DM, or if there are patients with no cognitive deficit at all. The aim of this study is to assess cognitive impairments among patients with different phenotypes of DM type 1 (DM1) and type 2 (DM2), and to potentially define cognitive clusters in these disorders. Study comprised 101 DM1 and 46 DM2 adult patients who were genetically confirmed. Patients underwent analysis of five cognitive domains (visuospatial, executive, attention, memory and language). Virtually all DM1 patients had cognitive defect with approximately 2–3 cognitive domains affected. On the other hand, one-third of DM2 patients had completely normal neuropsychological findings, and in other two-thirds approximately 1–2 domains were affected. Cluster analysis showed that in both diseases visuospatial and executive dysfunctions seemed to be the main cognitive defects, while memory and language impairments appeared in more severe phenotypes. Our results showed that a single form of DM1 or DM2 may consist of several cognitive clusters. Understanding of cognitive impairments in DM is very important to follow positive and side effects in ongoing and future clinical trials.     

Brain sonography insight into the midbrain in myotonic dystrophy type 2

Introduction: The aim of this study was to analyze transcranial sonography (TCS) findings in genetically confirmed myotonic dystrophy type 2 (DM2) patients. Methods: Forty DM2 patients and 38 gender‐ and age‐matched healthy controls (HCs) underwent TCS through the pre‐auricular acoustic bone window. Results: Substantia nigra hyperechogenicity was found in 20% of DM2 patients compared with 3% of HCs. Brainstem raphe (BR) hypoechogenicity was more common in DM2 patients compared with HCs (56% vs. 10%, P < 0.01), and it was more common in patients with fatigue and excessive daytime sleepiness (P < 0.05). Diameter of the third ventricle was increased in DM2 patients compared with HCs (5.8 ± 1.7 vs. 5.1 ± 1.0 mm, P < 0.05). Conclusions: Finding BR hypoechogenicity might have clinical implication because of the potential response to serotonin‐reuptake inhibitors. TCS revealed alterations in brain structures previously not seen in MRI studies. Muscle Nerve 53 : 700–704, 2016     

Predictors of work disabilities in patients with panic disorder with agoraphobia.

OBJECTIVE: The aim of this study was to ascertain predictors of work insufficiency in patients with panic disorder (PD) with agoraphobia (AG). METHOD: Linear regression was used to identify predictors of work insufficiency in a sample of 72 consecutive outpatients with PD with AG. Intensity of work insufficiency was ascertained from modified National Institute of Mental Health Panic Questionnaire (NIMH PQ). That represented dependent variable. Independent variables were demographic data, duration of illness, presence of comorbid current major depression episode, presence of any personality disorder and scores on the Panic and Agoraphobia Scale (PAS) subscales: panic attacks, AG (avoidance behavior), anticipatory anxiety and worries about health. RESULTS: Patients reported severe work insufficiency. The best predict variable for the work insufficiency in patients with PD with AG was high score on the PAS dimension of AG. CONCLUSION: Patients generally reported severe effects of PD with AG on work efficacy and the results suggested that the impaired work efficacy was the most associated with avoidance behavior. These results recommend that the treatment of PD with AG patients should be related to decreasing avoidance behavior in order to establish adequate work performance in patients.     

Silica zeolite scavenging of exhaled isoflurane: A preliminary report

PURPOSE: We evaluate the effectiveness of a silica zeolite (Deltazite) hydrophobic molecular sieve adsorbent, in removing exhaled isoflurane. METHODS: In three experiments, a simulated anesthesia mannequin was ventilated using 1% isoflurane in nitrous oxide and oxygen (1:1 ratio) at a gas flow of 3 L x min-1. Airway pressures, end-tidal carbon dioxide [ETCO2], inspired and end-tidal isoflurane were measured. The scavenging line was connected to a canister containing 750 g of the silica zeolite. Concentrations of isoflurane entering and exiting the canister were measured, as well as the pressure gradient across the canister and gas flow through the canister. In phase 1 (n = 3), the mannequin was ventilated for 6.5 hr, followed by phase 2 where a test lung replaced the simulator. The time (phase 1 plus phase 2) until isoflurane 'breakthrough' (> 0.02%) was noted. RESULTS: The average canister weight increase was 68 g, however 92 g of isoflurane were used. The isoflurane concentration exiting the canister remained undetectable throughout phase 1 in each experiment. The pressure gradient across the canister averaged 0.13 cm H2O and did not increase throughout phase 1. The time to 'breakthrough' (phase 1 plus phase 2) was 8.0 hr, 8.8 hr and 9.0 hr. CONCLUSIONS: Silica zeolite was effective at completely removing 1% isoflurane from exhaled gases for periods of eight hours. The technology shows promise in removing isoflurane emitted from anesthesia machine scavenging systems.