The paper reports acupuncture treatment for one case of oculomotor nerve palsy after cranial-orbital communicating tumor surgery. The acupoint prescription was composed of the local acupoints of the eyes (Yansanzhen, Tijian, Cuanzhu [BL2], Yuyao [EX-HN4] and Sizhukong [TE23]), the acupoints on the head, face and neck (Yangbai [GB14], Sibai [ST2] and Fengchi [GB20]), Guanyuan (CV4) on the abdomen, and those on the four limbs (Hegu [LI4], Zusanli [ST36], Shenmai [BL62] and Zhaohai [KI6]). The point-to-point needling technique with the eyelid lifted was operated at Tijian, Cuanzhu (BL2), Yuyao (EX-HN4), and Sizhukong (TE23). Warm needling with moxa cone placed on the needle handle was operated at Guanyuan (CV4) and Zusanli (ST36), and the usual needling technique was delivered at the rest acupoints. The treatment was given once daily, discontinued for 1 day after every 6 treatments. One course of treatment was composed of 7 days, and 6 courses were required. After treatment completion, the upper eyelids were basically symmetrical and the bilateral eye cracks were equal, the double vision appeared occasionally. No recurrence and no aggravation were reported in 1 month of follow up visit.
Humans ; Acupuncture Points ; Acupuncture Therapy ; Oculomotor Nerve Diseases/etiology* ; Orbital Neoplasms/surgery* ; Postoperative Complications/etiology*

This article reports a case of multiple cranial nerve injury after gamma knife radiosurgery treated with acupuncture and moxibustion combined with rehabilitation therapy. The patient presented with weakness of facial and tongue muscles, hoarseness, choking on water, and swallowing difficulties. The syndrome was attributed to qi and blood deficiency, and blood stasis obstructing the collaterals. The treatment principle focused on replenishing qi and blood, promoting blood circulation and unblocking collaterals. Yintang (GV24⁺), Lianquan (CV23), Qihai (CV6), Guanyuan (CV4), and Cuanzhu (BL2), Yangbai (GB14), Jingming (BL1), Sizhukong (TE23), Yingxiang (LI20), Sibai (ST2), Juliao (ST3), Quanliao (SI18), Dicang (ST4), Jiache (ST6), Xiaguan (ST7), Taiyang (EX-HN5) on the affected side, bilateral Jinjin (EX-HN12), Yuye (EX-HN13), Hegu (LI4), Zusanli (ST36), Sanyinjiao (SP6), Tianshu (ST25) were selected. Among these, bilateral Jinjin (EX-HN12) and Yuye (EX-HN13) were treated with pricking, Dicang (ST4) and Jiache (ST6) on the affected side were connected to an electroacupuncture device, and warming acupuncture was applied at Guanyuan (CV4). Rehabilitation therapy and electromyographic biofeedback were also incorporated. The treatments were given 2-3 times a week. After 18 months of intermittent treatment, the patient reported significant improvement, House Brackmann (H-B) facial nerve function grade was Ⅳ, and Sunnybrook facial nerve rating scale score was 53 points. After 2-month of follow-up, the patient reported normal swallowing ability and restored social engagement.
Humans ; Acupuncture Points ; Acupuncture Therapy ; Cranial Nerve Diseases/therapy*

Objective:To summarize the initial experience and evaluate the technical feasibility of the endoscopic transcanal transpromontorial approach（TTA） for vestibular schwannoma resection by analyzing long-term follow-up outcomes. Methods:A retrospective analysis was conducted on the perioperative and long-term follow-up data（mean follow-up time: 5 years） of patients who underwent endoscopic TTA for vestibular schwannoma resection in the Department of Otorhinolaryngology Head and Neck Surgery at Xiangya Hospital, Central South University, between January 2020 and December 2020. Long-term outcomes were systematically evaluated. Results:This study included two patients（one 41-year-old male and one 51-year-old female）. According to the AAO-HNS hearing classification system, preoperative hearing was Class C in one patient and Class D in the other. Preoperative imaging confirmed Koos stageⅠ tumors in both cases. Postoperative transient facial nerve paralysis（House-Brackmann Grade Ⅲ） recovered to Grade Ⅰ within 4 months. No complications such as cerebrospinal fluid leakage, intracranial infection, or intracranial hemorrhage occurred. No tumor recurrence was observed during the 5-year follow-up period. Conclusion:The endoscopic transcanal transpromontorial approach is minimally invasive, facilitates rapid recovery, and demonstrates satisfactory technical feasibility and safety when strict patient selection criteria（Koos stageⅠtumors with non-serviceable hearing） are applied.
Humans ; Neuroma, Acoustic/surgery* ; Male ; Adult ; Middle Aged ; Retrospective Studies ; Female ; Endoscopy/methods* ; Follow-Up Studies ; Treatment Outcome

OBJECTIVE

To describe the clinical presentation, management and outcome of a rare case of isolated oculomotor nerve palsy in an immunocompromised adult secondary to a central nervous system (CNS) tuberculoma.

This is a case report.

A Filipino male in his 30s developed severe throbbing headache followed by binocular diplopia and drooping of the right upper eyelid. Findings were compatible with a neurologically-isolated pupil-involving, complete oculomotor nerve palsy on the right. Brain magnetic resonance imaging demonstrated enlargement and contrast enhancement of the cisternal portion of the right oculomotor nerve. Serologic testing was positive for the human immunodeficiency virus (HIV) and syphilis. Cerebrospinal fluid (CSF) analysis showed lymphocytic pleocytosis and elevated protein. CSF polymerase chain reaction was positive for Mycobacterium tuberculosis (TB). The patient was treated with penicillin, quadruple anti-Koch’s, and anti-retrovirals. Eyelid position and ocular motility improved after treatment. Aberrant regeneration of the right oculomotor nerve was observed with elevation of the right eyelid on downgaze (pseudo-Graefe sign).

CNS tuberculoma may present as a neurologically-isolated oculomotor nerve palsy, particularly in immunocompromised individuals. In TB-endemic countries, like the Philippines, it should be considered in the differential diagnosis. Early recognition and appropriate antimicrobial therapy can lead to neurologic improvement.

Objective:This study aimed to analyze the results of auditory and vestibular function tests in patients with unilateral vestibular schwannoma and explore their association with tumor size. Methods:Clinical data from 81 patients diagnosed with unilateral vestibular schwannoma who underwent pure-tone audiometry（PTA）, cervical and ocular vestibular evoked myogenic potentials（c/oVEMP）, as well as video head impulse test（vHIT）, and subsequently underwent surgical treatment, were retrospectively analyzed. Patients were categorized into groups based on tumor size: small （≤ 15 mm）, medium（16-30 mm）, and large （>30 mm）, determined by the maximum tumor diameter on contrast-enhanced MRI scans. Results:PTA results indicated hearing loss in 73 cases（90.1%）; vestibular function tests revealed abnormal rates for the anterior semicircular canal, horizontal semicircular canal, posterior semicircular canal, utricle, and saccule at 29.6%, 77.8%, 54.3%, 90.1%, and 92.6%, respectively. Statistically, no significant differences were found in preoperative hearing test results among patients in different groups（F=0.393, P=0.676）. However, significant differences were observed in horizontal semicircular canal gain（r=-0.248, P=0.025）, abnormal rates of horizontal semicircular canal catch-up saccades（r=0.507, P<0.001）, as well as cVEMP（χ²=15.111, P=0.004） and oVEMP thresholds（χ²=18.948, P<0.001） across varying tumor size groups. Conclusion:The extent of hearing loss in patients with vestibular schwannoma is not correlated with tumor size, whereas the degree of vestibular dysfunction demonstrates a correlation with tumor size.
Humans ; Neuroma, Acoustic/physiopathology* ; Retrospective Studies ; Audiometry, Pure-Tone ; Vestibular Function Tests ; Vestibular Evoked Myogenic Potentials ; Female ; Male ; Head Impulse Test ; Middle Aged ; Adult ; Semicircular Canals/physiopathology* ; Aged ; Hearing Loss/physiopathology*

To explore the clinical features and influencing factors of first-onset neuromyelitis optica spectrum disease (NMOSD) within 1 year after delivery. A single center, observational cohort study was used to retrospectively analyze 12 patients with first-onset NMOSD within 1 year after delivery hospitalized in the Department of Neurology of Beijing Tong Ren Hospital from June 2015 to June 2018(short as the postpartum onset group). 12 patients with first-onset NMOSD without 1 year after delivery hospitalized in our department during the same period were selected (short as the control group). The results showed the next recurrence interval in the postpartum onset group was longer than the control group [the postpartum onset group: (6.1±3.5) years, the control group: (1.6±1.5) years, t=3.622,P=0.005], the times of relapses were less than the control group [the postpartum onset group: (1.8±1.4) times, the control group:4.0 (3.0, 7.3) times, Z=-3.122,P=0.002], and expanded disability status scale (EDSS) of the last follow-up was lower than the control group [the postpartum onset group: 3.0(2.3, 3.9), the control group: 4.5(4.0, 6.0), Z=-3.358,P=0.001] with statistically significant differences. The recurrence rates of 1 year, 3 years and 5 years in the postpartum onset group (0%, 16.7%, 33.3%) were lower than control group (58.3%, 83.3%, 91.7%) with statistically significant differences (χ²=8.000,P=0.014;χ²=10.667,P=0.003; χ²=8.711,P=0.009). After the second delivery, the recurrence rate in postpartum onset group was 100% (n=3) and in control group was 50%(n=2), but the difference was not statistically significant (χ²=2.100,P=0.429). In the postpartum onset group, combination of autoimmune disease was consistent with positive in serum AQP-4 antibody moderately (Kappa=0.5, P=0.046). Positive in other autoimmune antibodies were consistent with positive in serum AQP-4 antibody moderately (Kappa=0.5, P=0.046). Combination of autoimmune disease were consistent with positive in serum other autoimmune antibodies well (Kappa=0.667, P=0.021). In conclusion, the first-onset NMOSD within 1 year after delivery have longer next recurrence interval, less times of relapses, lower relapse rate, better long-term prognosis of central nervous system, and they have trend to suffering from recurrent after the second delivery. For the females, combined with autoimmune disease or autoimmune antibody, who are ready for pregnancy, could detect serum AQP-4; if serum AQP-4 positive, they are recommended to prevent the occurrence of NMOSD after delivery.
Pregnancy ; Female ; Humans ; Neuromyelitis Optica/diagnosis* ; Retrospective Studies ; Cohort Studies ; Postpartum Period ; Recurrence

To explore the clinical features and influencing factors of first-onset neuromyelitis optica spectrum disease (NMOSD) within 1 year after delivery. A single center, observational cohort study was used to retrospectively analyze 12 patients with first-onset NMOSD within 1 year after delivery hospitalized in the Department of Neurology of Beijing Tong Ren Hospital from June 2015 to June 2018(short as the postpartum onset group). 12 patients with first-onset NMOSD without 1 year after delivery hospitalized in our department during the same period were selected (short as the control group). The results showed the next recurrence interval in the postpartum onset group was longer than the control group [the postpartum onset group: (6.1±3.5) years, the control group: (1.6±1.5) years, t=3.622,P=0.005], the times of relapses were less than the control group [the postpartum onset group: (1.8±1.4) times, the control group:4.0 (3.0, 7.3) times, Z=-3.122,P=0.002], and expanded disability status scale (EDSS) of the last follow-up was lower than the control group [the postpartum onset group: 3.0(2.3, 3.9), the control group: 4.5(4.0, 6.0), Z=-3.358,P=0.001] with statistically significant differences. The recurrence rates of 1 year, 3 years and 5 years in the postpartum onset group (0%, 16.7%, 33.3%) were lower than control group (58.3%, 83.3%, 91.7%) with statistically significant differences (χ²=8.000,P=0.014;χ²=10.667,P=0.003; χ²=8.711,P=0.009). After the second delivery, the recurrence rate in postpartum onset group was 100% (n=3) and in control group was 50%(n=2), but the difference was not statistically significant (χ²=2.100,P=0.429). In the postpartum onset group, combination of autoimmune disease was consistent with positive in serum AQP-4 antibody moderately (Kappa=0.5, P=0.046). Positive in other autoimmune antibodies were consistent with positive in serum AQP-4 antibody moderately (Kappa=0.5, P=0.046). Combination of autoimmune disease were consistent with positive in serum other autoimmune antibodies well (Kappa=0.667, P=0.021). In conclusion, the first-onset NMOSD within 1 year after delivery have longer next recurrence interval, less times of relapses, lower relapse rate, better long-term prognosis of central nervous system, and they have trend to suffering from recurrent after the second delivery. For the females, combined with autoimmune disease or autoimmune antibody, who are ready for pregnancy, could detect serum AQP-4; if serum AQP-4 positive, they are recommended to prevent the occurrence of NMOSD after delivery.
Pregnancy ; Female ; Humans ; Neuromyelitis Optica/diagnosis* ; Retrospective Studies ; Cohort Studies ; Postpartum Period ; Recurrence

Central nervous system involvement in primary Sjögren's syndrome (pSS) is less common and usually presents as white matter lesions, neuromyelitis optica spectrum disorder (NMOSD), or transverse myelitis. NMOSD is an immune-mediated inflammatory demyelinating disease of the central nervous system with a high rate of relapse and significant disability. Studies have shown that patients with pSS combined with NMOSD have more severe symptoms and poorer prognosis. Here, we present a case of critical illness in pregnancy-associated NMOSD combined with Sjögren's syndrome. The patient was a 30-year-old pregnant woman with a history of Sjögren's syndrome who was diagnosed with NMOSD. She received combination therapy with steroids, intravenous immunoglobulin (IVIG), and hydroxychloroquine during pregnancy, resulting in partial resolution of numbness below the waist. However, due to irregular medication adherence outside the hospital setting, she developed weakness in her right lower limb accompanied by inability to move it, while her left lower limb still had some mobility but occasional numbness along with urinary and fecal incontinence. Ten days later, she was admitted to the emergency department where an emergency cesarean section was performed to deliver a healthy baby boy. However, her condition worsened postpartum as she developed high fever accompanied by bilateral lower limb paralysis and weakness along with loss of voluntary control over urination and defecation. The patient underwent ano-ther course of treatment consisting of steroids and IVIG; however there was limited improvement in symptoms observed after this intervention. Following administration of rituximab for the first time, the patient developed urinary tract infection which was successfully managed before continuing regular infusions. In later stages the patient could walk slightly with a limp and regained control over urination and defecation, allowing her to resume normal activities. This case suggests that combination therapy with steroids, IVIG, and hydroxychloroquine should be considered for the patients with pregnancy-associated NMOSD combined with Sjögren's syndrome. Rituximab can significantly improve symptoms such as postpartum paralysis in patients with NMOSD, however, there may be a risk of infection associated with its use.
Adult ; Female ; Humans ; Pregnancy ; Cesarean Section/adverse effects* ; Critical Illness ; Hydroxychloroquine/therapeutic use* ; Hypesthesia/complications* ; Immunoglobulins, Intravenous/therapeutic use* ; Inflammation/complications* ; Neuromyelitis Optica/diagnosis* ; Paralysis/complications* ; Pregnancy Complications/therapy* ; Rituximab/therapeutic use* ; Sjogren's Syndrome/complications* ; Steroids/therapeutic use* ; Vision Disorders

Wolfram syndrome is a rare genetic spectrum disorder characterized by diabetes insipidus, diabetes mellitus, optic atrophy, and deafness, accompanied by other variable clinical manifestations. At present, the prognosis of this syndrome is very poor, the specific molecular mechanism is not clear, effective treatments are lacking to delay, prevent or reverse the development of Wolfram syndrome, and many patients die prematurely due to severe neurological dysfunction. This increases the urgency of the research on the pathogenic molecular mechanism related to Wolfram syndrome and the development of new therapies. This article summarizes the research progress on the pathogenic molecular mechanism and treatment status of Wolfram syndrome, in order to provide reference for the further mechanism research, prevention and treatment of Wolfram syndrome.
Humans ; Wolfram Syndrome/therapy* ; Treatment Outcome ; Records

Objective: To investigate the application of cochlear nerve action potential (CNAP) monitoring in the resection of vestibular schwannoma, especially evaluating its significance for hearing preservation. Methods: From April 2018 to December 2021, 54 patients with vestibular schwannoma who underwent resection via retrosigmoid approach were collected in Chinese PLA General Hospital. Before surgery, all patients had effective hearing (AAO-HNS grade C or above). Brainstem auditory evoked potential (BAEP) combined with CNAP monitoring was performed during surgery. The CNAP monitoring was combined with continuous monitoring and cochlear nerve mapping. And patients were divided into hearing preservation group and non-preserved group according to postoperative AAO-HNS grade. SPSS 23.0 software was used to analyze the differences of CNAP and BEAP parameters between the two groups. Results: A total of 54 patients completed intraoperative monitoring and data collection, including 25 males (46.3%) and 29 females (53.7%), aged 27-71 years with an average age of 46.2 years. The maximum tumor diameter were (18.1±5.9) mm (range 10-34 mm). All tumors were totally removed with preserved facial nerve function (House-Brackmann grade I-II). The hearing preservation rate of 54 patients was 51.9% (28/54). During surgery, the V wave extraction rate of BAEP waveform was 85.2% (46/54) before tumor resection, 71.4% (20/28) in the hearing preservation group after tumor resection, and disappeared in the hearing preservation group (0/26). CNAP waveform was elicited in 54 patients during operation. Differences were found in the distribution of CNAP waveforms after tumor resection. The waveforms of the hearing-preserving group were triphasic and biphasic, while those in the non-preserving group were low-level and positive. For hearing preservation group, the amplitude of N1 wave after tumor resection was significantly higher than that before tumor resection[14.45(7.54, 33.85)μV vs 9.13(4.88, 23.35)μV, P=0.022]; However, for the non-preserved group, the amplitude of N1 wave after tumor resection was significantly lower than that before tumor resection [3.07(1.96, 4.60)μV vs 6.55(4.54, 9.71)μV, P=0.007]; After tumor resection, the amplitude was significantly higher than that of the unreserved group [14.45(7.54, 33.85)μV vs 3.07(1.96, 4.60)μV, P<0.001]. Conclusions: BAEP combined with CNAP monitoring is conducive to intraoperative hearing protection, and the application of cochlear nerve mapping can prompt the surgeon to avoid nerve injury. The waveform and N1 amplitude of CNAP after tumor resection have a certain value in predicting postoperative hearing preservation status.
Female ; Male ; Humans ; Middle Aged ; Neuroma, Acoustic/surgery* ; Action Potentials ; Evoked Potentials, Auditory, Brain Stem ; Cochlea ; Cochlear Nerve