Rasmussen's encephalitis(RE) is a rare and severe progressive epileptic syndrome with unknown etiology. Infection by viruses, including human cytomegalovirus(HCMV), has been speculated to be a potential trigger fo...Rasmussen's encephalitis(RE) is a rare and severe progressive epileptic syndrome with unknown etiology. Infection by viruses, including human cytomegalovirus(HCMV), has been speculated to be a potential trigger for RE. However, no viral antigens have been detected in the brains of patients with RE; thus, a possible clinical linkage between viral infections and RE has not been firmly established. In this study, we evaluated the expression of HCMV pp65 antigen in brain sections from 26 patients with RE and 20 non-RE patients by immunohistochemistry and in situ hybridization, and assessed the associations between HCMV infection and clinical parameters.Elevated expression of HCMV pp65 protein and DNA was observed in 88.5%(23/26) and 69.2%(18/26) of RE cases, respectively. In the non-RE group, HCMV pp65 antigen was detected only in two cases(10%), both of which were negative for DNA staining. Additionally, the intensity of HCMV pp65 staining was correlated with a shorter duration of the prodromal stage, younger age of seizure onset, and more severe unilateral cortical atrophy. Elevated expression of HCMV pp65 was observed in RE brain tissue and was correlated with the clinical features of RE disease. In summary, our results suggested that HCMV infection may be involved in the occurrence and progression of RE disease. Thus, further studies are needed to determine whether early treatment with anti-HCMV antibodies could modulate the course of RE.展开更多
The role of the ipsilaterally descending motor pathways in the recovery mechanisms after unilateral hemispheric damage is still poorly understood. Motor output reorganization was investigated in a 56-year-old male pat...The role of the ipsilaterally descending motor pathways in the recovery mechanisms after unilateral hemispheric damage is still poorly understood. Motor output reorganization was investigated in a 56-year-old male patient with acquired unilateral hemispheric atrophy due to Rasmussen encephalitis. In particular,the ipsilateral corticospinal pathways were explored using focal transcranial magnetic stimulation. In the first dorsal interosseous and wrist extensors muscles, the median amplitudes of the ipsilateral motor evoked potentials induced by transcranial magnetic stimulation in the patient were higher than those of 10 age-matched healthy control subjects. In the biceps brachii muscle, the median amplitudes of the ipsilateral motor evoked potentials were the second largest in the patient compared to the controls. This study demonstrated a reinforcement of ipsilateral motor projections from the unaffected motor cortex to the hemiparetic hand in a subject with acquired unihemispheric cortical damage.展开更多
Objective： There is no report on Bickerstaff s brainstem encephalitis （BBE） patients in China. We here report the first case of BBE in China. Methods： Clinical features, results of electromyography, eleetroencepha...Objective： There is no report on Bickerstaff s brainstem encephalitis （BBE） patients in China. We here report the first case of BBE in China. Methods： Clinical features, results of electromyography, eleetroencephalography （EEG）, magnetic resonance imaging （MRI） and cerebrospinal fluid （CSF） examination were studied to clarify the characteristics of this syndrome. Results： A 44-year-old man presented himself at our inpatient department with somnolence and dizziness as his initial symptoms. He developed multiple cranial nerves paralysis especially internal and external ophthalmoplegia, ataxia and tetraparesis within 1 week. His condition rapidly deteriorated, and he experienced coma. Electromyography showed indications of peripheral nerve dysfunction, electroencephalography revealed loss of basic rhythm, MR1 demonstrated high-intensity abnormalities on T2-weighted images of medulla oblongata, and CSF albuminocytological dissociation was defined abnormally as high protein. Ten months later, he almost completely recovered. Conclusion： BBE, fisher syndrome （FS） and Guillain-Barre syndrome （GBS） are similar clinically; BBE and FS were proposed to be the variant of GBS.展开更多
Cat scratch's disease caused by Bartonella henselae, is known to be a self-limited benign process in immunocompetent children. The association with neurologic manifestations is very uncommon especially in patient ...Cat scratch's disease caused by Bartonella henselae, is known to be a self-limited benign process in immunocompetent children. The association with neurologic manifestations is very uncommon especially in patient with no immunologic defects and in cases without specific treatment. A 7 years old male patient, without any immunocromised defect, presented an atypic presentation of the cat scratch disease. The patient came to the hospital in two opportunities in a status epilepticus, in both cases the diagnosis was encephalitis by Bartonella henselae and the evolution with treatment was monitored with PCR(polymerase chain reaction) in cerebrospinal fluid and blood, as well as IFI(IgM, IgG) serology(indirect immunofluorescence). The patient had a favorable clinical and laboratory evolution for 6 months showing no recurrence of the disease.展开更多
BACKGROUND Autoimmune encephalitis(AE)is a rare and recently described neuroinflammatory disease associated with specific autoantibodies.Anti-leucine-rich glioma inactivated 1(anti-LGI1)encephalitis is a rare but trea...BACKGROUND Autoimmune encephalitis(AE)is a rare and recently described neuroinflammatory disease associated with specific autoantibodies.Anti-leucine-rich glioma inactivated 1(anti-LGI1)encephalitis is a rare but treatable type of AE discovered in recent years.Alzheimer’s disease(AD)is a degenerative brain disease and the most common cause of dementia.AD may undergo a series of pathological physiological changes in brain tissue 20 years before the onset of typical symptoms.The stage of mild cognitive impairment(MCI)that occurs during this process,known as MCI due to AD,is the earliest stage with clinical symptoms.MCI is typically categorized into two subtypes:Amnestic MCI(aMCI)and non-aMCI.CASE SUMMARY This report describes a patient with rapid cognitive impairment,diagnosed with anti-LGI1 antibody-mediated AE and aMCI,and treated at Peking University Shenzhen Hospital in March 2023.The patient was hospitalized with acute memory decline for more than 3 months.Both the cerebrospinal fluid and serum were positive for anti-LGI1 antibodies,biomarkers of AD coexisting in the patient’s cerebrospinal fluid.Following combination treatment with immunoglobulin therapy and glucocorticoid,plus inhibition of acetylcholinesterase,the patient’s cognitive function significantly improved.Throughout the 3-month follow-up period,a sustained improvement in cognitive function was observed.The results of serum anti-LGI1 antibody were negative.CONCLUSION This case has raised awareness of the possible interaction between AE and early AD(including MCI due to AD),and alerted clinicians to the possibility of concurrent rare and common diseases in patients presenting with cognitive impairment.展开更多
基金supported by the following funds: the National Natural Science Foundation of China (81571275)the Beijing Municipal Natural Science Foundation (7144217)+5 种基金the Capital Applied Clinic Research Programs of Science and Technology (Z131107002213171)the Beijing Rising-star Plan of Science and Technology (Z141107001814042)the Open Research Fund of the Beijing Key Laboratory of Epilepsy Research (No. 2014DXBL02)Capital Medical University (15JL08)Scientific Research Common Program of Beijing Municipal Commission of Education (KM201610025001)Scientific Research Foundation for the Returned Overseas Chinese Scholars, State Education Ministry (2014 1685)
文摘Rasmussen's encephalitis(RE) is a rare and severe progressive epileptic syndrome with unknown etiology. Infection by viruses, including human cytomegalovirus(HCMV), has been speculated to be a potential trigger for RE. However, no viral antigens have been detected in the brains of patients with RE; thus, a possible clinical linkage between viral infections and RE has not been firmly established. In this study, we evaluated the expression of HCMV pp65 antigen in brain sections from 26 patients with RE and 20 non-RE patients by immunohistochemistry and in situ hybridization, and assessed the associations between HCMV infection and clinical parameters.Elevated expression of HCMV pp65 protein and DNA was observed in 88.5%(23/26) and 69.2%(18/26) of RE cases, respectively. In the non-RE group, HCMV pp65 antigen was detected only in two cases(10%), both of which were negative for DNA staining. Additionally, the intensity of HCMV pp65 staining was correlated with a shorter duration of the prodromal stage, younger age of seizure onset, and more severe unilateral cortical atrophy. Elevated expression of HCMV pp65 was observed in RE brain tissue and was correlated with the clinical features of RE disease. In summary, our results suggested that HCMV infection may be involved in the occurrence and progression of RE disease. Thus, further studies are needed to determine whether early treatment with anti-HCMV antibodies could modulate the course of RE.
文摘The role of the ipsilaterally descending motor pathways in the recovery mechanisms after unilateral hemispheric damage is still poorly understood. Motor output reorganization was investigated in a 56-year-old male patient with acquired unilateral hemispheric atrophy due to Rasmussen encephalitis. In particular,the ipsilateral corticospinal pathways were explored using focal transcranial magnetic stimulation. In the first dorsal interosseous and wrist extensors muscles, the median amplitudes of the ipsilateral motor evoked potentials induced by transcranial magnetic stimulation in the patient were higher than those of 10 age-matched healthy control subjects. In the biceps brachii muscle, the median amplitudes of the ipsilateral motor evoked potentials were the second largest in the patient compared to the controls. This study demonstrated a reinforcement of ipsilateral motor projections from the unaffected motor cortex to the hemiparetic hand in a subject with acquired unihemispheric cortical damage.
文摘Objective： There is no report on Bickerstaff s brainstem encephalitis （BBE） patients in China. We here report the first case of BBE in China. Methods： Clinical features, results of electromyography, eleetroencephalography （EEG）, magnetic resonance imaging （MRI） and cerebrospinal fluid （CSF） examination were studied to clarify the characteristics of this syndrome. Results： A 44-year-old man presented himself at our inpatient department with somnolence and dizziness as his initial symptoms. He developed multiple cranial nerves paralysis especially internal and external ophthalmoplegia, ataxia and tetraparesis within 1 week. His condition rapidly deteriorated, and he experienced coma. Electromyography showed indications of peripheral nerve dysfunction, electroencephalography revealed loss of basic rhythm, MR1 demonstrated high-intensity abnormalities on T2-weighted images of medulla oblongata, and CSF albuminocytological dissociation was defined abnormally as high protein. Ten months later, he almost completely recovered. Conclusion： BBE, fisher syndrome （FS） and Guillain-Barre syndrome （GBS） are similar clinically; BBE and FS were proposed to be the variant of GBS.
基金supported by the Programa Nacional de Innovación para la Competitividad y Productividad(Innóvate Perú)under the contract 116-PNICP-PIAP-2015
文摘Cat scratch's disease caused by Bartonella henselae, is known to be a self-limited benign process in immunocompetent children. The association with neurologic manifestations is very uncommon especially in patient with no immunologic defects and in cases without specific treatment. A 7 years old male patient, without any immunocromised defect, presented an atypic presentation of the cat scratch disease. The patient came to the hospital in two opportunities in a status epilepticus, in both cases the diagnosis was encephalitis by Bartonella henselae and the evolution with treatment was monitored with PCR(polymerase chain reaction) in cerebrospinal fluid and blood, as well as IFI(IgM, IgG) serology(indirect immunofluorescence). The patient had a favorable clinical and laboratory evolution for 6 months showing no recurrence of the disease.
基金Supported by the Shenzhen Science and Technology Program,No.JCYJ20220818102810022Shenzhen University Teaching Reform fund,No.JG2023166+1 种基金National Natural Science Foundation of China,No.12231018Science and Technology Innovation 2030-Major Project,No.2021ZD0201805.
文摘BACKGROUND Autoimmune encephalitis(AE)is a rare and recently described neuroinflammatory disease associated with specific autoantibodies.Anti-leucine-rich glioma inactivated 1(anti-LGI1)encephalitis is a rare but treatable type of AE discovered in recent years.Alzheimer’s disease(AD)is a degenerative brain disease and the most common cause of dementia.AD may undergo a series of pathological physiological changes in brain tissue 20 years before the onset of typical symptoms.The stage of mild cognitive impairment(MCI)that occurs during this process,known as MCI due to AD,is the earliest stage with clinical symptoms.MCI is typically categorized into two subtypes:Amnestic MCI(aMCI)and non-aMCI.CASE SUMMARY This report describes a patient with rapid cognitive impairment,diagnosed with anti-LGI1 antibody-mediated AE and aMCI,and treated at Peking University Shenzhen Hospital in March 2023.The patient was hospitalized with acute memory decline for more than 3 months.Both the cerebrospinal fluid and serum were positive for anti-LGI1 antibodies,biomarkers of AD coexisting in the patient’s cerebrospinal fluid.Following combination treatment with immunoglobulin therapy and glucocorticoid,plus inhibition of acetylcholinesterase,the patient’s cognitive function significantly improved.Throughout the 3-month follow-up period,a sustained improvement in cognitive function was observed.The results of serum anti-LGI1 antibody were negative.CONCLUSION This case has raised awareness of the possible interaction between AE and early AD(including MCI due to AD),and alerted clinicians to the possibility of concurrent rare and common diseases in patients presenting with cognitive impairment.
