Chorea-acanthocytosis(ChAc)is the most common subtype of neuroacanthocytosis syndrome,characterized by the presence of acanthocytes and neurological disorders.It is thought to be caused by VPS13A mutations.Characteris...Chorea-acanthocytosis(ChAc)is the most common subtype of neuroacanthocytosis syndrome,characterized by the presence of acanthocytes and neurological disorders.It is thought to be caused by VPS13A mutations.Characteristic movement disorders in ChAc is choreiform movements affecting both trunk and extremities and prominent orolingual dyskinesia is pathognomonic.Acanthocytosis in peripheral blood smear,elevated serum creatine kinase and atrophy of heads of caudate nuclei and dilation of the anterior horn of the lateral ventricles in magnetic resonance imaging could assist the diagnosis of ChAc.Botulinum toxin injection is a possible treatment for the typical orofacial dystonia.Deep brain stimulation is a novel surgical treatment modality.Most cases chose globus pallidus internus as target.Patients with dystonia as a major manifestation will benefit more from high-frequency stimulation and those with major findings of chorea and dysarthria are suitable for low-frequency stimulation.More evidence of long-term outcomes is warranted.展开更多
BACKGROUND Pediatric-onset systemic lupus erythematosus(SLE)is typically more severe than adult-onset SLE,with a higher incidence of nervous system involvement.Chorea is a relatively rare neurological complication rep...BACKGROUND Pediatric-onset systemic lupus erythematosus(SLE)is typically more severe than adult-onset SLE,with a higher incidence of nervous system involvement.Chorea is a relatively rare neurological complication reported in 2.4%-7%of SLE patients.In particular,chorea induced by glucocorticoid dose reduction is even rarer.Herein,we report the case of a girl with SLE,who developed chorea during the process of glucocorticoid therapy reduction.CASE SUMMARY We describe a 14-year-old girl who was diagnosed with SLE.She was treated with methylprednisolone and rituximab,and her symptoms improved.On the second day after the methylprednisolone dose was reduced according to the treatment guidelines,the patient developed chorea.Her condition improved after adjusting her glucocorticoid regimen.CONCLUSION This case is a reminder that extra attention to chorea is required in SLE patients during glucocorticoid dose reduction.展开更多
Introduction: Post-streptococcal chorea is the most common form of acquired chorea in children. Objective: The objective of this study was to contribute to better management of this condition by giving the characteris...Introduction: Post-streptococcal chorea is the most common form of acquired chorea in children. Objective: The objective of this study was to contribute to better management of this condition by giving the characteristics of the epidemiological, clinical, biological, etiological, therapeutic and evolutionary aspects. Methodology: This was a retrospective and descriptive study from January 2021 to December 2021 which took place in the pediatrics department of the Mother and Child University Teaching Hospital of N’Djamena (CHU-ME). All the children in whom we observed abnormal involuntary movements whose age was less than or equal to 15 years were concerned. Result: there were three female children. The average age was 7.5, of which the two are 7 years old and the third is 8 years old. Two had a history of angina. A notion of polyarthralgia and dental caries was found in the third. The diagnosis of post-streptococcal chorea was retained on the basis of clinical arguments: observation of abnormal movements of the limbs and the face, which are involuntary, sudden with an insidious and progressive onset, muscular hypotonia, and psychic disorder. Medical imaging, in particular echocardiography, which objectified two cases of associated rheumatic valvular disease. Remission was observed in all three children after two weeks of treatment with haloperidol, penicillin. Those with associated rheumatic valve disease also received corticosteroid therapy. Complete remission of chorea was observed in all three girls. Prophylaxis in two patients based on Penicillin V was instituted. Conclusion: Although less frequent, post-streptococcal chorea is still seen in our country and is still an important health problem that needs more real medical efforts.展开更多
Introduction: Chronic exposure to organic solvents may result in a variety of neurologic complications like dementia, cerebellar dysfunction, pyramidal syndrome, cranial nerve abnormalities, and neuropathy. Clinical P...Introduction: Chronic exposure to organic solvents may result in a variety of neurologic complications like dementia, cerebellar dysfunction, pyramidal syndrome, cranial nerve abnormalities, and neuropathy. Clinical Presentation: We report an unusual case of subacute chorea induced by occupational exposure. Brain magnetic resonance imaging showed diffuse white matter T2 hyperintensity. The screening of basic blood tests, and CSF studies to eliminate alternate diagnoses, were normal. The patient received 1000 mg/day of intravenous methylprednisolone for 3 days, with cessation of professional activity. We observed a regression of neurological symptoms after 3 months of follow-up. Conclusion: This case highlights the diversity of acute or chronic neurological complications of solvents.展开更多
Hemichorea associated with non-ketotic hyperglycemia(HC-NH)is a rare disease.The purpose of this case report is to introduce a patient with HC-NH and provide a schedule of examination and follow-up treatment.We also r...Hemichorea associated with non-ketotic hyperglycemia(HC-NH)is a rare disease.The purpose of this case report is to introduce a patient with HC-NH and provide a schedule of examination and follow-up treatment.We also reviewed the current understanding of pathophysiology and treatment and how to apply it to our patients.The case involved a 37-year-old Asian diabetic man who had a 9-day history of losing movement in left limbs and face.His initial blood glucose level was 10.13 mmol/L.HbA1c was 13.6%.Before admission,head CT scan showed suspicious small pieces of left brainstem with slightly high-density shadow and right putamen nucleus with high-density shadow.On the day of admission,head MRI showed punctate T1WI low signal shadow,T2WI high and low mixed signal shadow,T1WI high signal shadow and T2WI low signal shadow of right putamen nucleus.The case findings were consistent with his displayed motor pattern and with the HC-NH diagnosis.Gradual control of the blood glucose levels alleviates his choreiform symptoms.The endocrinology follow-up 6 months after discharge found that his symptoms did not recur after the outpatient’s medication compliance was improved.HC-NH is a rare manifestation of poor diabetes control,but it should also be noticed by clinicians.Early recognition and gradual treatment of elevated blood glucose levels seem to completely alleviate choreiform symptoms.展开更多
We report a rare case about chorea in a Chinese adult after pulmonary thromboendarterectomy （PTE） with deep hypothermia and circulatory arrest.A 41-year-old male was admitted to our hospital because of breathing dif...We report a rare case about chorea in a Chinese adult after pulmonary thromboendarterectomy （PTE） with deep hypothermia and circulatory arrest.A 41-year-old male was admitted to our hospital because of breathing difficulties for about three years and intermittent hemoptysis for about two months.Physical examination revealed mild cyanotic lips,lower extremity edema.He was classified as grade III heart failure according the classification system of the New York Heart Association （NYHA）.展开更多
In the treatment of 30 cases of minor chorea by puncturing Baihui (GV 20), Sishencong (Ex-HN 1) and Fengchi (GB 20), in combination of oral administration of herbal medicine, the total effective rate is 96.7%. Key Wor...In the treatment of 30 cases of minor chorea by puncturing Baihui (GV 20), Sishencong (Ex-HN 1) and Fengchi (GB 20), in combination of oral administration of herbal medicine, the total effective rate is 96.7%. Key Words Acupuncture Therapy - Chorea - Drug Therapy Author: ZHANG Shu-jun (1961-), female, lecturerTranslator: HUANG Guo-qiAn erratum to this article is available at http://dx.doi.org/10.1007/BF02850966.展开更多
文摘Chorea-acanthocytosis(ChAc)is the most common subtype of neuroacanthocytosis syndrome,characterized by the presence of acanthocytes and neurological disorders.It is thought to be caused by VPS13A mutations.Characteristic movement disorders in ChAc is choreiform movements affecting both trunk and extremities and prominent orolingual dyskinesia is pathognomonic.Acanthocytosis in peripheral blood smear,elevated serum creatine kinase and atrophy of heads of caudate nuclei and dilation of the anterior horn of the lateral ventricles in magnetic resonance imaging could assist the diagnosis of ChAc.Botulinum toxin injection is a possible treatment for the typical orofacial dystonia.Deep brain stimulation is a novel surgical treatment modality.Most cases chose globus pallidus internus as target.Patients with dystonia as a major manifestation will benefit more from high-frequency stimulation and those with major findings of chorea and dysarthria are suitable for low-frequency stimulation.More evidence of long-term outcomes is warranted.
基金This study was conducted with the approval of the ethics committee of Chongqing Hospital of Traditional Chinese Medicine(approval No.2023-GAKY-KS-XYQ).The patient gave explicit informed consent to report her clinical case.
文摘BACKGROUND Pediatric-onset systemic lupus erythematosus(SLE)is typically more severe than adult-onset SLE,with a higher incidence of nervous system involvement.Chorea is a relatively rare neurological complication reported in 2.4%-7%of SLE patients.In particular,chorea induced by glucocorticoid dose reduction is even rarer.Herein,we report the case of a girl with SLE,who developed chorea during the process of glucocorticoid therapy reduction.CASE SUMMARY We describe a 14-year-old girl who was diagnosed with SLE.She was treated with methylprednisolone and rituximab,and her symptoms improved.On the second day after the methylprednisolone dose was reduced according to the treatment guidelines,the patient developed chorea.Her condition improved after adjusting her glucocorticoid regimen.CONCLUSION This case is a reminder that extra attention to chorea is required in SLE patients during glucocorticoid dose reduction.
文摘Introduction: Post-streptococcal chorea is the most common form of acquired chorea in children. Objective: The objective of this study was to contribute to better management of this condition by giving the characteristics of the epidemiological, clinical, biological, etiological, therapeutic and evolutionary aspects. Methodology: This was a retrospective and descriptive study from January 2021 to December 2021 which took place in the pediatrics department of the Mother and Child University Teaching Hospital of N’Djamena (CHU-ME). All the children in whom we observed abnormal involuntary movements whose age was less than or equal to 15 years were concerned. Result: there were three female children. The average age was 7.5, of which the two are 7 years old and the third is 8 years old. Two had a history of angina. A notion of polyarthralgia and dental caries was found in the third. The diagnosis of post-streptococcal chorea was retained on the basis of clinical arguments: observation of abnormal movements of the limbs and the face, which are involuntary, sudden with an insidious and progressive onset, muscular hypotonia, and psychic disorder. Medical imaging, in particular echocardiography, which objectified two cases of associated rheumatic valvular disease. Remission was observed in all three children after two weeks of treatment with haloperidol, penicillin. Those with associated rheumatic valve disease also received corticosteroid therapy. Complete remission of chorea was observed in all three girls. Prophylaxis in two patients based on Penicillin V was instituted. Conclusion: Although less frequent, post-streptococcal chorea is still seen in our country and is still an important health problem that needs more real medical efforts.
文摘Introduction: Chronic exposure to organic solvents may result in a variety of neurologic complications like dementia, cerebellar dysfunction, pyramidal syndrome, cranial nerve abnormalities, and neuropathy. Clinical Presentation: We report an unusual case of subacute chorea induced by occupational exposure. Brain magnetic resonance imaging showed diffuse white matter T2 hyperintensity. The screening of basic blood tests, and CSF studies to eliminate alternate diagnoses, were normal. The patient received 1000 mg/day of intravenous methylprednisolone for 3 days, with cessation of professional activity. We observed a regression of neurological symptoms after 3 months of follow-up. Conclusion: This case highlights the diversity of acute or chronic neurological complications of solvents.
文摘Hemichorea associated with non-ketotic hyperglycemia(HC-NH)is a rare disease.The purpose of this case report is to introduce a patient with HC-NH and provide a schedule of examination and follow-up treatment.We also reviewed the current understanding of pathophysiology and treatment and how to apply it to our patients.The case involved a 37-year-old Asian diabetic man who had a 9-day history of losing movement in left limbs and face.His initial blood glucose level was 10.13 mmol/L.HbA1c was 13.6%.Before admission,head CT scan showed suspicious small pieces of left brainstem with slightly high-density shadow and right putamen nucleus with high-density shadow.On the day of admission,head MRI showed punctate T1WI low signal shadow,T2WI high and low mixed signal shadow,T1WI high signal shadow and T2WI low signal shadow of right putamen nucleus.The case findings were consistent with his displayed motor pattern and with the HC-NH diagnosis.Gradual control of the blood glucose levels alleviates his choreiform symptoms.The endocrinology follow-up 6 months after discharge found that his symptoms did not recur after the outpatient’s medication compliance was improved.HC-NH is a rare manifestation of poor diabetes control,but it should also be noticed by clinicians.Early recognition and gradual treatment of elevated blood glucose levels seem to completely alleviate choreiform symptoms.
基金This study was supported by a grant from the National Natural Science Foundation of China
文摘We report a rare case about chorea in a Chinese adult after pulmonary thromboendarterectomy （PTE） with deep hypothermia and circulatory arrest.A 41-year-old male was admitted to our hospital because of breathing difficulties for about three years and intermittent hemoptysis for about two months.Physical examination revealed mild cyanotic lips,lower extremity edema.He was classified as grade III heart failure according the classification system of the New York Heart Association （NYHA）.
文摘In the treatment of 30 cases of minor chorea by puncturing Baihui (GV 20), Sishencong (Ex-HN 1) and Fengchi (GB 20), in combination of oral administration of herbal medicine, the total effective rate is 96.7%. Key Words Acupuncture Therapy - Chorea - Drug Therapy Author: ZHANG Shu-jun (1961-), female, lecturerTranslator: HUANG Guo-qiAn erratum to this article is available at http://dx.doi.org/10.1007/BF02850966.
