Anti-IgLON 5 Disease.

PURPOSE OF REVIEW: This review aims to give an overview about the current knowledge of this novel neurological disorder associated to IgLON-5 antibodies and its treatment. RECENT FINDINGS: Anti-IgLON5 disease was first formally described in 2014. This newly discovered disorder recaps a complex neurological disorder with sleep, movement, and neuroimmunological and neurodegenerative aspects. The clinical manifestation of the anti-IgLON5 disease is very heterogeneous mostly including a sleep disorder with non-rapid eye movement (REM) sleep parasomnia and REM behavior disorder besides obstructive sleep apnea syndrome and stridor. Other neurological features (bulbar symptoms, gait abnormalities, cognitive dysfunction) are common. Until today, the mean age of diagnosis was mostly above the age of 60 with a balanced distribution of sex. Neuropathological examination showed neuronal loss and gliosis associated with an atypical tauopathy mainly involving the tegmentum of brainstem and hypothalamus. Although the function of the antibodies stays unclear so far, the evidence for the pathogenetic role of the antibody becomes more evident. Among the association to HLA-DRB1*10:01 and HLA-DQB1*05:01 as a potential factor for susceptibility, immunopathological findings are promising. So far, the pathophysiology of anti-IgLON5 disease is not sufficiently enlightened and needs more interdisciplinary approach to a better understanding of this interesting disorder at the border of autoimmunity and neurodegeneration. Immunotherapy has been frequently used but its therapeutic effect is limited.

Capsaicin 8 % as a cutaneous patch (Qutenza™): analgesic effect on patients with peripheral neuropathic pain.

Evaluation of the analgesic effect after a single application of the capsaicin 8 % cutaneous patch (Qutenza™) in 37 patients suffering from painful, distal symmetric polyneuropathy (PNP) for an average of 5 years. Patients ranged from 40 to 78 years of age and 22 subjects were HIV-positive. Patients were observed 4 weeks prior to 12 weeks post administration. An evaluation of the therapeutic effect of capsaicin 8 % as a dermal patch in terms of pain reduction, change of sleeping behavior and social activities was performed and statistical analysis of data was conducted using non-parametric methods. Patients were selected according to clinical criteria. Numerical rating scale (NRS 0-10) was used to inquire pain intensity and a pain score was calculated using the painDETECT(©) questionnaire Freynhagen R (Curr Med Res Opin 22:1911-1920, [2006]). A significant reduction of pain was achieved for up to 12 weeks, with a maximum after 2-4 weeks post administration. After patient education and before application of capsaicin patch, a significant reduction of three levels on the NRS was observed. Symptoms of painful PNP decreased over the period of investigation and 8 patients reported a reduction of systemic pain medication. In patients with an HIV infection, a significant extension of sleep was achieved for 2, 4 and 8 weeks after application. Thus, the application of the capsaicin 8 % patch resulted in a significant relief of neuropathic pain, a prolongation of sleep, a reduction of oral pain medication and a resumption of social activities.

Specific loss of cellular L-selectin on CD4(+) T cells is associated with progressive multifocal leukoencephalopathy development during HIV infection.

HIV(+) progressive multifocal leukoencephalopathy (PML) patients had a significantly lower expression of CD62L on CD4(+) T cells (P < 0.001) when compared with HIV(+) patients who did not develop PML. CD62L expression on CD4(+) T cells did not correlate with parameters such as CDC stage, CD4(+) cell percentage (of total CD3(+) T cells), CD4(+) cell counts, virus count, or clinical parameters. Measurement of CD62L might provide a biomarker for PML risk and could prompt a treatment change and/or close monitoring.

The intrinsic connectome of the rat amygdala.

The connectomes of nervous systems or parts there of are becoming important subjects of study as the amount of connectivity data increases. Because most tract-tracing studies are performed on the rat, we conducted a comprehensive analysis of the amygdala connectome of this species resulting in a meta-study. The data were imported into the neuroVIISAS system, where regions of the connectome are organized in a controlled ontology and network analysis can be performed. A weighted digraph represents the bilateral intrinsic (connections of regions of the amygdala) and extrinsic (connections of regions of the amygdala to non-amygdaloid regions) connectome of the amygdala. Its structure as well as its local and global network parameters depend on the arrangement of neuronal entities in the ontology. The intrinsic amygdala connectome is a small-world and scale-free network. The anterior cortical nucleus (72 in- and out-going edges), the posterior nucleus (45), and the anterior basomedial nucleus (44) are the nuclear regions that posses most in- and outdegrees. The posterior nucleus turns out to be the most important nucleus of the intrinsic amygdala network since its Shapley rate is minimal. Within the intrinsic amygdala, regions were determined that are essential for network integrity. These regions are important for behavioral (processing of emotions and motivation) and functional (memory) performances of the amygdala as reported in other studies.