Caregivers of preschool-aged children with TBI endorse that young children experience many typical post-concussive symptoms but also a range of other symptoms which may not be typically associated with TBI. Additional work is needed to determine whether specialized evaluation tools and educational materials may be useful for this age group.
Rapid-onset obesity with hypothalamic dysfunction, hypoventilation, and autonomic dysregulation (ROHHAD) is characterized by normal development followed by a sudden, rapid hyperphagic weight gain beginning during the preschool period, hypothalamic dysfunction, and central hypoventilation. [1][2][3] Without intervention, symptoms progress leading to substantial morbidity and mortality. [1][2][3] In addition, these patients display poorly characterized mood disorders, personality changes, and developmental regression, including loss of toilet-training, and behavior mimicking autism spectrum disorders. 1,2,[4][5][6] The etiology of ROHHAD is unknown and careful investigations have not revealed a genetic etiology. 2, 7-9 The association with neural crest tumors suggests an immune-mediated process resembling opsoclonus myoclonus ataxia syndrome. 2,5,7,10 Lymphocytic infiltration of the brain, 11, 12 presence of oligoclonal bands in the cerebrospinal fluid, 13 and response to immunomodulary therapy 5, 14 support an autoimmune process.High-dose cyclophosphamide (Hi-Cy) results in near total ablation of active lymphocytes without myeloablation, thereby "rebooting" the immune system, making it an efficacious and safe approach for selected patients with severe refractory autoimmune diseases. 5,15 We previously reported on a child with ROHHAD whose symptoms improved after Hi-Cy. 5 We now describe serial neuropsychological assessments of 2 consecutive, similarly treated abstract Rapid-onset obesity with hypothalamic dysfunction, hypoventilation, and autonomic dysregulation (ROHHAD) is a rare, generally progressive, and potentially fatal syndrome of unclear etiology. The syndrome is characterized by normal development followed by a sudden, rapid hyperphagic weight gain beginning during the preschool period, hypothalamic dysfunction, and central hypoventilation, and is often accompanied by personality changes and developmental regression, leading to substantial morbidity and mortality. We describe 2 children who had symptomatic and neuropsychological improvement after high-dose cyclophosphamide treatment. Our experience supports an autoimmune pathogenesis and provides the first neuropsychological profile of patients with rapid-onset obesity with hypothalamic dysfunction, hypoventilation, and autonomic dysregulation.
Adverse neurological side effects associated with childhood brain tumors and their treatments contribute to long-term neurocognitive morbidity. Measures designed to quantify tumor-related risk factors are lacking. The neurological predictor scale (NPS) is designed to assess treatment-related neurological risks. Preliminary validation established associations between the NPS and global cognitive functioning in this population, though its associations with specific neurobehavioral domains has yet to be addressed. Participants referred for outpatient neuropsychological assessment completed performance-based measures of intellectual, attentional, working memory, motor speed, and executive abilities. Caregivers completed ratings of adaptive functioning. Neuropsychological and adaptive data were available for 100 brain tumor survivors (51 % female), ages 6 to 22 years (M = 12.83, SD = 4.37). Total NPS scores were generated via retrospective medical record review. Total NPS scores were significantly associated with several neurocognitive composite scores including verbal reasoning and working memory, after controlling for years post-diagnosis (ps < .05). NPS scores also were significantly associated with performance-based measures of attention, executive functioning, and cognitive efficiency (ps < .05). No significant relationship was demonstrated between NPS scores and caregiver-reported adaptive behavior skills (ps > .05). Results indicate that the NPS is associated with performance-based neurocognitive functioning and executive skills but not with functioning in specific caregiver-reported adaptive behavior domains. The NPS offers some value as a resource for understanding associations between treatment-related neurological risks and select aspects of neurocognitive morbidity. Future studies should examine whether the NPS can aid in planning appropriate therapeutic intervention as survivors progress into early adulthood.
A longstanding question in working memory (WM) research concerns the fractionation of verbal and nonverbal processing. Although some contemporary models include both domain-specific and general-purpose mechanisms, the necessity to postulate differential processing of verbal and nonverbal material remains unclear. In the present two-experiment series we revisit the order reconstruction paradigm that Jones, Farrand, Stuart, and Morris (1995) used to support a unitary model of WM. Goals were to assess (1) whether serial position curves for dot positions differ from curves for letter names; and (2) whether selective interference can be demonstrated. Although we replicated Jones et al.'s finding of similar serial position curves for the two tasks, this similarity could reflect the demands of the order reconstruction paradigm rather than undifferentiated processing of verbal and nonverbal stimuli. Both generalised and material-specific interference was found, which can be attributed to competition between primary and secondary tasks for attentional resources. As performance levels for the combined primary and secondary tasks exceed active WM capacity limits, primary task items apparently are removed from active memory during processing of the secondary list and held temporarily in maintenance storage. We conclude that active WM is multimodal but maintenance stores may be domain specific.
In tropical and subtropical regions of the world, leishmaniasis is endemic and causes a range of clinical symptoms in people, from severe tegumentary forms (such as cutaneous, mucocutaneous, and diffuse leishmaniasis) to lethal visceral forms. The protozoan parasite of the genus Leishmania causes leishmaniasis, which is still a significant public health issue, according to the World Health Organization 2022. The public's worry about the neglected tropical disease is growing as new foci of the illness arise, which are exacerbated by alterations in behavior, changes in the environment, and an enlarged range of sand fly vectors. Leishmania research has advanced significantly during the past three decades in a few different avenues. Despite several studies on Leishmania, many issues, such as illness control, parasite resistance, parasite clearance, etc., remain unresolved. The key virulence variables that play a role in the pathogenicity‐host‐pathogen relationship of the parasite are comprehensively discussed in this paper. The important Leishmania virulence factors, such as Kinetoplastid Membrane Protein–11 (KMP‐11), Leishmanolysin (GP63), Proteophosphoglycan (PPG), Lipophosphoglycan (LPG), Glycosylinositol Phospholipids (GIPL), and others, have an impact on the pathophysiology of the disease and enable the parasite to spread the infection. Leishmania infection may arise from virulence factors; they are treatable with medications or vaccinations more promptly and might greatly shorten the duration of treatment. Additionally, our research sought to present a modeled structure of a few putative virulence factors that might aid in the development of new chemotherapeutic approaches for the treatment of leishmaniasis. The predicted virulence protein's structure is utilized to design novel drugs, therapeutic targets, and immunizations for considerable advantage from a higher understanding of the host immune response.
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