The Effects of Tyrosine Kinase Inhibitors (TKIs) in Monotherapy and with Add-on Treatments on Health-related Quality of Life of People with Chronic Myeloid Leukemia: A Systematic Review of Randomized-Controlled Trials.

Background: The era of establishing tyrosine kinase inhibitors (TKIs) in the treatment of chronic myeloid leukemia (CML) changed the outcome and the course of this life-threatening malignancy. People suffering from CML have now a better prognosis and a longer life expectancy due to the development of TKIs, even if it requires long-term, often lifelong, treatments that are nonetheless associated with improved Health-related Quality of life (HRQoL). However, data on the effects of TKIs on HRQoL are not always systematic; sometimes the data have been obtained by studies different from RCTs, or without a clear definition of what HRQoL is. The main purpose of this systematic review is to summarize all randomized-controlled trials (RCTs) including HRQoL as main or secondary outcome in patients with CML treated with TKIs or with TKIs plus an add-on treatment. Methods: A systematic review has been conducted by searching the relevant papers in PubMed/Medline and Web of Science with the following keywords: "quality of life" OR "health-related quality of life" OR "QoL" OR "HRQoL" OR "H-QoL" AND "chronic myeloid leukemia". Interval was set from January 2000 to December 2020. Results: 40 papers were identified through the search. Out of them, 7 RCTs were included. All the studies used standardized measures to assess HRQoL, even not always specific for CML. 5 RCTs randomized subjects to 2 or 3 arms to evaluate the effects of TKIs of the first, second and third generation in monotherapy. 2 RCTs randomized subjects to TKI therapy plus an add-on treatment versus TKI therapy as usual. The results of all these trials were examined and discussed. Conclusion: All the included RCTs pointed out significant findings regarding the positive effects of TKIs on HRQoL of people with CML, both when they were used in monotherapy or, notably, with an add-on treatment to enhance TKIs effects.

Development of a Clinical Rating Scale for the Severity of Apraxia of Eyelid Opening, Either Isolated or Associated with Blepharospasm.

BACKGROUND: Apraxia of eyelid opening is a movement disorder characterized by an inability to raise the eyelids without any overt contractions of the orbicularis oculi muscle. There is currently no clinical scale to rate the severity of this condition. OBJECTIVES: To develop and validate a novel scale that considers phenomenological aspects relevant to the severity of the condition. METHODS: The study sample included 20 patients with apraxia of eyelid opening, either isolated (9 patients) or associated with blepharospasm (11 patients). To validate the scale, selected features were checked for reliability, reliable items were combined to generate the scale, and clinimetric properties were evaluated. RESULTS: The novel severity scale yielded acceptable reliability, scaling assumptions, internal consistency, and sensitivity to change; a lack of floor and ceiling effects; and no correlation with the blepharospasm severity rating scale. CONCLUSIONS: We propose a severity scale that considers the most relevant apraxia of eyelid opening motor abnormalities based on objective criteria. This scale can be reliably administered by general neurologists after a brief training.

Staff Perception of Respect for Human Rights of Users and Organizational Well-being: A Study in Four Different Countries of the Mediterranean Area.

BACKGROUND: The perception by mental health service staff of respect for users' rights is a fundamental component of organizational well-being. The objective of this work is to examine how cultural differences and the working context can influence the perception of respect for users' rights in mental health professionals in the Mediterranean area. METHODS: An observational survey carried out in four different mental health networks in four countries of the Mediterranean area (Tunisia, North-Macedonia, Italy, Palestine). Each invited participant fulfilled a format on socio-demographic information and coded the Well-Being at Work and Respect Right Questionnaire (WWRR). All data were encrypted and analysed using the Statistical Package for Social Sciences (SPSS) version 20. The Games-Howell post-hoc test was used to assess differences between countries. The Games-Howell test does not assume equal variances and sample sizes. Eta-squared (η2) was used as a measure of effect size in the ANOVA (η2 around 0.01, 0.06, and 0.14 are considered small, medium, and large, respectively). RESULTS: The sample included 590 professionals working in the mental health field. The four countries showed statistically significant differences with regards to the quality rights assessment tool. Participants from Italy reported, on average, the highest scores across the questions. There were also differences across the countries about the perception of the impact of available resources on the effectiveness of care (η2 = 0.106). CONCLUSION: Our findings offer a useful insight into the perception of the quality of mental health services, especially from a users' rights point of view.

Bipolar disorder and antithyroid antibodies: review and case series.

Mood disorders and circulating thyroid antibodies are very prevalent in the population and their concomitant occurrence may be due to chance. However, thyroid antibodies have been repeatedly hypothesized to play a role in specific forms of mood disorders. Potentially related forms include treatment-refractory cases, severe or atypical depression, and depression at specific phases of a woman's life (early gestation, postpartum depression, perimenopausal). With regard to bipolar disorder, studies of specific subgroups (rapid cycling, mixed, or depressive bipolar) have reported associations with thyroid antibodies. Offspring of bipolar subjects were found more vulnerable to develop thyroid antibodies independently from the vulnerability to develop psychiatric disorders. A twin study suggested thyroid antibodies among possible endophenotypes for bipolar disorder. Severe encephalopathies have been reported in association with Hashimoto's thyroiditis. Cases with pure psychiatric presentation are being reported, the antithyroid antibodies being probably markers of some other autoimmune disorders affecting the brain. Vasculitis resulting in abnormalities in cortical perfusion is one of the possible mechanisms.

VNS induced desynchronization in gamma bands correlates with positive clinical outcome in temporal lobe pharmacoresistant epilepsy.

The vagus nerve stimulation (VNS) represents a diffuse non-pharmacological low-risk surgical option for epilepsy treatment. The aim of this study is to investigate the correlation between variations of global EEG synchronization and the clinical outcome in pharmacoresistant epileptic subjects implanted with VNS. Ten subjects affected by pharmacoresistant epilepsy were recruited on the basis of a clear-cut successful or unsuccessful outcome of the VNS add-on treatment. After five years from VNS surgery we examined the EEG in five subjects in each group. The investigation was led with the method of the phase lag index (PLI), which allows for the study of the global rate of synchronicity among the EEG signals before and after VNS implantation. The results of this study show that after five years from VNS surgery, in subjects whose seizures show a significant reduction, the desynchronization in the gamma frequency band is statistically decreased in comparison with patients who failed to show variations in the frequency and characteristics of their seizures. The other frequency bands are unaffected. This finding suggests that long lasting variations in gamma band desynchronization can be a new tool in assessing the efficacy of VNS. The possibility that GABA-mediated VNS-induced effects can also play a role in this result is discussed.

Affective psychosis, Hashimoto's thyroiditis, and brain perfusion abnormalities: case report.

BACKGROUND: It has recently become evident that circulating thyroid antibodies are found in excess among patients suffering from mood disorders. Moreover, a manic episode associated with Hashimoto's thyroiditis has recently been reported as the first case of bipolar disorder due to Hashimoto's encephalopathy. We report a case in which Hashimoto's thyroiditis was suspected to be involved in the deteriorating course of mood disorder and discuss potential pathogenic mechanisms linking thyroid autoimmunity with psychopathology. CASE PRESENTATION: A 43-year-old woman, with a history of recurrent depression since the age of 31, developed manic, psychotic, and soft neurological symptoms across the last three years in concomitance with her first diagnosis of Hashimoto's thyroiditis. The patient underwent a thorough medical and neurological workup. Circulating thyroperoxidase antibodies were highly elevated but thyroid function was adequately maintained with L-thyroxine substitution. EEG was normal and no other signs of current CNS inflammation were evidenced. However, brain magnetic resonance imaging evidenced several non-active lesions in the white matter from both hemispheres, suggestive of a non-specific past vasculitis. Brain single-photon emission computed tomography showed cortical perfusion asymmetry particularly between frontal lobes. CONCLUSION: We hypothesize that abnormalities in cortical perfusion might represent a pathogenic link between thyroid autoimmunity and mood disorders, and that the rare cases of severe Hashimoto's encephalopathy presenting with mood disorder might be only the tip of an iceberg.

Bed footboard peroneal and tibial neuropathy. A further unusual type of Saturday night palsy.

An uncommon cause of bilateral tibial and peroneal compression neuropathy is reported. After taking alcohol and drugs, a young heroin-addicted man lay unconscious overnight in supine position, with both legs crossing the wooden board at the end of the bed, the posterior aspect of the flexed knees pressing against its edge. The following day, he had weakness of foot flexion and extension and a sensory loss consistent with a bilateral tibial and peroneal neuropathy. Symptoms resolved rapidly in the left side; in the right side, a conduction block was still demonstrable 3 weeks later.

Electroneurography index based on nerve conduction study data: method and findings in control subjects.

We developed a summary score of data obtained from nerve conduction studies (NCS). The principle of such approach is that when a nonrandom trend to lower amplitudes or conduction velocities is present, it may be revealed by a summary nerve conduction score. In a group of normal subjects, peroneal, sural, ulnar, and superficial radial nerves were studied; age- and height-related F-wave and soleus H-reflex latencies were also examined. Z-scores of distal latencies, amplitudes, conduction velocities, F-wave latencies, and H-index were averaged in order to obtain three electroneurography (ENG) indices: a simple arithmetic (ENG index 1) and two weighted means (index 2 and index 3), assigning a double or triple weight to lower limb z-scores. Reference limits were established using multiple regression equations of ENG indices against age and height. This technique could be useful in providing a better cut-off between normal and diseased populations and in improving test-retest variability of NCS when follow-up studies are required.