Studies have shown that sensory nerve damage can activate the p38 mitogen-activated protein kinase(MAPK)pathway,but whether the same type of nerve injury after exercise activates the p38MAPK pathway remains unclear....Studies have shown that sensory nerve damage can activate the p38 mitogen-activated protein kinase(MAPK)pathway,but whether the same type of nerve injury after exercise activates the p38MAPK pathway remains unclear.Several studies have demonstrated that nerve growth factor may play a role in the repair process after peripheral nerve injury,but there has been little research focusing on the hypoglossal nerve injury and repair.In this study,we designed and established rat models of hypoglossal nerve crush injury and gave intraperitoneal injections of exogenous nerve growth factor to rats for 14 days.p38MAPK activity in the damaged neurons was increased following hypoglossal nerve crush injury;exogenous nerve growth factor inhibited this increase in acitivity and increased the survival rate of motor neurons within the hypoglossal nucleus.Under transmission electron microscopy,we found that the injection of nerve growth factor contributed to the restoration of the morphology of hypoglossal nerve after crush injury.Our experimental findings indicate that exogenous nerve growth factor can protect damaged neurons and promote hypoglossal nerve regeneration following hypoglossal nerve crush injury.展开更多
Objectives:Analyze the obstructive sleep apnea (OSA) treatment history in a group of participants who enrolled in a hypoglossal nerve stimulation trial Methods:Moderate-severe OSA patients with difficulty adhering to ...Objectives:Analyze the obstructive sleep apnea (OSA) treatment history in a group of participants who enrolled in a hypoglossal nerve stimulation trial Methods:Moderate-severe OSA patients with difficulty adhering to CPAP presented for enrollment in a multicenter trial.Self-reported history on prior OSA medical therapy was collected at enrollment,including OSA diagnosis date,CPAP start and stop dates,oral appliance trial,and reasons for discontinuation or non-adherence.Results:The cohort consisted of 929 participants,83% male,with a mean age (53.9 ± 10.5) years.Ninety percent (n =835) had complete CPAP information including 47% (n =435) who discontinued therapy prior to enrollment and 43% (n =400) who were still attempting CPAP but had inadequate adherence.Abandonment rates were 60% at 1-year,73% at 3-years,and 86% at 5-years.Oral appliance therapy was attempted by 171 patients for mean (1.8 ± 2.3) years,with 81% abandonment at 1 year,89% at 3-years,and 94% at 5-years.Conclusions:In this CPAP-refractory cohort,high rates of CPAP abandonment were reported in the first several years with approximately half of the participants not receiving any treatment despite being diagnosed for >5 years.Close clinical follow-up and consideration of alternative treatment options is indicated in all OSA patients in order to ensure adequate longitudinal care.展开更多
BACKGROUND Chondromyxoid fibroma(CMF) is a rare benign bone tumour of cartilaginous origin, which usually affects the metaphysis of the long bone. Involvement of the temporal bone is extremely rare. Patients with CMF ...BACKGROUND Chondromyxoid fibroma(CMF) is a rare benign bone tumour of cartilaginous origin, which usually affects the metaphysis of the long bone. Involvement of the temporal bone is extremely rare. Patients with CMF in the temporal bone can present some neurological deficits due to involvement of surrounding neural structures. CASE SUMMARY We present the first case of histopathologically proven CMF originating in the temporal bone and involving the hypoglossal canal in a 40-year-old woman. Hypoglossal nerve paralysis was identified on the cranial nerve examination. The patient underwent surgical excision and was neurologically normal except for mild left facial palsy on 5-mo follow-up examination after surgery. In the current report, the major characteristics and computed tomography/magnetic resonance imaging features of the lesion are discussed. Furthermore, previous literature regarding this pathology is reviewed.CONCLUSION The current study presents the first case of temporal bone CMF involving the hypoglossal canal.展开更多
Godtfredsen syndrome or clival syndrome is a rare syndrome of abducens and hypoglossal nerve palsies (cranial nerve 6th and 12th respectively) that localizes to a clival mass. There are few reported cases of this clin...Godtfredsen syndrome or clival syndrome is a rare syndrome of abducens and hypoglossal nerve palsies (cranial nerve 6th and 12th respectively) that localizes to a clival mass. There are few reported cases of this clinical presentation. The aim of this case report is to describe this rare manifestation observed in a woman with clival metastases arising from a thymoma. A previously well 34-year-old native lady presented to a district hospital in Sabah, Malaysia, with history of blurring of vision and headache for 1 month. Cranial nerve examination reveals right abducens nerve palsy (right 6th CN) and right hypoglossal nerve palsy (right 12th CN). Initial imaging with CT brain reveals a subtle extra-axial hyperdense mass adjacent to the clivus and a routine chest x-ray reveals a mediastinal mass. Further imaging of thorax showed right anterior mediastinal mass, which then proceeded with Video-Assisted Thoracoscopic Surgery (VATS) guided biopsy. The biopsy result was consistent with the finding of thymoma, type B2. She was then diagnosed with aggressive form of thymoma, which unfortunately has metastasized to the bone, lung, liver and brain. Despite prognosis at the time of diagnosis is guarded, she still opted to undergo chemotherapy. Despite the completion of 6 cycles of chemotherapy, her disease progressed, and she eventually succumbed to the illness. In short, the presence of combined 6th and 12th palsy should alert clinician to the possibility of clival mass or metastases and hence could earlier workup with appropriate imaging can lead to earlier diagnosis and better treatment outcome.展开更多
A 67-year-old woman underwent right radical neck dissection for cervical lymph node metastasis from maxillary gingival carcinoma. Two months later, metastasis in the left superior internal jugular lymph nodes were dis...A 67-year-old woman underwent right radical neck dissection for cervical lymph node metastasis from maxillary gingival carcinoma. Two months later, metastasis in the left superior internal jugular lymph nodes were discovered, and left radical neck dissection was performed. Postoperatively, airway obstruction occurred despite performing extubation after confirming that the patient had fully recovered from anesthesia. Bilateral hypoglossal nerve palsy was diagnosed and the patient was reintubated. After extubation on the following day, airway obstruction was relieved, but slurred speech and impaired swallowing were persistent. In view of this, hypoglossal nerve function should be examined before the second radical neck dissection on the contralateral side.展开更多
Schwannomas originating from the hypoglossal nerve are extremely rare neoplasms. Hypoglossal schwannomas usually occur between the third and fifth decades of life, with no sex predilection, commonly presenting as a pa...Schwannomas originating from the hypoglossal nerve are extremely rare neoplasms. Hypoglossal schwannomas usually occur between the third and fifth decades of life, with no sex predilection, commonly presenting as a painless, slow-growing, and lateral neck mass. The best treatment is complete surgical excision with preservation of the neural pathway, if possible. In fact, since these tumors are almost always benign, a conservative surgical approach is emphasized by most authors. We report a case of a large schwannoma of hypoglossal nerve origin in the upper neck in a 31-year-old male. Magnetic resonance imaging of the area demonstrated a 43 × 39 × 36 mm well-circumscribed mass with high and nonhomogeneous signal intensity on the right side of the upper neck. The lesion was successfully treated by extirpation, with no recurrence. The extracranial hypoglossal nerve sheath was as the origin of this tumor because the patient experienced remarkable disturbance of tongue motility after surgery.展开更多
Hypoglossal motor neurons(HMNs) innervate tongue muscles and play key roles in a variety of physiological functions,including swallowing,mastication,suckling,vocalization,and respiration.Dysfunction of HMNs is associa...Hypoglossal motor neurons(HMNs) innervate tongue muscles and play key roles in a variety of physiological functions,including swallowing,mastication,suckling,vocalization,and respiration.Dysfunction of HMNs is associated with several diseases,such as obstructive sleep apnea(OSA) and sudden infant death syndrome.OS A is a serious breathing disorder associated with the activity of HMNs during different sleep-wake states.Identifying the neural mechanisms by which the statedependent activities of HMNs are controlled may be helpful in providing a theoretical basis for effective therapy for OSA.However,the presynaptic partners governing the activity of HMNs remain to be elucidated.In the present study,we used a cell-type-specific retrograde tracing system based on a modified rabies virus along with a Cre/loxP gene-expression strategy to map the whole-brain monosynaptic inputs to HMNs in mice.We identified 53 nuclei targeting HMNs from six brain regions:the amygdala,hypothalamus,midbrain,pons,medulla,and cerebellum.We discovered that GAB Aergic neurons in the central amygdaloid nucleus,as well as calretinin neurons in the parasubthalamic nucleus,sent monosynaptic projections to HMNs.In addition,HMNs received direct inputs from several regions associated with respiration,such as the preBotzinger complex,parabrachial nucleus,nucleus of the solitary tract,and hypothalamus.Some regions engaged in sleep-wake regulation(the parafacial zone,parabrachial nucleus,ventral medulla,sublaterodorsal tegmental nucleus,dorsal raphe nucleus,periaqueductal gray,and hypothalamus) also provided primary inputs to HMNs.These results contribute to further elucidating the neural circuits underlying disorders caused by the dysfunction of HMNs.展开更多
Objective:Current guidelines for hypoglossal nerve stimulator(HGNS)implantation eligibility include drug-induced sleep endoscopy(DISE)findings and other patient characteristics but lead to highly variable rates of sur...Objective:Current guidelines for hypoglossal nerve stimulator(HGNS)implantation eligibility include drug-induced sleep endoscopy(DISE)findings and other patient characteristics but lead to highly variable rates of surgical success across institutions.Our objective was to determine whether additional factors seen on preoperative evaluation could be used as predictors of surgical success.Study design:Retrospective chart review.Setting:Single-institution academic tertiary care medical center.Subjects:and Methods:This study included patients with obstructive sleep apnea(OSA)who underwent HGNS implantation between 2015 and 2018.Surgical success was defined as a post-operative apnea-hypopnea index(AHI)of less than 20 events per hour and an AHI reduction of at least 50%.Preoperative polysomnogram(PSG)results,DISE findings,and physical parameters were compared between surgical successes and failures.Results:A total of 68 patients were included in the analysis.The overall surgical success rate was 79.4%(54/68).Elevated preoperative AHI was associated with an increased likelihood of treatment failure,with an AHI of(36.9±16.8)events/hour in the success group compared to(49.4±19.6)events/hour in the failure group(P=0.05).Patients observed to have partial lateral oropharyngeal collapse on DISE was more frequently associated with the treatment failure group than in the success group(P=0.04).Conclusion:Patients who underwent HGNS implantation overall had a very high treatment response rate at our institution.Factors that may predispose patients to surgical failure included the presence of lateral oropharyngeal collapse and a significantly elevated preoperative AHI.These should be considered when determining surgical candidacy for HGNS implantation.展开更多
Objective:To review the indications and clinical evidence supporting hypoglossal nerve stimulation (HNS) therapy for the treatment of moderate-to-severe obstructive sleep ap-nea (OSA). Methods:Peer reviewed literature...Objective:To review the indications and clinical evidence supporting hypoglossal nerve stimulation (HNS) therapy for the treatment of moderate-to-severe obstructive sleep ap-nea (OSA). Methods:Peer reviewed literature on hypoglossal nerve stimulation therapy for obstructive sleep apnea from 2001 to 2016. Results:The only currently FDA-approved HNS device for the treatment of moderate-to-severe OSA is produced by Inspire Medical Systems, which recently published its 36-month outcomes data from its Stimulation Therapy for Apnea Reduction (STAR) trial. HNS therapy is currently indicated for moderate-to-severe OSA patients who are CPAP-intolerant, have a body mass in-dex <32, apnea-hypopnea index <50, and without a concentric pattern of upper airway collapse on sleep endoscopy. Conclusions:Data from the STAR trial suggests that a subset of OSA patients can achieve a sig-nificant therapeutic response from hypoglossal nerve stimulation. However, these results may be limited in their generalizability to the broader OSA population.展开更多
Inflammation of a part or whole of the temporal bone and surrounding soft tissue is termed as malignant otitis externa,which typically spreads to skull base to involve cranial nerves VII.Rarely can it also effect one ...Inflammation of a part or whole of the temporal bone and surrounding soft tissue is termed as malignant otitis externa,which typically spreads to skull base to involve cranial nerves VII.Rarely can it also effect one or more of cranial nerves IX,X,XI,and XII.We present a case of malignant otitis externa which presented with symptomatic palsy of IX and XII nerves sparing the VII cranial nerve.The patient though later on had internal jugular vein thrombosis,which we presume is due to the involvement of the parapharyngeal space that prompted us to reconsider the diagnosis,and later on,to aggravate the therapy.With proper blood sugar control and appropriate long term antibiotics,not only that the patient is disease free at one year follow up,but the cranial nerve deficits also recovered.Apart from sharing the clinical and management details of this patient,we have reviewed the relevant literature in the discussion,which has shed some light onto some of the interesting facts about this condition and its prognosis.展开更多
文摘Studies have shown that sensory nerve damage can activate the p38 mitogen-activated protein kinase(MAPK)pathway,but whether the same type of nerve injury after exercise activates the p38MAPK pathway remains unclear.Several studies have demonstrated that nerve growth factor may play a role in the repair process after peripheral nerve injury,but there has been little research focusing on the hypoglossal nerve injury and repair.In this study,we designed and established rat models of hypoglossal nerve crush injury and gave intraperitoneal injections of exogenous nerve growth factor to rats for 14 days.p38MAPK activity in the damaged neurons was increased following hypoglossal nerve crush injury;exogenous nerve growth factor inhibited this increase in acitivity and increased the survival rate of motor neurons within the hypoglossal nucleus.Under transmission electron microscopy,we found that the injection of nerve growth factor contributed to the restoration of the morphology of hypoglossal nerve after crush injury.Our experimental findings indicate that exogenous nerve growth factor can protect damaged neurons and promote hypoglossal nerve regeneration following hypoglossal nerve crush injury.
文摘Objectives:Analyze the obstructive sleep apnea (OSA) treatment history in a group of participants who enrolled in a hypoglossal nerve stimulation trial Methods:Moderate-severe OSA patients with difficulty adhering to CPAP presented for enrollment in a multicenter trial.Self-reported history on prior OSA medical therapy was collected at enrollment,including OSA diagnosis date,CPAP start and stop dates,oral appliance trial,and reasons for discontinuation or non-adherence.Results:The cohort consisted of 929 participants,83% male,with a mean age (53.9 ± 10.5) years.Ninety percent (n =835) had complete CPAP information including 47% (n =435) who discontinued therapy prior to enrollment and 43% (n =400) who were still attempting CPAP but had inadequate adherence.Abandonment rates were 60% at 1-year,73% at 3-years,and 86% at 5-years.Oral appliance therapy was attempted by 171 patients for mean (1.8 ± 2.3) years,with 81% abandonment at 1 year,89% at 3-years,and 94% at 5-years.Conclusions:In this CPAP-refractory cohort,high rates of CPAP abandonment were reported in the first several years with approximately half of the participants not receiving any treatment despite being diagnosed for >5 years.Close clinical follow-up and consideration of alternative treatment options is indicated in all OSA patients in order to ensure adequate longitudinal care.
文摘BACKGROUND Chondromyxoid fibroma(CMF) is a rare benign bone tumour of cartilaginous origin, which usually affects the metaphysis of the long bone. Involvement of the temporal bone is extremely rare. Patients with CMF in the temporal bone can present some neurological deficits due to involvement of surrounding neural structures. CASE SUMMARY We present the first case of histopathologically proven CMF originating in the temporal bone and involving the hypoglossal canal in a 40-year-old woman. Hypoglossal nerve paralysis was identified on the cranial nerve examination. The patient underwent surgical excision and was neurologically normal except for mild left facial palsy on 5-mo follow-up examination after surgery. In the current report, the major characteristics and computed tomography/magnetic resonance imaging features of the lesion are discussed. Furthermore, previous literature regarding this pathology is reviewed.CONCLUSION The current study presents the first case of temporal bone CMF involving the hypoglossal canal.
文摘Godtfredsen syndrome or clival syndrome is a rare syndrome of abducens and hypoglossal nerve palsies (cranial nerve 6th and 12th respectively) that localizes to a clival mass. There are few reported cases of this clinical presentation. The aim of this case report is to describe this rare manifestation observed in a woman with clival metastases arising from a thymoma. A previously well 34-year-old native lady presented to a district hospital in Sabah, Malaysia, with history of blurring of vision and headache for 1 month. Cranial nerve examination reveals right abducens nerve palsy (right 6th CN) and right hypoglossal nerve palsy (right 12th CN). Initial imaging with CT brain reveals a subtle extra-axial hyperdense mass adjacent to the clivus and a routine chest x-ray reveals a mediastinal mass. Further imaging of thorax showed right anterior mediastinal mass, which then proceeded with Video-Assisted Thoracoscopic Surgery (VATS) guided biopsy. The biopsy result was consistent with the finding of thymoma, type B2. She was then diagnosed with aggressive form of thymoma, which unfortunately has metastasized to the bone, lung, liver and brain. Despite prognosis at the time of diagnosis is guarded, she still opted to undergo chemotherapy. Despite the completion of 6 cycles of chemotherapy, her disease progressed, and she eventually succumbed to the illness. In short, the presence of combined 6th and 12th palsy should alert clinician to the possibility of clival mass or metastases and hence could earlier workup with appropriate imaging can lead to earlier diagnosis and better treatment outcome.
文摘A 67-year-old woman underwent right radical neck dissection for cervical lymph node metastasis from maxillary gingival carcinoma. Two months later, metastasis in the left superior internal jugular lymph nodes were discovered, and left radical neck dissection was performed. Postoperatively, airway obstruction occurred despite performing extubation after confirming that the patient had fully recovered from anesthesia. Bilateral hypoglossal nerve palsy was diagnosed and the patient was reintubated. After extubation on the following day, airway obstruction was relieved, but slurred speech and impaired swallowing were persistent. In view of this, hypoglossal nerve function should be examined before the second radical neck dissection on the contralateral side.
文摘Schwannomas originating from the hypoglossal nerve are extremely rare neoplasms. Hypoglossal schwannomas usually occur between the third and fifth decades of life, with no sex predilection, commonly presenting as a painless, slow-growing, and lateral neck mass. The best treatment is complete surgical excision with preservation of the neural pathway, if possible. In fact, since these tumors are almost always benign, a conservative surgical approach is emphasized by most authors. We report a case of a large schwannoma of hypoglossal nerve origin in the upper neck in a 31-year-old male. Magnetic resonance imaging of the area demonstrated a 43 × 39 × 36 mm well-circumscribed mass with high and nonhomogeneous signal intensity on the right side of the upper neck. The lesion was successfully treated by extirpation, with no recurrence. The extracranial hypoglossal nerve sheath was as the origin of this tumor because the patient experienced remarkable disturbance of tongue motility after surgery.
基金supported by the National Natural Science Foundation of China (31530035,81420108015, 31671099,31871072,81570081,81770083 and 31971110)the National Basic Research Development Program of China (2015CB856401)+2 种基金the Program for Shanghai Outstanding Academic Leaders (to ZLH)the Shanghai Municipal Science and Technology Major Project (2018SHZDZX01)the National Key Research and Development Program of China (2018YFC1313600)。
文摘Hypoglossal motor neurons(HMNs) innervate tongue muscles and play key roles in a variety of physiological functions,including swallowing,mastication,suckling,vocalization,and respiration.Dysfunction of HMNs is associated with several diseases,such as obstructive sleep apnea(OSA) and sudden infant death syndrome.OS A is a serious breathing disorder associated with the activity of HMNs during different sleep-wake states.Identifying the neural mechanisms by which the statedependent activities of HMNs are controlled may be helpful in providing a theoretical basis for effective therapy for OSA.However,the presynaptic partners governing the activity of HMNs remain to be elucidated.In the present study,we used a cell-type-specific retrograde tracing system based on a modified rabies virus along with a Cre/loxP gene-expression strategy to map the whole-brain monosynaptic inputs to HMNs in mice.We identified 53 nuclei targeting HMNs from six brain regions:the amygdala,hypothalamus,midbrain,pons,medulla,and cerebellum.We discovered that GAB Aergic neurons in the central amygdaloid nucleus,as well as calretinin neurons in the parasubthalamic nucleus,sent monosynaptic projections to HMNs.In addition,HMNs received direct inputs from several regions associated with respiration,such as the preBotzinger complex,parabrachial nucleus,nucleus of the solitary tract,and hypothalamus.Some regions engaged in sleep-wake regulation(the parafacial zone,parabrachial nucleus,ventral medulla,sublaterodorsal tegmental nucleus,dorsal raphe nucleus,periaqueductal gray,and hypothalamus) also provided primary inputs to HMNs.These results contribute to further elucidating the neural circuits underlying disorders caused by the dysfunction of HMNs.
文摘Objective:Current guidelines for hypoglossal nerve stimulator(HGNS)implantation eligibility include drug-induced sleep endoscopy(DISE)findings and other patient characteristics but lead to highly variable rates of surgical success across institutions.Our objective was to determine whether additional factors seen on preoperative evaluation could be used as predictors of surgical success.Study design:Retrospective chart review.Setting:Single-institution academic tertiary care medical center.Subjects:and Methods:This study included patients with obstructive sleep apnea(OSA)who underwent HGNS implantation between 2015 and 2018.Surgical success was defined as a post-operative apnea-hypopnea index(AHI)of less than 20 events per hour and an AHI reduction of at least 50%.Preoperative polysomnogram(PSG)results,DISE findings,and physical parameters were compared between surgical successes and failures.Results:A total of 68 patients were included in the analysis.The overall surgical success rate was 79.4%(54/68).Elevated preoperative AHI was associated with an increased likelihood of treatment failure,with an AHI of(36.9±16.8)events/hour in the success group compared to(49.4±19.6)events/hour in the failure group(P=0.05).Patients observed to have partial lateral oropharyngeal collapse on DISE was more frequently associated with the treatment failure group than in the success group(P=0.04).Conclusion:Patients who underwent HGNS implantation overall had a very high treatment response rate at our institution.Factors that may predispose patients to surgical failure included the presence of lateral oropharyngeal collapse and a significantly elevated preoperative AHI.These should be considered when determining surgical candidacy for HGNS implantation.
文摘Objective:To review the indications and clinical evidence supporting hypoglossal nerve stimulation (HNS) therapy for the treatment of moderate-to-severe obstructive sleep ap-nea (OSA). Methods:Peer reviewed literature on hypoglossal nerve stimulation therapy for obstructive sleep apnea from 2001 to 2016. Results:The only currently FDA-approved HNS device for the treatment of moderate-to-severe OSA is produced by Inspire Medical Systems, which recently published its 36-month outcomes data from its Stimulation Therapy for Apnea Reduction (STAR) trial. HNS therapy is currently indicated for moderate-to-severe OSA patients who are CPAP-intolerant, have a body mass in-dex <32, apnea-hypopnea index <50, and without a concentric pattern of upper airway collapse on sleep endoscopy. Conclusions:Data from the STAR trial suggests that a subset of OSA patients can achieve a sig-nificant therapeutic response from hypoglossal nerve stimulation. However, these results may be limited in their generalizability to the broader OSA population.
文摘Inflammation of a part or whole of the temporal bone and surrounding soft tissue is termed as malignant otitis externa,which typically spreads to skull base to involve cranial nerves VII.Rarely can it also effect one or more of cranial nerves IX,X,XI,and XII.We present a case of malignant otitis externa which presented with symptomatic palsy of IX and XII nerves sparing the VII cranial nerve.The patient though later on had internal jugular vein thrombosis,which we presume is due to the involvement of the parapharyngeal space that prompted us to reconsider the diagnosis,and later on,to aggravate the therapy.With proper blood sugar control and appropriate long term antibiotics,not only that the patient is disease free at one year follow up,but the cranial nerve deficits also recovered.Apart from sharing the clinical and management details of this patient,we have reviewed the relevant literature in the discussion,which has shed some light onto some of the interesting facts about this condition and its prognosis.