Choreic Movements Research Articles

Clinical Response of Levodopa in CTNNB1-Related Dystonia

Abstractβ-catenin, which is encoded by the CTNNB1 gene, is essential for the development and functioning of the brain. There are a few documented cases of dystonia related to CTNNB1. Here, we report the case of an 11-year-old Spanish boy referred for movement disorders and gait disturbance. He had motor developmental delay and achieved unassisted walking at 3 years, with a tiptoe gait and valgus foot posture requiring ankle-foot orthoses. Blood tests showed elevated creatine kinase levels (1684 U/L, normal range 62–235). Molecular analysis revealed a deletion in exons 3-9 of the DMD gene, leading to the diagnosis of Becker muscular dystrophy. By age 8, he presents frequent falls due to a dystonic posture of the feet and abnormal movements in the upper and lower limbs. Whole-exome sequencing revealed a novel heterozygous, de novo pathogenic frameshift variant in the CTNNB1 gene (NM_001098209.1):p.Thr297fs/ c.889dupA. Treatment with levodopa/carbidopa (5.3 mg/kg/day) led to a partial clinical improvement, including a decrease in dystonia, measured by the Burke-Fahn-Marsden Dystonia Rating Scale, and choreic movements in all four limbs. We suggest that levodopa contributes to motor improvement in patients with CTNNB1-related dystonia, supporting its inclusion in the differential diagnosis of childhood dopa-responsive dystonia.

ADCY5-related dyskinesia - case series with literature review.

ADCY5-related dyskinesia is a rare neurological disease caused by mutations in the gene encoding the adenylyl cyclase 5 (ADCY5) isoform, a protein that plays an important role in intracellular transmission. Variants in ADCY5 are associated with a spectrum of neurological disease encompassing dyskinesia, chorea, and dystonia. State of the-art. ADCY5 mutations result in clinically heterogeneous manifestations which comprise a range of core and less to highly variable symptoms. Due to the heterogeneous nature and difficulty in diagnosis of the disorder, available treatments are highly limited. ADCY5-related dyskinesia was reported in 52 individuals in the literature over a five-year period (January 2017 to January 2022). We have listed all the symptoms and their frequency. The most common symptom reported in these patients was dystonia. Over 50% of patients developed dyskinesia and chorea. We report two cases of familial occurrence of symptomatic ADCY5-related dyskinesia. A 45-year-old patient presented with involuntary movements which had been occurring since childhood. The proband's neurological examination revealed dysarthria, involuntary myoclonic twitches, and choreic movements. The patient's 9-year-old son had developed involuntary movements, mainly chorea and dystonia. This paper aims to summarise the recent literature on ADCY5-related neurological disorders and to present a new case of a Polish family with ADCY5 mutation. Genetic diagnostics are important in the context of possible future targeted treatments.

A case of diabetic striatopathy due to uncontrolled type 2 diabetes.

Hemichorea-hemiballismus (HCHB) syndrome is a syndrome characterized by choreic movements which are irregular, nonrepetitive, and random movements, and ballismus which are spontaneous and violent movements. HCHB syndrome with a metabolic cause is a rare presentation that can be precipitated by uncontrolled diabetes. Presented here is a case of HCHB syndrome with right-sided neuroimaging findings and contralateral chorea due to uncontrolled type 2 diabetes mellitus. This patient was found to be obtunded with a blood glucose of greater than 500 mg/dL by EMS. After the administration of insulin, she was able to answer clarifying questions of noncompliance with her antihyperglycemic medications. She had a computed tomography without contrast of the head which showed hyperdense lesions in the right caudate nucleus and putamen consistent with HCHB syndrome. She was started on treatment for nonketotic hyperglycemia with insulin. As her mentation improved, she was able to cooperate with physical examination, which revealed irregular and violent movements in the left upper and lower extremities. Her hemichorea and hemiballismus improved with strict glycemic control, and she was able to be discharged to a skilled nursing facility for further rehabilitation. She would later have repeated hospitalizations for poor glycemic control, and repeat neuroimaging would reveal the resolution of hyperdensities after 4 months. HCHB syndrome due to uncontrolled diabetes has been termed diabetic striatopathy and is characterized by poor glycemic control, unilateral striatal hyperdensity on CT imaging, and contralateral choreic movements. Diabetic striatopathy remains a poorly understood disease, and the exact pathophysiologic mechanism has not been definitively elucidated. Diabetic striatopathy is a relatively new term for metabolic etiology of hemichorea-hemiballismus syndrome and was coined in 2009. The triad for diabetic striatopathy is poor glycemic control, unilateral striatal hyperdensity on CT imaging, and contralateral choreic movements. Multiple etiologies have been suggested for the cause of diabetic striatopathy including petechial hemorrhage, mineral deposition, myelin destruction, and infarction with reactive astrocytosis; however, the exact mechanism has yet to be determined. Antidopaminergic medications may be used to control the choreic movements of diabetic striatopathy; however, the mainstay of treatment is glycemic control, often with insulin therapy.

Aripiprazole induced reversible ptosis anda oromandibular dystonia

Introduction Aripiprazole is an atypical antipsychotic orally indicated for the treatment of schizophrenia, bipolar I, major depressive disorder. It is also indicated as an injection for agitation associated with schizophrenia or bipolar mania. Aripiprazole exerts its effects through agonism of dopaminergic and 5-HT1A receptors and antagonism of alpha-adrenergic and 5-HT2A receptors (Cosi et al., 2006). Ptosis is known as the drooping of the upper eyelid, and the patient usually presents with the complaint of the defect in vision and cosmesis (Shahzad & Siccardi, 2023). Orofacial dyskinesia and oromandibular dystonia are uncommon neurological disorders with involuntary, mainly choreic (dance-like) movements, or excessive, involuntary and sustained or repetitive muscle contractions that may involve the face, lips, tongue, and/or jaw (Bakke, 2016).ObjectivesThe aim of this study is to present a case of ptosis and orofacial spasm, which are neurological side effects that may be very rare side effects of aripiprazole.MethodsThe 22-year-old woman was referred to the psychiatric service via the emergency service, due to thoughts of harming herself and irritability. The patient was planned to be hospitalized due to decreased sleep, increased speech, and persecution delusions for the last days. After the patient stayed in the service for 25 days, the patient was prescribed olanzapine 10 mg for discharge. At the follow-up appointment, it was learned that the patient had gained weight due to olanzapine and was switched to aripiprazole on 4th of July. The patient’s aripiprazole dose was gradually increased to 10 mg/day. The patient’s relatives gradually noticed drooping eyelids, involuntary oral movements, and impaired speech due to aripiprazole. Cranial MRI and cranial MRI angiography performed to rule out organic pathologies resulted normal. Accordingly, the aripiprazole dose was reduced to 5 mg/day by his mother, and at the last follow-up appointment on August 4, aripiprazole was stopped and paliperidone was started, and the neurological symptoms completely resolved 4 days later.Results Since aripiprazole is frequently used in the field of psychiatry, its side effects are often wondered about. Extrapyramidal system side effects of antipsychotics are more common than ptosis. And In terms of the incidence of various extrapyramidal side effects, overall, no significant effects of age, sex, mean dose, study duration, or measuring method could be demonstrated. It is very important to distinguish between organic pathology and drug side effects, especially since ptosis accompanies neuromuscular diseases, ischemic, demyelinating brain lesions and intracranial aneurysms.ConclusionsAs a result, patients who are started on antipsychotics should be closely watched for side effects to increase patient comfort and drug compliance. Also organic pathologies must be excluded before making a final decision that it is a drug side effect.Disclosure of InterestNone Declared

P-27 Sweet moves - Hemichorea associated with non-ketotic hyperglycemia

Abstract Introduction Hemichorea is a movement disorder characterized by involuntary, irregular, and often violent movements of one hemibody, typically caused by dysfunction in the basal ganglia. There are several potential causes of hemichorea, namely vascular lesions, autoimmune conditions, structural lesions, drugs, or metabolic disturbances, among others. Hemichorea associated with non-ketotic hyperglycemia is a rare condition with a reported prevalence of 1 in 100.000. Although its exact mechanisms are not fully understood, it is believed to be linked to metabolic changes and vascular abnormalities associated with diabetes and its management involves optimizing blood sugar levels. Clinical Case This is the case of a female patient, 76 years old, caucasian with known medical history of hypertension, type 2 diabetes, and dyslipidemia. The woman, admitted to the Neurology department, presented with involuntary movements of the left hemibody and face, with sudden onset, one day before admission. There was no history of visual changes, headache, dizziness, syncope, or other symptoms and there were no recent changes in her usual medication. The neurological examination didn't show any defects other than the choreic movements of the left hemiface and hemibody. Blood tests revealed an HbA1c level of 9.3% and a glucose level of 254 mg/dL. A brain CT scan revealed a possible hypodensity of the right insula, besides signs of microangiopathic leukoencephalopathy. A brain MRI was ordered and showed, on the right putamen, hyperintensity on T1, mild hyperintensity on Flair (Fig.1), and mild hypointensity on T2, without areas of diffusion restriction suggesting ischaemic vascular damage - radiologic findings consistent with hemichorea associated with non-ketotic hyperglycemia. Strict glycemic control was achieved in cooperation with the Endocrinology department, with complete resolution of symptoms. Conclusion Although rare, hyperglycemia should be considered as a potential cause of hemichorea, as correction of the underlying condition will lead to rapid improvement of the movements and is the most important feature for diagnosis. This case shows the myriad of clinical presentations that a person living with Diabetes can have.Figure 1.Brain MRI scan.Brain MRI scan showing, on the right putamen, mild hyperintensity on Flair.

Huntington’s disease associated with psychotic features

Abstract: Huntington’s disease (HD) is a rare, progressive, fatal, and autosomal dominant neurodegenerative disorder characterized by a triad of abnormal choreatic movements, cognitive disorders, and psychotic symptoms. A very small group presents with psychotic features. In this report, we present a case of HD with psychotic features and a diagnosis of HD. We describe a 29-year-old male with a history of behavior changes, delusions of persecution, and auditory hallucinations, which on mental status examination were suggestive of organic delusional disorder (schizophrenia-like).

Clinical Neurology in Practice: The Tongue (part 2).

The tongue is an essential organ for the development of certain crucial functions such as swallowing and speech. The examination of the tongue can be very useful in neurology, as the various types of lingual alterations can lead to certain specific diagnoses, the tongue being a kind of 'mirror' of some neurological function. To discuss the elements of clinical examination of the tongue in relation to neurological disorders. After reviewing the different superficial lesions of the tongue, we deal with various movement disorders of the tongue (fasciculations/myokimia, orolingual tremor, choreic movements of the tongue, dystonia of the tongue, lingual myoclonus, and psychogenic movements), disorders of taste and lingual sensitivity and lingual pain. Examination of the tongue should not be limited to studying its motility and trophicity. It is equally important to check the sensory function and understand how to interpret abnormal movements involving the tongue. This study also aimed to demonstrate the importance of nonmotor tongue function in neurological practice.

Disappearance of hemichorea as the initial presentation of moyamoya disease after revascularization surgery regardless of residual hypoperfusion evaluated by quantitative N-isopropyl-p-123I-iodoamphetamine SPECT

Background and importanceAlthough hypoperfusion of the basal ganglia or the frontal subcortical matter is suspected, the pathology of chorea in moyamoya disease remains unclarified. Herein, we report a case of moyamoya disease presenting with hemichorea and evaluate pre- and postoperative perfusion using single photon emission computed tomography with N-isopropyl-p-123I-iodoamphetamine (123I-IMP SPECT). Clinical presentationAn 18-year-old woman presented with choreic movement of her left limbs. Magnetic resonance imaging revealed an ivy sign, and 123I-IMP SPECT demonstrated decreased cerebral blood flow (CBF) and cerebral vascular reserve (CVR) values in the right hemisphere. The patient underwent direct and indirect revascularization surgery to improve cerebral hemodynamic impairment. The choreic movements entirely resolved immediately after surgery. Although CBF and CVR values in the ipsilateral hemisphere demonstrated by quantitative SPECT increased, these did not reach the normal values threshold. ConclusionChoreic movement in moyamoya disease may be related to cerebral hemodynamic impairment. Further studies are required to elucidate its pathophysiological mechanisms.

Teaching Video NeuroImages: A treatable rare cause of chorea

A 72-year-old woman developed progressive choreic movements involving the right hemibody and the orofacial muscles (video 1). Her examination was otherwise unremarkable, without ataxia. Brain MRI, blood glucose, thyroid function, and rheumatologic panel were unremarkable. Genetic testing for Huntington disease and C9orf72 expansions were negative. Initial hematocrit and hemoglobin levels were normal, but increased to abnormal values (56% and 18.5 g/dL) 1 year after chorea onset. The JAK2V617F mutation was positive, suggesting a diagnosis of chorea due to polycythemia vera. Chorea disappeared with phlebotomy and hydroxyurea. Neurologists should suspect this rare treatable entity in elderly patients with chorea even with initially normal hematocrit levels.1

Two Cases of TMEM151A-Associated Paroxysmal Dyskinesia in a Single-Center Series of PRRT2-Negative Patients.

Two Cases of TMEM151A-Associated Paroxysmal Dyskinesia in a Single-Center Series of PRRT2-Negative Patients.

Síndrome cérebro-pulmão-tireoide: tratamento e prognóstico com o uso de cloridrato de metilfenidato

INTRODUCTION: The “brain-lung-thyroid syndrome” is caused by mutations in the NKX2-1 gene, which encodes the thyroid transcription factor 1, and is characterized by widely diverse clinical manifestations: choreic movements, respiratory disorders and hypothyroidism. CASE REPORT: Male patient, 10 years old, son of non-consanguineous parents, being treated for subclinical hypothyroidism with levothyroxine, was referred at 4 years of age for investigation of hypotonia. There were no reports of breathing problems at birth. Investigation for hypotonia (central and neuromuscular) including muscle biopsy was normal. There was a progressive improvement in hypotonia, but the patient developed choreic movements. In view of the new clinical finding, a genetic-molecular study was carried out directed towards the NKX2-1 gene, confirming the diagnosis of “brain-lung-thyroid syndrome”. The patient started treatment with methylphenidate hydrochloride with evident improvement in choreic movements. DISCUSSION: Manifestations related to the NKX2-1 gene may include hereditary benign chorea (HBC), congenital hypothyroidism and neonatal respiratory disorders. Generally, chorea begins in early childhood, in the patient’s first year of life (most common), in later childhood or even adolescence, and progresses into the second decade after which it remains static or (rarely) resolves. Recently, the use of methylphenidate in patients with a mutation in this gene proved to be a possible therapeutic approach to control choreic movements and improve motor coordination in patients affected by this rare disease.

Huntington disease in the Valencian Region

Huntington disease (HD) is a rare neurodegenerative disorder of the central nervous system characterized by unwanted choreatic movements, behavioral and psychiatric disturbances and dementia. Describe the geographical, age and sex distribution of HD in the Valencia Region (VR) and determine its prevalence and mortality. Cross-sectional study for the period 2010-2018. Confirmed cases of HD were identified through the Rare Disease Information System of the VR. Sociodemographic characteristics were described, and the prevalence and mortality rate were obtained. 225 cases were identified, 50.2% women. 52.0% lived in the province of Alicante. 68.9% were verified by their clinical diagnosis. The median age at diagnosis was 54.1 years, 54.7 years in men and 53.0 years in women. The prevalence in 2018 was 1.97/100,000 inhabitants (95%; CI: 0.39-2.37), showing a no significant increasing trend, overall and by sex. 49.8% died, 51.8% men. The median age at death was 62.7 years, being lower in men than in women. The mortality rate in 2018 was 0.32/100,000 inhabitants (95%; CI: 0.32-2.28), with no statistically significant differences. The prevalence obtained was within the range estimated by Orphanet (1-9/100,000). A difference between sexes was observed in the diagnosis age. Men are the group with the highest mortality and the earliest age of death. It is a disease with high mortality with an average of 6.5 years between diagnosis and death.

A Kinematic Data-Driven Approach to Differentiate Involuntary Choreic Movements in Individuals With Neurological Conditions.

The ability to differentiate similar choreic involuntary movements could lay the groundwork for the development of a minimally-invasive screening tool for their etiology and provide in-depth understandings of pathophysiology. As a first step, we investigate kinematic differences between Huntington's disease (HD) chorea and Parkinson's disease (PD) choreic levodopa-induced dyskinesia (LID), which have distinct pathological causes yet share a great kinematic resemblance. Twenty subjects with HD and ten subjects with PD stood with both upper limbs in front of them for approximately 60 seconds. The three-dimensional velocity time-series of involuntary movements of both hands were segmented into one-dimensional sub-movements abutted by velocity zero-crossings. A combination of unsupervised and supervised machine learning algorithms was employed to automatically select data features extracted from sub-movements and distinguish the two types of involuntary choreic movements. The trained model was able to accurately classify chorea vs. LID with an Area Under the Receiver Operating Characteristic Curve of 99.5%. A set of important features contributing to the construction of the classification model were identified and investigated. The trained model may serve as a tool for the automatic identification of different types of involuntary choreic movements, enabling continuous monitoring and personalized treatment for patients in various clinical settings. The results provide insights into kinematic characteristics of HD chorea and PD LID, which is the first step towards an improved general understanding of involuntary choreic movements.

Anti-inflammatory effects of ellagic acid and vanillic acid against quinolinic acid-induced rat model of Huntington's disease by targeting IKK-NF-κB pathway

Huntington disease (HD), an autosomal dominant neurodegenerative disorder characterized by involuntary choreatic movements with cognitive and behavioral disturbances. HD striatum has increased conversion of kynurenine to quinolinic acid (QA) which activates NMDA receptors leading to activation of microglia and increased levels of nuclear factor kappa B (NF-κB) leading to elevated transcription of inducible nitric oxide synthase (iNOS) and various cytokines causing neuronal death via neuroinflammation, oxidative stress, mitochondrial dysfunction and apoptosis. Therefore, inhibiting IKK-NF-κB pathway induced excitotoxicity, oxidative stress and neuroinflammation could be a potential intervention in slowing down the disease progression. QA injection intrastriatally (IS-QA) produce damage mimicking HD where neuroinflammation, oxidative stress and mitochondrial dysfunction play crucial role. Ellagic acid (EA) and vanillic acid (VA) are well reported to possess antioxidant and NF-κB inhibiting effect. Hence, in present study, rats administered IS-QA were treated with EA and VA for 21 days to explore their neuroprotective effects. Behavioral studies, biochemical estimations for oxidative stress and acetylcholinesterase assay were performed. Mitochondrial function was determined by estimating mitochondrial enzyme complexes; inflammatory markers like TNF-α, IL-6, NF-κB by ELISA and apoptosis by caspase-3 levels. Brain damage was determined by histopathology which revealed their neuroprotective effects. Various doses of EA and VA produced improved motor and cognitive functions, oxidative stress and neuroinflammation were also reduced and mitochondrial functioning was improved. In a nutshell, these results signify improved motor and cognitive functions by EA and VA in QA model of HD, along with declined oxidative stress, mitochondrial dysfunction and neuroinflammation.

Ataxia-telangiectasia syndrome

We describe the clinical case of a 12-year-old boy with no relevant personal or family medical history. He started to be evaluated in the developmental pediatrics because of global developmental delay evident since age 17 months. At age 3 years, spasticity began, which was more evident in the lower limbs, accompanied by axial and appendicular ataxia with progressive worsening. At age 9 years, he started to demonstrate cephalic and appendicular choreic movements, oromandibular dyskinesia, and oculomotor and orolingual apraxia.

Psychopathological Impact in Patients with History of Rheumatic Fever with or without Sydenham's Chorea: A Multicenter Prospective Study.

Sydenham’s chorea (SC) is a post-streptococcal autoimmune disorder of the central nervous system, and it is a major criterium for the diagnosis of acute rheumatic fever (ARF). SC typically improves in 12–15 weeks, but patients can be affected for years by persistence and recurrencies of both neurological and neuropsychiatric symptoms. We enrolled 48 patients with a previous diagnosis of ARF, with or without SC, in a national multicenter prospective study, to evaluate the presence of neuropsychiatric symptoms several years after SC’s onset. Our population was divided in a SC group (n = 21), consisting of patients who had SC, and a nSC group (n = 27), consisting of patients who had ARF without SC. Both groups were evaluated by the administration of 8 different neuropsychiatric tests. The Work and Social Adjustment Scale (WSAS) showed significantly (p = 0.021) higher alterations in the SC group than in the nSC group. Furthermore, 60.4% (n = 29) of the overall population experienced neuropsychiatric symptoms other than choreic movements at diagnosis and this finding was significantly more common (p = 0.00) in SC patients (95.2%) than in nSC patients (33.3%). The other neuropsychiatric tests also produced significant results, indicating that SC can exert a strong psychopathological impact on patients even years after its onset.

Prevalence of diabetic striatopathy and predictive role of glycated hemoglobin level

BackgroundDiabetic striatopathy is defined as a state of hyperglycemia associated with chorea/ballism, striatal hyperdensity at CT, or hyperintensity at T1-weighted MRI. It is considered a rare complication of uncontrolled diabetes but prevalence data are scarce.ObjectivesCharacterize diabetic striatopathy prevalence in the population afferent to the largest teaching hospital in Genova (Liguria, Italy) and investigate the role of glycated hemoglobin level in predicting the risk.MethodsData were retrospectively obtained from general population undergoing blood sampling for glycated hemoglobin and resulting with HbA1c values ≥ 8%, from January 2014 to June 2017. Brain neuroimaging of those who underwent at least a brain CT or MRI was examined in search of findings compatible with diabetic striatopathy and clinical information was collected. Logistic regression was used to predict the risk of diabetic striatopathy based on age and HbA1c values.ResultsSubjects with uncontrolled diabetes were 4603. Brain neuroimaging was available in 1806 subjects and three patients with diabetic striatopathy were identified, all of them reporting choreic movements. The prevalence of hemichorea due to diabetic striatopathy was therefore 3 cases out of 1806 (0.16%) in our population. Hepatic and hypoxic encephalopathies were the conditions most frequently mimicking diabetic striatopathy. Odds ratio of diabetic striatopathy and HbA1c level was significantly correlated (p = 0.0009).ConclusionsTo the best of our knowledge, this study is the first to evaluate the prevalence of diabetic striatopathy in Italy. High HbA1c values may have a role in predicting diabetic striatopathy.

Clinical and Genetic Aspects of Huntington's Disease in the Malian Population.

Huntington's disease (HD) is an autosomal dominant neurodegenerative disorder caused by mutation in the HTT gene and characterized by involuntary movements as well as cognitive and behavioral impairment. Since its first description 150 years ago, studies have been reported worldwide. However, genetically confirmed cases have been scarce in Africa. To describe the clinical and genetic aspects of HD in the Malian population. Patients with HD phenotype and their relatives were enrolled after obtaining consent. Symptoms were assessed using the Total Motor Scale (TMS) of the United Huntington's Disease Rating Scale (UHDRS) and the Mini-Mental State Examination (MMSE). Brain imaging and blood tests were performed to exclude other causes. DNA was extracted for HTT sequencing. Eighteen patients (13 families) with a HD phenotype were evaluated. A familial history of the disease was found in 84.6% with 55.5% of maternal transmission. The average length of the HTT CAG repeat was 43.6±11.5 (39-56) CAGs. The mean age at onset was 43.1±9.7years. Choreic movements were the predominant symptoms (100% of the cases) with an average TMS of 49.4±30.8, followed by cognitive impairment (average MMSE score: 23.0±12.0) and psychiatric symptoms with 22.2% and 44.4%, respectively. This is one of the largest HD cohorts reported in Africa. Increasing access to genetic testing could uncover many other HD cases and disease-modifying genetic variants. Future haplotype and psychosocial studies may inform the origin of the Malian mutation and the impact of the disease on patients and their relatives.

Effects of Exercise on Skeletal Muscle Pathophysiology in Huntington's Disease.

Huntington’s disease (HD) is a rare, hereditary, and progressive neurodegenerative disease, characterized by involuntary choreatic movements with cognitive and behavioral disturbances. In order to mitigate impairments in motor function, physical exercise was integrated in HD rehabilitative interventions, showing to be a powerful tool to ameliorate the quality of life of HD-affected patients. This review aims to describe the effects of physical exercise on HD-related skeletal muscle disorders in both murine and human models. We performed a literature search using PubMed, Scopus, and Web of Science databases on the role of physical activity in mouse models of HD and human patients. Fifteen publications fulfilled the criteria and were included in the review. Studies performed on mouse models showed a controversial role played by exercise, whereas in HD-affected patients, physical activity appeared to have positive effects on gait, motor function, UHDMRS scale, cognitive function, quality of life, postural stability, total body mass, fatty acid oxidative capacity, and VO2 max. Physical activity seems to be feasible, safe, and effective for HD patients. However, further studies with longer follow-up and larger cohorts of patients will be needed to draw firm conclusions on the positive effects of exercise for HD patients.

Acute onset movement disorders in diabetes mellitus: A clinical series of 59 patients

Background and purposeNo previous study has assessed the frequency and clinical–radiological characteristics of patients with diabetes mellitus (DM) and acute onset nonchoreic and nonballistic movements. We conducted a prospective study to investigate the spectrum of acute onset movement disorders in DM.MethodsWe recruited all the patients with acute onset movement disorders and hyperglycemia who attended the wards of three hospitals in West Bengal, India from August 2014 to July 2021.ResultsAmong the 59 patients (mean age = 55.4 ± 14.3 years, 52.5% men) who were included, 41 (69.5%) had choreic or ballistic movements, and 18 (30.5%) had nonchoreic and nonballistic movements. Ballism was the most common movement disorder (n = 18, 30.5%), followed by pure chorea (n = 15, 25.4%), choreoathetosis (n = 8, 13.6%), tremor (n = 5, 8.5%), hemifacial spasm (n = 3, 5.1%), parkinsonism (n = 3, 5.1%), myoclonus (n = 3, 5.1%), dystonia (n = 2, 3.4%), and restless leg syndrome (n = 2, 3.4%). The mean duration of DM was 9.8 ± 11.4 years (89.8% of the patients had type 2 DM). Nonketotic hyperglycemia was frequently (76.3%) detected. The majority (55.9%) had no magnetic resonance imaging (MRI) changes; the remaining showed striatal hyperintensity. Eight patients with MRI changes exhibited discordance with sidedness of movements. Most of the patients (76.3%) recovered completely.ConclusionsThis is the largest clinical series depicting the clinical–radiological spectrum of acute onset movement disorders in DM. Of note was that almost one third of patients had nonchoreic and nonballistic movements. Our findings highlight the importance of a capillary blood glucose measurement in patients with acute or subacute onset movement disorders, irrespective of their past glycemic status.