Index A Bon-bon sign , 202 Acute akathisia , 193–195 Botulinum neurotoxin (BoNT) , 72–73 Acute dystonic reactions Brainstem myoclonus, in childhood , 242 diagnosis , 193 Bucco-linguo-masticatory triad , 202 incidence , 192 Bursting , 9, 10, 12, 16 risk of , 192 Alpha-2 adrenergic agonists , 98 Altered receptive fi eld fi , 10 C Anticholinergic medications , 13, 69 ChAc. See Chorea-acanthocytosis (ChAc) Antisocial and oppositional behaviors, TS Chemodenervation, dystonia , 72–74 description , 92 Chorea treatment , 100–101 autoimmune choreas Anxiety and depression chorea gravidarum (CG) , 41 description , 93 sydenham chorea , 39–41 treatment , 100 benign chorea , 38–39 Aspiration pneumonia , 28 in childhood Attention defi cit hyperactivity disorder causes , 228 (ADHD) ego-dystonic movements , 227 description , 90–91 evaluation , 230–231 treatment , 100–101 features , 227 Autistic features, TS , 90 symptomatic treatment , 231–232 Autoimmune choreas defi nition and clinical manifestation fi , chorea gravidarum , 41 25–26 sydenham chorea , 39–41 differential diagnosis , 26–28 Autosomal dominant nocturnal frontal lobe drug-induced chorea , 43 epilepsy (ADNFLE) , 155 epidemic disorders , 25 genetic chorea ( see Genetic choreas) infectious chorea , 42 B metabolic/toxic choreas , 43 Basal ganglia and thalamocortical circuits origin , 25 anatomy of , 2–4 types , 25 neurochemistry , 5–7 vascular , 42 functional role , 5 Chorea-acanthocytosis (ChAc) , 34–35 physiological changes , 9–10 Choreoathetosis, in childhood Behavior therapy, TS , 99 description , 239 Benign hereditary choreas (BHC) , 38–39 etiology and evaluation , 240–241 Blepharospasm , 59, 65, 70, 73, 75, 202 treatment , 241 O. Suchowersky and C. Comella (eds.), Hyperkinetic Movement Disorders, 283 Current Clinical Neurology, DOI 10.1007/978-1-60327-120-2, © Springer Science+Business Media New York 2012 284 Index Chronic motor tic disorder (CMTD) , 87 myoclonus and asterixis , 199 Clinically established PMD , 173 neuroleptic malignant syndrome , 195–197 Clinically proven PMDs , 174 tardive dyskinesia ( see Tardive Clonazepam, myoclonus , 138 dyskinesia (TD)) Cortical myoclonus tremor , 198 in childhood , 242 Dystonia description , 125 in childhood causes , 235–237 defi nition fi , 232 D diffi culties fi , 233 Darting , 26, 202 hyperkinetic form , 233 Daytime bruxism , 194, 198 hypertonic form , 232 Deep brain stimulation (DBS) treatment , 238–239 choreoathetotic cerebral palsy , 241 classifi cation fi dystonia , 75–76, 235, 239, 260 age of onset , 59 dystonic storm , 72 disease distribution , 59–60 Huntingdon disease, 33Levodopa-induced disease etiology , 60–65 dyskinesia , 201 defi nition fi , 57 paroxysmal nonkinesigenic diagnosis dyskinesia , 156 primary dystonia , 65 posthypoxic myoclonus (Lance–Adams’ secondary dystonia , 65–67 Syndrome) , 128 dystonic storm , 58 psychogenic movement disorders geste antagoniste , 58 (PMDs) , 164 null point , 58 surgery , 272 paradoxical dystonia , 58 tardive dyskinesia (TD) , 205–206, 210 pathophysiology , 11–13 tremor treatment , 250 patterning , 57 uncontrolled tics , 99 surgery Delayed hyperkinetic movement disorders , clinical trials , 269–271 199–200 peripheral procedure , 263 Dentatorubral-pallidoluysian atrophy stereotactic procedure , 263–269 (DRPLA) , 33–34 tarda , 202 Depriming effect , 208 treatment Diphasic dyskinesia , 200 chemodenervation , 72–74 Disruptive behaviour, ADHD , 91 medication therapy , 68–72 Documented PMD , 172–173 physical therapy and assisting devices , Dopamine-modulating agents , 67–68 97–98 surgical intervention , 74–76 Dopa-responsive dystonia (DRD) , 147, 150, Dystonic storm , 58, 72 155, 176, 234, 238 DRPLA. See Dentatorubral-pallidoluysian atrophy (DRPLA) E Drug-induced chorea , 43 Ego-dystonic movements , 223, 227 Drug-induced dystonia , 72 Ego-syntonic movements , 223 Drug-induced hyperkinetic movement Epilepsia partialis continua , 242, 246, 248 disorders Epileptic myoclonus , 117, 119, 137 acute akathisia , 193–195 Essential myoclonus , 119, 126, 132, 137, acute dystonia , 191–193 245, 269 daytime bruxism , 198 Exaggerated startle refl ex, PMDs , 180 delayed hyperkinetic movement disorders , Excess involuntary movement, hyperkinetic 199–200 disorders faces of , 188–190 categories of , 224 levodopa-induced dyskinesia , 200–201 causes , 223 Index 285 F neuroimaging , 31 Factitious disorder, PMDs , 168–169 pathology and pathogenesis , 30 Fahn and Williams diagnosis surgery for clinically established PMD , 173 DBS surgery , 272 documented PMD , 172–173 foetal striatal transplantation , 271–272 possible PMD , 173 gene therapy , 272–273 probable PMD , 173 treatment , 31–33 Fixed dystonia , 177, 184, 232 westphal variant , 28 Focal disorders, in children , 225 Huntington disease-like (HDL) disorders , Focal dystonia , 12, 59 36–37 Foetal striatal transplantation , 271–272 Hydroxyacylglutathione hydrolase (HAGH) , 155 5-Hydroxytryptophan, myoclonus , 139 G Hyperkinetic movement disorders GABA. See g -aminobutyric acid (GABA) in childhood GABAergic drugs, dystonia , 70–71 chorea , 226–232 Gait disorders, PMDs , 179 choreoathetosis , 239–241 g -aminobutyric acid (GABA) , 122, 123, 155 dystonia , 232–239 Generalized disorders, in children , 225 excess involuntary movement , 223–224 Genetic choreas movement disorder classifi cation fi , DRPLA , 33 224–225 Fahr disease , 38 myoclonus , 241–247 Huntington disease ( see Huntington principles of treatment , 225–226 disease (HD)) secondary movement disorders , 222 Huntington disease-like (HDL) disorders , tremor , 247–250 36–37 drug-induced , 188–210 neuroacanthocytosis ( see Drug-induced hyperkinetic chorea-acanthocytosis , 34–35 movement disorders) HARP syndrome , 36 neurophysiology of , 7–9 McLeod syndrome , 35 surgery for pantothenate kinase-associated anatomy and physiology , 260–261 degeneration , 36 dystonia , 262–271 Wilson’s disease , 37–38 Huntington disease , 271–273 Gerber and Shill diagnostic criteria , 173–174 tardive dyskinesia , 273 Geste antagoniste , 58, 202 Tourette syndrome , 273 Glut-1 defi ciency syndrome fi , 148 Hypertonic disorders , 222 Hypnogenic dystonia , 149 Hypochondriasis , 168, 182 H HARP syndrome , 36 Hemiballism, pathophysiology , 10–11, 16 I Hemibody disorders, in children , 225 ICCA syndrome. See Infantile convulsions Hiccups , 130–131 and paroxysmal choreoathetosis High-potency neuroleptics , 41 (ICCA) syndrome Histrionic personality disorder , 169 Impulse-control problems, treatment of , 100 Huntington disease (HD) Infantile convulsions and paroxysmal chore- clinical features , 28–29 oathetosis (ICCA) syndrome , 154 diagnostic work-up , 30–31 Infectious choreas , 42 electrophysiology , 31 Intention myoclonus , 117, 128 epidemiology , 26 genetics , 29 genetic testing , 31 J motor disorder , 28 Jaw dystonia , 191 286 Index L 5-hydroxytryptophan , 139 Lance–Adam’s syndrome. See Posthypoxic levetiracetam , 136–137 myoclonus (PHM) piracetam , 137 Lateropolaris (Lpo) , 4 primidone , 138 Levetiracetam , 42, 71, 99, 125, 127, 129, 133, sodium oxybate , 138–139 134, 136–137, 209, 231, 247, 250 valproic acid , 137 Levodopa-induced dyskinesia , 200–201 zonisamide , 138 Low-threshold spiking (LTS cells) , 6 PMDs , 177–178 posthypoxic , 128–130 propriospinal , 127–128 M psychogenic , 135 Malingering , 169 refl ex , 126 Medication therapy, dystonia SSM , 126–127 anticholinergic medications , 69 antidopaminergic medications , 70 dopaminergic medications , 69 N GABAergic drugs , 70–71 Negative myoclonus , 128 pharmacologic treatment Neuroacanthocytosis drug-induced dystonia and dystonic chorea-acanthocytosis , 34–35 storm , 72 HARP syndrome , 36 Wilson’s disease , 71–72 McLeod syndrome , 35 Meige’s syndrome , 60 PKAN , 36 MERRF. See Myoclonic epilepsy with ragged Neuroleptic malignant syndrome (NMS) , red fi lters (MERRF) 195–197 Metabolic/toxic choreas , 43 Nocturnal dystonia , 149, 152 Minocycline , 32 Nondegenerative dystonia-plus syndrome , 62 Mobile dystonia , 232, 239 Multifocal disorders, in child , 225 Myoclonic epilepsy with ragged red fi lters O (MERRF) , 115, 119, 121, 125, 134, Obsessive-compulsive disorder (OCD) , 40 245, 246 Obsessive-compulsive symptoms (OCS) , Myoclonus 89–90, 100 in childhood OCS. See Obsessive-compulsive causes , 244 symptoms (OCS) epilepsia partialis continua , 242 Off-period dystonia , 200 evaluation , 246 Olanzapine , 192 forms of , 243 Oppenheim’s dystonia , 60 mechanism , 244 Opsoclonus–myoclonus–ataxia syndrome , treatment , 247 244, 245 classifi cation fi , 118–122 Oral zinc therapy, dystonia fl , 72 clinical features , 117, 118 Overfl ow dystonia , 58 cortical , 125 defi nition fi , 115 diagnosis , 123–124 P epidemiology , 116–117 Palatal myoclonus , 131–132, 242 essential myoclonus/myoclonus-dystonia , Pantothenate kinase-associated degeneration , 36 132–134 Paradoxical dystonia , 58 hiccups , 130–131 clinical manifestations MERRF , 134 neurochemistry , 153 negative myoclonus , 128 neuropathology , 153 origin , 116 paroxysmal hypnogenic dyskinesia , palatal , 131–132 148–149 pathophysiology , 15–16, 122–123 pathophysiology , 151–152 pharmacotherapy PED , 148 clonazepam , 138 PKD , 146–147 Index 287 PNKD , 147–148 Possible PMD , 173 description , 146 Propriospinal myoclonus , 127–128, 242, 243 diagnosis , 156 Pseudoakathisia , 203 genetics , 153–155 Pseudoepilepsy , 167 prognosis , 156 Psychogenic movement disorders (PMDs) secondary causes , 149–151 clinical features treatment , 157–158 chorea/athetosis , 178–179 Paroxysmal exertion-induced dyskinesia (PED) dystonia , 176–177 clinical manifestations , 148 exaggerated