LongTerm Mental Problems in KleineLevin Malady
Sixty percent thought that their decision would be legally binding in relatives who do not have capacity.
This study showed that wishes for religious rites were common, however many do not know how to make them known. learn more If they do know about services, then people are highly likely to engage with the ACP process.
This study showed that wishes for religious rites were common, however many do not know how to make them known. If they do know about services, then people are highly likely to engage with the ACP process.It is often difficult for people with cancer to make decisions for their care. The aim of this review is to understand the importance of shared decisionmaking (SDM) in Indian clinical scenario and identify the gaps when compared to practices in the Western world. A systematic search (2000-2019) was executed in Medline and Google Scholar using predefined keywords. Of the approximate 400 articles retrieved, 43 articles (Indian 5; Western 38) were selected for literature review. Literature review revealed the paucity of information on SDM in India compared to the Western world data. This may contribute to patientreported physical or psychological harms, life disruptions, or unnecessary financial costs. Western world data demonstrate the involvement and sharing of information by both patient and physician, collective efforts of the two to build consensus for preferred treatment. In India, involvement of patients in the planning for treatment is largely limited to tertiary care centers, academic institutes, or only when the cost of therapy is high. In addition, cultural beliefs and prejudices impact the extent of participation and engagement of a patient in disease management. Communication failures have been found to strongly correlate with the medicolegal malpractice litigations. Research is needed to explore ways to how to incorporate SDM into routine oncology practice. India has a high unmet need towards SDM in diagnosis and treatment of cancer. link2 Physicians need to involve patients or their immediate family members in decision making, to make it a patient-centric approach as well. SDM enforces to avoid uninformed decisionmaking or a lack of trust in the treating physician's knowledge and skills. Physician and patient education, development of tools and guiding policies, widespread implementation, and periodic assessments may advance the practice of SDM.A literature search was started with the objective of finding works pertaining to the use of operations research techniques in planning for human resources in palliative care. Since the search indicated that there is no such work, in this paper, we report on the literature on workforce planning and human resource planning for palliative care personnel. Using our findings, we discuss the factors that influence the supply and demand for the palliative care workforce. Our results show that the enhancement of efficiency, training more primary caregivers to deliver palliative care, and allowing for mid-career specialist training are practical ways to compensate for the gap between the supply and demand in the palliative care workforce.Three prime repair exonuclease 1 (TREX1) degrades single- and double-stranded DNA with 3'-5' exonuclease activity. TREX1 mutations are related to type 1 interferon-mediated autoinflammation owing to accumulated intracellular nucleic acids. Several cases of systemic lupus erythematosus, Aicardi-Goutieres syndrome (AGS), familial chilblain lupus (FCL), and retinal vasculopathy-cerebral leukodystrophy caused by TREX1 mutations have been reported, so far. In this report, we described five patients with TREX1 mutations from three families with three different disorders, which include AGS, FCL, and FCL with central nervous system vasculitis.We report a 2.5-year-old Turkish boy who first presented with nystagmus, lack of eye contact, and hypotonia at 2 months of age and developed refractory seizures when 6 months old. Extensive metabolic tests and imaging being noncontributory, whole-exome sequencing was carried out which revealed a heterozygote NM_001134407.2C.3299A>G (p.Glu1100Gly) novel mutation in GRIN2A gene. Topiramate was started and seizures were rapidly brought under control. GRIN2A mutations may result in altered GluN2A membrane trafficking and response to glutamate. This report illustrates the clinical variability of GRIN2A mutations according to the age of onset of symptoms and suggests considering mutations in this gene in cases of global developmental delay, refractory epilepsy, and nystagmus.Megaconial congenital muscular dystrophy (OMIM 602541) is defined by early-onset hypotonia, mildly elevated serum creatine kinase (CK) levels, muscle wasting, cardiomyopathy, psychomotor developmental delay and intellectual disability. The disease is caused by loss-of-function mutations in Choline kinase beta gene (CHKB) and has specific muscle biopsy findings. Here we investigate two patients with weakness of proximal muscles and generalized muscle atrophy, skin changes, agressiveness, social communication and empathy difficulties. Both patients had mildly elevated serum CK levels. Whole exome sequencing (WES) performed for both patients and homozygous c.818+1G>A and homozygous c.1031+1G>A variants were detected in patient 1 and patient 2, respectively. We would like to draw the attention of autism spectrum disorder in early diagnosis of congenital muscular dystrophies.Strategic cortical lesions involving the hand motor cortex (HMC) presenting acutely as distal upper limb pure motor weakness certainly do need to be differentiated on clinical grounds from "pseudoperipheral palsy." This rare phenotype can imitate peripheral motor nerve deficits and should not be easily overlooked. The isolated "central hand and finger weakness" presenting as an acute onset of varying combinations such as pseudomedian, pseudoradial, and/or pseudoulnar nerve palsy is intriguing to the novice. In literature, this phenotype has been reported solely to result from cortical cerebral infarction and documented to occur in less then 1% of all ischemic strokes. The apropos of six "unforgettable patients" here highlights the heterogeneous pathophysiologic etiologies and mechanisms that included not only the conventional stroke risk factors but also hyperhomocysteinemia, common carotid artery thrombosis due to hyperhomocysteinemia and severe iron-deficiency anemia, biopsy-proven giant cell arteritis (GCA), cerebral metastasis, and dilated cardiomyopathy-related left ventricular thrombosis. Physicians and neurologists alike, as clinicians, need to be familiar with the peculiarities and clinical presentations of central hand control network cortical lesions.West Nile Virus (WNV) encephalitis CSF findings are usually described as polymorphonuclear pleocytosis initially followed by lymphocytic pleocytosis. We report a 68-year-old female with WNV encephalitis monocytic pleocytosis and flaccid quadriparesis with ventral roots enhancement. We suggest the inclusion of neuroinvasive WNV in the differential of encephalopathy with flaccid paralysis despite a monocytic pleocytosis. We also suggest the consideration of neuroinvasive in acute polyneuropathies unresponsive to immunotherapies.
Lack of compliance to medication and uncontrolled risk factors are associated with increased risk of recurrent stroke and acute coronary syndrome in patients with recent stroke. Multimodal patient education may be a strategy to improve the compliance to medication and early adoption of nonpharmacological measures to reduce the vascular risk factor burden in patients with stroke. We thus aim to develop multilingual short messaging services (SMS), print, and audio-visual secondary stroke prevention patient education package. The efficacy of the package will be tested in a randomized control trial to prevent major cardiovascular and cerebrovascular events.
In the
stage, intervention materials (SMS, video, and workbook) were developed. In the
stage, the package was independently assessed and modified by the stakeholders involved in the stroke patient care and local language experts. The modified stroke prevention package was tested for implementation issues (
stage).
Sixty-nine SMS, six videos, and ingual patient education material could be developed in a stepwise manner. The efficacy of the package to prevent major adverse cardiovascular events is being tested in the SPRINT INDIA study.
The availability and affordability of antiepileptic drugs (AEDs) are critical to the success of public health initiatives enabling care for people with epilepsy in the community.
To pilot survey the availability and affordability of AEDs in the community.
Field workers used standard WHO-Health Action International approaches and collected data on the availability of, and maximum retail prices of originator brands and least price generics of AEDs in 46 randomly selected public (
= 29), private (
= 8), and charitable (
= 9) pharmacy outlets. Median price ratios were computed apropos international reference prices of corresponding medications and affordability gauged with reference to daily wage of lowest paid worker.
Only 10 outlets (7 - private, 3 - public, and none - charitable) stocked at least one essential AED. Median price ratios varied between 1.1 and 1.5 essentially reflecting the difference between the least price generics and originator brands. Of note, carbamazepine-retard, 200 mg put up the slightest difference in prices of originator and least price generic brands and also was the most affordable AED.
The availability and affordability of most AEDs were poor and hence, this needs to be studied on a wider scale and thereafter efforts to improve both the availability and affordability are desirable in order to address the huge treatment gap for epilepsy in India.
The availability and affordability of most AEDs were poor and hence, this needs to be studied on a wider scale and thereafter efforts to improve both the availability and affordability are desirable in order to address the huge treatment gap for epilepsy in India.
A wide variety of neurological diseases result in clinical and/or radiological enlargement of nerves, roots and plexuses. link3 With the advancement in techniques and use of magnetic resonance neurography (MRN), aided by electrophysiology, proximal segments of the lower motor neuron (LMN) can be well studied. The relative merits of investigative modalities have not been well defined and comprehensive information on this subject is sparse.
This retrospective study included data from January 2010 to June 2018. Patients having clinical and/or radiological enlargements of lower motor neuron were included. Clinical and laboratory work up, electrophysiology, MRN and biopsy studies were documented and analyzed.
133 patients fulfilled the inclusion criteria. The diagnostic categories were of leprosy (32%), immune neuropathies (27.8%), nerve infiltrations (8.2%), inherited neuropathies (9%), diabetic radiculopathies (9%) and others (12.7%). MRN was essential to diagnosis in 24.8% and supportive in 31.5% patients. Electrophysiology was essential in diagnosis in 70.6%, biopsy in 45.8% and genetic studies in 6.4% patients.
The manuscript presents a large cohort of diseases causing enlargement of LMN with clinical and investigative aspects of 7 patients of the most unusual condition of chronic immune sensorimotor polyradiculopathy (CISMP) and details of 7 other patients with chronic mononeuropathies at non-entrapment sites. A table of comparative utility and an algorithm depicting the optimization of investigations has been presented.
The manuscript presents a large cohort of diseases causing enlargement of LMN with clinical and investigative aspects of 7 patients of the most unusual condition of chronic immune sensorimotor polyradiculopathy (CISMP) and details of 7 other patients with chronic mononeuropathies at non-entrapment sites. A table of comparative utility and an algorithm depicting the optimization of investigations has been presented.