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hyperkinetic disorders

Navneet Kaur, Puneet Kumar, Sumit Jamwal, Rahul Deshmukh, Vinod Gauttam
BACKGROUND: Tetrabenazine (TBZ) is the only US Food and Drug Administration-approved drug for the treatment of chorea related to Huntington's disease and other hyperkinetic disorders. TBZ was first synthesized in 1950, and was then used for the treatment of psychosis. But later its potential in treating hyperkinetic disorders was proved by its ability to block vesicular monoamine transporters 2 and deplete monoamine stores. There is still lack of awareness about the therapeutic potential of this drug...
September 2016: Annals of Neurosciences
Hortensia Gimeno, Jean-Pierre Lin
The multidisciplinary team (MDT) approach illustrates how motor classification systems, assessments and outcome measures currently available have been applied to a national cohort of children and young people with dystonia and other hyperkinetic movement disorders (HMD) particularly with a focus on dyskinetic cerebral palsy (CP). The paper is divided in 3 sections. Firstly, we describe the service model adopted by the Complex Motor Disorders Service (CMDS) at Evelina London Children's Hospital and King's College Hospital (ELCH-KCH) for deep brain stimulation...
September 8, 2016: European Journal of Paediatric Neurology: EJPN
Yilmaz Yildiz, Emine Pektas, Aysegul Tokatli, Goknur Haliloglu
Hereditary dopamine transporter deficiency syndrome (DTDS) is a neurotransmitter disorder caused by a defect in the neuronal uptake of dopamine. To date, 20 patients are reported in the literature, and we present 2 additional patients with DTDS harboring novel homozygous SLC6A3 gene mutations. Patient A is an 8-month-old male with neonatal-onset hypotonia, who developed orolingual dyskinetic movements and oculogyric crises after 4 months of age, with evolution to status dystonicus episodes. Patient B is a 4-year-old male who also had hypotonia since birth, with additional severe limb contractions and oculogyric crises after the age of 3 months, with a misdiagnosis of epileptic encephalopathy...
September 30, 2016: Neuropediatrics
Ujjawal Roy, Shyamal Kumar Das, Adreesh Mukherjee, Debsadhan Biswas, Koushik Pan, Atanu Biswas, Ajay Panwar
BACKGROUND: Hemichorea-hemiballism (HCHB) is a hyperkinetic movement disorder with features of both chorea and ballism occurring on the same side. CASE REPORT: We present a case of HCHB due to nonketotic hyperglycemia (NKH) that was the initial presentation of diabetes and was irreversible clinically even after 6 months of optimal blood sugar control. DISCUSSION: Although HCHB due to hyperglycemia is a potentially reversible condition in the majority of patients, prolonged uncontrolled hyperglycemia may cause ischemic insult and persistent symptoms...
2016: Tremor and Other Hyperkinetic Movements
David L Jung, Peter Hwang
No abstract text is available yet for this article.
September 2016: PM & R: the Journal of Injury, Function, and Rehabilitation
Zoé Senta-Loys, Sandrine Bourgeois, Cyril Pailler-Mattei, Géraldine Agusti, Stéphanie Briançon, Hatem Fessi
OBJECTIVES: Orodispersible films (ODF) were formulated to facilitate tetrabenazine (TBZ) administration to paediatric population for the treatment of hyperkinetic movement disorders. METHODS: ODF were obtained by solvent casting/evaporation method using four different polymers (HPMC, PVP, pullulan and HEC). Physicochemical, mechanical and biopharmaceutical characterizations as well as API state in ODF by thermal analysis were investigated to define and compare formulations...
September 27, 2016: Journal of Pharmacy and Pharmacology
Howard D Weiss
Hyperkinetic movements, such as tremor, myoclonus, chorea, and dystonia, occur in many neurologic and medical conditions. Accurate clinical evaluation is the important first step for the proper diagnosis and treatment of patients with abnormal movements.
August 2016: Seminars in Neurology
Bi-Chun Duan, Wen-Chin Weng, Kuang-Lin Lin, Lee Chin Wong, Sung-Tse Li, Mei-Hsin Hsu, Jainn-Jim Lin, Pi-Chaun Fan, Ming-I Lin, Nan-Chang Chiu, Yu-Ching Lin, Huei-Shyong Wang, Kun-Long Hung, Wang-Tso Lee
Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is one of the most common autoimmune encephalitis that presents with a wide variety of movement disorders. The purpose of our study is to review the manifestations and duration of movement disorders in different ages with NMDAR encephalitis.A retrospective cohort of 28 patients (20 females and 8 males) with positive cerebrospinal fluid (CSF) anti-NMDAR antibody in a 5-year period from major hospitals in Taiwan was enrolled. They were categorized into 3 age groups: 7 patients were ≤10 years, 14 patients were 10 to 18 years, and 7 patients were >18 years...
September 2016: Medicine (Baltimore)
Leonie A Menke, Marc Engelen, Mariel Alders, Vincent J J Odekerken, Frank Baas, Jan M Cobben
In 2 unrelated patients with axial hypotonia, developmental delay and a hyperkinetic movement disorder, a missense mutation was found in codon 209 of the GNAO1 gene. From the still scarce literature on GNAO1 mutations, a clear genotype-phenotype correlation emerged. From the 26 patients reported thus far, 12 patients had epileptic encephalopathy, and 14 had a developmental delay and a hyperkinetic movement disorder. All but 1 of the latter patients had missense mutations in GNAO1 codon 209 or 246, which thus appear to be mutation hotspots...
September 12, 2016: Journal of Child Neurology
Carsten Schröder, Michael Dörks, Bianca Kollhorst, Tilo Blenk, Ralf W Dittmann, Edeltraut Garbe, Oliver Riedel
Studies from different countries showed increasing use of antipsychotics in pediatric patients. However, these studies were methodologically limited and could not assess underlying diagnoses and off-label use sufficiently. This is the first study to examine antipsychotic prescriptions in a representative sample of minors over a long period, looking at changes regarding substances and drug classes, underlying diagnoses, and the rate of off-label use. Claims data of about two million pediatric subjects were used to calculate annual prevalences and incidence rates of antipsychotic prescriptions for the years 2004-2011...
September 13, 2016: European Child & Adolescent Psychiatry
Alexandre Kreisler, Anne-Caroline Verpraet, Solène Veit, Odile Pennel-Ployart, Hélène Béhal, Alain Duhamel, Alain Destée
Purpose: To better define the clinical characteristics of idiopathic oromandibular dystonia, we studied voice, speech, and swallowing disorders and their impact on activities of daily living. Method: Fourteen consecutive patients with idiopathic oromandibular dystonia and 14 matched, healthy control subjects were included in the study. Results: Dysarthria was the most common disorder and its characteristics varied from one patient to another...
September 8, 2016: Journal of Speech, Language, and Hearing Research: JSLHR
Madhu Nagappa, Parayil Sankaran Bindu, Shwetha Chiplunkar, Periasamy Govindaraj, Gayathri Narayanappa, Ayyappan Krishnan, M M Srinivas Bharath, Aarthi Swaminathan, Jitender Saini, Hanumanthapura R Arvinda, Sanjib Sinha, Pavagada S Mathuranath, Arun B Taly
Deficiency of gamma-amino-butyrate aminotransferase (ABAT) is a rare inherited disorder. A six-month-old girl presented with hyper-somnolence, hyperkinetic movements of distal extremities during wakefulness, hypotonia, bi-pyramidal signs, and impaired response to sound and visual stimuli. Brain MRI at five months showed restricted diffusion along the internal capsule and genu of corpus callosum. A follow up MRI at 18months, showed hyperintensities in brainstem, external and internal capsule, 'trilaminated' appearance of posterior limb of internal capsule and dysmyelination of sub-cortical white matter...
September 2, 2016: Brain & Development
Noreen Samad, Muhammad Abdul Haleem, Darakhshan Jabeen Haleem
Effect of administration of Rice bran oil (RBO) was evaluated on haloperidol elicited tardive dyskinesia in rats. Albino Wistar rats treated with haloperidol in drinking water at a dose of 0.2mg/kg/day and RBO by oral tubes at a dose of 0.4 mL/day for 5 weeks. Motor coordination, VCMs and 8-hydroxy-2-(di-n-propylamino) tetraline)[8-OH-DPAT] _syndrome were monitored. Striatal serotonin (5-hydroxytryptamine; 5-HT) and 5-hydroxyindolacetic acid (5-HIAA) levels were determined by high performance liquid chromatography (HPLC-EC)...
July 2016: Pakistan Journal of Pharmaceutical Sciences
Lucia Ricciardi, Michela Ebreo, Adriana Graziosi, Marianna Barbuto, Chiara Sorbera, Letterio Morgante, Francesca Morgante
INTRODUCTION: Speech disturbances in Parkinson's disease (PD) are heterogeneous, ranging from hypokinetic to hyperkinetic types. Repetitive speech disorder has been demonstrated in more advanced disease stages and has been considered the speech equivalent of freezing of gait (FOG). We aimed to verify a possible relationship between speech and FOG in patients with PD. METHODS: Forty-three consecutive PD patients and 20 healthy control subjects underwent standardized speech evaluation using the Italian version of the Dysarthria Profile (DP), for its motor component, and subsets of the Battery for the Analysis of the Aphasic Deficit (BADA), for its procedural component...
August 15, 2016: Parkinsonism & related Disorders
Toni S Pearson
BACKGROUND: The autosomal recessive ataxias are a heterogeneous group of disorders that are characterized by complex neurological features in addition to progressive ataxia. Hyperkinetic movement disorders occur in a significant proportion of patients, and may sometimes be the presenting motor symptom. Presentations with involuntary movements rather than ataxia are diagnostically challenging, and are likely under-recognized. METHODS: A PubMed literature search was performed in October 2015 utilizing pairwise combinations of disease-related terms (autosomal recessive ataxia, ataxia-telangiectasia, ataxia with oculomotor apraxia type 1 (AOA1), ataxia with oculomotor apraxia type 2 (AOA2), Friedreich ataxia, ataxia with vitamin E deficiency), and symptom-related terms (movement disorder, dystonia, chorea, choreoathetosis, myoclonus)...
2016: Tremor and Other Hyperkinetic Movements
Fu-Jung Hsiao, Wan-Yu Hsu, Wei-Ta Chen, Rou-Shayn Chen, Yung-Yang Lin
Paroxysmal kinesigenic dyskinesia (PKD) is a rare group of hyperkinetic movement disorders characterized by brief attacks of choreoathetosis or dystonia. To clarify the alterations of the functional connectivity within the somatosensory network in PKD patients, magnetoencephalographic (MEG) responses to paired median-nerve electrical stimulation were recorded in 10 PKD patients treated by carbamazepine or oxcarbamazepine and 22 age-matched controls. In patients, MEG recordings were obtained during drug-on and -off periods...
August 11, 2016: Clinical EEG and Neuroscience: Official Journal of the EEG and Clinical Neuroscience Society (ENCS)
Mihaela Boca, Katie Lloyd, Marcus Likeman, Philip Jardine, Alan Whone
A previously well 16-year-old boy developed a rapid-onset hypokinetic syndrome, coupled with a radiological appearance of extensive and highly symmetrical basal ganglia and white matter change. The diagnostic process was challenging and we systematically considered potential causes. After excluding common causes of this clinico-radiological picture, we considered common disorders with this unusual radiological picture and vice versa, before finally concluding that this was a rare presentation of a rare disease...
August 8, 2016: Practical Neurology
José Fidel Baizabal-Carvallo, Marlene Alonso-Juarez
INTRODUCTION: Traditionally, deep brain stimulation (DBS) for movement disorders (MDs) is provided using stimulation frequencies equal to or above 100 Hz. However, recent evidence suggests that relatively low-frequency stimulation (LFS) below 100 Hz is an option to treat some patients with MDs. OBJECTIVES: We aimed to review the clinical and pathophysiological evidence supporting the use of stimulation frequencies below 100 Hz in different MDs. RESULTS: Stimulation of the subthalamic nucleus at 60 Hz has provided benefit in gait and other axial symptoms such as swallowing and speech...
October 2016: Parkinsonism & related Disorders
L Defebvre, P Krystkowiak
Stroke may be associated with different types of movement disorders, such as hyperkinetic syndromes (hemichorea-hemiballism, unilateral asterixis, limb-shaking, dystonia, tremor, myoclonus) and hypokinetic syndromes (especially vascular parkinsonism). However, movement disorders are rare and transient in acute stroke and, as a permanent consequence, are more often delayed. While ischemic and hemorrhagic strokes can happen at any level of the frontal-subcortical motor system, they can be explained most of the time by a dysfunction in the basal ganglia motor circuit...
August 2016: Revue Neurologique
T A Kupriyanova, E V Koren'
A clinical case of a child with hyperkinetic conduct disorder is presented, with symptoms of hyperkinetic disorder itself closely intertwining with symptoms of conduct disorder. The qualitative changes in the child's condition are noted, including not only achieved control over the symptoms, but also improved social functioning level, achieved by means of Pantogam (neuroprotective drug) addition to atomoxetine therapy. The proposed strategy can contribute to improvement of treatment results for children with certain clinical types of hyperkinetic conduct disorder...
2016: Zhurnal Nevrologii i Psikhiatrii Imeni S.S. Korsakova
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