Ictal piloerection is associated with high-grade glioma and autoimmune encephalitis-Results from a systematic review.

PURPOSE: To comprehensively analyze ictal piloerection (IP) in a large number of subjects. METHODS: We performed a systematic review on case report studies of patients diagnosed with IP (1929-2017) with additional cases included from the Department of Neurology of University of Pécs, the National Institute of Clinical Neurosciences, and Odense University Hospital. Each included case was characterized regarding patient history, IP seizure characteristics, diagnostic work-up, and therapy. Comparative analyses were also carried out based on sex and pathology. RESULTS: Altogether, 109 cases were included. We observed a strong male predominance (p < 0.001). The mean age at onset of epilepsy was 39.5 ± 20.7 years (median: 38, IQR:24-57). The seizure onset zone was temporal (p < 0.001), and was lateralized to the ipsilateral hemisphere in unilateral localization (p = 0.001). The seizure was accompanied by cold shiver in 53%, and by other autonomic symptoms in 47% of cases. In 53% of patients, IP never progressed into complex partial or generalized tonic-clonic seizure; 16% of the patients reported occasional, and 31% regular generalization. Seizure frequency was higher among females (median:25/day, IQR:3-60) than among males (median:3/day, IQR:1-11) (p = 0.017). The two most common underlying pathologies were limbic encephalitis (23%) and astrocytoma (23%, among them 64% WHO III-IV astrocytoma). CONCLUSION: IP was particularly associated with autoimmune encephalitis and high-grade glioma, suggesting IP's particular clinical importance in directing diagnostic work-up.

"The Fathers have eaten Sour Grapes, and the Children's Teeth are set on Edge": Differentiating the Emotional Experiences of Grima and Disgust.

The Spanish term grima refers to the aversive emotional experience typically evoked when one hears, for example, a scratch upon a chalkboard. Whereas Spanish speakers can distinguish between the concepts of grima and disgust, English and German speakers lack a specific word for this experience and typically label grima as disgust. In the present research, we tested the degree of differentiation between the two aversive experiences in Spanish speakers. Study 1 addressed whether Spanish speakers apply spontaneously the term grima rather than disgust to grima-eliciting experiences. Study 2 systematically addressed the constitutive features of both grima and disgust by mapping their internal structures. Results showed that the noise of a chulk on a blackboard and scraping fingernails on a blackboard, along with the physical manifestation of goose bumps, were the most typical features of the category. Whereas both grima and disgust were characterized as unpleasant sensations, t(193) = 1.21, ns, they differed with respect to their physiological signatures (e.g., producing shivers was characteristic of grima, as compared to disgust, t(194) = 12.02, p = .001, d = 1.72) and elicitors (e.g., a fractured bone was a characteristic elicitor of grima; t(193) = 5.78, p = .001, d = .83, whereas pederasts and pedophiles were the most characteristic elicitor of disgust, t(193) = 8.46, p = .001, d = 1.21). Thus, both grima and disgust are conceptually different experiences, whose shared features hold different degrees of typicality. The present research suggests that grima and disgust are two distinct emotion concepts.

Moment-to-moment changes in feeling moved match changes in closeness, tears, goosebumps, and warmth: time series analyses.

Feeling moved or touched can be accompanied by tears, goosebumps, and sensations of warmth in the centre of the chest. The experience has been described frequently, but psychological science knows little about it. We propose that labelling one's feeling as being moved or touched is a component of a social-relational emotion that we term kama muta (its Sanskrit label). We hypothesise that it is caused by appraising an intensification of communal sharing relations. Here, we test this by investigating people's moment-to-moment reports of feeling moved and touched while watching six short videos. We compare these to six other sets of participants' moment-to-moment responses watching the same videos: respectively, judgements of closeness (indexing communal sharing), reports of weeping, goosebumps, warmth in the centre of the chest, happiness, and sadness. Our eighth time series is expert ratings of communal sharing. Time series analyses show strong and consistent cross-correlations of feeling moved and touched and closeness with each other and with each of the three physiological variables and expert-rated communal sharing - but distinctiveness from happiness and sadness. These results support our model.

Visceral motor neuron diversity delineates a cellular basis for nipple- and pilo-erection muscle control.

Despite the variety of physiological and target-related functions, little is known regarding the cellular complexity in the sympathetic ganglion. We explored the heterogeneity of mouse stellate and thoracic ganglia and found an unexpected variety of cell types. We identified specialized populations of nipple- and pilo-erector muscle neurons. These neurons extended axonal projections and were born among other neurons during embryogenesis, but remained unspecialized until target organogenesis occurred postnatally. Target innervation and cell-type specification was coordinated by an intricate acquisition of unique combinations of growth factor receptors and the initiation of expression of concomitant ligands by the nascent erector muscles. Overall, our results provide compelling evidence for a highly sophisticated organization of the sympathetic nervous system into discrete outflow channels that project to well-defined target tissues and offer mechanistic insight into how diversity and connectivity are established during development.

Pilomotor seizures: an autonomic semiology of limbic encephalitis?

PURPOSE: Ictal piloerection is an infrequent seizure semiology that is commonly overlooked as an ictal epileptic manifestation. Piloerection is considered to be principally caused by temporal lobe activity although frontal and hypothalamic seizure origins have been reported. The described etiology has shown a wide variety of structural causes such as mesial temporal sclerosis, tumors, posttraumatic, cavernomas and cryptogenic epilepsies. METHODS: We retrospectively reviewed the incidence of ictal piloerection in the clinical records of patients who underwent video-EEG monitoring (VEEGM) between 2007 and 2013 in a multicenter cooperative study. All patients presented refractory epilepsies and were evaluated with a protocol that included brain MRI, neuropsychology and VEEGM. RESULTS: A total of 766 patients were evaluated in four tertiary centers in Spain. Five patients showed piloerection as principal seizure semiology (prevalence 0.65%). The mean age at seizure onset was 39.6 years and the average epilepsy duration was 5.2 years (range 2-14) before diagnosis. Four patients were additionally examined with FDG-PET and/or SPECT-SISCOM. All presented temporal lobe epilepsy (TLE), three right-sided and two left-sided. A typical unilateral hippocampal sclerosis was described in 3 cases. The etiology detected in all cases was limbic encephalitis. Three had LGI1, one anti-Hu, and another Ma2 antibodies. CONCLUSION: Our series describes a so far not well-recognized autoimmune association of pilomotor seizures to limbic encephalitis. This etiology should be ruled out through a comprehensive diagnostic work-up even in cases of long-lasting TLE with typical hippocampal atrophy on MRI.

'Goose bumps' as presenting feature of intraventricular glioblastoma multiforme.

'Goose-bumps' seizures are rare manifestations of epilepsy. They are rarely reported by patients and can be easily dismissed by clinicians. Clinically, it carries some diagnostic localising value especially with unilateral onset. In this report, we present a case of intraventricular glioblastoma multiforme with ipsilateral goose bumps and review the literature.

Alpha(1A)-adrenergic control of piloerection and palpebral fissure width in rats.

We determined the receptor subtypes of α1-adrenoceptor, which is involved in autonomic functions induced by methamphetamine (METH) in rats. An intraperitoneal injection of METH provoked the autonomic responses piloerection, eyelid retraction, and ejaculation. Pretreatment with prazosin, a nonselective α1-adrenoceptor antagonist, completely abolished the above METH-induced responses. Prazosin also provoked eyelid ptosis in saline controls. The effects of prazosin were mimicked only by a selective α1A-adrenoceptor antagonist, silodosin, not by selective α1B or α1D antagonists. These results suggest that α1A-adrenoceptor participates in the regulation of piloerection, palpebral fissure width, and ejaculation in rats.

Quantitative pilomotor axon reflex test: a novel test of pilomotor function.

BACKGROUND: Cutaneous autonomic function can be quantified by the assessment of sudomotor and vasomotor responses. Although piloerector muscles are innervated by the sympathetic nervous system, there are at present no methods to quantify pilomotor function. OBJECTIVE: To quantify piloerection using phenylephrine hydrochloride in humans. DESIGN: Pilot study. SETTING: Hospital-based study. PARTICIPANTS: Twenty-two healthy volunteers (18 males,4 females) aged 24 to 48 years participated in 6 studies. INTERVENTIONS: Piloerection was stimulated by iontophoresis of 1% phenylephrine. Silicone impressions of piloerection were quantified by number and area. The direct and indirect responses to phenylephrine iontophoresis were compared on both forearms after pre treatment to topical and subcutaneous lidocaine and iontophoresis of normal saline. RESULTS: Iontophoresis of phenylephrine induced piloerection in both the direct and axon reflex­mediated regions, with similar responses in both arms. Topical lidocaine blocked axon reflex­mediated piloerection post-iontophoresis (mean [SD], 66.6 [19.2] for control impressions vs 7.2 [4.3] for lidocaine impressions;P.001). Subcutaneous lidocaine completely blocked piloerection.The area of axon reflex­mediated piloerection was also attenuated in the lidocaine-treated region postiontophoresis (mean [SD], 46.2 [16.1]cm2 vs 7.2 [3.9]cm2; P.001). Piloerection was delayed in the axon reflex region compared with the direct region. Normal saline did not cause piloerection. CONCLUSIONS: Phenylephrine provoked piloerection directly and indirectly through an axon reflex­mediated response that is attenuated by lidocaine. Piloerection is not stimulated by iontophoresis of normal saline alone.The quantitative pilomotor axon reflex test (QPART) may complement other measures of cutaneous autonomic nerve fiber function.

Pilomotor seizures: a video case report.

Piloerection is a rare ictal manifestation usually associated with temporal lobe epilepsy. We present video and electrographic documentation of pilomotor seizures in a 75-year-old man with left temporal lobe epilepsy and remote herpes zoster encephalitis. In general, piloerection is most often localised to the temporal lobe. Unilateral piloerection is usually associated with an ipsilateral ictal onset. Bilateral piloerection has a less certain lateralising value, although earlier reports suggest left hemispheric predominance [Published with videosequences].

Single follicular unit transplantation reconstructs arrector pili muscle and nerve connections and restores functional hair follicle piloerection.

The autologous transplantation of hair follicles that have been separated into single follicular units is an accepted treatment for androgenetic alopecia. Recent studies demonstrate that the multiple stem cell populations and surrounding cutaneous tissues coordinately regulate the hair follicle functions and skin homeostasis. Therefore, the critical issues for consideration regarding functional hair restoration therapy are reproduction the correct connectivity and cooperation with host cutaneous tissues, including the arrector pili muscle (APM) and nerve system. We report successful establishment of mouse single follicular transplantation model and autonomous restoration of transplanted hair follicle piloerection in mouse skin. Transplanted hair follicles were responsive to the neurotransmitter acetylcholine and formed proper connections with surrounding host tissues such as APM and nerve fibers, which in turn connect with not only the hair follicle bulge region but also the APM. These results demonstrate that the piloerection ability of transplanted hair follicles can be estimated quantitatively. This study makes a substantial contribution towards the development of transplantation therapy that will facilitate future functional regeneration therapy for skin and skin appendages.

Physiological correlates and emotional specificity of human piloerection.

Piloerection is known as an indicator of strong emotional experiences. However, little is known about the physiological and emotional specificity of this psychophysiological response. In the presented study, piloerection was elicited by audio stimuli taken from music and film episodes. The physiological response accompanying the incidence of piloerection was recorded with respect to electrodermal, cardiovascular and respiratory measures and compared to a matched control condition. The employment of an optical recording system allowed for a direct and objective assessment of visible piloerection. The occurrence of piloerection was primarily accompanied by an increase of phasic electrodermal activity and increased respiration depth as compared to a matched control condition. This physiological response pattern is discussed in the context of dominant theories of human piloerection. Consideration of all available evidence suggests that emotional piloerection represents a valuable indicator of the state of being moved or touched.

Semiology of the rare seizure subtype piloerection.

OBJECTIVE: To report piloerection and vasomotor instability as a sole manifestation of partial seizures. DESIGN: Case report. SETTING: Inpatient tertiary care center. PATIENT: A 72-year-old man with acute onset of repetitive autonomic events. MAIN OUTCOME MEASURES: Extensive cardiovascular evaluation, electroencephalographic testing, radiology, laboratory assessment, and frequency of clinical events (seizures). RESULTS: We characterize an uncommon manifestation of right temporal lobe partial seizures, initially thought to be cardiovascular in origin, as pilomotor seizures accompanied by other autonomic phenomena. The ictal electroencephalogram recordings established the diagnosis of right temporal lobe seizures, and head magnetic resonance imaging demonstrated right mesial temporal T2 signal change, enhancement, and subsequent atrophy. The underlying etiology was not uncovered despite an extensive laboratory and radiological evaluation. However, given the history and imaging findings, an infectious or secondary immunological etiology was suspected. CONCLUSIONS: Pilomotor events with other transient autonomic features, such as tachycardia and blood pressure fluctuations, may represent localization-related epilepsy. The acute onset of these events accompanied by other autonomic phenomena in the setting of focal magnetic resonance imaging abnormalities within the hippocampal region raises questions concerning pilomotor seizures as well as the differential diagnosis of acute seizure activity in the setting of neuroimaging findings characteristic for limbic encephalitis.

Dissociation between neurovegetative signs and subjective symptoms in a case of idiopathic pilomotor seizures.

OBJECTIVE: Pilomotor seizure (PS) is a rare subtype of simple and complex partial seizures, often related to temporal lobe epilepsy and occasionally linked to alterations of amygdala. The physiologic role played by this latter region in the coordination of autonomic responses to fear-induced emotional changes raises the question as to whether the involvement of amygdala in PS might elicit a disconnection between subjective symptoms and neurovegetative signs. METHODS: We report a case of idiopathic bilateral PS studied with video electroencephalogram, polygraphic 24-hour Holter electroencephalogram, and magnetic resonance imaging, plus spectral functional magnetic resonance imaging, in which the seizures were associated with abrupt tachycardia occurring in a state of emotional neutrality, without either clouding or loss of consciousness. RESULTS: Electroencephalogram documented PS episodes occurring during waking, rapid eyes movements, and nonrapid eye movement sleep stages. Although no morphologic alteration was detected, spectral magnetic resonance imaging visualized alterations of the metabolic ratios of N-acetylaspartate and creatine-phosphocreatine in hippocampus and amygdala, whereas no apparent involvement of the temporal lobe was found. CONCLUSIONS: These findings suggest that the limited involvement of amygdala and hippocampus in PS triggers the repertoire of fear-related sympathetic responses uncoupled from alterations in emotional status. This phenomenon supports the possibility that autonomic responses involved in fear or extreme alertness follow a kind of "ethological" modularity.

[A study of skin deformation by optical coherent microscopy].

The deformation of rat skin in response to cooling and stimulation of nerve fibers innervating the muscles piloerectors and vasoconstrictors has been studied by optical coherent microscopy. It has been found that the deformation of the skin on cooling occurs due to the contraction of bundles of collagen fibers rather than smooth muscles of vessels and piloerectors.

Objective and continuous measurement of piloerection.

Increasing attention is drawn to the investigation of piloerection (or goose bumps) in response to strong emotional experiences. This research is complicated by the need to rely on self-report measures. This article proposes an efficient method for the objective and continuous measurement of piloerection. It is based on an optical recording device combined with a discrete Fourier transform analysis quantifying the frequency power related to visible piloerection. The validity of the method was demonstrated in a proof-of-principle experiment involving a person with the exceptional ability to control the erection of his hair. The method reliably mapped all occurrences of visible piloerection and provided insight into the temporal dynamics of the underlying physiological process. It thus proved suitable for future experimental investigation of human piloerection.

The effects of rewarding ventral tegmental area stimulation and environmental enrichment on lipopolysaccharide-induced sickness behavior and cytokine expression in female rats.

Responding for rewarding brain stimulation has been used to track hedonic status in animals. In addition to neurochemical alterations, stimulation of the lateral hypothalamus or ventral tegmentum has been shown to influence immunological processes, including elevation of peripheral natural killer cell activity. In the present study, we examined whether ventral tegmental area (VTA) stimulation or environmental enrichment altered the severity of lipopolysaccharide (LPS)-induced sickness behaviors and the provocation of cytokine expression induced by the endotoxin. Accordingly, rats received either trials of brain stimulation reward or exposure to an enriched environment and subsequently challenged with 150 ug/kg i.p. of LPS. Groups receiving LPS and saline injections without further manipulation were also included. Using the real-time RT-PCR and a multiplex bead assay, mRNA and protein levels for several cytokines and their receptors were determined to evaluate how these may vary as a consequence of reward. Both brain stimulation and environmental enrichment similarly diminished sickness behaviors associated with the endotoxin. Receptor gene levels were generally stable across groups. Levels of IL-6 within the VTA were increased in the group receiving LPS challenge alone and environmental enrichment was associated with modestly reduced IL-6 levels within this brain region. Taken together, these data suggest that rewarding brain stimulation and environmental enrichment buffer against malaise provoked by endotoxin challenge. Moreover, IL-6 expression within the VTA may influence the development of sickness behavior following inflammatory stimuli.

Pilomotor seizures associated with sequential changes in magnetic resonance imaging.

Piloerection is rarely described in seizures. This symptom has been most frequently observed in patients with temporal lobe epilepsy and is rarely the principal clinical feature of seizures. No specific etiology of epilepsy associated with pilomotor seizures has been reported. We present the first case of a patient who experienced sudden and transitory epilepsy with pilomotor seizures occurring several times a day for months, and associated with sequential changes of the left hippocampus demonstrated by magnetic resonance imaging. [Published with video sequences].

Skin biopsy for assessment of autonomic denervation in Parkinson's disease.

Autonomic dysfunction in Parkinson's disease (PD) is considered a late complication of the disease or an adverse effect of anti-parkinsonian medications. Morphological changes are demonstrated only by postmortem examination. The study objective was to evaluate peripheral autonomic neural involvement in PD using punch skin biopsy. The study sample included 22 patients (mean age 50 +/- 7.7 years, mean disease duration 5.3 +/- 3.8 years) and 19 controls. Four-millimeter skin biopsies were immunohistochemically stained with anti-PGP 9.5 antibody. Autonomic innervation of the blood vessels, sweat glands, and erector pili muscles was assessed and rated from 0 (normal) to 2 (severe). Cutaneous autonomic innervation was decreased in patients compared to controls. Semi quantitative analysis demonstrated reduced autonomic innervation of the blood vessels (1.0 +/- 0.8 vs. 0.42 +/- 0.8 in controls; p < 0.02), of sweat glands (0.95 +/- 0.67 vs. 0.47 +/- 0.61; p < 0.02) and of the erector pili muscles (1.06 +/- 0.55 vs 0.21 +/- 0.42; p < 0.001). This method demonstrates that the peripheral autonomic system is affected in PD at early stage of the disease and that autonomic involvement in PD may be more prevalent than previously thought.