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*

Objective: To investigate the misdiagnosis of area postrema syndrome (APS) manifesting as intractable nausea, vomiting and hiccups in neuromyelitis optic spectrum disease (NMOSD) and reduce the risk of misdiagnosis. Methods: We retrospectively analyzed data from NMOSD patients attending the Department of Neurology at the First Medical Center of PLA General Hospital between January 2019 and July 2021. SPSS25.0 was then used to analyze the manifestations, misdiagnosis, and mistreatment of APS. Results: A total of 207 patients with NMOSD were included, including 21 males and 186 females. The mean age of onset was 39±15 years (range: 5-72 years). The proportion of patients who were positive for serum aquaporin 4 antibody was 82.6% (171/207). In total, 35.7% (74/207) of the NMOSD patients experienced APS during the disease course; of these patients, 70.3% (52/74) had APS as the first symptom and 29.7% (22/74) had APS as a secondary symptom. The misdiagnosis rates for these conditions were 90.4% (47/52) and 50.0% (11/22), respectively. As the first symptom, 19.2% (10/52) of patients during APS presented only with intractable nausea, vomiting and hiccups; 80.8% (42/52) of patients experienced other neurological symptoms. The Departments of Gastroenterology and General Medicine were the departments that most frequently made the first diagnosis of APS, accounting for 54.1% and 17.6% of patients, respectively. The most common misdiagnoses related to diseases of the digestive system and the median duration of misdiagnosis was 37 days. Conclusions: APS is a common symptom of NMOSD and is associated with a high rate of misdiagnosis. Other concomitant symptoms often occur with APS. Gaining an increased awareness of this disease/syndrome, obtaining a detailed patient history, and performing physical examinations are essential if we are to reduce and avoid misdiagnosis.
Male ; Female ; Humans ; Child, Preschool ; Child ; Adolescent ; Young Adult ; Adult ; Middle Aged ; Aged ; Neuromyelitis Optica/diagnosis* ; Area Postrema ; Retrospective Studies ; Hiccup/complications* ; Vomiting/etiology* ; Nausea/etiology* ; Inflammation ; Syndrome ; Autoantibodies ; Diagnostic Errors ; Aquaporin 4

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

OBJECTIVE: To report on a rare case of Neurofibromatosis type 2 (NF2) manifesting as oculomotor nerve palsy and explore its genetic basis. METHODS: A patient with NF2 who had presented at Beijing Ditan Hospital Affiliated to Capital Medical University on July 10, 2021 was selected as the study subject. Cranial and spinal cord magnetic resonance imaging (MRI) was carried out on the patient and his parents. Peripheral blood samples were collected and subjected to whole exome sequencing. Candidate variant was verified by Sanger sequencing. RESULTS: MRI revealed bilateral vestibular Schwannomas, bilateral cavernous sinus meningiomas, popliteal neurogenic tumors, and multiple subcutaneous nodules in the patient. DNA sequencing revealed that he has harbored a de novo nonsense variant of the NF2 gene, namely c.757A>T, which has replaced a codon (AAG) encoding lysine (K) at position 253 with a stop codon (TAG). This has resulted in removal of the Merlin protein encoded by the NF2 gene from position 253 onwards. The variant was not found in public databases. Bioinformatic analysis suggested that the corresponding amino acid is highly conserved. Based on the guidelines from the American College of Medical Genetics and Genomics (ACMG), the variant was rated as pathogenic (PVS1+PS2+PM2_Supporting+PP3+PP4). CONCLUSION The heterozygous nonsense variant c.757A>T (p.K253*) of the NF2 gene probably underlay the disease in this patient with an early onset, atypical but severe phenotype.
Male ; Humans ; Neurofibromatosis 2/genetics* ; Genes, Neurofibromatosis 2 ; Oculomotor Nerve Diseases/genetics* ; Computational Biology ; Genomics ; Mutation

Objective: To explore the value of electrically evoked auditory brainstem response (EABR) monitoring combined with brainstem auditory evoked potential (BAEP) and compound action potential (CAP) monitoring during vestibular schwannoma resection for the protection of the cochlear nerve. Methods: Clinical data from 12 patients with vestibular schwannomas who had useful hearing prior to surgery were analyzed at the PLA General Hospital from January to December 2021. Among them, there were 7 males and 5 females, ranging in age from 25 to 59 years. Before surgery, patients underwent audiology assessments (including pure tone audiometry, speech recognition rate, etc.), facial nerve function evaluation, and cranial MRI. They then underwent vestibular schwannoma resection via the retrosigmoid approach. EABR, BAEP, and CAP were simultaneously monitored during surgery, and patients' hearing preservation was observed and analyzed after surgery. Results: Prior to surgery, the average PTA threshold of the 12 patients ranged from11 to 49 dBHL, with a SDS of 80% to 100%. Six patients had grade A hearing, and six patients had grade B hearing. All 12 patients had House-Brackman grade I facial nerve function prior to surgery. The MRI indicated tumor diameters between 1.1 and 2.4 cm. Complete removal was achieved in 10/12 patients, while near-total removal was achieved in 2/12 patients. There were no serious complications at the one-month follow-up after surgery. At the three-month follow-up, all 12 patients had House-Brackman grade I or II facial nerve function. Under EABR with CAP and BAEP monitoring, successful preservation of the cochlear nerve was achieved in six of ten patients (2 with grade B hearing, 3 with grade C hearing, and 1 with grade D hearing). Successful preservation of the cochlear nerve was not achieved in another four patients (all with grade D hearing). In two patients, EABR monitoring was unsuccessful due to interference signals; however, Grade C or higher hearing was successfully preserved under BAEP and CAP monitoring. Conclusion: The application of EABR monitoring combined with BAEP and CAP monitoring during vestibular schwannoma resection can help improve postoperative preservation of the cochlear nerve and hearing.
Male ; Female ; Humans ; Adult ; Middle Aged ; Neuroma, Acoustic/complications* ; Hearing/physiology* ; Evoked Potentials, Auditory, Brain Stem/physiology* ; Cochlear Nerve ; Hearing Loss, Sensorineural/etiology* ; Retrospective Studies ; Postoperative Complications/prevention & control*