Chorea, Ataxia, and other Movement Disorders. Dr. Fred Stephen Sarfo (MD, PhD) Komfo Anokye Teaching Hospital Kumasi, Ghana
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1 Chorea, Ataxia, and other Movement Disorders Dr. Fred Stephen Sarfo (MD, PhD) Komfo Anokye Teaching Hospital Kumasi, Ghana
2 Control of motor function
3 Control of motor function The activities of the basal ganglia and cerebellum modulate the corticospinal and cortical-brainstem-spinal systems. Often referred to as extra-pyramidal system
4 Movement disorders Hyperkinetic Movements Hypokinetic Movements
5
6 Epidemiology of hyperkinetic movement disorders in Africa per 100,000 Study Chorea Athetosis Dystonia Cerebellar ataxia El Tallawy et al (Al Quseir, Egypt) El Tallawy et al (Al Kharga, Egypt) Kandil et al (Assiut, Egypt)
7 Kumasi, neurology clinic Hypokinetic Movement Disorders Frequency Valid percent Idiopathic PD Drug induced PD Vascular PD Hyperkinetic Movement disorders Essential tremor Cerebellar/ thalamic tremor Myokymia Choreo-athetosis Dystonia PD plus syndrome Tics Writers cramps Tardive dyskinesia Restless leg Total
8 Hyperkinesias Due to imbalance of activity in the complex basal ganglia circuits Related to excessive dopaminergic activity in the basal ganglia
9 Cortico-Basal-thalamic circuits
10 Basal ganglia circuits
11 Chorea, athetosis, ballism, dystonia Not a single entity, but a spectrum Myoclonus Ballismus Chorea Athetosis Dystonia Movements become less violent / explosive / jerky - smoother and more flowing - more sustained
12 Chorea Chorea means dance Irregular, unpredictable brief jerky/fidgety movements that are usually of low amplitude Semi-purposeful If subtle, it can be missed May be generalised/segmental/localised Videos
13 Causes of chorea Hereditary Dominant- Huntington s disease Recessive- Wilson s disease Autoimmune Rheumatic chorea (Sydenham s) Chorea gravidarum Systemic lupus erythematosus Metabolic Hypo-hypernatraemia, hypo-hyperglycaemia, hypocalcaemia, renal failure Toxins- mercury, carbon monoxide Drugs- neuroleptics (ie TARDIVE DYSKINESIA), metoclopramide, L-dopa, anticonvulsants, steroids, oral contraceptives Inflammatory HIV/AIDS encephalitis Vascular Basal ganglia infarcts
14 Causes of chorea in Africa 62/100,000 Rheumatic chorea 21/100,000 Huntington s chorea 17/100,000 Vascular disease 12/100,000 Post-encephalitic Sample of 42,000 participants from 7,000 families in Assiut, Egypt. (Kandil et al., 1994)
15 Athetosis Athetosis is a slow continuous stream of sinuous, writhing movements, typically of the hands and feet Causes - dyskinetic motor fluctuation in PD - athetoid cerebral palsy If athetosis becomes faster, it sometimes blends with chorea i.e. choreoathetosis or choreoathetoid movements
16 Myoclonus Brief, isolated, random, non-purposeful jerks of muscle groups in the limbs. Generalised/ segmental/ focal Rx. Valproate drug of choice. May respond to benzodiazepines eg clonazepam Video
17 Ballismus Essentially a focal form of chorea (dramatic) Hemiballism proximal high-amplitude flailing chorea limited to one side of the body Biballism if bilateral Monoballism if in a single limb Causes Usually vascular (eg, basal ganglia infarct; usually of the sub-thalamic nucleus) Metabolic Good response to antipsychotics, usually self-limiting
18 Tics Consist of abnormal movements and abnormal sounds Vary in severity over time Usually preceded by uncomfortable feeling or sensory urge that is relieved by carrying out the movement May be simple or complex Often suppressible Video
19 Stereotypy Refers to coordinated movements that repeat continually and identically Resemble motor tics but there is no driving urge Often repeat themselves in a uniform, repetitive fashion for long periods of time
20 Akathisia Unable to sit still Feeling of inner, general restlessness that is reduced or relieved by moving about Complex and usually stereotyped movements Can be both generalized and focal Can usually be briefly suppressed Most common cause is iatrogenic
21 Approach to a patient with hyperkinesia History taking - Patient description of problems - Course of present illness - Age at onset - Past medical history - Birth and family history - Infections - Drug/toxin exposure (eg neuroleptics, alcohol)
22 Approach to a patient with hyperkinesia Neurological examination - spend time observing the patient overall Establish type of movement disorder - hyperkinetic Establish pattern of movement disorder - Focal vs segmental vs generalised - Particular characteristics of the movement disorder Rhythmic/intermittent Rest/posture/action Precipitating features (startle/posture/task specific/drugs Associated features eg. Dementia, epilepsy Establish aetiology of movement disorder
23 Aetiology of dyskinesias Hereditary - Huntington s dx - Wilson s dx - Neuroacanthosis - Porphyria Childhood - Cerebral birth injury - Kernicterus - Cerebral palsy Infective/inflammatory - Post-streptococcal - Henoch-Scholein purpura - Creutzfeldt-Jakob disease - SLE Drugs - Levodopa/dopamine agonists - Phenothiazines - tricyclics Vascular - CVA (infarct / hemorrhagic) - Arteriovenous malformation - Antiphospholipid antibody syndr Endocrine - Pregnancy - Thyrotoxicosis - Hypoparathyroidism - hypoglycaemia
24 ATAXIA Ataxia is the inability to perform smooth, accurate and coordinated movements Ataxia can arise from disorders of Cerebellum (most common) Sensory pathways (sensory ataxia) Frontal lobe lesions (via fronto-cerebellar associative fibres)
25 Cerebello-cortical loops
26 Motor ataxia Motor Ataxia Caused by cerebellar disorders Intact sensory receptors and afferent pathways Integration of proprioception is faulty Midline cerebellar lesions cause truncal ataxia Lateral cerebellar lesions cause limb ataxia Thalamic infarcts may cause contralateral ataxia with sensory loss
27 Sensory ataxia Sensory Ataxia Failure of proprioceptive information to the CNS May be due to disorders of spinal cord or peripheral nerves Can be compensated for by visual inputs
28 Approach to patient History Onset Rapidity Previous symptoms PMH Medications Social Alcohol intake Illicit drug use Associated Symptoms Headache Drowsiness Dizziness Vertigo Tinnitus Fever Nausea/vomiting Weakness Paresthesia
29 Approach to patient Physical Exam Gait testing Tandem gait Orthostatic VS Full neurologic exam Cerebellar function Dysmmetria Dysdiadochokinesia Dyssynergia Stewart-Holmes rebound sign Rhomberg
30
31 ATAXIA CAUSES Acquired vs inherited Acute vs subacute vs chronic
32 Ataxia causes Acquired Infection (abscess, cerebellitis) Vascular (bleed, infarct, TIA) Demyelinating (MS, PN) Malignancy (tumor) Toxin (INH, Li, cyclosporin, cytosine, phenytoin) Paraneoplastic Metabolic (Vit E deficiency, hypothyroidism)
33 Ataxia causes Inherited Intermittent hyperammonia Progressive Mitochondrial encephalomyopathy Wilson s disease
34 Investigations Rule out acquired causes CT scan or MRI LP EMG, NCS TFT Lipids
35 THANK YOU FOR LISTENING
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The Urge to React : Obsessive Compulsive Disorder and Huntington s Disease John Barkenbus, MD North Carolina Neuropsychiatry Charlotte Clinic Disclaimer The information provided by speakers in workshops,