ABSTRACT
BACKGROUND: Patients with Multiple System Atrophy (MSA) frequently report non-motor symptoms, and several research groups have highlighted this. OBJECTIVE: We systematically searched for and reviewed papers assessing prevalence of non-motor symptoms (NMS) in MSA patients as reported in the scientific literature. METHODS: We performed a systematic review of studies of subjects with MSA (involving > 10 patients) who were assessed for NMS, published in the English literature in PUBMED and EMBASE databases from 1947-2022. RESULTS: 23 research papers, with data from 2648 clinically diagnosed and 171 pathologically verified cases of MSA were included, along with 238 controls. Mean age for MSA cases was 61.3 (9.2) years, mean disease duration 3.6 (2.7) years. 57.9% were male. Our analysis showed that the prevalence of cognitive issues in MSA varied widely (between 15-100%); dementia per se was uncommon, but assessment in advanced stages of MSA is impacted by unintelligible speech (which may be noted in a quarter of cases). The prevalence of depressive symptoms in MSA was between 44-88%. Sleep disturbances were reported by 17-89%, with REM-sleep behaviour disorder (RBD) rates as high as 75%. Pain was reported by 40-47% of patients: rheumatic or musculoskeletal sources of pain being commonest. Fatigue was reported by 29-60% of patients. Symptoms of autonomic failure in MSA were seen in 34-96.5% patients at baseline. CONCLUSION: In routine clinical practice, NMS in MSA are under-recognised by clinicians. These impact hugely on patient quality of life and contribute to their overall morbidity. A methodical ascertainment of these complaints will address an unmet need, and lead to a more holistic approach of care for individuals with MSA.
ABSTRACT
Background: Cerebellar ataxias comprise a large group of heterogeneous disorders with both motor and non-motor symptoms (NMS). Objective: We wanted to ascertain the reported prevalence of NMS in different subtypes of hereditary cerebellar ataxias. Methods: Systematic review of studies of hereditary cerebellar ataxias (involving >5 patients) who were assessed for NMS, published in the English literature in PUBMED and EMBASE databases from 1947 to 2021. Results: A total of 35 papers, with data from 1311 autosomal dominant spinocerebellar ataxia (SCA), 893 autosomal recessive cerebellar ataxia (ARCA), and 53 X-linked ataxia cases were included with a total of 450 controls. Mean age for SCA cases at diagnosis was 47.6 (SD, 14.9) years, for ARCA cases was 34.6 (SD, 14.7) years and for X-linked ataxia cases was 68.6 (9.1) years. The prevalence of cognitive problems in SCAs was between 23% and 75% (ranging from mild to severe), being least prevalent in SCA6. The prevalence of depression in SCAs was between 13% and 69% and sleep disorders were between 7% and 80%. Pain was reported by 18% to 60% of patients, especially in SCA3, and fatigue by 53% to 70%. The prevalence of reported cognitive dysfunction in ARCA was 12.5% to 100% and depression between 14% and 51%. The prevalence of anxiety in X-linked ataxias (FXTAS) was 17 % and depression 55%. Conclusions: The presence of NMS in hereditary cerebellar ataxias is common. The prevalence and spectrum of NMS in SCAs, ARCAs, and X-linked ataxias vary. In routine clinical practice, NMS in cerebellar ataxias are under-recognized and certainly under-reported. Therefore, they are unlikely to be addressed adequately. Improved ascertainment of NMS in cerebellar ataxias in clinical practice will enable holistic treatment of these patients.
ABSTRACT
Background and Objectives: Spontaneous, non-traumatic intracerebral hemorrhages (ICH) is the second most common cause of stroke after acute ischemia. However, it causes significant mortality and morbidity and has comparatively worse outcomes. Most studies show that ICH when treated with good medical care has lower mortality. Our primary aim was to evaluate how ICH is currently managed at the National hospital of Sri Lanka (NHSL), the premier tertiary care hospital in the country, whilst identifying strengths, weaknesses and deficiencies. We plan to develop a comprehensive management protocol for ICH patients and to reevaluate the treatment practices after applying them. NHSL is a research centre for the international, multicenter INTERACT2 trial, which emphasizes intensive blood pressure, blood sugar and temperature control and managing coagulopathy in ICH leading to better outcomes. We are recruiting new ICH cases to this trial. Methodology: The prospective standard audit was conducted at NHSL for a period of 3 months and the study population consisted of 76 patients with spontaneous ICH. The 2015 AHA/ASA guideline for the management of spontaneous intracerebral hemorrhage was used as the standard guide for the management. Results: Of 76 patients with ICH 46 (60.5%) were males and mean age was 61.3+- 12.6 years. The blood pressure (BP) and blood sugar control was achieved only in 51.3% (39) and 15.8% (12) respectively. Although prophylactic anti-epileptics are not recommended in ICH almost 70% were started on them, in spite of only 15.8% having overt convulsions. Steroids though not recommended to lower intracranial pressure in ICH 51% were on them. Of those ICH admitted to medical and neurology wards 81% were referred to neurosurgery irrespective of the location and the nature of the bleed and when not indicated (based on AHA/ASA guidelines) delaying and interfering with optimal medical care. Conclusions: Several deficiencies were identified in the current management routines of ICH at NHSL including delayed, suboptimal medical care, unnecessary widespread use of AEDs and steroids. Large majority of neurosurgical referrals too were unnecessary and not indicated.
