Hyperkinetic Movement Disorders Flashcards
Define Hypokinetic and Hyperkinetic
Hypokinetic : excess movement
Hyperkinetic: lack movement
List Akinetic Rigid Syndromes
- Parkinson’s Disease
- Lewy body dementia
- Atypical Parkinsonism : Multiple System Atrophy (MSA) , Progressive Supranuclear Palsy (PSP), Corticobasal Degeneration (CBD)
- Drug-induced Parkinsonism
- Vascular Parkinsonism
- Chronic traumatic encephalopathy
What is Bradykinesia
Slowness of movement
Define Tardive
Late occurring
Define Dyskenesia
Difficulty or abnormality in movement.
Involuntary movement , fragmentary/incomplete
Define Akinetic
No movement
Signs of Hyperkinetic disorders ( Dyskenesia)
- Tremor
- Dystonia
- Chorea
- Hemiballismus
- Tics
- Myoclonus
What is a Resting tremor
Occurs in a body part that is not activated and completely supported against gravity
What is an Action tremor
Occurs with voluntary muscle contraction
• Postural
• Isometric
• Kinetic
Causes of tremor
- Essential tremor
- Genetic tremor disorders : Wilson’s
- Degenerative disorders : PD
- Metabolic diseases : Hypothyroid
- Peripheral neuropathies
- Drug-induced : salbutamol, lithium
- Toxins : heavy metal
- Functional
Explain essential tremor
Most common tremor syndrome in adults
• Bilateral UL action tremor -Hands, arms, head (titubation), trunk, speech
• Frequency 4-7Hz ( fast )
• diagnosed when No other neuro signs (dystonia, ataxia, parkinsonism)
• Slowly progressive, rarely very disabling
• Improves with alcohol, B-blockers, DBS
• Handwriting is shaky but not small
• Patients often have a family history
Describe Dystonia , give examples and how it’s classified
Involuntary sustained contractions of opposing muscles groups causing twisting movements or abnormal postures.
ex: head tilted to side / protruded forward , inverted foot
Classified by area affected, age of onset or secondary to degenerative disease
Causes of Dystonia
Genetic , birth trauma or secondary to disease
How does a patient with Dystonia present
- Abnormal posture ( head twisting / spasming )
- cramps
- pain
- may be associated with tremor
What is Laryngeal Dystonia
Spasmodic dysphonia
Affects muscles of larynx
- Adductor vocal cords slam together and stiffen , making it difficult for vibration = voice production , speech sounds strangled , words cut off
- Abductor : cords fold open and don’t vibrate = voice is weak and breathy
How is Laryngeal Dystonia diagnosed
ENT via Larynoscopy
Treatment for Laryngeal Dystonia
botulinum toxin
injections
What is Generalized dystonia and what is the cause
Primary dystonia " torsion " all over the body - Usually onset <25 years of age - Typically lower limb onset - 50% have DYT1 mutation (AD) ( dominant inheritance)
What is Chorea
Rapid jerky movements that flit from one part of body to another (‘dancing hands and feet’)
- generalized
- short-lasting
- distal : affects legs and hands more
- asymmetrical
- can’t stick out tongue for 10 seconds
Causes of Chorea
- Huntington’s disease
- Rheumatic fever (Sydenham’s chorea)
- Drugs (OCP, L-dopa)
- Pregnancy (chorea gravidarum)
Treatment of Chorea
dopamine receptor blockers (tetrabenazine
Describe Huntington’s Disease
Hyperkinetic (chorea), psychiatric and cognitive neurodegenerative disorder.
- Autosomal dominant caused by triple repeat disorder
- loss of nerve cells in putamen and caudate nucleus = reduction in GABA
- prognosis: 10-20 y from onset
HD presentation
- jerky uncontrollable movements (not just a tremor)
- unsteady gait,
- dysarthria
- dysphagia
- anxiety, depression, insomnia
- turn head to saccade ( look at something ) = abnormal eye movements
HD treatment
- Tetrabenzine can reduce the chorea
- antipsychotics often
required - gene silencing ( still research )
What is Hemiballismus
Severe proximal Chorea
- Violent flinging movements of one side of the body
Cause of Hemiballismus
infarction/SOL/ haemorrhage in the contralateral (opposite) subthalamic nucleus
Treatment of Hemiballismus
not always required, settles spontaneously, may need dopamine blockers or surgery in extreme cases
Describe Tics
Sudden, repetitive, non-rhythmic, same movement over and over again or vocalization
- preceded by urge : ex- need to yawn , sneeze , or scratch
- not involuntary , suppressible but irresistible
- build up of tension which patient consciously chooses to release , relieving urge
- increases with stress, excitement
- decrease when concentrating on absorbing activity
Explain Types of tics
Motor • simple: blinking, pouting, facial grimacing • complex: kicking, jumping, hopping
Vocal • Simple (phonic): grunting, sniffing, coughing • Complex: echolalia (repeating words spoken by someone else), coprolalia (spontaneous utterance of obscene words)
Causes of Tics
Primary:- • Simple transient tics of childhood - goes away on it's on in 4 y old boys • Gilles de la Tourette’s syndrome
Secondary • Neurodegenerative disease (e.g. HD, Wilson’s disease) • Associated with LDs (e.g. Down syndrome, Autism, Rett’s syndrome) • Infection (e.g. Sydenham’s chorea, PANDAS)
What is Tourette’s Syndrome
Severe expression of tic disorder
•Onset <18 years of age (usually 5-7 years old), M>F
•Multiple motor & at least one vocal tic, occuring many times
a day, nearly every day
- 10% have coprolalia
- Prognosis : improves with maturity, normal life expectancy and intelligence
Treatment of Tourette’s
Psychobehavioural therapy, reassurance, rarely
neuroleptic medication needed
Describe Myoclonus
Brief involuntary twitch (lighting fast jerk) of a muscle or group of muscles
- not a tremor , no rhythm , irregular
- stimulus sensitivity
Causes of Myoclonus
- Neuro & metabolic
disorders e.g. Epilepsy, CJD, ureamia, liver failure - Often drug-induced
- may not be a disease but just a normal jerk
Positive myoclonus vs negative
Positive : contractions
Negative : relaxations
Drugs most commonly to induce Dyskinesia
Salbutamol , Lithium , Valproate , Levodopa
Explain presentation of Tardive dyskinesia , what is the cause
- Mouthing & smacking of lips
- Grimaces with contortion of face & neck
- Occurs after several years of treatment with Neuroleptics
Explain presentation of Acute Dystonic reactions, what is the cause and treatment
Spasmodic torticollis, trismus ( locked jaw ) , oculogyric crises (episodes of sustained upward gaze)
• Can occur after just a single dose, appearing rapidly
• Treated with IV Anticholinergic (e.g. Benztropine, Procyclidine)
Describe Athetosis
- Involuntary irregular, slow writing movements of the extremities, = exaggerated postures of the limbs
- Pathology – injury to striatum, globus pallidus
- Cause – birth trauma (cerebral palsy)
Describe Akathisia
unpleasant sensation of restlessness, manifests as inability to sit still or
remain motionless
- feeling not involuntary movement
Describe Wilson’s disease
Mixed movement disorder due to accumulation of Copper in brain , liver and cornea
- presentation : wing beating tremor , dystonia, chorea
- rigidity
- dysarthria
- cognitive and psychiatric impairment
- Kayser Fleischer ring in blue eyes ( brown ring )
Treatment of Wilson’s disease
copper chelating drugs (e.g. penicillamine, trietene)
Describe ataxia
Lack of co-ordination of voluntary motor acts impairing their smooth performance (rate, range, timing, direction and force of movement maybe affected).
Causes : cerebellar , sensory , optic and frontal dysfunction
Describe Sensory ataxia
- Impaired proprioception
- Cause : disease of the dorsal (posterior) columns of spinal cord, neuropathies or neuronopathies (dorsal root ganglia)
- exacerbated by removal of visual cues (Romberg’s sign)
- pseudoatheosis shown : loss of proprioception causing involuntary movements
Cerebellar ataxia presentation
- Dysarthria
- Intention tremor
- Dysmetria (past pointing)
- Dysdiadochokinesia
- Titubation (head tremor)
- Gait ataxia
- Abnormal eye movements (nystagmus, saccadic intrusions - side to side movement when fixating on point )
- Rebound phenomenon
- Often limb hypotonia
What causes ipsilateral limb ataxia
Cerebellar hemisphere lesions
What causes Truncal & gait ataxia
Midline ( vermis ) cerebellar lesions
Causes of cerebellar ataxia
Localised • Demyelination (Multiple sclerosis) • Tumour • Infarct • Haemorhage
Global
• Infective – often kids (VZV
cerebellitis)
• Endocrine e.g. myxoedema
• Immune-mediated, paraneoplastic : Opsoclonus-myoclonus , Miller Fisher syndrome
• Degenerative : Multiple system atrophy (MSA), Spinocerebellar ataxias (SCAs), Episodic ataxias
• Toxic/Poisoning : DDT, mercury salts, 5-fluorouracil
Circumducting gait causes
Post stroke and Hemiballism
To little movement , touching opposing fingers slows down , little facial expression, resting tremor
What is the likely diagnosis
Parkinson’s disease : Bradykinesia
If person’s movement is slow but speed and amplitude is not decreasing by increments is it Bradykinesia ?
No , might be slowing of movements due to MND
