Aims: To evaluate the behavioural and psychological symptoms of dementia (BPSD), to determine their correlation with types and stages of dementia and patient demographics, and to assess the impact on caregiver distress. Methods: This cross-sectional study recruited consecutive dementia patients and caregivers who attended our cognitive clinic. Standard criteria were used to classify types of dementia. BPSD were assessed with the Neuropsychiatric Inventory, and its distress scale was used for caregiver distress. Results: Of a total 107 patients, nearly all (99.1%) had at least one BPSD; 71% had ≥4 symptoms. Most frequent were apathy and agitation, followed by irritability, sleep and appetite disorders, and mood disorders; disinhibition and euphoria were least frequent. BPSD were less prominent with increasing age; males showed more agitation. Apathy and eating disorders were more prevalent in the rural community. BPSD were highest in frontotemporal dementia (FTD), followed by dementia with Lewy bodies (DLB), and least in vascular dementia. Hallucinations were more common in DLB, aberrant motor behaviour in FTD. All domains of BPSD, except for anxiety and euphoria, were more prominent with increasing severity of dementia. Increasing BPSD (except for euphoria) caused higher caregiver distress. Conclusion: BPSD are universally present, bear correlates with dementia type and severity, and cause significant caregiver distress.
Purpose To report the clinical features of eight patients presenting with emulsification of the heavier than water vitreous substitute, Densiron-68. Methods Two patients underwent primary inferior retinal detachment (RD) surgery, two patients underwent giant retinal tear repair, three patients had repair of inferior RD complicated by proliferative vitreoretinopathy and one patient had inferior RD surgery following repair of a scleral rupture. All patients had insertion of Densiron-68. Results Significant emulsification of Densiron-68 was seen within 12 weeks of surgery in eight cases out of a total of 40 patients receiving Densiron-68. Conclusion Despite adequate Densiron fills, emulsification necessitated its removal. Emulsified Densiron may have contributed to significant intraocular inflammation, epiretinal membrane formation and cystoid macular oedema. Without removal, prolonged presence of emulsified Densiron may lead to keratopathy, secondary glaucoma and retinal toxicity secondary to partitioning of perfluorohexyloctane. This has potentially significant implications on the indications for Densiron-68 use and warrants consideration before deciding on the optimal surgical intervention for inferior RDs.
Type 2 diabetes mellitus (T2DM) can lead to death without treatment and it has been predicted that the condition will affect 215 million people worldwide by 2010. T2DM is a multifactorial disorder whose precise genetic causes and biochemical defects have not been fully elucidated, but at both levels, calpains appear to play a role. Positional cloning studies mapped T2DM susceptibility to CAPN10, the gene encoding the intracellular cysteine protease, calpain 10. Further studies have shown a number of noncoding polymorphisms in CAPN10 to be functionally associated with T2DM while the identification of coding polymorphisms, suggested that mutant calpain 10 proteins may also contribute to the disease. Here we review recent studies, which in addition to the latter enzyme, have linked calpain 5, calpain 3, and its splice variants, calpain 2 and calpain 1 to T2DM-related metabolic pathways along with T2DM-associated phenotypes, such as obesity and impaired insulin secretion, and T2DM-related complications, such as epithelial dysfunction and diabetic cataract.
Premature visual impairment due to lens opacification is a debilitating characteristic of untreated diabetes. Lens opacification is primarily due to the insolubilization of crystallins, proteins essential for lens optical properties, and recent studies have suggested that a major cause of this insolubilization may be the unregulated proteolysis of crystallins by calpains. These are intracellular cysteine proteases whose activation requires the presence of calcium (Ca2+) and elevated levels of lens Ca2+ is a condition associated with both diabetic cataractogenesis and other forms of the disorder. A number of calpains have been identified in the lens, including calpain 2, calpain 10 and two isozymes of calpain 3: Lp82 and Lp85. The use of animal hereditary cataract models have suggested that calpain 2 and/or Lp82 may be the major calpains involved in murine cataractogenesis with contributions from calpain 10 and Lp85. However, calpain 2 appears to be the major calpain involved in murine diabetic cataractogenesis and the strongest candidate of the calpains for a role in human types of cataractogenesis. Here, we present an overview of recent evidence on which these observations are based with an emphasis on the ability of calpains to proteolyse lens crystallins and calpain structural features, which appear to be involved in the Ca2+-mediated activation of these enzymes.
Calpain inhibitors show the potential to serve as non-surgical alternatives in treating diabetic cataract and other types of these disorders. Here, we have tested the recently developed calpain inhibitor, SJA6017, for its ability to inhibit cataractogenesis in porcine lenses. These lenses were incubated in increasing levels of extralenticular calcium (Ca2+; 5-30 mM). Atomic absorption spectroscopy was used to determine total internal lens Ca2+ and a correlation between porcine lens Ca2+ uptake and levels of lens opacification were found with a total internal lens Ca2+ level of 5.8 microM Ca2+ g(-1) wet lens weight corresponding to the onset of catarctogenesis. A total internal lens Ca2+ level of 8.0 microM Ca2+ g(-1) wet lens weight corresponded to cataract occupying approximately 70% of the lens cell volume. This degree of cataract was reduced by approximately 40%, when SJA6017 (final concentration 0.8 microM) was included in the extralenticular medium, suggesting that the Ca2+-mediated activation of calpains may be involved in the observed opacification. Supporting this suggestion atomic absorption spectroscopy showed that the effect of SJA6017 (final concentration 0.8 microM) on lens opacification was not due to the compound restricting porcine lens Ca2+ uptake. The results indicate that calpain-induced cataractogenesis is dependent on extracellular Ca2+ and the calpain inhibitor SJA6017 (0.8 microM) had no significant effect on Ca2+ uptake by lens. Its inhibitory effect on lens opacification may be due to a direct action on the activity of calpain.
Hematohidrosis is a very rare condition in which an individual sweats blood. It may occur in an individual who is suffering from extreme levels of stress. Various causative factors have been suggested like component of systemic disease, vicarious menstruation, excessive exertion, psychogenic, and unknown causes. Fear and intense mental contemplation are the most frequent causes. It may also occur in bleeding disorders. We here report a case where bloody sweat was discharged from the forehead, face, and body episodically in a 12-year-old healthy girl with no bleeding disorder or any other underlying cause. All investigations done were within normal limits, except low intelligent quotient and loss of insight. The patient was given atropine sulphate transdermal patch with marked improvement in severity.
Background: Anti-N-methyl D-aspartate receptor (anti NMDAR) antibody encephalitis is an immune-mediated entity characterised by a constellation of neuro-psychiatric symptoms. Objective: To describe clinical profile and treatment outcomes of patients with anti NMDAR antibody encephalitis. Settings and Design: Subjects were selected by screening for all patients satisfying Graus et al .'s criteria for probable anti NMDAR antibody encephalitis, admitted in neurology department of a tertiary care centre in Eastern India. Materials and Methods: A prospective, longitudinal study was conducted by identifying 25 patients with anti NMDAR antibodies in CSF and or serum, between September 2018 to February 2020. Statistical Analysis: Chi square test was used to compare variables. Results: Out of 98 patients screened, 25 subjects (14 females: 11 male) were positive for anti NMDAR autoantibodies, with a mean age of 17 years. 13 subjects belonged to paediatric age group. Most common presenting feature was memory/learning deficit (88%) followed by behavioural abnormalities (84%) and seizures (68%). 11 patients (44%) patients needed escalation to second line therapy, rituximab. Seven (28%) and twelve (48%) patients underwent complete (mRS 0-1) and partial recovery (mRS 2-3) respectively, while 4 (16%) became disabled (mRS 4-5). Mortality was 8%. Paediatric population had a better outcome in terms of disability (p = 0.043). Conclusion: Anti NMDAR-Ab encephalitis is the most common cause of antibody positive autoimmune encephalitis worldwide. There are important clinical markers and investigational profiles which carry prognostic significance.
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