CAPOS syndrome is an autosomal dominant neurological disorder caused by mutations in the ATP1A3 gene. Initial symptoms, often fever-induced, include recurrent acute ataxic encephalopathy in childhood, featuring cerebellar ataxia, optic atrophy, areflflexia, sensorineural hearing loss, and in some cases, pes cavus. This report details a case of CAPOS syndrome resulting from a maternal ATP1A3 gene mutation. Both the child and her mother exhibited symptoms post-febrile induction,including severe sensorineural hearing loss in both ears, ataxia, areflexia, and decreased vision. Additionally, the patient's mother presented with pes cavus. Genetic testing revealed a c. 2452G>A（Glu818Lys） heterozygous mutation in theATP1A3 gene in the patient . This article aims to enhance clinicians' understanding of CAPOS syndrome, emphasizing the case's clinical characteristics, diagnostic process, treatment, and its correlation with genotypeic findings.
Humans ; Child ; Female ; Cerebellar Ataxia/diagnosis* ; Talipes Cavus ; Hearing Loss, Sensorineural/diagnosis* ; Optic Atrophy/diagnosis* ; Mutation ; Phenotype ; Sodium-Potassium-Exchanging ATPase/genetics* ; Foot Deformities, Congenital ; Reflex, Abnormal

Breathing is an intrinsic natural behavior and physiological process that maintains life. The rhythmic exchange of gases regulates the delicate balance of chemical constituents within an organism throughout its lifespan. However, chronic airway diseases, including asthma and chronic obstructive pulmonary disease, affect millions of people worldwide. Pathological airway conditions can disrupt respiration, causing asphyxia, cardiac arrest, and potential death. The innervation of the respiratory tract and the action of the immune system confer robust airway surveillance and protection against environmental irritants and pathogens. However, aberrant activation of the immune system or sensitization of the nervous system can contribute to the development of autoimmune airway disorders. Transient receptor potential ion channels and voltage-gated Na⁺ channels play critical roles in sensing noxious stimuli within the respiratory tract and interacting with the immune system to generate neurogenic inflammation and airway hypersensitivity. Although recent studies have revealed the involvement of nociceptor neurons in airway diseases, the further neural circuitry underlying airway protection remains elusive. Unraveling the mechanism underpinning neural circuit regulation in the airway may provide precise therapeutic strategies and valuable insights into the management of airway diseases.
Humans ; Pulmonary Disease, Chronic Obstructive ; Neurons ; Respiration ; Hypersensitivity ; Reflex

Animals ; Reflex, Startle ; Models, Animal ; Acoustics

OBJECTIVE: To compare the clinical efficacy between wrist-ankle acupuncture and conventional acupuncture on shoulder-hand syndrome (SHS) phaseⅠafter stroke. METHODS: A total of 64 patients with SHS phaseⅠafter stroke were randomized into a wrist-ankle acupuncture group and a conventional acupuncture group, 32 cases in each group. On the basis treatment of internal medicine and conventional rehabilitation, wrist-ankle acupuncture was applied at upper 4 area, upper 5 area and upper 6 area on the affected side in the wrist-ankle acupuncture group, while acupuncture was applied at Jianyu (LI 15), Quchi (LI 11), Shousanli (LI 10), etc. on the affected side in the conventional acupuncture group. The treatment was given 30 min each time, once a day, 5 days a week for 3 weeks in both groups. Before and after treatment, the visual analogue scale (VAS) score, degree of hand swelling, shoulder-hand syndrome scale (SHSS) score, Fugl-Meyer assessment for upper extremity (FMA-UE) score and modified Barthel index (MBI) score were observed, and the clinical therapeutic effect was evaluated in both groups. RESULTS: After treatment, the VAS scores, degree of hand swelling and SHSS scores were decreased (P<0.05), and the FMA-UE scores and MBI scores were increased (P<0.05) compared before treatment in both groups; in the wrist-ankle acupuncture group, the VAS score, degree of hand swelling and SHSS score were lower (P<0.05), and the FMA-UE score and MBI score were higher (P<0.05) than those in the conventional acupuncture group. The total effective rate was 96.9% (31/32) in the wrist-ankle acupuncture group, which was superior to 90.6% (29/32) in the conventional acupuncture group (P<0.05). CONCLUSION Wrist-ankle acupuncture can effectively relieve pain and hand swelling, improve motor function of upper extremity and self-care ability of daily life in patients with shoulder-hand syndrome phaseⅠafter stroke, the therapeutic effect is superior to conventional acupuncture.
Acupuncture Points ; Acupuncture Therapy ; Ankle ; Humans ; Reflex Sympathetic Dystrophy/therapy* ; Stroke/therapy* ; Upper Extremity ; Wrist

OBJECTIVE: To compare the clinical efficacy differences between WANG Ju-yi 's meridian diagnosis method combined with Bobath rehabilitation training and Bobath rehabilitation training alone for post-stroke shoulder-hand syndrome (SHS) typeⅠ. METHODS: A total of 106 patients with post-stroke SHS typeⅠwere randomly divided into an observation group (53 cases, 2 cases dropped off ) and a control group (53 cases, 3 cases dropped off ). The patients in the both groups were treated with medications for basic diseases and conventional acupuncture at Waiguan (TE 5), Shousanli (LI 10) and Jianyu (LI 15) on the affected side. In addition, the patients in the control group were treated with Bobath rehabilitation training, 20 minutes each time; on the basis of the control group, the patients in the observation group were treated with WANG Ju-yi's meridian diagnosis method to adjust the abnormal parts in meridians of the hand taiyin and hand yangming on the affected side, 20 minutes each time. Both groups were treated once a day, 5 times a week for 8 weeks. The scores of visual analogue scale (VAS), upper-limb Fugl-Meyer assessment (FMA) and Barthel index (BI) were recorded before and after treatment as well as 6 weeks after treatment (follow-up), and the clinical efficacy of the two groups was evaluated after treatment. RESULTS: Compared before treatment, the VAS scores were reduced and the scores of upper-limb FMA and BI were increased in the two groups after treatment and in the follow-up (P<0.05). The VAS score in the observation group was lower than that in the control group (P<0.05), and the scores of upper-limb FMA and BI in the observation group were higher than those of the control group (P<0.05). The total effective rate in the observation group was 82.4% (42/51), which was higher than 62.0% (31/50) in the control group (P<0.05). CONCLUSION WANG Ju-yi 's meridian diagnosis method combined with Bobath rehabilitation training could effectively treat post-stroke SHS typeⅠ, reduce pain symptoms and improve joint motor dysfunction, and improve the quality of life. Its curative effect is better than Bobath rehabilitation training alone.
Acupuncture Therapy ; Humans ; Meridians ; Quality of Life ; Reflex Sympathetic Dystrophy/therapy* ; Stroke/complications* ; Stroke Rehabilitation ; Treatment Outcome

OBJECTIVE: To assess the efficacy on relieving pain and improving the range of motion of shoulder joint in post-stroke shoulder-hand syndrome of phlegm-stasis obstruction in treatment of the combined therapy of eye acupuncture, Tengliao (Chinese herbal warm dressing technique) and rehabilitation training (eye acupuncture + Tengliao + rehabilitation) as compared with the combined treatment of Tengliao and rehabilitation training (Tengliao + rehabilitation) and the simple rehabilitation training (rehabilitation). METHODS: A total of 356 patients with post-stroke shoulder-hand syndrome of phlegm-stasis obstruction were randomized into an eye acupuncture + Tengliao + rehabilitation group (group A, 122 cases, 2 cases dropped off), a Tengliao + rehabilitation group (group B, 120 cases, 3 cases dropped off) and a rehabilitation group (group C, 114 cases, 1 case dropped off). In the group C, the basic treatment was combined with routine rehabilitation training. In the group B, on the base of the treatment as the group C, Tengliao was exerted. A medical bag composed of over 20 Chinese herbal materials was heated and dressed at the affected area, 30 min each time, 5 times weekly. In the group A, besides the treatment as the group B, eye acupuncture was applied to heart region, kidney region, upper jiao region and lower jiao region, 30 min each time, 5 times weekly. The treatment lasted 28 days in all of three groups. Separately, before treatment, in 7, 14, 21 and 28 days of treatment, as well as in 14 days after treatment of follow-up, the score of visual analogue scale (VAS) for pain, the score of guides to evaluation of permanent impairment (GEPI) and the score of National Institutes of Health stroke scale (NIHSS) were observed in each group. RESULTS: The scores of VAS, GEPI and NIHSS were all improved with the treatment lasting in the three groups (P<0.000 1). In 7, 14, 21 and 28 days of treatment and in follow-up as well, VAS scores in the group A were all lower than the group C (P<0.05). After 14 days of treatment, GEPI score showed increasing trend, while NIHSS score showed decreasing trend in the group A compared with the group B. Before treatment, GEPI score was lower and NIHSS score was higher in the group A compared with the group C (P<0.05). It was suggested that the illness was slightly serious in the group A. After propensity score matching, in 14, 21 and 28 days as well as in follow-up, GEPI scores in the group A were higher than the group C respectively (P<0.05). Regarding NIHSS score at each time point, the difference had no statistical significance between the group A and the group C (P>0.05). CONCLUSION The combined therapy of eye acupuncture, Tengliao and rehabilitation training obtains a better efficacy on post-stroke shoulder-hand syndrome of phlegm-stasis obstruction as compared with rehabilitation training.
Acupuncture Points ; Acupuncture Therapy/methods* ; Humans ; Pain ; Reflex Sympathetic Dystrophy/therapy* ; Stroke/complications* ; Stroke Rehabilitation ; Treatment Outcome

OBJECTIVE: A network Meta-analysis of randomized controlled trials (RCT) of 4 commonly used acupuncture therapies (electroacupuncture, fire needling, warming acupuncture and filiform needling) for shoulder hand syndrome (SHS) after stroke was performed. METHODS: The RCTs regarding electroacupuncture, fire needling, warming acupuncture and filiform needling for SHS after stroke before March 10, 2020 were searched in databases of CNKI, Wanfang, VIP, SinoMed, PubMed, EMbase and Cochrane Library. The included literature was screened and evaluated by Cochrane bias risk assessment tool, and the data analysis was performed by RevMan5.3, Gemtc0.14.3 and Stata14.2. RESULTS: A total of 21 RCTs were included, involving 1508 patients, 814 cases in the observation group and 694 cases in the control group. In term of effective rate and visual analogue scale (VAS) score, warming acupuncture, electroacupuncture and fire needling needling were superior to western medication and rehabilitation ( CONCLUSION The curative effect of 4 acupuncture therapies for SHS after stroke is better than the western medication and rehabilitation, and warming acupuncture has the best clinical efficacy.
Acupuncture Points ; Acupuncture Therapy ; Humans ; Network Meta-Analysis ; Reflex Sympathetic Dystrophy ; Stroke/therapy* ; Treatment Outcome

OBJECTIVE: To observe the clinical effects of zheng's massage combined with electroacupuncture in the treatment ofreflex sensory dystrophy syndrome of the wrist. METHODS: From October 2016 to September 2018, 48 cases of reflex sensory dystrophy syndrome of the wrist were divided into the observation group and the control group. In the observation group, there were 24 cases, including 10 males and 14 females, ranging in age from 54 to 76 years old, with an average age of (61.41 ±7.90) years old. The patients in the observation group were treated with massage combined with electroacupuncture. The control group consisted of 24 patients, including 9 males and 15 females, ranging in age from 52 to 75 years old, with an averageage of (58.71±8.11 ) years old. The patients in the control group were treated with electroacupuncture alone. All the patients in both groups were treated for 6 weeks. The clinical symptoms and signs, visual anglogue scale (VAS), Cooney wrist score and clinical efficacy evaluation were compared between the two groups before and after treatment, and statistical analysis was conducted. RESULTS: After 6 weeks of treatment, VAS in the control group was 4.9±1.8, and Cooney wrist score was 74.63±1.72; VAS in the observation group was 2.2±1.4, and Cooney wrist score was 86.31±2.53. The comprehensive scores of VAS and Cooney wrist joint between two groups were improved after treatment, and the observation group was better than control group(<0.05). The curative effect of the observation group was better than that of the control group. CONCLUSION Zheng's massage combined with electroacupuncture has the following advantages in the treatment of reflex sympathetic dystrophy syndrome of the wrist, such as small trauma, patients' willingness promoting functional rehabilitation, which is worthy of clinical promotion.
Aged ; Electroacupuncture ; Female ; Humans ; Male ; Massage ; Middle Aged ; Reflex Sympathetic Dystrophy ; therapy ; Treatment Outcome ; Wrist ; Wrist Joint

PURPOSE: To report the clinical manifestations and computed tomography (CT) findings of patients with a trapdoor type medial orbital wall blowout fracture.METHODS: From March 2009 to October 2016, the clinical records and computed tomography findings of patients who underwent surgical treatment for a trapdoor type medial orbital wall blowout fracture were retrospectively analyzed.RESULTS: A total of eight patients (six males and two females) were enrolled with a combined mean age of 14.4 years. Clinical manifestations were eyeball movement limitation (abduction and adduction) and ocular motility pain (eight patients, 100%), diplopia (seven patients, 87.5%), and nausea and vomiting (four patients, 50%). On CT, the distance from the orbital apex to the fracture site was an average of 22.0 mm and occurred in the middle position of the entire wall. Two patients had missed rectus completely dislocated into the ethmoid sinus through the fracture gap and six patients had definite involvement in the fracture gap and edema of the medial rectus muscle. The medial rectus muscle cross-sectional area was 47.7 mm² which was edematous compared to the contralateral eye (40.1 mm²). Orbital wall reconstruction was performed an average of 4.1 days after the injury. In all patients with oculocardiac reflex-like nausea and vomiting immediately improved after surgery. Six out of eight patients who had eyeball movement limitations (abduction and adduction) preoperatively showed adduction limitation after surgery. The eyeball movement limitation and diplopia disappeared 11.7 days and 46.7 days after surgery, respectively.CONCLUSIONS: Patients with trapdoor type medial wall blowout fracture showed characteristic computed tomographic findings and clinical manifestations such as eyeball movement limitation, ocular motility pain, diplopia, and oculocardiac reflex. An understanding of clinical findings and quick surgical treatment are therefore required. The type of eyeball movement limitation was abduction and adduction limitation preoperatively and adduction limitation postoperatively.
Diplopia ; Edema ; Ethmoid Sinus ; Humans ; Male ; Nausea ; Orbit ; Reflex, Oculocardiac ; Retrospective Studies ; Vomiting

Cough is a physiological defense reflex for protecting the airways from aspiration and irritation. Thus, roles of environmental triggers are postulated in the pathogenesis of chronic cough. There are several lines of epidemiological evidence demonstrating the relationships between environmental irritant and pollutant exposure and chronic cough. However, positive findings from cross-sectional studies just reflect the protective nature of cough but may not properly address the true impact of environmental triggers. If harmful inhalation is repeated, cough may be seen as chronic but indeed is protective in nature. Therefore, long-term residual outcomes would be the key for understanding the effects of environmental triggers on chronic cough. The present review aims to summarize the associations between chronic cough and environmental pollutants or irritant exposure, with a focus on the long-term residual effects of (1) chronic persistent exposure and (2) acute high-intensity exposure on chronic cough, and also to examine (3) whether childhood irritant/pollutant exposure may increase the risk of chronic cough in adulthood.
Cough ; Cross-Sectional Studies ; Environmental Pollutants ; Epidemiology ; Hypersensitivity ; Inflammation ; Inhalation ; Reflex