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Seizure May 2022Ictal piloerection (IP) is a rare manifestation of focal epilepsy. Autoimmune limbic encephalitis (LE) and malignant brain tumours are the most frequent recognized... (Review)
Review
BACKGROUND
Ictal piloerection (IP) is a rare manifestation of focal epilepsy. Autoimmune limbic encephalitis (LE) and malignant brain tumours are the most frequent recognized aetiologies.
METHODS
We selected all patients diagnosed with LE in our Institute from 2004 to 2020 and manifesting with IP. We performed a literature review on LE patients presenting IP.
RESULTS
Of 15 patients diagnosed with LE (13.3%), two manifested IP as prominent ictal feature. One of them also had stiff-limb syndrome. Video-EEG documented ictal discharges from the right temporal regions with concomitant sympathetic skin response (SSR) recording. Antibody testing showed elevated serum and CSF titres of GAD65 antibodies (Ab), in both cases. Despite a combination of several anti-seizure medications and first- and second-line immunotherapy, they showed a poor clinical outcome after 2 and 9 years of follow-up, respectively. The literature review yielded 13 papers reporting 26 LE cases with IP. LGI1 Ab were the most frequently associated (73.1%) followed by VGKC-complex (7.7%), GAD65 (7.7%), NMDAr (3.8%), Ma2 (3.8%) and Hu (3.8%) Ab. Cases with LGI1 Ab showed a good response to immunotherapy.
DISCUSSION AND CONCLUSION
The prevalence of IP in our LE cohort was of 13.3%, higher than expected. According to the literature review, most cases were associated with LGI1 Ab and showed a good response to immunotherapy. With the contribution of our cases, GAD65 emerged as the second most frequently detected Ab, showing a poor outcome. Our findings widen the spectrum of IP-associated Ab, with the respective prognostic implications.
Topics: Autoantibodies; Autoimmune Diseases; Electroencephalography; Humans; Limbic Encephalitis
PubMed: 35427850
DOI: 10.1016/j.seizure.2022.03.025 -
Stem Cells and Development Mar 2022Skin bears essential appendages and diverse cell types that function importantly in protection, thermoregulation, mechanosensation, piloerection, and social...
Skin bears essential appendages and diverse cell types that function importantly in protection, thermoregulation, mechanosensation, piloerection, and social communication. The hair follicle is a tiny skin appendage with intricate structure and has versatile functions in mammals. Hair follicles evolve stem cells that regenerate cyclically to produce hairs and to accommodate the rapidly changing environment. Sharing the same bulge niche with hair follicle stem cells (HFSCs), melanocyte stem cells (McSCs) undergo cyclic activation in synchrony with HFSCs, to pigment the hairs, which can protect the skin from ultraviolet radiation. Disorders of HFSCs or McSCs result in skin diseases such as hair loss, canities, vitiligo, and even melanoma, compromising senses of well-being and posing psychosocial distress to the affected individuals. The activation or quiescence of these stem cells is not only regulated by intrinsic factors within the follicle, but is also largely influenced by the extrinsic environmental factors, including the neighboring cells, systemic factors, and the external environment. Although great progress has been made to elucidate the intrinsic regulation of HFSCs or McSCs, understanding the environmental modulation of these stem cells can provide novel insight for the development of new therapeutic strategies for treating alopecia and skin pigmentation-related disorders.
Topics: Alopecia; Animals; Hair; Hair Follicle; Humans; Mammals; Melanocytes; Skin Pigmentation; Ultraviolet Rays
PubMed: 35285756
DOI: 10.1089/scd.2022.29011.wwu -
Frontiers in Zoology Mar 2022Sexual selection has driven sexual dimorphism in agonistic behaviour in many species. Agonistic behaviour is fundamentally altered by domestication and captivity, but it...
BACKGROUND
Sexual selection has driven sexual dimorphism in agonistic behaviour in many species. Agonistic behaviour is fundamentally altered by domestication and captivity, but it is unclear whether ancestral sex differences remain. We aimed to evaluate the effect of sex on agonistic behaviour, fighting ability and contest costs. We studied this in domestic pigs (Sus scrofa) where aggression compromises welfare, and sexual dimorphism in aggression has been inconclusively demonstrated. Behaviour and physiology of 827 male and female juvenile pigs were studied during resident-intruder tests and dyadic contests at various ages, while accounting for the relative body weight difference between the opponents.
RESULTS
Males won in 79% of contests against females, even when at a large weight disadvantage. The effect of sex increased with age, with males having a 138 times higher likelihood of winning than females when 13 weeks old. In dyadic contests, males invested more time in non-damaging agonistic display behaviour and took longer before escalating into damaging aggression. Males showed ritualized display that included foaming from the mouth and piloerection of the neck hair, which was nearly always absent in females. Contest costs in terms of contest duration, blood lactate and skin lesions where higher for males, especially when fighting another male.
CONCLUSIONS
Profound sex differences were present for agonistic behaviour, fighting ability and contest costs, and became more pronounced as animals got older. Males invested more in ritualized display before escalating into costly fights, whereas females attacked sooner but also terminated contests more rapidly and with fewer costs. The sexual dimorphism in agonistic behaviour in juvenile domestic pigs is in line with the evolutionary relevance for females' maternal defence and males' competition for females.
PubMed: 35279172
DOI: 10.1186/s12983-022-00458-9 -
Case Reports in Neurology 2021Autonomic status epilepticus (Aut SE) is a condition characterized by ongoing focal autonomic seizure lasting for >30 min. Aut SE can show a variety of clinical...
Autonomic status epilepticus (Aut SE) is a condition characterized by ongoing focal autonomic seizure lasting for >30 min. Aut SE can show a variety of clinical manifestations including vomiting, nausea, changes in heart rate, piloerection, pupillary abnormalities, and visual abnormalities. Although Aut SE is a common finding in childhood in the context of Panayiotopoulos syndrome, few reports have described this condition during adulthood. In the present report, we describe a case of Aut SE in an adult patient with parasellar meningioma and bilateral frontotemporal epileptiform activity on EEG record.
PubMed: 35111030
DOI: 10.1159/000519944 -
PLoS Pathogens Dec 2021Kyasanur Forest disease virus (KFDV) and the closely related Alkhurma hemorrhagic disease virus (AHFV) are emerging flaviviruses that cause severe viral hemorrhagic...
Kyasanur Forest disease virus (KFDV) and the closely related Alkhurma hemorrhagic disease virus (AHFV) are emerging flaviviruses that cause severe viral hemorrhagic fevers in humans. Increasing geographical expansion and case numbers, particularly of KFDV in southwest India, class these viruses as a public health threat. Viral pathogenesis is not well understood and additional vaccines and antivirals are needed to effectively counter the impact of these viruses. However, current animal models of KFDV pathogenesis do not accurately reproduce viral tissue tropism or clinical outcomes observed in humans. Here, we show that pigtailed macaques (Macaca nemestrina) infected with KFDV or AHFV develop viremia that peaks 2 to 4 days following inoculation. Over the course of infection, animals developed lymphocytopenia, thrombocytopenia, and elevated liver enzymes. Infected animals exhibited hallmark signs of human disease characterized by a flushed appearance, piloerection, dehydration, loss of appetite, weakness, and hemorrhagic signs including epistaxis. Virus was commonly present in the gastrointestinal tract, consistent with human disease caused by KFDV and AHFV where gastrointestinal symptoms (hemorrhage, vomiting, diarrhea) are common. Importantly, RNAseq of whole blood revealed that KFDV downregulated gene expression of key clotting factors that was not observed during AHFV infection, consistent with increased severity of KFDV disease observed in this model. This work characterizes a nonhuman primate model for KFDV and AHFV that closely resembles human disease for further utilization in understanding host immunity and development of antiviral countermeasures.
Topics: Animals; Chlorocebus aethiops; Cytokines; Disease Models, Animal; Encephalitis Viruses, Tick-Borne; Encephalitis, Tick-Borne; Female; HEK293 Cells; Hemorrhagic Fevers, Viral; Humans; Lymph Nodes; Macaca nemestrina; Vero Cells; Viremia
PubMed: 34855915
DOI: 10.1371/journal.ppat.1009678 -
Nederlands Tijdschrift Voor Geneeskunde Sep 2021Depending on the level and severity of spinal cord injury (SCI), SCI patients may suffer from loss of autonomic nervous system function besides the well-known motor and...
Depending on the level and severity of spinal cord injury (SCI), SCI patients may suffer from loss of autonomic nervous system function besides the well-known motor and sensory function loss. Changes in the autonomic control of the cardiovascular system can lead to the life-threatening phenomenon of autonomic dysreflexia (AD), especially in patients with cervical or high thoracic SCI. AD is defined as a sudden increase in systolic blood pressure of at least 20 mmHg above baseline. It results from an uncontrolled reaction of the sympathetic nervous system to a stimulus below the neurological SCI level. The characteristic symptoms above the neurological level are caused by vasodilation (headache, flushing, sweating, nasal congestion) whereas the symptoms below the neurological level are caused by vasoconstriction (piloerection). Recognition and appropriate management of AD are essential in preventing life-threatening complications of hypertensive crisis as well as identifying underlying disease requiring further treatment.
Topics: Autonomic Dysreflexia; Autonomic Nervous System; Blood Pressure; Humans; Hypotension; Spinal Cord; Spinal Cord Injuries
PubMed: 34854619
DOI: No ID Found -
Royal Society Open Science Oct 2021Consolation has been observed in several species, including marmoset monkeys, but it is often unclear to what extent they are empathy-based. Marmosets perform well in at...
Consolation has been observed in several species, including marmoset monkeys, but it is often unclear to what extent they are empathy-based. Marmosets perform well in at least two of three components of empathy-based consolation, namely understanding others and prosociality, but it is unknown to what extent they show matching with others. We, therefore, tested whether non-aroused individuals would become aroused themselves when encountering an aroused group member (indicated by piloerection of the tail). We found a robust contagion effect: group members were more likely to show piloerection themselves after having encountered an aroused versus relaxed conspecific. Moreover, group members offered consolation behaviours (affiliative approaches) towards the aroused fellow group members rather than the latter requesting it. Importantly, this pattern was shown by both aroused and non-aroused individuals, which suggests that they did not do this to reduce their own arousal but rather to console the individual in distress. We conclude that marmosets have all three components of empathy-based consolation. These results are in line with observations in another cooperative breeder, the prairie vole.
PubMed: 34729211
DOI: 10.1098/rsos.211255 -
Annals of Indian Academy of Neurology 2021
PubMed: 34728956
DOI: 10.4103/aian.AIAN_1124_20 -
Environmental Science and Pollution... Mar 2022The RhoA gene showed an important genotypic association with nicotine dependence and smoking initiation. The current study aims to investigate the effect of the Rho...
The RhoA gene showed an important genotypic association with nicotine dependence and smoking initiation. The current study aims to investigate the effect of the Rho GTPase inhibitor ML141 in the progression of nicotine dependence in a mice model of precipitated nicotine withdrawal syndrome by mecamylamine.The experimental procedure involved administration of 2.5 mg/kg nicotine dissolved in normal saline subcutaneously (s.c) four times a day consecutively for 7 days and last single dose in the morning on 8 day. ML-141 was dissolved in dimethyl sulfoxide (DMSO) and was administered daily with nicotine as corrective treatment at a dose of 1,5 and 10 mg/kg (p < 0.05). An injection of 3 mg/kg of mecamylamine intraperitoneal (ip) was given an hour later than the last nicotine dose on the day 8 to precipitate withdrawal of nicotine and withdrawal severity was assessed by measuring hyperalgesia, piloerection, jumping frequency, tremors, and withdrawal severity score (WSS). Various behavioural changes such as hyperalgesia, piloerection, jumping frequency, and tremors were monitored and WSS was calculated. ML-141 a selective Rho GTPase inhibitor was found to show dose-dependent effect on all these parameters. Inhibition of Rho GTPase was found to reduce the severity of withdrawal syndrome; therefore, it can be concluded that Rho GTPase would serve as a suitable biological target by regulating the reward system in brain and could be used as new target for drug discovery.
Topics: Animals; Mecamylamine; Mice; Nicotine; Substance Withdrawal Syndrome; Tobacco Use Disorder; rho GTP-Binding Proteins
PubMed: 34665416
DOI: 10.1007/s11356-021-17059-4 -
Epileptic Disorders : International... Oct 2021Musicogenic epilepsy (ME), a peculiar form of reflex epilepsy, represents a neurological rarity and yet another demonstration of the extraordinary power of music on the...
Musicogenic epilepsy (ME), a peculiar form of reflex epilepsy, represents a neurological rarity and yet another demonstration of the extraordinary power of music on the human brain. Despite the heterogeneity of the reported musical triggers, patients' emotional response to music is thought to play a crucial role in provoking seizures. Accordingly, the mesial temporal structures (especially of the non-dominant hemisphere) appear most involved in seizure generation, although a more complex fronto-temporal epileptogenic network was documented in some cases. Autoimmune encephalitis has been recently included among the many possible aetiologies of ME based on a few reports of music-induced seizures in patients with anti-glutamic acid decarboxylase 65 antibodies. Here, we describe the case of a 25-year-old man, educated in music over a long period of time, who had suffered from drug-resistant temporal lobe epilepsy following seronegative limbic encephalitis related to non-Hodgkin lymphoma. Along with spontaneous events, the patient also developed musicogenic seizures later in the disease course. After detecting five music-induced episodes via 24-hour ambulatory EEG, we performed prolonged video-EEG monitoring during which the patient presented a right temporal seizure (characterized by déjà-vu, piloerection and gustatory hallucinations) while listening to a hard rock song through headphones (which he had not previously heard). This observation allowed us to confirm the provoking effect of the music on our patient's seizures, despite the lack of any emotional drive, which suggests that a "cognitive" trigger was more likely in this case. Our report further highlights that autoimmune encephalitis should be investigated as a novel potential cause of musicogenic epilepsy, regardless of autoantibody status.
Topics: Adult; Drug Resistant Epilepsy; Electroencephalography; Epilepsy, Reflex; Humans; Limbic Encephalitis; Male; Music; Seizures
PubMed: 34612817
DOI: 10.1684/epd.2021.1322