Hypomyelinating leukodystrophies (HLDs) are a heterogeneous group of disorders caused by primary deficit in myelin development; they are radiologically characterized by mild T2 hyperintensity with near normal T1 signal of the cerebral white matter. While most HLDs occur during infancy or childhood, adult-onset phenotypes are reported as well. To date, HLDs have not been extensively discussed in the literature on movement disorders apart from segregated case reports. From the perspective of movement disorders, HLDs commonly manifest as spastic ataxia, except for disorders such as hypomyelination with atrophy of the basal ganglia and cerebellum (H-ABC) and fucosidosis, where dystonia predominates. In addition, dystonia can be associated with the 18q deletion syndrome and KIF1C- and NKX6-2-related spastic ataxia. Chorea can be observed in the striatal variant of POLR3A, 18q deletion syndrome, and KIF1C-related disorders. Associated morphological features such as facial dysmorphism, hypodontia, early cataract, and skeletal and limb dysmorphism often provide vital clues to recognize these HLDs. Additional imaging clues include striatal atrophy in the H-ABC syndrome, spinal cord T2 hyperintensities in leukoencephalopathy with brainstem and spinal cord involvement and lactate elevation, intracranial calcification in Cockayne syndrome, and pallidal T2 hypointensity in fucosidosis. Early recognition of these clinicoradiological clues will be helpful in ordering a comprehensive genetic panel to confirm the diagnosis and determine the prognosis and therapeutic outcome.
{"title":"Hypomyelinating leukodystrophy and movement disorders","authors":"Jacky Ganguly, Jigyasha Sinha, P. Basu, Anushree Pal, Banashree Mondal, Mona Tiwari, Hrishikesh Kumar","doi":"10.4103/aomd.aomd_1_23","DOIUrl":"https://doi.org/10.4103/aomd.aomd_1_23","url":null,"abstract":"Hypomyelinating leukodystrophies (HLDs) are a heterogeneous group of disorders caused by primary deficit in myelin development; they are radiologically characterized by mild T2 hyperintensity with near normal T1 signal of the cerebral white matter. While most HLDs occur during infancy or childhood, adult-onset phenotypes are reported as well. To date, HLDs have not been extensively discussed in the literature on movement disorders apart from segregated case reports. From the perspective of movement disorders, HLDs commonly manifest as spastic ataxia, except for disorders such as hypomyelination with atrophy of the basal ganglia and cerebellum (H-ABC) and fucosidosis, where dystonia predominates. In addition, dystonia can be associated with the 18q deletion syndrome and KIF1C- and NKX6-2-related spastic ataxia. Chorea can be observed in the striatal variant of POLR3A, 18q deletion syndrome, and KIF1C-related disorders. Associated morphological features such as facial dysmorphism, hypodontia, early cataract, and skeletal and limb dysmorphism often provide vital clues to recognize these HLDs. Additional imaging clues include striatal atrophy in the H-ABC syndrome, spinal cord T2 hyperintensities in leukoencephalopathy with brainstem and spinal cord involvement and lactate elevation, intracranial calcification in Cockayne syndrome, and pallidal T2 hypointensity in fucosidosis. Early recognition of these clinicoradiological clues will be helpful in ordering a comprehensive genetic panel to confirm the diagnosis and determine the prognosis and therapeutic outcome.","PeriodicalId":7973,"journal":{"name":"Annals of Movement Disorders","volume":"6 1","pages":"58 - 71"},"PeriodicalIF":0.0,"publicationDate":"2023-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"42280413","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Sruthi Kola, R. Kandadai, R. Alugolu, S. Jabeen, R. Borgohain
AIM: To study the impact of trajectory parameters on Microelectrode recording during Subthalamic nucleus deep brain stimulation surgery in PD patients MATERIALS AND METHODS: This is a retrospective study. On the day of surgery MRI was taken with frame on which planning was done using stereocalc and navigation systems and final coordinates of X, Y, Z, mid sagittal angle, axial angles were obtained. During surgery Microelectrode recording (MER) was done and with bed side examination final lead placement decided. Impact of trajectory angles on MER was studied using appropriate statistical analysis. RESULTS: Among 40 patients studied,mean age of patients and duration of disease were 55.65years and 7.95 years. Mean UPDRS OFF and ON scores were 60.7 and 15.4 respectively and mean MOCA score was 26.6. Distribution of type of recording differs significantly across mid sagittal angles among <15o vs >15o (P <0.05). Group of <15o for midsagittal angle and >75o for axial angle had highest percentage (60%) showing no recording when compared to others. Significantly higher proportion of cases with higher duration of PD had higher incidence of no recording and vice-versa (P <0.05). CONCLUSION: The study found a correlation between approach angle and the quality of MER. Results showed that a midsagittal angle > 15o and an axial angle < 75o produced better MERs. Although not statistically significant, this could suggest approach angles may have a role to achieve good microelectrode recordings. Larger prospective studies may be helpful to understand this further.
{"title":"Study of Impact of Stereotactic parameters on Microelectrode recording in Parkinson’s disease patients who underwent Subthalamic nucleus Deep brain stimulation","authors":"Sruthi Kola, R. Kandadai, R. Alugolu, S. Jabeen, R. Borgohain","doi":"10.4103/aomd.aomd_6_23","DOIUrl":"https://doi.org/10.4103/aomd.aomd_6_23","url":null,"abstract":"AIM: To study the impact of trajectory parameters on Microelectrode recording during Subthalamic nucleus deep brain stimulation surgery in PD patients MATERIALS AND METHODS: This is a retrospective study. On the day of surgery MRI was taken with frame on which planning was done using stereocalc and navigation systems and final coordinates of X, Y, Z, mid sagittal angle, axial angles were obtained. During surgery Microelectrode recording (MER) was done and with bed side examination final lead placement decided. Impact of trajectory angles on MER was studied using appropriate statistical analysis. RESULTS: Among 40 patients studied,mean age of patients and duration of disease were 55.65years and 7.95 years. Mean UPDRS OFF and ON scores were 60.7 and 15.4 respectively and mean MOCA score was 26.6. Distribution of type of recording differs significantly across mid sagittal angles among <15o vs >15o (P <0.05). Group of <15o for midsagittal angle and >75o for axial angle had highest percentage (60%) showing no recording when compared to others. Significantly higher proportion of cases with higher duration of PD had higher incidence of no recording and vice-versa (P <0.05). CONCLUSION: The study found a correlation between approach angle and the quality of MER. Results showed that a midsagittal angle > 15o and an axial angle < 75o produced better MERs. Although not statistically significant, this could suggest approach angles may have a role to achieve good microelectrode recordings. Larger prospective studies may be helpful to understand this further.","PeriodicalId":7973,"journal":{"name":"Annals of Movement Disorders","volume":"6 1","pages":"85 - 92"},"PeriodicalIF":0.0,"publicationDate":"2023-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"47680329","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Shreyashi Jha, Ravi Yadav, V. Holla, N. Kamble, Pramod Kumar Pal, Dwarkanath Srinivas
Tardive dystonia (TD) is a disabling neurological disorder and is usually refractory to medical therapy. Over the past decade, several case reports and case series have demonstrated remarkable benefits of deep brain stimulation of the globus pallidus interna for the treatment of refractory TD. In this case report, we present an illustrative case of refractory TD treated with globus pallidus interna–deep brain stimulation, with long-term sustained improvement of the dystonia and psychiatric comorbidity. In addition, the patient had a dorsal cord schwannoma, producing pyramidal signs in the lower limbs, which highlights the need for meticulous clinical examination for optimum patient management.
{"title":"Long-term efficacy of pallidal deep brain stimulation in tardive dystonia: A case report and follow-up of 4 years","authors":"Shreyashi Jha, Ravi Yadav, V. Holla, N. Kamble, Pramod Kumar Pal, Dwarkanath Srinivas","doi":"10.4103/aomd.aomd_28_22","DOIUrl":"https://doi.org/10.4103/aomd.aomd_28_22","url":null,"abstract":"Tardive dystonia (TD) is a disabling neurological disorder and is usually refractory to medical therapy. Over the past decade, several case reports and case series have demonstrated remarkable benefits of deep brain stimulation of the globus pallidus interna for the treatment of refractory TD. In this case report, we present an illustrative case of refractory TD treated with globus pallidus interna–deep brain stimulation, with long-term sustained improvement of the dystonia and psychiatric comorbidity. In addition, the patient had a dorsal cord schwannoma, producing pyramidal signs in the lower limbs, which highlights the need for meticulous clinical examination for optimum patient management.","PeriodicalId":7973,"journal":{"name":"Annals of Movement Disorders","volume":"6 1","pages":"100 - 102"},"PeriodicalIF":0.0,"publicationDate":"2023-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"48021883","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Ashna Kumar, Michelle Rosario, S. Siddiqui, Divyani Garg, A. Shukla, Suvasini Sharma
Peripheral demyelinating polyneuropathy, central dysmyelination, Waardenburg syndrome, and Hirschsprung’s disease is a rare genetic disorder caused by de novo variants in the SOX10 gene. The SOX10 gene is expressed in the neural crest cells during early embryonic development and in the glial cells of the peripheral and central nervous systems during late embryonic development, as well as in adults. Here, we describe our findings in a 9-month-old male infant presenting with failure to thrive, global developmental delay, seizures, hypotonia, heterochromia iridis, hypopigmented skin macules, pendular nystagmus, Hirschsprung’s disease, and hearing impairment. Nerve conduction studies were suggestive of sensorimotor demyelinating polyneuropathy. Brain magnetic resonance imaging showed diffuse hypomyelination. Targeted genetic testing revealed a novel stop-loss variant in the SOX10 gene (NM_006941.4). This case highlights the importance of clinical phenotyping that can aid in targeted genetic testing.
{"title":"Developmental delay and assessment in an infant with PCWH syndrome: A case report","authors":"Ashna Kumar, Michelle Rosario, S. Siddiqui, Divyani Garg, A. Shukla, Suvasini Sharma","doi":"10.4103/aomd.aomd_34_22","DOIUrl":"https://doi.org/10.4103/aomd.aomd_34_22","url":null,"abstract":"Peripheral demyelinating polyneuropathy, central dysmyelination, Waardenburg syndrome, and Hirschsprung’s disease is a rare genetic disorder caused by de novo variants in the SOX10 gene. The SOX10 gene is expressed in the neural crest cells during early embryonic development and in the glial cells of the peripheral and central nervous systems during late embryonic development, as well as in adults. Here, we describe our findings in a 9-month-old male infant presenting with failure to thrive, global developmental delay, seizures, hypotonia, heterochromia iridis, hypopigmented skin macules, pendular nystagmus, Hirschsprung’s disease, and hearing impairment. Nerve conduction studies were suggestive of sensorimotor demyelinating polyneuropathy. Brain magnetic resonance imaging showed diffuse hypomyelination. Targeted genetic testing revealed a novel stop-loss variant in the SOX10 gene (NM_006941.4). This case highlights the importance of clinical phenotyping that can aid in targeted genetic testing.","PeriodicalId":7973,"journal":{"name":"Annals of Movement Disorders","volume":"6 1","pages":"96 - 99"},"PeriodicalIF":0.0,"publicationDate":"2023-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"48216915","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Jaslovleen Kaur, Abhishek Lenka, Jonathan Isaacson, Stuart Isaacson
Psychosis is a debilitating non-motor symptom of Parkinson’s disease that commonly manifests with illusions, presence/passage hallucinations, and well-formed visual hallucinations. Parkinson’s disease psychosis (PDP) is associated with several negative repercussions such as increased caregiver distress and high rates of nursing home placement, healthcare expenditure, and mortality. Several neurotransmitters have been implicated in the pathogenesis of PDP; these include dopamine, acetylcholine, and serotonin. Most antipsychotics have a variable degree of dopamine-blocking property that may worsen parkinsonism or result in the emergence of other drug-induced movement disorders. Therefore, atypical antipsychotics with minimal dopamine-blocking property (quetiapine, clozapine) are commonly prescribed to treat PDP. Pimavanserin, which modulates serotonergic transmission (5-HT2A inverse agonist), is the only drug approved by the US Food and Drug Administration to treat PDP; however, it is not globally available. Therefore, it is crucial to continue the search for effective pharmacotherapy of PDP. Other serotonergic targets of interest include selective 5-HT3 receptor antagonist ondansetron. Licensed for use as an antiemetic, open-label studies on ondansetron in the 1990s have shown encouraging results in the treatment of hallucinations in PD. However, ondansetron was not further studied in PDP as it was cost-prohibitive. In this article, we highlight the role of abnormal serotonergic transmission in the pathogenesis of PDP, revisit the studies that investigated the role of ondansetron in treating PDP, and discuss its potential as an effective therapeutic option for PDP.
{"title":"Ondansetron for the treatment of Parkinson’s disease psychosis: Rationale and literature review","authors":"Jaslovleen Kaur, Abhishek Lenka, Jonathan Isaacson, Stuart Isaacson","doi":"10.4103/aomd.aomd_53_22","DOIUrl":"https://doi.org/10.4103/aomd.aomd_53_22","url":null,"abstract":"Psychosis is a debilitating non-motor symptom of Parkinson’s disease that commonly manifests with illusions, presence/passage hallucinations, and well-formed visual hallucinations. Parkinson’s disease psychosis (PDP) is associated with several negative repercussions such as increased caregiver distress and high rates of nursing home placement, healthcare expenditure, and mortality. Several neurotransmitters have been implicated in the pathogenesis of PDP; these include dopamine, acetylcholine, and serotonin. Most antipsychotics have a variable degree of dopamine-blocking property that may worsen parkinsonism or result in the emergence of other drug-induced movement disorders. Therefore, atypical antipsychotics with minimal dopamine-blocking property (quetiapine, clozapine) are commonly prescribed to treat PDP. Pimavanserin, which modulates serotonergic transmission (5-HT2A inverse agonist), is the only drug approved by the US Food and Drug Administration to treat PDP; however, it is not globally available. Therefore, it is crucial to continue the search for effective pharmacotherapy of PDP. Other serotonergic targets of interest include selective 5-HT3 receptor antagonist ondansetron. Licensed for use as an antiemetic, open-label studies on ondansetron in the 1990s have shown encouraging results in the treatment of hallucinations in PD. However, ondansetron was not further studied in PDP as it was cost-prohibitive. In this article, we highlight the role of abnormal serotonergic transmission in the pathogenesis of PDP, revisit the studies that investigated the role of ondansetron in treating PDP, and discuss its potential as an effective therapeutic option for PDP.","PeriodicalId":7973,"journal":{"name":"Annals of Movement Disorders","volume":"6 1","pages":"72 - 78"},"PeriodicalIF":0.0,"publicationDate":"2023-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"49185571","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"A patient with Parkinson’s disease carrying a rare variant in the kinase domain of LRRK2","authors":"H. Onder, V. Topçu, S. Comoglu","doi":"10.4103/aomd.aomd_14_22","DOIUrl":"https://doi.org/10.4103/aomd.aomd_14_22","url":null,"abstract":"","PeriodicalId":7973,"journal":{"name":"Annals of Movement Disorders","volume":"6 1","pages":"108 - 110"},"PeriodicalIF":0.0,"publicationDate":"2023-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"47733612","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Nishtha Yadav, H. Pendharkar, Chandrajit Prasad, R. Kenchaiah, N. Kamble
{"title":"Chorea in moyamoya disease with ipsilateral basal ganglia atrophy and mineralization","authors":"Nishtha Yadav, H. Pendharkar, Chandrajit Prasad, R. Kenchaiah, N. Kamble","doi":"10.4103/aomd.aomd_12_22","DOIUrl":"https://doi.org/10.4103/aomd.aomd_12_22","url":null,"abstract":"","PeriodicalId":7973,"journal":{"name":"Annals of Movement Disorders","volume":"6 1","pages":"103 - 107"},"PeriodicalIF":0.0,"publicationDate":"2023-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"46621590","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"The effect of “liquid gold” levodopa–carbidopa ascorbic acid solution in patients with Parkinson’s disease","authors":"J. Rissardo, A. Caprara","doi":"10.4103/aomd.aomd_42_22","DOIUrl":"https://doi.org/10.4103/aomd.aomd_42_22","url":null,"abstract":"","PeriodicalId":7973,"journal":{"name":"Annals of Movement Disorders","volume":"6 1","pages":"111 - 113"},"PeriodicalIF":0.0,"publicationDate":"2023-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"43870581","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
C. Nagpal, Ayush Agarwal, V. Venkateswaran, K. Soni, M V Srivastava, A. Trikha
Ifosfamide, an analog of cyclophosphamide, is commonly used as a chemotherapeutic agent to treat sarcomas and solid tumors. However, neurotoxicity is a rare side effect of this drug. When present, the symptoms range from confusion, agitation, and delirium in mild cases to mutism, visual blurring, hallucinations, seizures, stupor, and even coma in extreme cases. Within this spectrum, extrapyramidal symptoms are extremely rare, and when present, may not revert with drug discontinuation. Sequelae may occasionally persist even after discontinuation of the drug. Our case illustrates a rare occurrence of ifosfamide-induced extrapyramidal neurotoxicity in a patient with metastatic phyllodes tumor of the breast and concomitant COVID-19 illness. Ifosfamide-induced extrapyramidal neurotoxicity is a clinical diagnosis of exclusion and requires ruling out of other possible causes. The diagnosis is supported by a temporal correlation with drug administration, presence of risk factors, and improvement after infusion cessation, along with normal brain imaging.
{"title":"Ifosfamide-induced extrapyramidal neurotoxicity with COVID-19","authors":"C. Nagpal, Ayush Agarwal, V. Venkateswaran, K. Soni, M V Srivastava, A. Trikha","doi":"10.4103/aomd.aomd_24_22","DOIUrl":"https://doi.org/10.4103/aomd.aomd_24_22","url":null,"abstract":"Ifosfamide, an analog of cyclophosphamide, is commonly used as a chemotherapeutic agent to treat sarcomas and solid tumors. However, neurotoxicity is a rare side effect of this drug. When present, the symptoms range from confusion, agitation, and delirium in mild cases to mutism, visual blurring, hallucinations, seizures, stupor, and even coma in extreme cases. Within this spectrum, extrapyramidal symptoms are extremely rare, and when present, may not revert with drug discontinuation. Sequelae may occasionally persist even after discontinuation of the drug. Our case illustrates a rare occurrence of ifosfamide-induced extrapyramidal neurotoxicity in a patient with metastatic phyllodes tumor of the breast and concomitant COVID-19 illness. Ifosfamide-induced extrapyramidal neurotoxicity is a clinical diagnosis of exclusion and requires ruling out of other possible causes. The diagnosis is supported by a temporal correlation with drug administration, presence of risk factors, and improvement after infusion cessation, along with normal brain imaging.","PeriodicalId":7973,"journal":{"name":"Annals of Movement Disorders","volume":"6 1","pages":"93 - 95"},"PeriodicalIF":0.0,"publicationDate":"2023-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"46098209","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
A. Ranjan, Sanaullah Mudassir, Neetu Sinha, Abhishek Kumar
OBJECTIVE: Diabetic striatopathy (DS) is characterized by a hyperglycemic state associated with chorea/ballism, and/or striatal hyperdensity on computed tomography, or hyperintensity on T1-weighted magnetic resonance imaging. To date, there have been only a few case series reported in the literature on this topic. In the present study, we report four cases of DS associated with movement disorders. METHODS: The patients were recruited based on the presence of hyperglycemia associated with chorea/ballism or striatal hyperintensity on T1-weighted magnetic resonance imaging. RESULTS: Four patients with DS (two men and two women), with a mean age of 61 years, were included in our study. Three out of the four patients had a previous diagnosis of type 2 diabetes mellitus. The mean blood glucose level on admission and glycated hemoglobin were 390.25 mg/dl and 12.45%, respectively. Hemiballism was present in two patients: one patient had dystonia and the other had choreiform movement at presentation. The putamen was affected in all patients, with involvement of the globus pallidus and caudate nucleus in one patient. All patients had resolution of their abnormal movements after glucose-lowering therapy, with additional use of anti-chorea medication in three patients. CONCLUSION: DS should be considered in elderly patients who present with chorea/ballism/dystonia and should be accordingly managed with resolution of abnormal movements. In addition, dystonia can be a presenting symptom in DS.
{"title":"Diabetic striatopathy: A case series of rare and treatable movement disorder","authors":"A. Ranjan, Sanaullah Mudassir, Neetu Sinha, Abhishek Kumar","doi":"10.4103/aomd.aomd_62_21","DOIUrl":"https://doi.org/10.4103/aomd.aomd_62_21","url":null,"abstract":"OBJECTIVE: Diabetic striatopathy (DS) is characterized by a hyperglycemic state associated with chorea/ballism, and/or striatal hyperdensity on computed tomography, or hyperintensity on T1-weighted magnetic resonance imaging. To date, there have been only a few case series reported in the literature on this topic. In the present study, we report four cases of DS associated with movement disorders. METHODS: The patients were recruited based on the presence of hyperglycemia associated with chorea/ballism or striatal hyperintensity on T1-weighted magnetic resonance imaging. RESULTS: Four patients with DS (two men and two women), with a mean age of 61 years, were included in our study. Three out of the four patients had a previous diagnosis of type 2 diabetes mellitus. The mean blood glucose level on admission and glycated hemoglobin were 390.25 mg/dl and 12.45%, respectively. Hemiballism was present in two patients: one patient had dystonia and the other had choreiform movement at presentation. The putamen was affected in all patients, with involvement of the globus pallidus and caudate nucleus in one patient. All patients had resolution of their abnormal movements after glucose-lowering therapy, with additional use of anti-chorea medication in three patients. CONCLUSION: DS should be considered in elderly patients who present with chorea/ballism/dystonia and should be accordingly managed with resolution of abnormal movements. In addition, dystonia can be a presenting symptom in DS.","PeriodicalId":7973,"journal":{"name":"Annals of Movement Disorders","volume":"6 1","pages":"26 - 29"},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"49203882","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
京公网安备 11010802042870号
京ICP备2023020795号-1
扫码关注我们
求助内容：
应助结果提醒方式：