Guillain-Barré syndrome is a rare but fatal autoimmune disease of unknown origin. Infectious disease is the most common etiology of Guillain-Barré syndrome. We had a 75-year-old female patient with Guillain-...Guillain-Barré syndrome is a rare but fatal autoimmune disease of unknown origin. Infectious disease is the most common etiology of Guillain-Barré syndrome. We had a 75-year-old female patient with Guillain-Barré syndrome and a 90-year-old male patient with facial nerve palsy admitted to our hospital. Both patients experienced recovery from early Guillain-Barré syndrome and peripheral facial nerve palsy after receiving intravenous infusion of NaCl with KCl solution and taking vitamin C.展开更多
BACKGROUND Implant vagus nerve stimulation is an adjunctive treatment for intractable epilepsy when patients are not suitable for resective surgery.AIM To identify the safety and efficacy of vagus nerve stimulation in...BACKGROUND Implant vagus nerve stimulation is an adjunctive treatment for intractable epilepsy when patients are not suitable for resective surgery.AIM To identify the safety and efficacy of vagus nerve stimulation in children with intractable epilepsy and analyze the effects on different epilepsy syndromes.METHODS Eligible children with intractable epilepsy were admitted to the study.We collected data from preoperative assessments as the baseline.During the followup time,we recorded the process of seizures(frequency,duration,and seizure type),the changes of drugs or parameters,the complications,etc.The mean reduction rate of seizures,response rate,and McHugh scale were chosen as the outcomes.RESULTS A total of 213 patients were implanted with Tsinghua Pins vagus nerve stimulators,and the average age was 6.6 years.In the follow-up time of postoperative 3 mo,6 mo,12 mo,18 mo,and 24 mo,the average reduction rate was 30.2%,49.5%,56.3%,59.4%,and 63.2%,while the response rate was 21.8%,62.5%,57.1%,69.2%,and 70.7%.In addition,implanted vagus nerve stimulation had different effects on epilepsy syndromes.The reduction rate of West syndrome increased from 36.4%(postoperative 6 m)to 74.3%(postoperative 24 m).The reduction rate of Lennox-Gastaut syndrome improved from 25.4%to 73.1%in 24 mo.The chi-square test of the five efficacy grades showed P<0.05.The comparison between the 3-mo follow-up and the 6-mo follow-up showed P<0.05,and the comparison between the 6-mo follow-up and the 24-mo follow-up showed P>0.05.CONCLUSION Vagus nerve stimulation is safe and effective in children with intractable epilepsy,and the seizure reduction occurred in a time-dependent manner.Moreover,patients with West syndrome may get the most benefits.展开更多
Hoarseness of voice is a common symptom of many laryngeal and extralaryngeal conditions. It is very crucial to identify the culprit behind it, keeping in mind all the various differentials. We re-port a case of a 35-y...Hoarseness of voice is a common symptom of many laryngeal and extralaryngeal conditions. It is very crucial to identify the culprit behind it, keeping in mind all the various differentials. We re-port a case of a 35-year-old woman presenting with complaint of hoarseness of voice. Upon car-diac evaluation, the patient was diagnosed with bicuspid aortic valve, with severe aortic stenosis and dilation of ascending aorta. We suspected that the plausible reason for hoarseness of voice was compression of recurrent laryngeal nerve due to dilation of aorta, i.e., Ortner’s syndrome. But to our surprise, CT of aorta and neck revealed extensive elongated cervical vagus nerve schwannoma. Such type of association is unique and new to voluminous medical literature.展开更多
We present a case of an elderly man, who initially presented with right facial nerve palsy, ipsilateral headache, elevated erythrocyte sedimentation rate(ESR) and no fever. A presumptive diagnosis of giant cell arteri...We present a case of an elderly man, who initially presented with right facial nerve palsy, ipsilateral headache, elevated erythrocyte sedimentation rate(ESR) and no fever. A presumptive diagnosis of giant cell arteritis was made and the patient was treated with highdose steroids. A temporal artery biopsy was negative. Several months later, while on 16 mg of methylprednisolone daily, he presented with severe sensorimotor peripheral symmetric neuropathy, muscle wasting and inability to walk, uncontrolled blood sugar and psychosis. A work-up for malignancy was initiated with the suspicion of a paraneoplastic process. At the same time a biopsy of the macular skin lesions that had appeared on the skin of the left elbow and right knee almost simultaneously was inconclusive, whereas a repeat biopsy from the same area of the lesions that had become nodular, a month later, was indicative of Kaposi'ssarcoma. Finally, a third biopsy of a similar lesion, after spreading of the skin process, confirmed the diagnosis of Kaposi's sarcoma. He was treated with interferon α and later was seen in very satisfactory condition, with no clinical evidence of neuropathy, normal muscle strength, no headache, normal electrophysiologic nerve studies, involution of Kaposi's lesions and a normal ESR.展开更多
Background Lennox-gastaut syndrome(LGS)is an epileptic encephalopathy often associated with behavioral and psychiatric disorders.Vagus nerve stimulation(VNS)has been approved effective for LGS treatment.Surgical resec...Background Lennox-gastaut syndrome(LGS)is an epileptic encephalopathy often associated with behavioral and psychiatric disorders.Vagus nerve stimulation(VNS)has been approved effective for LGS treatment.Surgical resection is also an option for LGS patients with focal pathology,offering a high probability of seizure control.However,it is challenging to accurately localize the seizure focus.Case presentation The case presented here is a 19-year-old male with a 16-year history of epilepsy with comorbid severe cognitive and psychiatric disorders.He was diagnosed with LGS due to generalized slow spike-wave discharges and multiple seizure types.He was treated with VNS in 2017 at the age of 15.After that,the frequency of the short tonic seizures decreased from 4–5 times per day to 2–5 times per year,and the generalized tonic–clonic seizure pattern did not recur,which had a frequency of 2–4 times per month before the surgery.In 2019,the generalized abnormal interictal epileptiform discharges changed to be localized in the right frontal–temporal lobe at the age of 17 years(2019).Conclusions This case report suggested that the generalized epileptiform discharges evolve into localized discharges after VNS treatment,which may help reveal the primary seizure focus for resection surgery in patients with LGS.展开更多
Melkersson-Rosenthal syndrome(MRS) is a rare, noncaseating granulomatous disorder of unknown etiology and undefined diagnostic criteria. The classical triad of recurrent orofacial edema, relapsing facial paralysis, an...Melkersson-Rosenthal syndrome(MRS) is a rare, noncaseating granulomatous disorder of unknown etiology and undefined diagnostic criteria. The classical triad of recurrent orofacial edema, relapsing facial paralysis, and fissured tongue, is not frequently seen in its complete form, and many patients remain misdiagnosed or undiagnosed for years. The purpose of this study is to review the findings in the literature describing theMelkersson-Rosenthal syndrome with aim to identify its clinical and histopathological characteristics and correlate them with definitive diagnostic criteria and effective treatment modalities. A systematic review and analysis of more than 100 publications met eligibility criteria performed by the authors. Orofacial edema of unknown etiology is the most typical clinical feature of the Melkersson-Rosenthal syndrome. Its coexistence with of facial nerve palsy or fissured tongue could be characterized as an oligosymptomatic MRS. Many investigators suggest cheilitis granulomatosa as a monosymptomatic form of MRS, while patients with facial palsy and fissured tongue, without orofacial edema, should not be considered having MRS.Histological evidence is not necessary. Corticosteroids are generally accepted as the mainstay treatment.展开更多
Cerebellopontine angle (CPA) lesions account for up to 10% of all intracranial tumors. Most CPA tumors are benign, but can cause nerve damage or compress the surrounding structures if left untreated. The typical prese...Cerebellopontine angle (CPA) lesions account for up to 10% of all intracranial tumors. Most CPA tumors are benign, but can cause nerve damage or compress the surrounding structures if left untreated. The typical presentation is with adult-onset sensorineural hearing loss or non-pulsatile tinnitus. In some patients, this goes unnoticed, and presentation is delayed until the lesion is much larger and presents with symptoms related to mass effect. We present the case study of 63 years old gentleman, who had suspected left CPA lesion on CT head done few years ago for dizziness and left-sided facial numbness. MRI could not be done at that time due to his MRI incompatible pacemaker leading to delay in his management eventually causing loss of patient to the follow up. He later developed progressive difficulty in walking which was initially attributed to as secondary to vasovagal syncope and postural hypotension. He eventually presented to us with intractable nausea and vomiting, worsening headache and ataxia. He had an urgent CT head which showed significant growth in the lesion with compression of the surrounding structures and obstructive hydrocephalus. He was given steroids which improved his nausea and vomiting, followed by undergoing surgery in regional center leading to significant improvement in his gait within few days of surgery. He unfortunately continued to have a degree of ataxia and facial numbness. This case illustrates a rare presentation of CPA tumor with symptoms of nausea and vomiting as a result of mass effect of the growing tumor. In addition, this review also shows the importance of regularly following up the patients with suspected CPA lesions on initial scans which will help with identifying the increase in size of lesion promptly and potentially preventing advanced complications of CPA tumors. We suggest regular monitoring of these patients to timely manage the lesion and avoid the potential life-threatening complications.展开更多
文摘Guillain-Barré syndrome is a rare but fatal autoimmune disease of unknown origin. Infectious disease is the most common etiology of Guillain-Barré syndrome. We had a 75-year-old female patient with Guillain-Barré syndrome and a 90-year-old male patient with facial nerve palsy admitted to our hospital. Both patients experienced recovery from early Guillain-Barré syndrome and peripheral facial nerve palsy after receiving intravenous infusion of NaCl with KCl solution and taking vitamin C.
文摘BACKGROUND Implant vagus nerve stimulation is an adjunctive treatment for intractable epilepsy when patients are not suitable for resective surgery.AIM To identify the safety and efficacy of vagus nerve stimulation in children with intractable epilepsy and analyze the effects on different epilepsy syndromes.METHODS Eligible children with intractable epilepsy were admitted to the study.We collected data from preoperative assessments as the baseline.During the followup time,we recorded the process of seizures(frequency,duration,and seizure type),the changes of drugs or parameters,the complications,etc.The mean reduction rate of seizures,response rate,and McHugh scale were chosen as the outcomes.RESULTS A total of 213 patients were implanted with Tsinghua Pins vagus nerve stimulators,and the average age was 6.6 years.In the follow-up time of postoperative 3 mo,6 mo,12 mo,18 mo,and 24 mo,the average reduction rate was 30.2%,49.5%,56.3%,59.4%,and 63.2%,while the response rate was 21.8%,62.5%,57.1%,69.2%,and 70.7%.In addition,implanted vagus nerve stimulation had different effects on epilepsy syndromes.The reduction rate of West syndrome increased from 36.4%(postoperative 6 m)to 74.3%(postoperative 24 m).The reduction rate of Lennox-Gastaut syndrome improved from 25.4%to 73.1%in 24 mo.The chi-square test of the five efficacy grades showed P<0.05.The comparison between the 3-mo follow-up and the 6-mo follow-up showed P<0.05,and the comparison between the 6-mo follow-up and the 24-mo follow-up showed P>0.05.CONCLUSION Vagus nerve stimulation is safe and effective in children with intractable epilepsy,and the seizure reduction occurred in a time-dependent manner.Moreover,patients with West syndrome may get the most benefits.
文摘Hoarseness of voice is a common symptom of many laryngeal and extralaryngeal conditions. It is very crucial to identify the culprit behind it, keeping in mind all the various differentials. We re-port a case of a 35-year-old woman presenting with complaint of hoarseness of voice. Upon car-diac evaluation, the patient was diagnosed with bicuspid aortic valve, with severe aortic stenosis and dilation of ascending aorta. We suspected that the plausible reason for hoarseness of voice was compression of recurrent laryngeal nerve due to dilation of aorta, i.e., Ortner’s syndrome. But to our surprise, CT of aorta and neck revealed extensive elongated cervical vagus nerve schwannoma. Such type of association is unique and new to voluminous medical literature.
文摘We present a case of an elderly man, who initially presented with right facial nerve palsy, ipsilateral headache, elevated erythrocyte sedimentation rate(ESR) and no fever. A presumptive diagnosis of giant cell arteritis was made and the patient was treated with highdose steroids. A temporal artery biopsy was negative. Several months later, while on 16 mg of methylprednisolone daily, he presented with severe sensorimotor peripheral symmetric neuropathy, muscle wasting and inability to walk, uncontrolled blood sugar and psychosis. A work-up for malignancy was initiated with the suspicion of a paraneoplastic process. At the same time a biopsy of the macular skin lesions that had appeared on the skin of the left elbow and right knee almost simultaneously was inconclusive, whereas a repeat biopsy from the same area of the lesions that had become nodular, a month later, was indicative of Kaposi'ssarcoma. Finally, a third biopsy of a similar lesion, after spreading of the skin process, confirmed the diagnosis of Kaposi's sarcoma. He was treated with interferon α and later was seen in very satisfactory condition, with no clinical evidence of neuropathy, normal muscle strength, no headache, normal electrophysiologic nerve studies, involution of Kaposi's lesions and a normal ESR.
基金Natural Science Foundation of Yunnan Province(202105AC160029,KYRLMY20200026 and YNGXCXTD2018012)Major Science and Technology Special Project of Yunnan Province(202002AA100204).
文摘Background Lennox-gastaut syndrome(LGS)is an epileptic encephalopathy often associated with behavioral and psychiatric disorders.Vagus nerve stimulation(VNS)has been approved effective for LGS treatment.Surgical resection is also an option for LGS patients with focal pathology,offering a high probability of seizure control.However,it is challenging to accurately localize the seizure focus.Case presentation The case presented here is a 19-year-old male with a 16-year history of epilepsy with comorbid severe cognitive and psychiatric disorders.He was diagnosed with LGS due to generalized slow spike-wave discharges and multiple seizure types.He was treated with VNS in 2017 at the age of 15.After that,the frequency of the short tonic seizures decreased from 4–5 times per day to 2–5 times per year,and the generalized tonic–clonic seizure pattern did not recur,which had a frequency of 2–4 times per month before the surgery.In 2019,the generalized abnormal interictal epileptiform discharges changed to be localized in the right frontal–temporal lobe at the age of 17 years(2019).Conclusions This case report suggested that the generalized epileptiform discharges evolve into localized discharges after VNS treatment,which may help reveal the primary seizure focus for resection surgery in patients with LGS.
文摘Melkersson-Rosenthal syndrome(MRS) is a rare, noncaseating granulomatous disorder of unknown etiology and undefined diagnostic criteria. The classical triad of recurrent orofacial edema, relapsing facial paralysis, and fissured tongue, is not frequently seen in its complete form, and many patients remain misdiagnosed or undiagnosed for years. The purpose of this study is to review the findings in the literature describing theMelkersson-Rosenthal syndrome with aim to identify its clinical and histopathological characteristics and correlate them with definitive diagnostic criteria and effective treatment modalities. A systematic review and analysis of more than 100 publications met eligibility criteria performed by the authors. Orofacial edema of unknown etiology is the most typical clinical feature of the Melkersson-Rosenthal syndrome. Its coexistence with of facial nerve palsy or fissured tongue could be characterized as an oligosymptomatic MRS. Many investigators suggest cheilitis granulomatosa as a monosymptomatic form of MRS, while patients with facial palsy and fissured tongue, without orofacial edema, should not be considered having MRS.Histological evidence is not necessary. Corticosteroids are generally accepted as the mainstay treatment.
文摘Cerebellopontine angle (CPA) lesions account for up to 10% of all intracranial tumors. Most CPA tumors are benign, but can cause nerve damage or compress the surrounding structures if left untreated. The typical presentation is with adult-onset sensorineural hearing loss or non-pulsatile tinnitus. In some patients, this goes unnoticed, and presentation is delayed until the lesion is much larger and presents with symptoms related to mass effect. We present the case study of 63 years old gentleman, who had suspected left CPA lesion on CT head done few years ago for dizziness and left-sided facial numbness. MRI could not be done at that time due to his MRI incompatible pacemaker leading to delay in his management eventually causing loss of patient to the follow up. He later developed progressive difficulty in walking which was initially attributed to as secondary to vasovagal syncope and postural hypotension. He eventually presented to us with intractable nausea and vomiting, worsening headache and ataxia. He had an urgent CT head which showed significant growth in the lesion with compression of the surrounding structures and obstructive hydrocephalus. He was given steroids which improved his nausea and vomiting, followed by undergoing surgery in regional center leading to significant improvement in his gait within few days of surgery. He unfortunately continued to have a degree of ataxia and facial numbness. This case illustrates a rare presentation of CPA tumor with symptoms of nausea and vomiting as a result of mass effect of the growing tumor. In addition, this review also shows the importance of regularly following up the patients with suspected CPA lesions on initial scans which will help with identifying the increase in size of lesion promptly and potentially preventing advanced complications of CPA tumors. We suggest regular monitoring of these patients to timely manage the lesion and avoid the potential life-threatening complications.
