Anti-C4c+C4b polyclonal antibody [FITC]

Aim: Structural connectivity in the reorganizing spinal cord after injury dictates functional connectivity and hence the neurological outcome. As magnetic resonance imaging (MRI)-based structural parameters are mostly accessible across spinal cord injury (SCI) patients, we studied MRI-based spinal morphological changes and their relationship to neurological outcome in the rat model of cervical SCI. Introduction: Functional connectivity assessments on patients with SCI rely heavily on MRI-based approaches to investigate the complete neural axis (both spinal cord and brain). Hence, underlying MRI-based structural and morphometric changes in the reorganizing spinal cord and their relationship to neurological outcomes is crucial for meaningful interpretation of functional connectivity changes across the neural axis. Methods: Young adult rats, aged 1.5 months, underwent a precise mechanical impact hemicontusion incomplete cervical SCI at the C4/C5 level, after which sensorimotor behavioral assessments were tracked during the reorganization period of 1-6 weeks, followed by MRI of the cervical spinal cord at 8 weeks after SCI. Results: A significant ipsilesional forelimb motor debilitation was observed from 1 to 6 weeks after injury. Heat sensitivity testing (Hargreaves) showed ipsilesional forelimb hypersensitivity at 5 and 6 weeks after SCI. MRI of the cervical spine showed ipsilateral T1- and T2-weighted lesions across all SCI rats compared with no significant lesions in sham rats. Morphometric assessments of the lesional and nonlesional changes showed the diverse nature of their interindividual variability in the SCI receiving rats. While the various T1 and T2 MRI lesional volumes associated weakly or moderately with neurological outcome, the nonlesional spinal morphometric changes associated much more strongly. The results have important implications for interpreting functional MRI-based functional connectivity after SCI by providing vital underlying structural changes and their relative neurological impact. Impact statement Functional connectivity assessments on patients with SCI relies heavily upon MRI based approaches. Hence, underlying MRI based structural and morphometric changes in the reorganizing spinal cord and its relationship to neurological outcomes is vital for meaningful interpretation of functional connectivity changes across the complete neural axis (both spinal cord and the brain).

BACKGROUND Ethylmalonic encephalopathy (EE) is a rare autosomal recessive metabolic disorder caused by impaired mitochondrial sulfur dioxygenase. Due to poor therapeutic effect of current conventional treatments, progressive psychomotor regression and neurological impairment usually contribute to early death in the first decade of life. Liver transplantation (LT) is emerging as a novel therapeutic option for EE; however, worldwide experience is still limited. CASE SUMMARY An 18-mo-old patient with the diagnosis of EE received a living donor liver transplant in our institution, which, to our knowledge, is the first case in Asian-Pacific countries. During 20 mo of follow-up, the longitudinal metabolic evaluations revealed a wild fluctuation in urinary EMA levels, still far beyond the normal range. Urinary 2-methylsuccinic acid levels gradually restored to normal, whereas the concentrations of urinary isobutyrylglycine and plasma C4- and C5-acylcarnitines fluctuated markedly and still remained above the reference limits. Only mild amelioration of petechiae and ecchymosis was observed, and no dramatic reversion of chronic mucoid diarrhea and orthostatic acrocyanosis occurred. Although brain magnetic resonance imaging suggested a certain improvement in basal ganglia lesions, the patient still presented developmental delay and neurologic disability. CONCLUSION LT may bring about a partial but not complete cure to EE. Given its definite role in defending against the devastating natural progression of EE, LT should still be considered for patients with EE in the absence of other superior therapeutic options. Early establishment of diagnosis and initiation of conventional treatment pre-transplant, timely LT, and continuous administration of metabolism-correcting medications post-transplant may be helpful in minimizing the neurologic impairment and maximizing the therapeutic value of LT in EE.