Hyperglycemia-induced hemichorea-hemiballismus syndrome - a systematic review.

Nonketotic hyperglycemia may occur as a cause of chorea in patients with chronic decompensated diabetes. Because it is rare and consequently poorly studied, diagnosis and treatment can be delayed. Therefore, our objective was to summarize clinical and radiological features, as well as treatments performed, from previously reported cases to facilitate adequate management in clinical practice. We searched MEDLINE/PubMed, EMBASE, Cochrane, CINAHL, Web of Science, Scopus, and LILACS databases for studies published before April 23, 2021. We included case reports and case series of adults (aged ≥ 18 years) that described hyperglycemic chorea with measurement ofglycated hemoglobin (HbA1c) and cranial magnetic resonance imaging (MRI). Studies were excluded if participants were pregnant women, aged < 18 years, and had no description of chorea and/or physical examination. We found 121 studies that met the inclusion criteria, for a total of 214 cases. The majority of the included studies were published in Asia (67.3%). Most patients were women(65.3%) aged > 65 years (67.3%). Almost all patients had decompensated diabetes upon arrival at the emergency department (97.2%). The most common MRI finding was abnormalities of the basal ganglia (89.2%). There was no difference in patient recovery between treatment with insulin alone and in combination with other medications. Although rare, hyperglycemic chorea is a reversible cause of this syndrome; therefore, hyperglycemia should always be considered in these cases.

Scapular Dyskinesia: How to Differentiate Between Etiologies.

It is important to discuss the importance of synchronous balance between periscapular muscles for scapulothoracic motion and resultant scapulohumeral rhythm. Abnormalities in this balance can lead to scapular dyskinesia and winging, affecting shoulder motion and leading to impingement. Strategies exist to diagnose and differentiate between pathologies such as muscle paralysis (eg, trapezius or serratus anterior) or overactivity (eg, pectoralis minor). The physician should be aware of the role of diagnostic imaging, as well as the unique considerations for patients with Ehlers-Danlos syndrome. Overall, a comprehensive physical examination to accurately diagnose and treat scapular pathologies is particularly important.

Case-Based Understanding of the Role of the Scapula in Common Shoulder Pathologies.

Scapular dyskinesis is an extremely common component of shoulder pathology, especially in the overhead athlete; despite its prevalence, proper diagnosis and management of scapular disorders remains an enigma for many clinicians. An understanding of the contribution of the scapula to pathomechanics and clinical symptoms is necessary to optimize both surgical and nonsurgical treatment of shoulder disorders. Without correction of scapular dysfunction, even effective management of the pathoanatomy is unlikely to produce optimal results. It is important to examine the role of the scapula in common shoulder pathologies and elucidate a case-based treatment strategy for both surgical and nonsurgical shoulder disorders.

Resolving the Dance: A Case Study on Non-Ketotic Hyperglycemic Hemichorea-Hemiballismus in a Patient with Long-Standing Type 2 Diabetes.

BACKGROUND Non-ketotic hyperglycemic hemichorea-hemiballism (HCHB) is a rare complication of diabetes, which is mainly described in case reports. This condition occurs more commonly in older women and is known to be associated with T1 hyperintensity basal ganglia lesions on magnetic resonance imaging (MRI). The pathophysiology of non-ketotic hyperglycemic HCHB is not well defined, although a combination of regional metabolic failure and ischemia due to hyperglycemia is suspected to occur. Treatment entails tight blood glucose control, although antipsychotic medications such as risperidone may be helpful in refractory cases. CASE REPORT We describe a case of a middle-aged man with long-standing type 2 diabetes who experienced 3 weeks of progressive unilateral arm, leg, and face choreiform movements. Laboratory testing performed just prior to symptom onset was notable for a hemoglobin A1C of >15% and a serum blood glucose of 566 mg/dl. MRI revealed diffuse T1 hyperintensity in the left lentiform nucleus. Our patient's insulin regimen was adjusted, resulting in improvement in average serum glucose (A1C of 9.4%). However, his symptoms did not improve significantly. A trial of benzodiazepine was attempted, without success. When risperidone was started, the patient experienced resolution of symptoms. Recurrence of non-ketotic hyperglycemic HCHB while off risperidone has not occurred to date. CONCLUSIONS Non-ketotic hyperglycemic HCHB is a rare but important diagnosis to consider in patients with hyperglycemia and new-onset choreiform movements. Patients with long-standing type 2 diabetes may be affected, especially when glycemic control worsens. When tight blood glucose control does not resolve symptoms, a short course of antipsychotic agents may provide relief.

Diabetic striatopathy in type 1 diabetes mellitus: a rare conundrum.

Diabetic striatopathy is a clinicoradiological syndrome characterised by acute hyperkinetic movement disorder in the form of hemichorea-hemiballism with basal ganglia abnormalities in neuroimaging. The hallmark basal ganglia abnormalities appear as hyperdensities in CT brain and hyperintensities in MRI brain, which could mislead the clinician towards an erroneous diagnosis of cerebral haemorrhage. It is classically described in elderly patients with type 2 diabetes mellitus, and its occurrence in type 1 diabetes is extremely rare. This case report entails the clinical details of a young man in his 20s with type 1 diabetes mellitus who had uncontrolled blood glucose levels and presented with a recent onset of abnormal movements in his left upper and lower limbs. The semiology, biochemistry and radiological investigation findings and treatment are detailed. A clear understanding of the condition could lead to an early diagnosis, spare the patient unnecessary investigations and improve treatment outcomes.

Prediction of dyskinesia in Parkinson's disease patients using machine learning algorithms.

Dyskinesias are non preventable abnormal involuntary movements that represent the main challenge of the long term treatment of Parkinson's disease (PD) with the gold standard dopamine precursor levodopa. Applying machine learning techniques on the data extracted from the Parkinson's Progression Marker Initiative (PPMI, Michael J. Fox Foundation), this study was aimed to identify PD patients who are at high risk of developing dyskinesias. Data regarding clinical, behavioral and neurological features from 697 PD patients were included in our study. Our results show that the Random Forest was the classifier with the best and most consistent performance, reaching an area under the receiver operating characteristic (ROC) curve of up to 91.8% with only seven features. Information regarding the severity of the symptoms, the semantic verbal fluency, and the levodopa treatment were the most important for the prediction, and were further used to create a Decision Tree, whose rules may guide the pharmacological management of PD symptoms. Our results contribute to the identification of PD patients who are prone to develop dyskinesia, and may be considered in future clinical trials aiming at developing new therapeutic approaches for PD.

[A case of a pathological variant of the PRRT2 gene in twins with paroxysmal kinesiogenic dyskinesia]. / Sluchai patologicheskogo varianta gena PRRT2 u bliznetsov s paroksizmal'noi kineziogennoi diskineziei.

Paroxysmal dyskinesia is a clinically and etiologically polymorphic group of diseases, the main clinical manifestation of which is transient attacks of extrapyramidal movements, with different conditions of occurrence. Paroxysmal kinesigenic dyskinesia belongs to the group of primary dyskinesias, which also includes paroxysmal non-kinesigenic dyskinesia and exercise-induced paroxysmal dyskinesia. The most common cause of paroxysmal kinesiogenic dyskinesia is mutations in the PRRT2 gene; in cases of non-kinesiogenic dyskinesia, a mutation in the MR1 gene is detected. The diagnosis of primary dyskinesias causes significant difficulty for clinicians due to the rarity of occurrence, as well as the large spectrum of conditions occurring with paroxysmal motor disorders in childhood. The article describes the clinical observation of 16-year-old twin brothers with transient attacks of dystonic, choreic and ballistic hyperkinesis that suddenly arose during movement. Patients were treated for tics and epilepsy for 12 years. Taking into account the clinical picture - transient attacks of hyperkinesis, their connection with movement, as well as data from video-electroencephalographic monitoring, a diagnosis of paroxysmal kinesiogenic dyskinesia was established, which in a further diagnostic search was confirmed by targeted sequencing of the pathological variant of the PRRT2 gene previously described in patients with kinesiogenic dyskinesia. The administration of carbamazepine, which is the drug of choice in the treatment of this category of patients, has achieved significant control over hyperkinesis in twins. Thus, molecular genetic diagnosis helps confirm the diagnosis of paroxysmal dyskinesias, but careful analysis of the clinical picture, considering the provoking factor, remains the basis of diagnosis.

Dyskinesia and impulsive compulsive behaviour in Parkinson's disease are not related: Insights from a study with a wearable sensor.

INTRODUCTION: Previous studies have suggested an association between Impulsive Compulsive Behaviour (ICB) and dyskinesia in Parkinson's disease (PD). However, none of these studies have employed an objective home-based measure of dyskinesia. OBJECTIVES: To evaluate in advanced PD the relationship between ICB and dyskinesia, objectively measured with a wearable device. METHODS: In this cross-sectional study, ICB and other neuropsychiatric symptoms were assessed by means of structured clinical interview and specific screening instruments. Presence and severity of motor fluctuations and dyskinesia were rated with patient's and clinician's based rating instruments. Motor fluctuations and dyskinesia were also measured at home for 5-days using a validated wearable devise, the Parkinson's KinetiGraph™(PKG). RESULTS: We included 89 subjects with PD (29 females, 62 ± 7 years, disease duration 10.3 ± 4.5), of whom 36 (40%) had ICB. Patients with and without ICB did not differ by presence and severity of dyskinesia measured by clinical scales and PKG. There was no association between the presence of ICB and dyskinesia in the whole sample. CONCLUSION: Our data suggest that ICB and dyskinesia are common but unrelated disorders in advanced PD.

Effect of acupuncture treatment on nonketotic hyperglycemic hemichorea-hemiballismus: a case report.

Nonketotic hyperglycemic hemichorea-hemiballismus is a rare syndrome in the clinic, and treatment is often delayed. Hypoglycemic therapy is the most widely used and effective treatment, but some patients experience a slower improvement. Other symptomatic treatment medicines have some degree of side effects. Acupuncture treatment is beneficial for hemichorea-hemiballismus. A male patient, aged 59 years, first visited our hospital outpatient department due to motor agitation with involuntary movements of the right limb. He had a history of type 2 diabetes mellitus and poor blood glucose control. His serum glucose was 26.5 mmol/L (normal: 4.4-6.1 mmol/L), and magnetic resonance imaging demonstrated an irregular area of high signal intensity in T1-weighted imaging, low signal intensity on T2-weighted imaging, and high signal intensity in the left corpus striatum in T2-FLAIR imaging. Hospitalization was recommended for the patient. After ruling out other possibilities, he was eventually diagnosed with nonketotic hyperglycemic hemichorea-hemiballismus. Intensive glycemic control was immediately started with subcutaneous injection and acupuncture treatment at "governor vessel 13 acupoints", and the involuntary movements completely disappeared on the ninth day of hospitalization. The pathophysiology of nonketotic hyperglycemic hemichorea-hemiballismus is unclear. Different patient histories lead to different brain tissue conditions, and relapses and uncontrolled blood glucose add difficulties to treatment. According to Traditional Chinese Medicine theory, insufficient kidney essence leads to brain dystrophy and causes the symptoms of hemichorea-hemiballismus. Research evidence has shown that acupuncture at "governor vessel 13 acupoints" has a beneficial treatment effect on nonketotic hyperglycemic hemichorea-hemiballismus.

Scapular Dyskinesis in the Athletic Patient: A Sport-Specific Review.

¼: Scapular dyskinesis is an alteration of normal scapular kinematics. It is essential that each patient be evaluated holistically and that sport-related factors be taken into account. ¼: The presentation of scapular dyskinesis may be highly variable depending on the underlying etiology or associated pathology, but the onset of symptoms is often gradual. ¼: Sport-specific literature on scapular dyskinesis is most commonly reported in the context of baseball, swimming, and tennis. Treatment is most often conservative and involves physical therapy directed at the scapular stabilizers.

Content Validation of the Movement Disorder-Childhood Rating Scale (MD-CRS) for Dyskinetic Cerebral Palsy.

BACKGROUND: Dyskinetic cerebral palsy (DCP), a lifelong neurological disorder beginning in early childhood, manifests with hyperkinetic movements and dystonia. The Movement Disorder-Childhood Rating Scale (MD-CRS) is a clinician-reported outcome measure assessing the intensity of movement disorders and their effect on daily life in pediatric patients. Content validity of clinical outcome assessments is key to accurately capturing patient perspective. Evidence demonstrating content validity of the MD-CRS in patients with DCP is needed. This study captures input from patients with DCP and their caregivers regarding the content validity of the MD-CRS. METHODS: This qualitative, noninterventional, cross-sectional study included interviews with children/adolescents (aged six to 18 years) with DCP and caregivers of children with DCP. Participants were asked to describe body regions and daily functions affected by DCP. Caregivers also reviewed MD-CRS Part I to evaluate the relevance of the items and corresponding response options. Descriptions of DCP were coded and mapped to MD-CRS items and response options. Caregiver feedback on MD-CRS Part I was analyzed using inductive content analysis. RESULTS: Eight patients and 12 caregivers were interviewed. Participants confirmed that the body regions and activities listed in the MD-CRS were affected by DCP and that involuntary movements interfered with all motor, oral/verbal, self-care, and video protocol activities. Caregivers endorsed the response options for 12 of 15 items in MD-CRS Part I and suggested clarifications for others. CONCLUSIONS: Participants confirmed that affected body regions and activities listed in the MD-CRS were relevant to their experience with DCP, demonstrating the content validity of this tool in children/adolescents with DCP.

An Apple a Day to Keep the Parkinson's Disease Doctor Away?

It is challenging to reliably assess the motor features of Parkinson's disease in real-time. This has motivated the search for new digital outcomes that can objectively and remotely measure the severity of parkinsonian motor impairments over an extended period of time. The United States Food and Drug Administration (FDA) has recently granted a 510(k) clearance to the Rune Labs Kinematics System, an ambulatory, smartwatch-based monitoring system to remotely track tremor and dyskinesias in persons with Parkinson's disease. The FDA clearance means that this new digital approach can be regarded as being safe for use in daily practice, with acceptable correlations to clinically based measures. However, the immediate implications for clinicians are limited, because it remains to be demonstrated whether the digital signals correlate well to clinically meaningful outcomes at patient level. The impact on research is also restricted for now, as more validation studies are needed before this new digital approach can be used as primary or secondary endpoint in clinical trials. ANN NEUROL 2023;93:681-685.

Development of a Clinical Framework for the Assessment of Dyskinesia and Function in the Upper Limb in Children with Cerebral Palsy.

OBJECTIVE: Dyskinesia in cerebral palsy (CP) is a complex movement disorder that can significantly impact upper limb function. Despite a range of available tools, there is no consensus on best practice assessment of upper limb function in children with CP and dyskinesia. This study aimed to develop a clinical framework for the assessment of the impact of dyskinesia on upper limb function in children with CP. DESIGN: Modified Delphi study using expert consensus. METHODS: An expert panel of six highly experienced Australian therapists ranked assessment tools sourced from existing evidence-based literature using a five-point Likert scale. Tools rated as important for use "most" or "all" of the time, by 80% of respondents were accepted into the framework following two survey rounds and a third stage discussion. RESULTS: Of 21 tools, 12 were included in the framework under five categories: (i) screening for dyskinesia; (ii) measuring the severity of dyskinesia; (iii) Classifying upper limb functional ability; (iv) measuring upper limb functional ability; and (v) measuring upper limb movement. CONCLUSIONS: The framework for assessing the impact of dyskinesia on upper limb function in CP aims to guide clinicians to improve assessment consistency and facilitate individualized goal-directed management. Further studies with a larger number of expert clinicians and researchers will further strengthen the utility of the framework.

The Dyskinesia Impairment Scale, Second Edition: Development, construct validity, and reliability.

AIM: To create a shortened, more user-friendly Second Edition of the Dyskinesia Impairment Scale (DIS-II) to assess dystonia and choreoathetosis, and evaluate its construct validity and reliability. METHOD: Scale development included an online expert meeting (n = 21) and iterative discussions within the research group (n = 6). A Rasch measurement model analysis on DIS scores from individuals with dyskinetic cerebral palsy or inherited/idiopathic dystonia (n = 123, 74 males, mean age 14 years, SD 5 years) was performed to evaluate the construct validity and reliability of the DIS-II. RESULTS: The DIS-II evaluates dystonia and choreoathetosis in action and rest in 11 body regions, with action items scored from 0 to 3 and rest items 0 to 2. The number of videos to record are reduced from 26 to 14 and the items to score are reduced from 144 to 88. Rating scale functioning, goodness-of-fit evaluation, principal component analysis, and targeting met the predefined quality criteria of the study and construct validity was therefore considered good. Furthermore, person reliability indicated that the DIS-II can separate individuals into eight distinct ability levels. INTERPRETATION: The DIS-II provides valid and reliable measures for dystonia and choreoathetosis, and reduces the administration and scoring time compared with the DIS. The DIS-II logit scores (interval level data) enhance comparison over time and between individuals in clinical practice and research. WHAT THIS PAPER ADDS: Compared with the Dyskinesia Impairment Scale (DIS), the shortened edition (DIS-II) requires half of the number of videos to be scored. The DIS-II has a simplified rating scale, requiring scoring of 88 instead of 144 items. The DIS-II has shown excellent reliability and good construct validity. The interval properties of the DIS-II are superior to the ordinal level outcome measures of the DIS.

[Functional (Psychogenic) Movement Disorder Focusing on Involuntary Movements: A Review].

Functional neurological disorders differ from malingering/factitious disorders and are diagnosed based solely on careful history taking and neurological evaluation. Some clinical characteristics, including distractibility, entrainability, and variability are important to identify the positive physical signs that indicate functional alterations. Thorough investigations are not essential to exclude organic pathologies, whereas electrophysiological and radiological findings are sometimes useful. Neurologists play an important role when they explain the diagnosis to the patient. The explanation itself may be therapeutic, when delivered successfully and may help patients to understand the nature and mechanisms underlying their movement disorder. A multidisciplinary team approach, including coordination between rehabilitation therapists and psychotherapists may produce positive treatment outcomes, particularly in movement disorder centers. In this article, we discuss some functional neurological disorders, including those in patients with functional movement disorders (involuntary movements) together with video presentations.

Abdominal Wall Dyskinesia in a Child Presenting as Belly Dancers' Syndrome: A Case Report.

Belly dancer's dyskinesia or syndrome is a rare condition characterized by involuntary, undulating, infrequent diaphragm movements. The etiologies for this disorder include nervous system disorders (peripheral or central), drug-induced, psychological, or idiopathic. This article describes a 10-year-old boy with an underlying psychological stressor who suddenly experienced involuntary abdominal wall movements after salbutamol nebulization. After a detailed history, physical examination, and abdominal ultrasound that revealed rapid rhythmic diaphragm movements, the child was diagnosed with salbutamol-induced belly dancer's dyskinesia with an underlying psychological problem. These movements subsided with medical and psychological therapy for two weeks. Belly dancer's dyskinesia is a complex disorder that is difficult to diagnose but can be managed with medical treatment and psychological counseling alone in a few patients. In contrast, in other cases, surgical intervention may be required. Keywords: case reports; dyskinesias; salbutamol.

Hemiballismus-hemichorea syndrome in an acute rehabilitation setting: two case reports.

BACKGROUND: We report two similar cases of patients diagnosed with hemiballismus-hemichorea syndrome secondary to uncontrolled hyperglycemia. Both patients were treated at an inpatient rehabilitation center and made a significant recovery in both function and activities of daily living. Although hemiballismus-hemichorea syndrome has known pharmacological treatments, little has been reported on the effectiveness of acute rehabilitation on managing and treating this syndrome. CASE PRESENTATION: The first case involves a 44-year-old Hispanic male with past medical history of type 2 diabetes mellitus, hypertension, anxiety, and depression who presented with continuous, uncontrollable, unilateral movements 1 month following a hospital admission for hyperglycemia. Magnetic resonance imaging findings showed lesions in the basal ganglia, confirming the diagnosis of hemiballismus-hemichorea syndrome. The patient was started on antipsychotic medications and antihyperglycemic medications controlling glucose levels, but continued to have hemiballismus symptoms. The second case involves a 78-year-old Haitian female with past medical history of type 2 diabetes mellitus and hypertension who presented with weakness and continuous, involuntary movements in her upper and lower extremities 1 month following a hospital admission for hyperglycemia and encephalopathy. Magnetic resonance imaging findings were positive for bilateral lesions in the basal ganglia, establishing a diagnosis of hemiballismus-hemichorea syndrome. After a 2-week stay at an acute rehabilitation center, both patients made a significant recovery in function and activities of daily living. CONCLUSION: The aim in presenting these cases is to elucidate the etiology and progression of a rare disease process known as hemiballismus-hemichorea syndrome and to provide evidence for the potential positive impact of acute rehabilitation on patients with unresolved hemiballismus-hemichorea following an episode of hyperglycemia.

Motor abnormalities are associated with poor social and functional outcomes in schizophrenia.

BACKGROUND: Up to 50% of patients with schizophrenia are suffering from motor abnormalities, which may contribute to decreased quality of life, impaired work capacity, and a reduced life expectancy by 10-20 years. However, the effect of motor abnormalities on social and global functioning, as well as, functional capacity is not clear. We hypothesized, that the presence of motor abnormalities is associated with poorer functional outcomes in patients with schizophrenia. METHODS: We collected data on 5 different motor abnormalities in 156 patients suffering from schizophrenia spectrum disorders: parkinsonism, catatonia, dyskinesia, neurological soft signs and psychomotor slowing (PS). Additionally, we used three different scales to evaluate the functional outcomes in these patients: the Global Assessment of Functioning (GAF) and the Social and Occupational Functioning Assessment Scale (SOFAS) which use clinicians' judgment; and one using a performance-based measure of functional capacity, the brief version of the UCSD Performance-based Skills Assessment (UPSA-B). RESULTS: Our analysis demonstrated that patients with catatonia (all F > 4.5; p < 0.035) and parkinsonism (all F > 4.9; p < 0.027) scored lower on GAF and SOFAS compared to patients without catatonia and parkinsonism. In contrast, no significant difference on functional outcomes between patients with dyskinesia versus without dyskinesia exist in our study. Furthermore, there are statistically significant negative correlations for parkinsonism and PS with GAF, SOFAS and UPSA-B (all tau are at least -0.152, p-value <0.036). We also found significant negative correlations between catatonia and both GAF & SOFAS (all tau are at least -0.203, p-value<0.001) and between NES and SOFAS (tau = -0.137, p-value = 0.033). CONCLUSION: Here, we showed that four of the most common motor abnormalities observed in schizophrenia were associated with at least one of the patients' functional outcomes. The stronger the motor impairment was the worse the global and social functioning. Future studies need to test, whether amelioration of motor abnormalities is linked to improved community functioning.