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Cleveland Clinic Journal of Medicine Jul 2012The management of choreic disorders presents significant challenges, including identifying the etiology of the disorder, treating and preventing motor symptoms, and... (Review)
Review
The management of choreic disorders presents significant challenges, including identifying the etiology of the disorder, treating and preventing motor symptoms, and managing a range of other neurologic and behavioral complications. Chorea may occur in several neurodegenerative, genetic, or drug-related conditions, and a thorough diagnostic evaluation is needed to identify the specific underlying causes. Some choreic disorders have specific treatable underlying etiologies, such as vitamin B(12) deficiency or drug-induced dyskinesia. Autoimmune disorders such as Sydenham chorea may be treated with penicillin, corticosteroids, intravenous immunoglobulin, or plasma exchange. Heredodegenerative choreas such as Huntington disease often respond to treatment with tetrabenazine or amantadine. Many other agents may be used nonspecifically for symptom control, including benzodiazepines, neuroleptics, and antiepileptic medications. In addition to motor symptoms, patients with Huntington disease or other choreic disorders often experience increasing depression, bradykinesia, cognitive impairment, aggressive behaviors, and other complications as the disease progresses. Caring for the caregiver is also a significant concern in the long-term treatment of choreic disorders.
Topics: Chorea; Humans; Huntington Disease
PubMed: 22761268
DOI: 10.3949/ccjm.79.s2a.06 -
Artificial Intelligence in Medicine Feb 2016After several years of treatment, patients with Parkinson's disease (PD) tend to have, as a side effect of the medication, dyskinesias. Close monitoring may benefit...
BACKGROUND
After several years of treatment, patients with Parkinson's disease (PD) tend to have, as a side effect of the medication, dyskinesias. Close monitoring may benefit patients by enabling doctors to tailor a personalised medication regimen. Moreover, dyskinesia monitoring can help neurologists make more informed decisions in patient's care.
OBJECTIVE
To design and validate an algorithm able to be embedded into a system that PD patients could wear during their activities of daily living with the purpose of registering the occurrence of dyskinesia in real conditions.
MATERIALS AND METHODS
Data from an accelerometer positioned in the waist are collected at the patient's home and are annotated by experienced clinicians. Data collection is divided into two parts: a main database gathered from 92 patients used to partially train and to evaluate the algorithms based on a leave-one-out approach and, on the other hand, a second database from 10 patients which have been used to also train a part of the detection algorithm.
RESULTS
Results show that, depending on the severity and location of dyskinesia, specificities and sensitivities higher than 90% are achieved using a leave-one-out methodology. Although mild dyskinesias presented on the limbs are detected with 95% specificity and 39% sensitivity, the most important types of dyskinesia (any strong dyskinesia and trunk mild dyskinesia) are assessed with 95% specificity and 93% sensitivity.
CONCLUSION
The presented algorithmic method and wearable device have been successfully validated in monitoring the occurrence of strong dyskinesias and mild trunk dyskinesias during activities of daily living.
Topics: Accelerometry; Antiparkinson Agents; Dyskinesias; Humans; Levodopa; Monitoring, Physiologic; Parkinson Disease; Support Vector Machine
PubMed: 26831150
DOI: 10.1016/j.artmed.2016.01.001 -
Epileptic Disorders : International... Jun 2002Recognition and classification of neonatal seizures remain problematic, particularly when clinicians rely only on clinical criteria. Physicians should utilize... (Review)
Review
Recognition and classification of neonatal seizures remain problematic, particularly when clinicians rely only on clinical criteria. Physicians should utilize synchronized video-EEG-polygraphic recordings to correlate suspicious behaviors with electrographic seizures, to help limit misdiagnosis and overtreatment of either normal or abnormal nonepileptic behaviors. Since neonatal seizures, particularly status epilepticus, predict an increased risk for later epilepsy and other neurological sequelae, accurate diagnoses are needed for aggressive antiepileptic drug use in the NICU, as well as family counseling and anticipatory medical care after discharge. Neurophysiological documentation of neonatal seizures also must be integrated with an appreciation of pathophysiological mechanisms responsible for brain lesions that cause seizures. Maternal-fetal-placental diseases, as well as genetic vulnerabilities may be responsible for seizures long before diagnosis in the newborn period. Alternatively, the severity of seizures in neonates with perinatal asphyxia or other etiologies may be independently associated with brain injury. Seizures in the newborn are one of the few, neonatal neurological emergencies. While prompt diagnostic and therapeutic plans are necessary, unresolved medical issues continue to challenge the physician's evaluation of the newborn with suspected seizures (table I) [1-3]. Recognition of the seizure state remains the foremost challenge to overcome. Clinical and electroencephalographic manifestations of neonatal seizures vary dramatically from those of older children. This generalization is underscored by the brevity and subtlety of the clinical repertoire of the newborn's neurological examination. Environmental restrictions surrounding the sick infant in an intensive care setting, including confinement within an isolette, intubation and attachment to multiple catheters limit accessibility. Medication alters arousal and muscle tone, which further limit the clinician's ability to consider these clinical neurological signs as surrogate markers of underlying brain disorders. Brain injury from antepartum factors may later be expressed as neonatal seizures, as part of a postnatal, encephalopathic clinical picture which may only be precipitated by stress during the intrapartum and neonatal periods [4]. Alternatively, medical conditions during parturition or after birth may cause seizures, with coincident injury. For medication options to effectively treat seizures, new agents need to be designed for specific etiologies, timing of injury, and the unique cellular/molecular organization of the immature brain. This review addresses the challenges and controversies regarding neonatal seizure recognition in the context of current clinical practices. Experimental models of neonatal seizures elucidate the pathophysiological mechanisms and adverse consequences of seizures on brain development. Since 20% to 50% of children with neonatal seizures experience later epilepsy, diagnostic accuracy is essential [5, 6], particularly for the newborn who suffers a prolonged seizure state.
Topics: Brain; Diagnosis, Differential; Dyskinesias; Electroencephalography; Epilepsy; Humans; Infant, Newborn; Seizures; Time Factors
PubMed: 12105077
DOI: No ID Found -
Tidsskrift For Den Norske Laegeforening... Sep 2008Early Parkinson's disease is dominated by a motor syndrome called parkinsonism, but as the disease develops motor complications and non-motor problems may occur as well.... (Review)
Review
BACKGROUND
Early Parkinson's disease is dominated by a motor syndrome called parkinsonism, but as the disease develops motor complications and non-motor problems may occur as well. This paper describes how to diagnose Parkinson's disease and the various motor complications and gives recommendations on how to treat the symptoms in these patients.
MATERIAL AND METHODS
The paper builds on international evidence-based publications and the Norwegian guidelines for treatment of Parkinson's disease.
RESULTS AND INTERPRETATION
Motor symptoms such as tremor at rest, akinesia, rigidity and postural instability are the cardinal signs in Parkinson's disease. After diagnosing a patient with the disease we recommend to start with selegiline as a disease-modifying treatment strategy. When symptoms lead to functional impairment, symptomatic treatment should be started in addition. Dopamine agonists are primarily recommended in younger patients and levodopa in the older ones. When the patients develop motor complications it is important to first thoroughly evaluate the problems to arrive at the best possible treatment strategy. If a sufficient response is not obtained both deep brain stimulation and treatment with continuous delivery of medication should be considered.
Topics: Age Factors; Antiparkinson Agents; Deep Brain Stimulation; Dopamine Agonists; Dyskinesias; Dystonia; Humans; Levodopa; Muscle Rigidity; Parkinson Disease; Selegiline; Treatment Outcome; Tremor
PubMed: 18846123
DOI: No ID Found -
BMJ Case Reports Feb 2021A 10-year-old girl presented with a month long history of episodic limb movements. She had a normal neurological examination and after thorough investigation, she was...
A 10-year-old girl presented with a month long history of episodic limb movements. She had a normal neurological examination and after thorough investigation, she was thought to have possible tics. Anxiety was reported as being a trigger. Unusually, these 'tics' were not directly witnessed during hospital visits. Eighteen months after the initial presentation, the clinician observed dystonic posturing after the child stood up from having been seated during a consultation. Paroxysmal kinesigenic dyskinesia (PKD) was then suspected and confirmed on genetic testing. She was successfully treated with carbamazepine. In hindsight, it became apparent that her anxiety was related to a fear of uncontrolled movements, rather than it being a trigger. The abnormal involuntary movements in PKD are precipitated by sudden voluntary movement. Lack of recognition of this typical feature, normal examination and/or features such as coexisting anxiety can lead to misdiagnosis or delayed diagnosis of this easily treatable condition.
Topics: Anticonvulsants; Carbamazepine; Child; Diagnosis, Differential; Dystonia; Female; Humans
PubMed: 33547116
DOI: 10.1136/bcr-2020-235112 -
Journal of Endocrinological... Jan 2024Diabetic striatopathy (DS) is a rare complication of poorly controlled diabetes mellitus (DM), characterized by hyperglycemia associated with chorea/ballism and... (Review)
Review
PURPOSE
Diabetic striatopathy (DS) is a rare complication of poorly controlled diabetes mellitus (DM), characterized by hyperglycemia associated with chorea/ballism and characteristic reversible basal ganglia abnormalities on computed tomography (CT) and/or magnetic resonance imaging (MRI). We propose a narrative review of the literature on this topic, currently unknown to most, and about which physicians should be aware. We intend to summarize, critically review, and take to mean the evidence on this disorder, describing its typical features.
METHODS
We searched Pubmed for English-language sources using the following keywords in the title and the abstract: diabetic striatopathy, hyperglycemic non-ketotic hemichorea/hemiballism, chorea/hemichorea associated with non-ketotic hyperglycemia, diabetic hemiballism/hemichorea, chorea, hyperglycemia, and basal ganglia syndrome. We collected scientific articles, including case reports, reviews, systematic reviews, and meta-analyses from the years 1975 to 2023. We eliminated duplicate, non-English language or non-related articles.
RESULTS
Older Asian women are more frequently affected. Suddenly or insidiously hemichorea/hemiballism, mainly in the limbs, and high blood glucose with elevated HbA1c in the absence of ketone bodies have been observed. Furthermore, CT striatal hyperdensity and T1-weighted MRI hyperintensity have been observed. DS is often a treatable disease following proper hydration and insulin administration. Histopathological findings are variable, and no comprehensive hypothesis explains the atypical cases reported.
CONCLUSION
DS is a rare neurological manifestation of DM. If adequately treated, although treatment guidelines are lacking, the prognosis is good and life-threatening complications may occur occasionally. During chorea/hemiballism, we recommend blood glucose and HbA1c evaluation. Further studies are needed to understand the pathogenesis.
Topics: Humans; Female; Chorea; Blood Glucose; Glycated Hemoglobin; Dyskinesias; Diabetes Mellitus; Magnetic Resonance Imaging; Hyperglycemia
PubMed: 37578646
DOI: 10.1007/s40618-023-02166-5 -
The Cochrane Database of Systematic... 2003Abnormal involuntary movements known as dyskinesias are amongst the most disabling side-effects of levodopa therapy. It is thought that amantadine, an NMDA-receptor... (Review)
Review
BACKGROUND
Abnormal involuntary movements known as dyskinesias are amongst the most disabling side-effects of levodopa therapy. It is thought that amantadine, an NMDA-receptor antagonist, may reduce dyskinesias in patients with Parkinson's disease without worsening Parkinsonian symptoms.
OBJECTIVES
To compare the efficacy and safety of adjuvant amantadine therapy versus placebo in treating dyskinesia in patients with Parkinson's disease, already established on levodopa, and suffering from motor complications.
SEARCH STRATEGY
Electronic searches of The Cochrane Controlled Trials Register (The Cochrane Library Issue 3, 2001), MEDLINE (1966-2001), EMBASE (1974-2001), SCISEARCH (1974-2001), BIOSIS (1993-2001), GEROLIT (1979-2001), OLDMEDLINE (1957-1965), LILACS (1982-2001), MedCarib (17th Century - 2001), PASCAL (1973-2001), JICST-EPLUS (1985-2001), RUSSMED (1973-2001), DISSERTATION ABSTRACTS (2000-2001), SIGLE (1980-2001), ISI-ISTP (1990-2001), Aslib Index to Theses (2001), Clinicaltrials.gov (2001), metaRegister of Controlled Trials (2001), NIDRR (2001) and NRR (2001) were conducted. Grey literature was hand searched and the reference lists of identified studies and reviews examined. The manufacturers of amantadine were contacted.
SELECTION CRITERIA
Randomised controlled trials comparing amantadine with placebo in the treatment of dyskinesia in patients with a clinical diagnosis of idiopathic Parkinson's disease.
DATA COLLECTION AND ANALYSIS
Data was abstracted independently by NC and KD onto standardised forms and disagreements were resolved by discussion.
MAIN RESULTS
Three randomised controlled trials were found comparing amantadine with placebo in the treatment of dyskinesia in patients with idiopathic Parkinson's disease. Three trials were excluded on the basis that they had no control group and a further three did not state whether they randomised the treatment that participants received. The included trials were double-blind cross-over studies involving a total of 53 patients. All three studies failed to present data from the first arm, instead presenting results as combined data from both treatment arms and both placebo arms. Two trials had no wash-out interval between the treatment periods. In view of the risk of a carry-over effect into the second arm, the results of these trials were not analysed. The final trial had a one week wash-out interval but only examined 11 participants. One study reported side-effects of amantadine in 8 of the 18 participants, including confusion and worsening of hallucinations. Another reported reversible edema of both feet in one of eleven participants.
REVIEWER'S CONCLUSIONS
Due to lack of evidence it is impossible to determine whether amantadine is a safe and effective form of treatment for levodopa-induced dyskinesias in patients with Parkinson's disease.
Topics: Amantadine; Antiparkinson Agents; Dyskinesias; Humans; Parkinson Disease; Randomized Controlled Trials as Topic
PubMed: 12804468
DOI: 10.1002/14651858.CD003467 -
Journal of Veterinary Internal Medicine 2023Autoimmune mechanisms represent a novel category for causes of seizures and epilepsies in humans, and LGI1-antibody associated limbic encephalitis occurs in cats.
BACKGROUND
Autoimmune mechanisms represent a novel category for causes of seizures and epilepsies in humans, and LGI1-antibody associated limbic encephalitis occurs in cats.
HYPOTHESIS/OBJECTIVES
To investigate the presence of neural antibodies in dogs with epilepsy or dyskinesia of unknown cause using human and murine assays modified for use in dogs.
ANIMALS
Fifty-eight dogs with epilepsy of unknown cause or suspected dyskinesia and 57 control dogs.
METHODS
Serum and CSF samples were collected prospectively as part of the diagnostic work-up. Clinical data including onset and seizure/episode type were retrieved from the medical records. Screening for neural antibodies was done with cell-based assays transfected with human genes for typical autoimmune encephalitis antigens and tissue-based immunofluorescence assays on mouse hippocampus slices in serum and CSF samples from affected dogs and controls. The commercial human und murine assays were modified with canine-specific secondary antibody. Positive controls were from human samples.
RESULTS
The commercial assays used in this study did not provide unequivocal evidence for presence of neural antibodies in dogs including one dog with histopathologically proven limbic encephalitis. Low titer IgLON5 antibodies were present in serum from one dog from the epilepsy/dyskinesia group and in one dog from the control group.
CONCLUSION AND CLINICAL IMPORTANCE
Specific neural antibodies were not detected using mouse and human target antigens in dogs with epilepsy and dyskinesia of unknown origin. These findings emphasize the need for canine-specific assays and the importance of control groups.
Topics: Humans; Dogs; Animals; Mice; Cats; Limbic Encephalitis; Epilepsy; Antibodies; Seizures; Dyskinesias; Dog Diseases; Cell Adhesion Molecules, Neuronal; Cat Diseases
PubMed: 37232512
DOI: 10.1111/jvim.16744 -
Tremor and Other Hyperkinetic Movements... 2022Chorea can be due to a large number of etiologies. Unilateral chorea is classically related to a contralateral structural lesion, e.g. of the putamen or subthalamic... (Review)
Review
BACKGROUND
Chorea can be due to a large number of etiologies. Unilateral chorea is classically related to a contralateral structural lesion, e.g. of the putamen or subthalamic nucleus, however, based upon personal impressions, we have observed that systemic disease, in particular metabolic or autoimmune conditions, can also lead to a unilateral or markedly asymmetric presentations. We sought to investigate this impression by reviewing the literature.
METHODS
A PubMed search was conducted using the terms asymmetric" AND "chorea" OR "hemichorea" OR "unilateral" AND "chorea" OR "monochorea" OR "right greater than left" AND "chorea" OR "left greater than right" AND "chorea" OR "right more than left" AND "chorea" OR "left more than right" AND "chorea" as well as "hemiballismus" NOT "stroke" NOT "infarct" NOT "dyskinesia. A total of 243 sources were felt to meet criteria and were reviewed.
RESULTS
The most common etiology of reported hemi- or asymmetric chorea was diabetic non-ketotic hyperglycemic hemichorea/hemiballismus. Other common diagnoses were Sydenham's disease, antiphospholipid syndrome and drug-induced chorea. The vast majority of patients with hemi- or asymmetric chorea had acquired rather than genetic, degenerative or congenital causes.
CONCLUSION
Despite the potential limitations of our literature review, the evidence presented here supports the observation that the vast majority of asymmetric or unilateral chorea presentations are due to acquired causes, and in this situation an exhaustive search for reversible etiology should be undertaken. However, presentation with symmetric, generalized chorea does not exclude reversible causes, and investigations should address these in addition to genetic and neurodegenerative etiologies.
Topics: Chorea; Dyskinesias; Humans; Movement Disorders; Putamen; Subthalamic Nucleus
PubMed: 35136702
DOI: 10.5334/tohm.675 -
Revista de NeurologiaThe clinical, pathophysiological and genetic features of some of the paroxysmal movement disorders are reviewed. Paroxysmal idiopathic dyskinesias are genetically... (Review)
Review
AIM AND DEVELOPMENT
The clinical, pathophysiological and genetic features of some of the paroxysmal movement disorders are reviewed. Paroxysmal idiopathic dyskinesias are genetically heterogeneous. Although some motor manifestations of epilepsy and paroxysmal dyskinesia may be difficult to differentiate clinically, the current understanding is that the two disorders are clinically distinct. However there are several recent reports of families in which different individuals have either disorder or both manifestations, with age-related expressions. Co-occurrence makes it likely that a common, genetically determined pathophysiologic abnormality is variably expressed in the cerebral cortex and in basal ganglia.
CONCLUSION
The pathophysiology of paroxysmal dyskinesia is unknown. To date, despite strong suspicions, no ion channel gene mutations have been isolated.
Topics: Child; Dyskinesias; Humans
PubMed: 14730492
DOI: No ID Found