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Hyperkinetic & Hypokinetic Movement Disorders Approach Hypokinetic Parkinson s disease Idiopathic Secondary PD PD Plus Hyperkinetic Tremors Dystonia

Myoclonus Chorea Ballismus Athetosis Tics Tremor Involuntary oscillation of body part Alternating/ synchronous contractions of reciprocally innervated muscles May be y Fast/ slow y Coarse/ fine y Uniplanar/ biplanar May involve y Upper & lower limbs y Lips y Tongue y Neck y Voice Types Resting PD

Hyperkinetic Definitions Abnormal involuntary movements excessive movements Involves interaction between y Brain cortex y Basal ganglia y Thalamus y Cerebellum y Spinal cord y Muscle unit

Postural Thyrotoxicosis Anxiety Physiologic Drug induced

Intention/ action Cerebellar

Dystonic Dystonic Writer s cramp

Etiology Hereditary Non-hereditary primary hyperkinetic MD Degenerative Secondary y Ischemia/ Post-hypoxic y Demyelinating y Tumours y Post-traumatic y Inflammatory y Infection y Immunological y Endocrine y Metabolic Drug induced

Approach to Tremor Resting tremor Slow, resting, biplanar, pill-rolling, 4-5Hz Body part is at complete rest against gravity Precipitated by stress, anxiety, contralateral hand clenching Parkinson disease y Mask-like face y Asymmetry of tremor y Cogwheel rigidity y Bradykinesia y Festinant gait Postural tremor (most common) Occurs during writing, eating, dressing Upper limbs frequently affected Initially asymmetric Uniplanar (typically), flexion-extension movement of hand Action (Intention) tremor Tremor amplitude during terminal portion of targeted movement Involve cerebellum & its connection (Cerebellar MRI brain) Dystonic Wilson s disease Writer s cramp (Kayser-Fleischer ring in eyes, serum ceroplasmin, 24h urine Cu) Titubation Spasmus mutans (look for head tilt, Nystagmus)(MRI) Enhanced physiological Amplitude fine tremor (6-12Hz) (barely visible to naked eye) Anxiety Hypoglycaemia Drugs beta, agonists, dopamine agonists Toxins alcohol withdrawal, mercury, lead Othostatic tremor Variant of essential tremor Occur in legs immediately on standing, relieved by sitting down Frequency (14-18Hz) (No other clinical signs, symptoms present) Finger-to-Nose Test

A Normal

B Cerebellar hemisphere dysfunction

C Parkinsonian

D Essential tremor

Investigations Thyroid function test Serum ceruloplasmin 24h urine copper MRI brain Treatment Benzhexol, Valproate, Primidone, Propanolol Surgical Deep brain stimulation

Dystonia Sustained muscle contractions Twisting, repetitive movements Abnormal postures/ positions Due to lesion of outer segment of putamen Age of onset Childhood/ young adulthood 25 y/o Progresses from focal limb dystonia Involve craniocervical muscles to severe generalized form Remain localized/ segmental Not progressive Anatomical distribution Focal dystonia (single region) Segmental dystonia Cervical dystonia 2 adjacent regions (spasmodic torticollis) Meige syndrome Blepharospasm Multifocal dystonia Oromandibular dystonia 2 nonadjacent regions Largyngeal dystonia Hemidystonia (spasmodic dysphonia) Ipsilateral arm & leg Limb dystonia Generalized dystonia Leg(s), trunk, 1 other region Focal dystonias

Secondary Dystonia Environmental factors/ insults that affect brain Spinal cord/ peripheral injury Cerebral palsy Arteriovenous malformations (AVM) Brain tumours Brainstem lesions Head injury Inflammatory, infectious Cerebral infarct/ haemorrhage Multiple sclerosis (MS) Thalamotomy Cervical spinal cord injury Lumbar canal stenosis Peripheral trauma Hypoparathyroidism Prolonged exposure to toxic environment Phenytoin, Carbamazepine, Levodopa Dystonia-Plus Syndromes Dopa-responsive dystonia (DRD)/ Segawa syndrome Genetic disorder (apparent in 6-16 y/o) Abnormal, stiff-legged gait, plantar flexion or eversion, tendency to walk on toe Rapid-onset dystonia-parkinsonism (RDP) Autosomal dominant (chromosome 19q (DYT12)) Abrupt development over hours, days, weeks of combined dystonia & parkinsonism Myoclonus dystonia Heredodegenerative Dystonia X-linked dystonia-parkinsonism (Lubag) Huntington s disease Wilson s disease Neuroacanthocytosis Rett syndrome Parkinson s disease Juvenile parkinsonism Other heredodegenerative dystonias Treatment Injection therapy with (for focal dystonia) y Botulinum neurotoxin type A (Botox) y Botulinum neurotoxin type B (Myobloc) Physical therapy, bracing, relaxation, learning techniques Diazepam, Clonazepam Intrathecal baclofen Surgery y Lesioning y Deep brain stimulation (DBS)

Causes Primary (1) (Idiopathic) Dystonia is the only sign 2 cause is ruled out Secondary (2) (Symptomatic) Primarily from environmental causes, underlying disease May/ may not be genetic, injury to brain Dystonia-plus syndromes Non-degenerative, neurochemical disorders Associated with other neurologic features (eg. myoclonus, parkinsonism) Heredodegenerative dystonia Hereditary, neurodegenerative disorder Other neurologic symptoms are present in addition to dystonia Primary Dystonia Familial/ non-familial (sporadic) Causes y DYT1 Gene Mutation o Dystonia musculorum deformans (DMD) o Early-onset primary dystonia o Idiopathic torsion dystonia (ITD) o Primary torsion dystonia o Primary generalized dystonia y DYT6, DYT7, DYT2, DYT4 Dystonia y Primary Adult-onset Focal/ Segmental Dystonias

Myoclonus Sudden, brief, shock-like movements May be +ve Contraction of a muscle Contraction of multiple muscles

-ve Brief loss of muscle tone Tightening (contraction) of other muscles (result in flapping-type motion)

Characteristic saw-tooth pattern Classifications Physiologic Cortical y Arising from an area of sensorimotor cortex y Eg. Progressive myoclonic epilepsy Subcortical y Result of hypoxia/ metabolic process (renal failure) y Involve thalamus, brainstem Spinal Peripheral y Hemifacial spasm (caused by compression of facial nerve) y Persist during sleep Clinical Anatomic distribution y Focal (spinal, propiospinal, arm, palatal) y Segmental y Multifocal y Generalized Provocative factors y Spontaneous (encephalopathy) y Reflex y Action/ intention Contraction pattern y Rhythmic y Arrhythmic y Oscillatory Etiological Physiologic y Hypnic jerks (sleep start) y Hiccups y Benign sleep myoclonus of infancy Essential y Autosomal-dominant pattern of inheritance y Occurs along with tremor, dystonia Progressive Myoclonic Epilepsy y Lysosomal lipid storage diseases y Mitochondrial disorder y Cystatin B deficiency y Glycogen-storage disease Secondary/ symptomatic y Inborn errors of metabolism y Trauma y Neurodegenerative disease

Chorea ( Dance ) Irregular, unpredictable brief, jerky movement Flow from 1 body part to another in a non-stereotyped fashion Distal (usually) Severity Mild Severe Fidgetiness in children Interfering with y Speech y Swallowing y Maintaining posture y Ability to walk Due to y Intracortical inhibition of motor cortex y Contralateral thalamus (disinhibition due to loss of normal pallidal inhibitory input) Causes Rheumatic chorea Thyrotoxicosis Huntington s disease Neuroacantocythosis Other causes Metabolic hyperthyroidism, hypothyroidism Toxins alcohol, carbon monoxide poisoning Immunological SLE Drugs Excess L-dopa, phenothiazines, OCP, phenytoin Pregnancy Treatment Tetrabenazine Reserpine Neuroleptics Haloperidol Huntington s Disease Genetic disorder Autosomal dominant Characteristics y Onset of symptoms 4th 5th decade y Chorea y Progressive dementia y Untreatable, death 15-20 years (median 16.2 years) after onset of symptoms Results from degeneration of striatal neurons

Normal Brain

Huntington s Disease Ventricles enlarged Due to atrophy of head of caudate (from neuronal loss)

Ballismus Severe form of chorea Large-amplitude Proximal, flinging of a limb/ body part Types Unilateral Hemiballismus Bilateral Paraballismus Biballismus

Tics Intermittent, repeated, stereotyped movements/ sounds Occur in an infrequent/ almost continuous manner Can be y Simple sudden, brief, repetitive (involve limited number of muscle groups) y Complex distinct, coordinated patterns of movements (involve several muscle groups) y Motor y Vocal Classification Motor Tics Eye blinking Head jerking Shoulder shrugging Eye darting Finger flexing Sticking the tongue out Hiccupping Yelling Throat clearing Barking Vocal Tics Touching nose Touching other people Smelling objects Obscene gestures Flapping the arms Hopping Using different voice intonations Repeating one s own words/ phrases Repeating others words/ phrases Obscene language

Causes Result from vascular lesion within subthalamic nucleus on side opposite to disorder

Simple Tics Athetosis Slower writhing, twisting movement Hands, feet Slow writhing movements often alternate with holding parts of limbs in certain positions (postures) to produce a continuous, flowing stream of movement

Complex Tics

Causes Primary (idiopathic) Tourette s syndrome (chronic motor & vocal tic disorder) Transient tic disorder Drug/ toxin induced Stimulants (amphetamines, Ritalin, caffeine) Carbamazepine Steroids Neuroleptics Carbon monoxide Para-infectious Post-infectious autoimmune neuropsychiatric disorder (associated with streptococcus (PANDAS)) Post-encephalitis Others Neuroacanthocytosis Huntington s disease Wilson s disease Pantothenate kinase associated neurogeneration Pervasive developmental disorder (autism spectrum) Tourette s Syndrome Inherited neurobehavioral disorder Manifested by motor, vocal tics Started during childhood Accompanied by OCD, ADHD, poor impulse control Result from abnormalities in activity of neurotransmitters (particularly dopamine) within basal ganglia

Hypokinetic Idiopathic Parkinson s Disease (IPD) Neurodegenerative disease Progressive loss of dopaminergic neurons Dopamine deficiency Acetylcholine excess Cardinal Features Bradykinesia Rest tremor Cogwheel rigidity Postural instability Associated Features Sustained, significant levodopa effect Armswing Difficulty rising from a low chair Impaired olfaction Hypophonia Stooped posture Depression/ anxiety Shuffling/ festinating/ freezing gait Unilateral symptoms onset Difficulty turning in bed Drooling Constipation Micrographia Sleep disturbance/ REM sleep behaviour disorder Seborrhea Parkinson s Disease Hoehn & Yahr stages Stages I Hemi-Parkinsonism II Bilateral No postural instability III Bilateral Mild postural instability IV Bilateral Moderate postural instability V Bilateral Severe postural instability (wheel-chair/ bed-bound) Pathology Depigmentation of Substantia nigra Degeneration of nigra cells Potential Causes of PD Genes -synuclein Parkin UCH-L1 Susceptible genes

Environment Pesticides Rural living

Pathogenic Mechanisms Protein aggregation Mitochondrial dysfunction Oxidative stress Inflammation Excitotoxicity Apoptosis (cell death) Drugs Dopaminergic agents y Levodopa (Madopar/ Sinemet) y Dopamine agonists Ergot DA (Bromocriptine, Cabergoline, Peribedil) y Non-Ergot Selective D2 agonist (Pramipexole/ Ropinorole) COMT Inhibitors (Entacopone) MAO-B Inhibitors (Selegiline/ Rasagiline) Anticholinergics (Artane) Antiviral (Amantadine)