Systematic review of the patient burden of generalised myasthenia gravis in Europe, the Middle East, and Africa.

BACKGROUND: Myasthenia gravis (MG) is a rare autoimmune disease characterised by muscle weakness, and progression from ocular (oMG) to generalised (gMG) symptoms results in a substantial negative impact on quality of life (QoL). This systematic review aimed to provide an overview of the patient burden experienced by people living with gMG. METHODS: Electronic database searches (conducted March 2022), supplemented by interrogation of grey literature, were conducted to identify studies reporting patient burden outcomes in patients with gMG in Europe, the Middle East and Africa. Results were synthesised narratively due to the heterogeneity across trials. RESULTS: In total, 39 patient burden publications (representing 38 unique studies) were identified as relevant for inclusion in the systematic review, consisting of 37 publications reporting formal patient-reported outcome measures (PROMs), and two publications describing alternative qualitative assessments of patient experience. The studies included a variety of measures including generic and disease-specific PROMs, as well as symptom-specific PROMs focusing on key comorbidities including depression, anxiety, fatigue and sleep disturbance. The findings showed some variation across studies and PROMs; however, in general there was evidence for worse QoL in patients with gMG than in healthy controls or in patients with oMG, and a trend for worsening QoL with increasing MG severity. CONCLUSIONS: This review highlights the importance of considering patient QoL when developing and assessing treatment and management plans for patients with gMG. However, the heterogeneity identified across studies illustrates the need for further representative and well-powered studies in large cohorts administering consistent, validated questionnaires. TRIAL REGISTRATION: The protocol for this systematic review was registered in PROSPERO: CRD42022328444.

Cognitive failure in patients with terminal cancer: a prospective study.

In a prospective open study, 61 consecutive patients with terminal cancer admitted to the hospital underwent cognitive assessment using the Mini-Mental State Questionnaire three times a week between admission and discharge or death. Mini-Mental State Questionnaire score upon admission was 28 +/- 1.5 in patients who were discharged (N = 14), and 25 +/- 3 in patients who died in the hospital (N = 47, P less than 0.01). The forty-seven patients who died in the hospital presented a total of 66 episodes of cognitive failure (CF) that were defined as a score of less than 24 or a drop of greater than 30% in the score on the Mini-Mental State Questionnaire. Of these 47 patients, 39 (83%) presented CF an average of 16 days before death. Upon detection of CF, a complete medical examination, laboratory evaluation, computerized tomography of the brain if indicated by abnormal findings on medical examination, and a complete medication review were performed. The cause of CF could not be established in 37 (56%) cases. Drugs, sepsis, and brain metastasis were the most frequently detected causes and were present in 6, 4, and 4 cases, respectively. In addition, 22 episodes (33%) of CF improved (10 episodes spontaneously and 12 episodes as a result of treatment). Our findings suggest that CF is extremely prevalent during the last weeks of life and, consequently, informed consent for therapeutic or research procedures or resuscitation may be impossible to obtain reliably at that stage.

Preoperative cardiac evaluation.

A thorough history and a complete physical examination will detect most cardiac problems that can affect perioperative management or outcome. Further diagnostic tests, including an electrocardiogram, a chest radiograph and more sophisticated studies, should be performed if indicated by the history and physical examination. Congestive heart failure and a myocardial infarction within the six months before surgery are the most important risk factors for postoperative cardiac events, but almost all cardiovascular diseases have some impact on perioperative management and risk stratification. Regional anesthesia is no safer than general anesthesia, except under a few circumstances.

Primary splenic hairy cell leukaemia: a case report and review of the literature.

Hairy cell leukaemia affecting primarily the spleen is a very rare feature of this disease at presentation. Splenectomy in such cases would seem to provide a cure. We report a case of primary splenic hairy cell leukaemia in which clinical and haematological remission were achieved after splenectomy, and we review the literature.

Acute appendicitis in the elderly: a 5-year retrospective study.

Acute appendicitis in elderly patients has not been studied extensively in the United Kingdom. Studies were performed prior to the regular use of broad spectrum antibiotics and tended to include younger patients. We studied retrospectively all elderly patients (n = 30, mean age = 72 years) who presented in the years 1980-85 and a younger group (n = 30, mean age = 23 years) who had a confirmed pathological diagnosis of appendicitis. We found a significant reduction in accurate pre-operative diagnoses, a longer duration of hospital stay and increased complication and mortality rates in the elderly group. Our study suggests that diagnostic accuracy, mortality and morbidity have not improved in comparison to earlier overseas studies.

Carcinoid tumour presenting as recurrent gastric outlet obstruction: a case of long-term survival.

A rare gastric tumour which presented on two occasions with gastric outlet obstruction is described. The important feature is that a gastric tumour presenting in this manner is not necessarily carcinoma.