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Abnormal movements.
• Movement disorders are typically heterogenous both clinically and pathologically.
• Characterized by impairment of movement.
○ Planning
○ Control
○ Execution
• Examples of movement disorders include.
○ Ataxia
○ Dystonia
○ Gait disorders
○ Chorea
○ Myoclonus
○ Parkinsonism
○ Spasticity
○ Tardive dyskinesia
○ Tics
○ Tremor
Tremor
• Note.
○ Frequency
○ Amplitude
○ Exacerbating factors.
Stress
Fatigue
• Rest tremor.
○ Abolished by voluntary movement.
○ Seen in Parkinsonism
• Intention tremor.
○ Irregular
○ Large amplitude
○ Worst at the end of purposeful acts.
○ Suggests cerebellar disease.
MS
Stroke
• Postural tremor.
○ Absent at rest
○ Present on maintained posture
Arms stretch out in front of patient.
○ May persist, but isn’t exaggerated by, movment.
○ Causes.
Benign essential tremor.
• Autosomal dominant
• Reduced by alcohol
Thyrotoxicosis
Anxiety
β – agonists.
• Re – emergent tremor.
○ Postural tremor that develops after a delay of about 10 seconds.
○ Seen in Parkinsonism.
• Asterixis.
○ Coarse, non – rhythmic hand flap.
○ Worsened by extending arms and cocking back wrists
○ Causes.
Liver failure
Renal failure
Carbon dioxide narcosis.
○ Management.
Surgery
Deep brain stimulation.
Chorea
• Non – rhythmic, jerky and purposeless
• Flit from one place to another.
○ Facial grimacing
○ Raising shoulders
○ Flexing and extending fingers
• Causes.
○ Huntingdon’s
○ Sydenham’s
○ Strep infection
Rare complication.
Hemiballismus.
• Large – amplitude
• Flinging
• Hemichorea
○ Affects proximal muscles.
• Contralateral to vascular lesion of subthalamic nucleus.
• Often occurs in elderly diabetics.
• Recovers spontaneously over months.
Athetosis
• Slow
• Sinuous
• Purposeless
• Especially.
○ Digits
○ Hands
○ Face
○ Tongue
• Often difficult to distinguish from chorea.
• Commonest cause is cerebral palsy.
Tics
• Brief
• Repeated
• Stereotyped.
• Able to be suppressed for a while.
• Common in children.
○ Usually resolve.
• Tourette’s syndrome.
○ Motor tics
○ Vocal tics
○ Management.
Psychological support
If tics are severe.
• Clonazepam
• Clondine
• Haloperidol.
○ Risk of tardive dyskinesia.
Myoclonus.
• Sudden
• Involuntary
• Jerky
• Generalised or focal
• Seen in.
○ Metabolic problems.
Eg. Renal failure
○ Neurodegenerative disease.
Eg. Lysosomal storage enzyme defects.
○ CJD
○ Myoclonic epilepsy.
○ Benign essential myoclonus.
General myoclonus
Begins in childhood
Autosomal dominant inheritance.
No other consequences.
• Myoclonus may respond to.
○ Valproate
○ Clonazepam
○ Piracetam.
Tardive dyskinesia
• Occur after chronic exposure to dopamine receptor blockers.
○ Antiemetics
○ Antipsychotics.
• Can be permanent, even when causative drug is stopped.
• Can cause significant distress and disability.
• Classification.
○ Tardive dyskinesia.
Orobuccolingual
Truncal
Choreiform
Dystonia
• Prolonged muscle spasm.
• Causes abnormal posture or repetitive movements.
• For example, writers cramp., inability to move hand to mouth.
• Can be classified by.
○ Age of onset.
Childhood is < 12 years
Adolescence is 13 – 20 years
Adulthood is > 20 years.
○ Part of the body affected.
○ Cause.
• Childhood onset suggests idiopathic generalized dystonia.
○ Often starts in one leg.
○ Spreads to cover that side of the body over next 5 – 10 years.
○ Often autosomal dominant condition.
Often deletion in DTY1.
○ Treatment is challenging.
Exclude Wilson’s disease
Exclude dopa – responding dystonia
○ Management.
High – dose trihexyphenidyl.
• Eg. benzhexol
• Anti – cholinergic.
Deep brain stimulation
• Dystonia may be confined to one part of the body, ie. A focal dystonia.
○ Eg. Spasmodic torticollis (head pulled to one side).
○ Blepharospasm.
○ Writer’s cramp
• Focal dystonias in adults tend to be idiopathic.
○ Rarely generalize
○ Worsened by stress
○ May develop geste antagonistique.
To try and resist dystonic posturing.
Eg. Touching finger to jaw in spasmodic torticollis.
○ Effective control of focal dystonia is usually achievable with botox injections into the
dysfunctioning muscle.
May be side effects.
• Acute dystonia.
○ May occur in young men who are starting neuroleptics.
○ Clinical picture.
Head pulled back
Eyes drawn upwards
Trismus
○ Management.
Anti – cholinergics.
Eg. Benzotropine 1 – 2 mg IV.
• Writer’s cramp.
○ Look for.
Hand and forearm spasm.
Dystonic arm posture
Focal tremor/ myoclonus
Dominant hand muscle hypertrophy
○ Associated with OCD.
○ EMG may show.
Reduced reciprocal inhibition of wrist flexors
Increased co – contraction of antagonist forearm muscles during voluntary
movement.
○ EEG may show abnormal motor command.
Sensorimotor region β region.
○ Management.
Simple measures often fail.
• β – blockers
• Valproate.
Things that often work include.
• Arm cooling
• Breath holding
• Botox
• EMG biofeedback.
Epilepsy.
• Recurrent tendency to spontaneous, intermittent, abnormal brain activity.
• Manifests as seizures.
• May take many forms.
○ Tend to be stereotype for specific patient.
• Convulsions.
○ Motor signs of electrical activity.
○ Most normal patients would have convulsions under certain metabolic circumstances.
Hyponatraemia
Hypoxia
• Incontinence and myoclonic jerks are not particularly associated with epilepsy.
• Tongue biting and prolonged post – ictal phase are very suggestative.
• Prevalence of active epilepsy is about 1%
• Clinical picture
○ Prodrome lasting hours – days.
Rare.
Change in mood or behaviour.
○ Aura.
Part of seizure.
Strange feeling in abdomen
Sensation of déjà vu.
Strange smells
Flashing lights
Implies partial seizure.
• Often Temporal lobe
Diagnosis
• Detailed description of the fit from a witness is vital.
○ Be very cautious of diagnosing epilepsy in error.
Major problem due to.
• Toxic drug side effects
• Stigmatising illness
• Employment problems.
• Expensive insurance
• Driving bans.
• Decide what type of fit it is.
○ Main concern is with the onset of the attack.
○ If the seizure starts with focal symptoms, it is partial.
However fast it generalizes.
• Precipitants.
○ Drugs
○ TV
Almost always generalized
Rarely require drugs.
• Partial seizures.
○ Features refer to a part of one hemisphere.
○ Suggests structural disease.
○ Elementary symptoms.
Consciousness unimpaired.
Eg. Focal motor seizure.
○ Complex symptoms.
Consciousness impaired.
Eg. Olfactory aura presceding autosomatism.
Usually temporal lobe.
○ Partial seizures with secondary generalisation.
Electrical disturbances starting focally.
Progresses to generalized seizure.
Causes.
• Often none found.
• Structural.
○ Trauma
○ Space occupying lesion
○ Stoke
○ Tuberouls sclerosis
○ SLE
○ PAN
○ Sarcoid
○ Vascular malformations.
• Metabolic.
○ Alcohol withdrawl.
○ Benzodiaepine withdrawl
○ Hyper or hypoglycaemia
○ Hypoxia
○ Uraemia
○ Hypernatraemia
○ Hypocalcaemia
○ Liver disease.
• Drugs.
○ Phenothiazides
○ Tricyclics
○ Cocaine
• Infection.
○ Encephalitis
○ SYphiolis
○ Cysticercosis
○ HIV.
Generalized.
• Try valproate as first line.
○ Monitor LFTs & INR during first 6 months.
○ Most hepatic failure occurs in patients < 3 years on
polytherapy.
○ Toxic effects.
Sedation
Tremor
Weight gain
Hair thinning
Ankel swelling
Hyperammonaemia
• Encephalopathy
Liver failure.
○ Drug levels aren’t helpful.
• If valproate not effective/ tolerated, try lamotrigine.
○ May be more effective and better tolerated.
Absence seizures.
• Valproate.
• Ethozuximide.
Partial seizures ± generalization.
• Carbamazepine.
○ Slow release form useful if side effects occur at peaks.
○ Toxic effects include.
Rash
Nausea
Diplopia
Dizziness
Fluid retention
Hyponatraemia
Blood dyscrasias.
• 2nd line.
○ Valproate
○ Levetiracetam
○ Topiramate.
Phenytoin.
• Effective and well established.
• Not 1st line due to toxicity.
○ Nystagmus
○ Diplopia
○ Tremor
○ Dysarthria
○ Ataxia
○ Decreased intellect
○ Depression
○ Poor drive
○ Polyneuropathy
○ Acne
○ Coarse facial features
○ Gum hypertrophy
○ Blood dyscrasias
• Dosage is difficult & needs blood level monitoring.
Lamotrigine.
• Useful monotherapy for primary generalized seizures..
• Also used as add on in secondary generalized seizures.
• Halve monotherapy dose when combined with valproate.
• Double monotherapy dose when combined with carbamazipine or
phenytoin.
• Side effects.
○ Rash.
May be serious
Normally occurs within 8 weeks of starting therapy.
Especially when co – therapy with valproate.
See doctor immediately if rash or ‘flu – symptoms
associated with hypersensitivity develops.
Check.
• FBC
• U&E
• LFTs
• INR
○ Fever
○ Malaise
○ ‘Flu symptoms
○ Drowsiness
○ Raised LFTs
○ Photosensitivity
○ Diplopia
○ Reduced vision
○ Vomiting
○ Aggression
○ Tremor
○ Agitation
○ Drug interactions
Other anticonvulsants
Antimalarials
Antidepressants.
Levetiracetam & Topiramate.
• New anti – epileptics.
• Used for secondary generalized seizures.
• Side effects.
○ Diarrhoea
○ Vomiting
○ Dyspepsia
○ Depression
○ Drowsiness
○ Diplopia
○ Neutropaenia.
Zonisamide.
• Partial seizures
• Secondary generalized seizures.
Status Epilepticus.
• Seizure lasting > 30 minutes.
• Continuous seizures without regaining consciousness.
• Length of attack increases.
○ Mortality
○ Risk of permanent brain damage.
• Aim to terminate seizures as soon as possible.
• Investigations.
○ EE|G can be very helpful.
○ Check for pregnancy.
If positive, then diagnosis is likely o be eclampsia.
Check urine and BP
Call senior obstetrician.
Immediate delivery may be required.
○ Telemetry
○ Bloods.
Glucose
Blood Gases
U&E
Calcium
FBC
Anticonvulsant levels
Toxicology screen
Blood cultures
• Management.
○ Basic Life Support.
○ Get IV access.
○ Lorazepam.
Beware respiratory arrest during end of infusion with all benzodiazepines.
Alternative is diazepam.
• Less long lasting.
• Can also be given rectally.
Buccal midazolam can also be given.
○ Phenytoin.
If fits continue lorazepam
Beware hypotension
Contraindicated if
• Bradycardia
• Heart block
Monitor.
• BP
• ECG
○ Diazepam.
If fits continue after phenytoin
Requires close monitoring.
• Especially of respiratory function.
Very rare for status not to respond once this stage of protocol is reached.
• If no response, consider pseudoseizure.
○ Particularly if odd features such as.
Pelvic thrusts
Resisting attempt to open lids.
Resisting attempts to passive movements
Flailing arms and legs.
• Further management.
○ Dexamethasone.
If it is possible that seizures are due to.
• |Vasculitis
• Cerebral oedema
• Tumour.
○ General anaesthesia.
Requires admission to ITU.
IV infusion phase.
Phenytoin 15 mg/kg at < 50 mg/min
Diazepam 8 mg/hour in 5% dextrose.
• Investigations.
○ Nerve conduction studies.
Slowed conduction
○ Lumbar puncture.
Protein often increased to > 5.5 g/L
Normal CSF WCC
• Treatment.
○ Respiratory involvement requires transfer to ITU.
Ventilate sooner than later.
• FVC < 1.5 L
• PaO2 < 10 kPa
• PaCO2 > 6 kPa
Check FVC ever 4 hours
○ IV immunoglobulins
For 5 days.
○ Plasma exchange
○ Corticosteroids have no role.
• |Prognosis.
○ Good
○ 85% make a complete recovery.
○ 10% are unable to walk alone at 1 year
○ Mortality of 10%
• Tests.
○ Nerve conduction studies not normally needed.
May be negative anyway.
• Treatment.
○ Carpal tunnel injections.
Not certain if there is any benefit over placebo beyond 1 month.
○ Wrist splints at night.
May relieve nocturnal pain.
○ Flexor retinaculum division.
Decompresses carpal tunnel.
Gives more permanent result.