Autonomic Signs Research Articles

Investigating the Prevalence of Autonomic Neuropathy in Diabetic Patients with Urinary Irritation Symptoms Without a Known Cause.

Evaluating pseudomotor performance can be a valuable tool for investigating the peripheral autonomic nervous system in diabetic patients. Sudoscan, a simple and non-invasive method for assessing pseudomotor performance, has been developed in recent years. This study aimed to investigate autonomic neuropathy using Sudoscan in diabetic patients with lower urinary tract symptoms (LUTS) of unknown cause. In this cross-sectional study conducted from April 2022 to April 2023, we included 195 patients with type 2 diabetes who were referred to the urology clinic. We extracted demographic, clinical, and laboratory data from the patient files and evaluated urinary symptoms using the International Prostate Symptom Score (IPSS) questionnaire. Patients underwent Sudoscan testing to evaluate autonomic neuropathy in the physical medicine and rehabilitation clinic. To further assess urinary irritative symptoms, patients underwent urodynamic studies (UDS) and ultrasonography. The Sudoscan test results showed that autonomic neuropathy was present in 77 patients (40%), with 43 (22.1%) having moderate and 44 (22.6%) having severe neuropathy. Patients with autonomic neuropathy were found to be older, had longer diabetes durations, higher average blood glucose levels, and higher creatinine levels. Additionally, we found a significant correlation between autonomic neuropathy and signs of high post-void residue on ultrasound and detrusor contraction disorders on UDS (p-value < 0.05). Our study found a higher prevalence of autonomic neuropathy in diabetic patients with LUTS using Sudoscan (40%). Longer diabetes duration and poor glycemic control were associated with an increased risk of autonomic neuropathy linked with LUTS, such as urge incontinence.

Anesthetic Challenges in Hirayama Disease Patients Undergoing Cervical Spine Surgery—A Case Series

AbstractHirayama disease (HD) is a rare disease, resulting from cervical compressive myelopathy, manifesting as upper limb muscular atrophy, and rarely autonomic and upper motor neuron signs. Anesthesia management is challenging—careful neck positioning during bag-mask ventilation and endotracheal intubation, avoidance of drugs that release histamine, multimodal monitoring to avoid delayed recovery, anticipation of hypotension, and blood loss due to autonomic dysfunction—all this is necessary for successful outcome of general anesthesia in HD patients. This case series demonstrates that preexisting autonomic dysfunction in HD patients should alert the anesthesiologists regarding higher likelihood of hemodynamic perturbations and blood loss, compared with patients who have normal autonomic functions, and henceforth take appropriate precautionary measures.

The Role of the Autonomic Nervous System in Epilepsy and Migraine: A Narrative Review.

Autonomic symptoms may be local and general clinical manifestations of both epilepsy and migraine caused by the dysfunction of brain areas best known as the central autonomic network. Despite their prevalence, autonomic signs are often misdiagnosed and their treatment is undervalued. This review aims to describe the autonomic manifestations reported during seizures and migraineur attacks according to their presentation, focusing on the role of the central autonomic network (CAN) and on the parasympathetic outflow that often-induced cranial autonomic symptoms (CAS) during migraineur attacks. Further, our purpose is to analyze the pathophysiological meanings and whether their presence influences the prognosis and therapy of these disorders.

Anomalies of the Craniocervical Junction (Chiari Malformations)

AbstractArnold Chiari malformations include a combination of posterior fossa, hindbrain, and cervical occipital junction abnormalities, sometimes associated with spinal cord abnormalities such as spina bifida, syringomyelia, and syringobulbia. The most frequent form is Chiari I syndrome but two other variants, progressively more severe, have been described. Chiari malformations are the result of defective development of posterior fossa and can be due to genetic mutations, skeletal malformations, and intrautero factors. Clinical manifestations depend on the compression of the nerve structures within the foramen magnum and the spinal canal and mainly consist in headache or neck pain, gait disturbances, sensory or motor abnormalities, and autonomic signs. However, a high number of cases of Chiari I is asymptomatic and the diagnosis is occasional. Diagnosis is performed through nuclear magnetic resonance imaging of the brain and cervical tract, although other investigations may support the diagnosis. First-line treatment for candidate patients is a surgical procedure that involves decompression of the posterior cranial fossa and the craniocervical junction, as well as correction of associated malformations with techniques that depend on the severity of the case. Anyhow, some symptomatic patients benefit from conservative medical treatment with nonsteroidal anti-inflammatory drugs.

Dysautonomia in anti-Hu paraneoplastic neurological syndromes.

Dysautonomia has been associated with paraneoplastic neurological syndrome (PNS)-related mortality in anti-Hu PNS, but its frequency and spectrum remain ill-defined. We describe anti-Hu patients with dysautonomia, estimate its frequency, and compare them to patients without dysautonomia. Patients with anti-Hu antibodies diagnosed in the study centre (1990-2022) were retrospectively reviewed; those with autonomic signs and symptoms were identified. Among 477 anti-Hu patients, 126 (26%) had dysautonomia (the only PNS manifestation in 7/126, 6%); gastrointestinal (82/126, 65%), cardiovascular (64/126, 51%), urogenital (24/126, 19%), pupillomotor/secretomotor (each, 11/126, 9%), and central hypoventilation (10/126, 8%). Patients with isolated CNS involvement less frequently had gastrointestinal dysautonomia than those with peripheral (alone or combined with CNS) involvement (7/23, 30% vs. 31/44, 70% vs. 37/52, 71%; P = 0.002); while more frequently central hypoventilation (7/23, 30% vs. 1/44, 2.3% vs. 2/52, 4%; P < 0.001) and/or cardiovascular alterations (18/23, 78% vs. 20/44, 45% vs. 26/52, 50%; P = 0.055). Median [95% CI] overall survival was not significantly different between patients with (37 [17; 91] months) or without dysautonomia (28 [22; 39] months; P = 0.78). Cardiovascular dysautonomia (HR: 1.57, 95% CI [1.05; 2.36]; P = 0.030) and central hypoventilation (HR: 3.51, 95% CI [1.54; 8.01]; P = 0.003) were associated with a higher risk of death, and secretomotor dysautonomia a lower risk (HR: 0.28, 95% CI [0.09; 0.89]; P = 0.032). Patients with cardiovascular dysautonomia dying ≤ 1year from clinical onset had severe CNS (21/27, 78%), frequently brainstem (13/27, 48%), involvement. Anti-Hu PNS dysautonomia is rarely isolated, frequently gastrointestinal, cardiovascular and urogenital. CNS dysfunction, particularly brainstem, associates with lethal cardiovascular alterations and central hypoventilation, while peripheral involvement preferentially associates with gastrointestinal or secretomotor dysautonomia, being the latest more indolent.

Autonomous traffic sign detection for self-driving car system using convolutional neural network algorithm

The accurate detection of traffic signs is a critical component of self-driving systems, enabling safe and efficient navigation. In the literature, various methods have been investigated for traffic sign detection, among which deep learning-based approaches have demonstrated superior performance compared to other techniques. This paper justifies the widespread adoption of deep learning due to its ability to provide highly accurate results. However, the current research challenge lies in addressing the need for high accuracy rates and real-time processing requirements. In this study, we propose a convolutional neural network based on the YOLOv8 algorithm to overcome the aforementioned research challenge. Our approach involves generating a custom dataset with diverse traffic sign images, followed by conducting training, validation, and testing sets to ensure the robustness and generalization of the model. Experimental results and performance evaluation demonstrate the effectiveness of the proposed method. Extensive experiments show that our model achieved remarkable accuracy rates in traffic sign detection, meeting the real-time requirements of the input data.

P-10 A rare case of Triple A Syndrome with familial involvement

Abstract Introduction Alacrimia, achalasia cardia, and adrenocorticotrophic hormone (ACTH)-resistant adrenal insufficiency make up the triple-A syndrome. It is an uncommon disease with an autosomal recessive inheritance pattern. The AAAS gene, which codes for the ALADIN protein and is located on chromosome 12q13, is the cause of Allgrove syndrome. The initial and typical symptoms are achalasia cardia and adrenal insufficiency. The majority of neurological disorders have autonomic signs, and they typically show in later life. The other typical neurological conditions are polyneuropathy, amyotrophy, and optic atrophy. Clinical Case A 25-year-old female patient presented to our clinic with complaints of weakness, dizziness, and hyperpigmentation of the skin. Her medical history revealed a recent intensive care unit (ICU) stay due to hypotension, during which her condition was managed with steroid therapy. However, she did not continue the steroid treatment after discharge. Notably, the patient had a family history of achalasia with operative intervention in all three siblings. The patient and her family members were also under the care of a gastroenterology specialist. Additionally, the patient reported experiencing dry eyes, for which she was using artificial tears. Physical examination revealed hyperpigmentation of tongue, gums and buccal mucosa. Upon examination, her baseline investigations revealed a hemoglobin of 11.4 g/dL, sodium 137 mmol/L and potassium 4.0 mmol/L. The patient's adrenocorticotropic hormone (ACTH) level was measured at 1250 pg/ml, with a basal cortisol level of 0.5 ug/dL, leading to the diagnosis of adrenal insufficiency. Hydrocortisone treatment was initiated at a dosage of 2×10mg, resulting in a decline of the ACTH level to 31. Given the combination of symptoms including achalasia, alacrima, and adrenal insufficiency, genetic testing was conducted. Both alleles revealed a homozygous c.1432C&amp;gt;T (p.Arg478*) mutation, confirming the diagnosis of Triple A Syndrome. Following the genetic diagnosis, a familial investigation was initiated. This investigation revealed the presence of the homozygous positive mutation in two siblings and two cousins. The constellation of symptoms and the genetic findings confirmed the occurrence of Triple A Syndrome within this family. Conclusion This case highlights the significance of recognizing Triple A Syndrome as an underlying cause of adrenal insufficiency in the presence of achalasia, alacrima, and a suggestive clinical history. In order to confirm the diagnosis and identify any affected family members, genetic testing is crucial. To avoid potential complications brought on by adrenal insufficiency and other systemic Triple A Syndrome manifestations, early identification and treatment are crucial.

Neuropathic pain as a symptom in autonomic neuropathies and other rare diseases : Small fiber neuropathy: its recognition, diagnosis, and treatment

Neuropathic pain is difficult to diagnose and treat. Small fiber neuropathy (SFN) flies under the radar of nerve conduction studies. The importance of astructured patient history and physical examination in the context of neuropathic pain is emphasized. Describing SFN as an important cause, the authors consider rare but partially treatable differential diagnoses. They conclude that autonomic symptoms are frequently associated, often presenting with diverse symptoms. Aselective literature research to present SFN symptoms as well as differential diagnostic and therapeutic steps in the context of SFN and rare diseases focusing on the autonomic nervous system. Neuropathic pain significantly reduces quality of life. To shorten the time until diagnosis and to initiate therapy, the authors recommend astructured patient history including sensory plus and minus symptoms and non-specific autonomic signs. If the initial search for the cause is not successful, rare causes such as treatable transthyretin (ATTR) amyloidosis and Fabry's disease or autoimmune causes should be considered, particularly in the case of progressive and/or autonomic symptoms. The diagnosis and therapy of rare SFN requires interdisciplinary collaboration and, in many cases, areferral to specialized centers to achieve the best patient care.

Epidemiology and clinical features of paroxysmal hemicrania: Asystematic review and meta-analysis.

To assess the prevalence or relative frequency of paroxysmal hemicrania and its clinical features in the adult general population and among adult patients evaluated for headache in tertiary care. Paroxysmal hemicrania is a rare trigeminal autonomic cephalalgia with characteristic attacks of headache, associated cranial autonomic symptoms and signs, and an absolute response to indomethacin. Its epidemiological burden remains unknown in both the adult general population and among adult patients evaluated for headache in a tertiary care setting. Moreover, the frequencies of the clinical features associated with paroxysmal hemicrania have not been well established. A literature search of PubMed and Embase was conducted from January 1, 1988, to January 20, 2023. Eligible for inclusion were observational studies reporting the point prevalence or relative frequency of paroxysmal hemicrania or its clinical features in the adult general population or among adult patients evaluated for headache in tertiary care. Two independent investigators (M.J.H. and J.G.L.) performed the title, abstract, and full-text article screening. Each included study's risk of bias was critically appraised using the Joanna Briggs Institute Critical Appraisal Checklist for Studies Reporting Prevalence Data. Estimates of prevalence or relative frequency were calculated using a random-effects meta-analysis. The between-study heterogeneity was assessed using the I2 statistic and further explored with meta-regression. This study was pre-registered on PROSPERO (identifier: CRD42023391127). A total of 17 clinic-based studies and one population-based study met the eligibility criteria. Importantly, an overall high risk of bias was observed across the eligible studies. The relative frequency of paroxysmal hemicrania was estimated to be 0.3% (95% CI, 0.2%-0.5%) among adult patients evaluated for headache in tertiary care with considerable heterogeneity (I2 = 76.4%). No cases with paroxysmal hemicrania were identified among 1,838 participants in a population-based sample. Moreover, the most prevalent cranial autonomic symptoms were lacrimation (77.3% [95% Cl, 62.7%-87.3%]), conjunctival injection (75.0% [95% Cl, 60.3%-85.6%]), and nasal congestion (47.7% [95% Cl, 33.6%-62.3%]). Our findings suggest that paroxysmal hemicrania is a rare disorder among adults evaluated for headache in tertiary care, while its prevalence in the general population remains unknown. Further studies focusing on the clinical features of paroxysmal hemicrania are warranted.

Orofacial migraine and neurovascular orofacial pain—new insights into characteristics and classification

Orofacial migraine (OFM) and neurovascular orofacial pain (NVOP) are both recognized as migraine-related entities affecting the facial and orofacial regions, according to the International Classification of Orofacial Pain (ICOP). However, the distinction between these two conditions and the question of whether NVOP should be considered a separate entity remain subjects of ongoing debate. The aim of this study is to compare the diagnostic characteristics of OFM and NVOP to reassess whether they should continue to be classified as two distinct diagnoses. The cohort comprised 75 patients, 12 males and 63 females, 40 were diagnosed as NVOP and 35 as OFM according to ICOP criteria. Patients were recruited from the tertiary orofacial pain clinic in Hadassah Medical Center between the years 2016 to 2023. NVOP and OFM patients did not differ in age, sex, pain intensity and other pain characteristics. However, OFM patients have significantly more cranial autonomic signs (36.4%) than NVOP patients (10.3%), and also more migraine symptoms such as nausea and photophobia. (68.6% vs. 41%) OFM patients reported significantly more awakening from sleep (52.9%) than NVOP patients (26.3%). Also, OFM pain was concomitant with headache in about two third of cases (66.7%), compared to only a third (30.8%) of NVOP cases. Most NVOP patients have pain that mimics toothache (85%), rarely detected in OFM (11.4%). The diagnostic features of OFM and NVOP indicate that there are many similarities between the two. But also, unique features that allows for separating OFM and NVOP into two distinct diagnostic entities, in accordance with the ICOP classification. Inclusion of patients with associated headaches enhanced this separation, and suggests expanding the definition of ICOP and include it under OFM and NVOP. At present there is justification to maintain the separate ICOP classifications of NVOP and OFM, particularly for research purposes.

Midline Swaddling and Traditional swaddling Position and Their Influence on Physical and Autonomic sign on Newborn Babies.

The traditional method of swaddling is done with the arms and legs straightened and wrapped tightly with the hips in an adduction position, and the newborns are mostly carried out by traditional swaddling in Indonesia. When swaddling is properly done, infants show less awakening and longer sleep, better neuromuscular development, less physiological stress, better motor organization, and better self-regulation abilities. Improper swaddling can result in conditions that are detrimental for newborns, like SIDS, DDH, which cause stress. The early exposure of the neonates to stress results to residual symptoms in behavior and neurobiological disorders. This study aims to determine the effect of midline position compared to the traditional swaddling regarding physical and autonomics stress sign. The study population consisted of 194 newborns. The midline and traditionally swaddled were observed and recorded, physical and autonomic signs at birth, 10 minutes and 2 hours were documented. This study found that there is statistically significant difference between the groups in terms of cries at 10 minute and 2 hours and so with the heart rate and respiration rate at birth (p&lt;0,05), and a significant difference in oxygen saturation at 10 minutes and 2 hours (p&lt;0,05). Additionally, midline swaddling had a larger average decrease in heart rate and respiration rate, and increase in the oxygen saturation. Therefore, this study can deduce that, midline position swaddling has less stressful effect than the traditional position. The same research elsewhere in Indonesia will be costly. Future research would suggest for larger sample that may suggest a more accurate outcome.

Autonomous Traffic Sign Detection for Self-Driving Car System Using Convolutional Neural Network Algorithm

Autonomous Traffic Sign Detection for Self-Driving Car System Using Convolutional Neural Network Algorithm

Clinical spectrum, etiology, and treatment response in neonatal autonomic seizures: A case series

AbstractBackgroundNeonatal autonomic signs such as apnea, cyanosis, pallor, and desaturation rarely occur as isolated ictal phenomena and require a high degree of clinical suspicion in combination with continuous video electroencephalogram (vEEG) to establish a diagnosis.MethodsWe review the clinical profile, etiology, and treatment response in five neonates who presented with apnea as the primary seizure semiology.ResultsIctal apneic episodes were confirmed on continuous vEEG in all five infants within 24–48 h of symptom onset. Seizure etiologies included structural, infectious, and genetic, including a neonate with ANKRD11‐associated KBG syndrome, in which ictal apnea has not been previously described. Acute seizures resolved in all neonates following treatment with a single or combination of antiseizure medications.ConclusionsAbrupt onset and clustering episodes of apnea and oxygen desaturation in term infants should raise suspicion for epileptic seizures. Genetic testing should be considered as part of the diagnostic evaluation for autonomic seizures of unknown etiology. Early diagnosis and treatment of neonatal autonomic seizures may lead to excellent treatment response in the acute setting.

Orofacial Migraine and Other Idiopathic Non-Dental Facial Pain Syndromes: A Clinical Survey of a Social Orofacial Patient Group.

Background: Orofacial pain syndromes (OFPs) are a heterogeneous group of syndromes mainly characterized by painful attacks localized in facial and oral structures. According to the International Classification of Orofacial Pain (ICOP), the last three groups (non-dental facial pain, NDFP) are cranial neuralgias, facial pain syndromes resembling primary headache syndromes, and idiopathic orofacial pain. These are often clinical challenges because the symptoms may be similar or common among different disorders. The diagnostic efforts often induce a complex diagnostic algorithm and lead to several imaging studies or specialized tests, which are not always necessary. The aim of this study was to describe the encountered difficulties by these patients during the diagnostic-therapeutic course. Methods: This study was based on the responses to a survey questionnaire, administered to an Italian Facebook Orofacial Patient Group, searching for pain characteristics and diagnostic-therapeutic care courses. The questionnaire was filled out by patients affected by orofacial pain, who were 18 years and older, using a free online tool available on tablets, smartphones, and computers. Results: The sample was composed of 320 subjects (244F/76M), subdivided by age range (18-35 ys: 17.2%; 36-55 ys: 55.0%; >55 ys 27.8%). Most of the patients were affected by OFP for more than 3 years The sample presented one OFP diagnosis in 60% of cases, more than one in 36.2% of cases, and 3.8% not classified. Trigeminal neuralgia is more represented, followed by cluster headaches and migraines. About 70% had no pain remission, showing persisting background pain (VAS median = 7); autonomic cranial signs during a pain attack ranged between 45 and 65%. About 70% of the subjects consulted at least two different specialists. Almost all received drug treatment, about 25% received four to nine drug treatments, 40% remained unsatisfied, and almost 50% received no pharmacological treatment, together with drug therapy. Conclusion: To the authors' knowledge, this is the first study on an OFP population not selected by a third-level specialized center. The authors believe this represents a realistic perspective of what orofacial pain subjects suffer during their diagnostic-therapeutic course and the medical approach often results in unsatisfactory outcomes.

Dysautonomia in Amyotrophic Lateral Sclerosis.

Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease, characterized in its typical presentation by a combination of lower and upper motor neuron symptoms, with a progressive course and fatal outcome. Due to increased recognition of the non-motor symptoms, it is currently considered a multisystem disorder with great heterogeneity, regarding genetical, clinical, and neuropathological features. Often underestimated, autonomic signs and symptoms have been described in patients with ALS, and various method analyses have been used to assess autonomic nervous system involvement. The aim of this paper is to offer a narrative literature review on autonomic disturbances in ALS, based on the scarce data available to date.

LGI1 and CASPR2-related Morvan syndrome - diagnostic challenges in a pediatric case

Morvan syndrome is a rare immune-mediated pathology involving the central, peripheral and autonomic nervous systems. Although it was described in adults, mostly as a paraneoplastic syndrome, it is rarely seen in pediatric patients. In this report, we present the case of a 15-year-old male patient who experienced insomnia, peripheral nerve hyperexcitability and autonomic signs, highly suggestive for Morvan syndrome, but prone to diagnostic dilemmas as well. The diagnosis was confirmed by the presence of antibodies against voltage-gated potassium channels (VGKC). This article aims to highlight the unique clinical picture of a rare syndrome, diagnostic pitfalls and treatment options in pediatric patients.

Drug-resistant epicrania fugax: Responding to onabotulinumtoxinA.

Epicrania fugax (EF) is a primary headache consisting of brief paroxysms of pain, lasting 1-10 s, that move through different nerve territories of one hemicranium with a linear or zigzag trajectory, although there are some clinical variants. Preventive therapy with anti-seizure medication such as gabapentin and lamotrigine are most commonly used in patients presenting with frequent and non-remitting attacks. In some cases, greater occipital nerve blockades are used for short- or long-term relief. Here, we report two patients with a paroxysmal EF-type pain who meet the criteria for EF of the International Classification of Headache Disorders, 3rd edition, with clear triggers and autonomic ocular signs and who failed multiple preventive treatments, but had a sustained response to onabotulinumtoxinA.

THE EFFECT OF POSTPARTUM PHYSICAL THERAPY ON AUTONOMIC SYMPTOMS OF POST-COVID-19 SYNDROME IN WOMEN AFTER CAESAREAN SECTION WHO EXPERIENCED CORONAVIRUS DISEASE DURING PREGNANCY

Purpose: to determine the effectiveness of restoring the state of women health with post-COVID-19 syndrome who underwent cesarean section, based on the dynamics of parameters of the autonomic nervous system.&#x0D; Methods. 44 women were examined at the beginning of the late postpartum period. The control group consisted of 15 women who gave birth vaginally and did not suffer from COVID-19 during pregnancy and observation. The comparison group consisted of 15 women after cesarean section who suffered from COVID-19 during pregnancy, recovered during pregnancy and the postpartum period according to general recommendations for the rehabilitation of patients with coronavirus infection. The main group consisted of 14 women after caesarean section, suffered from COVID-19 during pregnancy, performed a proven program of physical therapy for 2 months using breathing therapeutic exercises, therapeutic exercises with elements of yoga for the muscles of the back, abdomen, upper and lower limbs, taking into account movement limitations due to a scar of the anterior abdominal wall; massage of the chest, back, abdomen; aromatherapy sessions; autogenic training sessions. They tried to restore the state of women through a balance between the sympathetic and parasympathetic departments due to the normalizing effect of the applied agents on the hormonal background and psycho-emotional state, reducing the feeling of bodily discomfort, general strengthening of the body, accelerating the recovery of the organs of the abdominal cavity and pelvis, accelerating adaptation to changes in habitual activity due to a violation daily routine and childcare needs. The condition of the women was assessed by the results of the test with isometric load, the Kerdo index, the dynamics of 24-hour heart rate variability monitoring (power in the range of low LF frequencies and high HF frequencies, LF/HF ratio, Baevsky tension index).&#x0D; Research results. During the first examination, an imbalance between the activity of the sympathetic and parasympathetic links of the autonomic nervous system towards sympathicotonia was detected in all women, which was most pronounced in women with post-COVID-19 syndrome in all parameters. During re-examination, the best result in terms of all studied parameters was found by women of the control group, who demonstrated the dynamics of the normal course of the postpartum period after vaginal delivery and increased activity of the parasympathetic department against the background of suppression of the sympathetic department. The results of the women of the main group according to the Kerdo index, tests with isometric tension, the results of daily blood pressure monitoring, which were determined, were statistically significantly better than those of the women of the comparison group, testifying to the reduction of the imbalance of the links of the autonomic nervous system and the advantages of the rehabilitation program for the correction of the autonomic signs of post-COVID-19 syndrome, which was created taking into account the physical and psychoemotional state of women after cesarean section, in comparison with the general rehabilitation program.&#x0D; Conclusions. The use of physical therapy, taking into account the imbalance of the links of the autonomic nervous system (sympatheticotonia), is pathogenetically and practically justified in the process of physical therapy of women with post-COVID-19 syndrome in the postpartum period after abdominal delivery.

Continuous hemicrania as the initial manifestation of Ramsay Hunt syndrome: a case report

IntroductionRamsay-Hunt syndrome, also called otic zoster, is a rare complication of herpes zoster. The syndrome is characterized by peripheral facial nerve palsy and an erythematous vesicular eruption in the ear or mouth. Preceding the appearance of the vesicles, unilateral otalgia or neck pain may occur more commonly. However, persistent hemicrania is infrequent in the pre-eruptive phase.ObjectivesTo present an atypical case of Ramsay Hunt syndrome with continuous unilateral headache preceding the onset of other symptoms and signs of the syndrome.Case reportReport of a 69-year-old woman who presented subacute onset of moderate to severe left hemicrania with no autonomic signs. Eight days after the start and continuous headache maintenance, she presented with peripheral facial paralysis. After four days, she noticed the presence of vesicles in the left ear and odynophagia. She developed nausea with several episodes of vomiting and severe imbalance that made it impossible for her to walk unassisted. On physical examination, she presented vesicles in the left ear and oropharynx, left peripheral facial palsy (House Brackmann grade IV), left hypoacusis, nystagmus, and vestibular gait. Diagnostic tests for screening several metabolic diseases and diagnosis of infection (including HIV) were unremarkable. Brain computed tomography and cerebrospinal fluid analysis showed no abnormalities.ConclusionsRamsay-Hunt syndrome mainly involves the facial and vestibulocochlear nerves, causing peripheral facial palsy, otalgia, hypoacusis, and, less frequently, imbalance. Although pain is a frequent manifestation of the pre-eruptive phase of RHS, unilateral headache is not common in this scenario. On the other hand, it is a prevalent complaint in the emergency department and has several different etiologies. Hence, diagnosing RHS when patients present exclusively unilateral headaches is challenging for clinicians. Physicians must consider RHS a vital differential diagnosis of sided-locked headaches, avoiding diagnostic errors and treatment delays.

The Prevalence of Bladder and Bowel Dysfunction in Children with Cerebral Palsy and its Association with Motor, Cognitive, and Autonomic Function

ABSTRACT Purpose To describe the prevalence of bladder and bowel dysfunction (BBD) in 8–10-year-old children with cerebral palsy and its association with motor, cognitive, and autonomic dysfunction. Methods A cross-sectional, random sample study of parents of 8–10-year-old children with cerebral palsy. Tools: The Enuresis/Urinary Incontinence Parental Questionnaire, the Functional Independence Measure children’s version, the autonomic signs questionnaire, and the Gross Motor Function Classification System. Results 39 out of 59 parents consented to participate, whereas 25.64% reported complete continence. Of the 29 children with BBD, 21 (72.4%) had lower urinary tract symptoms and bowel problems. Only two of the children received conservative and noninvasive treatments. Lastly, motor, cognitive and autonomic impairments were associated with incontinence. Conclusions BBD is common in 8–10-year-old children with cerebral palsy at all levels of functioning. Most having both lower urinary tract symptoms and bowel problems.