GB, PD, HD Flashcards
Aetiology and RF of Guillain-Barre?
1) Virus: CMV, EBV, HIV
2) Bacteria: Herpes Zoster, Campylobacter jejuni
3) In some cases no obvious infection
Signs/Symptoms of GB syndrome?
1) 1-3 weeks post infection
2) Symmetrical, ascending muscle weakness and/or numbness - begins in distal muscles and progresses to proximal muscles over 4 weeks.
3) Glove + Stocking pattern
4) Loss of reflexes, poor coordination.
5) The 4 weeks is followed by recovery.
Severe Cases:
Temporary paralysis - legs, arm, face, vocal cords and respiratory muscles.
Autonomic dysfunction - sweating/tachycardia/incontinence.
CN involvement - blurred/double vision and chewing/swallowing.
Diagnosis of GBS? What does this show?
1) Nerve conduction studies (EMG) - slow conduction, prolonged distal motor latency.
2) Lumbar Puncture - CSF has raised proteins but WCC normal
3) Spirometry considered to monitor FVC - admit to ITU if decreased.
Treatment of GBS?
1) If FVC < 1.5L/80% then ventilate and admit to ITU - monitor FVC 4 hourly
2) IV immunoglobulins for 5 days to decrease duration and severity of paralysis (Contraindicated in its with IgA deficiency - screen first!!)
3) Plasma exchange
How does Parkinson’s disease present?
1) Insidious, asymmetrical onset - impaired dexterity, unilateral foot drop.
2) Parkinsonian triad - Bradykinesia, rigidity, resting tremor (pill-rolling, improves with activity)
3) Other signs: Cog wheeling, micrographia, serpentine stare/mask face/less blinking, monotonous voice, brisk reflexes, postural instability.
4) Parkinsonian gait - festinate, stooped posture, shuffling steps, narrow base, reduced arm swing.
5) Non-motor - depression/dementia/psychosocial, ED, constipation and urinary Sx.
6) NO SENSATION OR POWER PROBLEMS.
Diagnosis of Parkinsonism?
1) Mainly clinical based on history and examination.
2) Test response to Levadopa
3) MRI will initially be normal but later show atrophy.
Treatment of Parkinsonism?
1) MDT approach
2) Levadopa with carbidopa (delay till late due to complications)
3) Dopamine agonists - Ropinirole.
4) MAOB inhibitors - Selegiline/Rasigiline
5) COMT Inhibitors -
Entacapone/Tolcapone
6) Neuropsychiatric problems with antidepressants and antipsychotics.
Aetiology of Huntington’s chorea?
1) Autosomal dominant, progressive neurodegenerative disorder with onset between 30-50 years.
2) Repeat expression of CAG sequence - extended glutamine sequence in the Huntington gene.
3) Cerebral atrophy of the caudate nucleus and the pitman of the basal ganglia (loss of GABA and cholinergic neurons)
Signs and symptoms of Huntington’s Disease?
1) Chorea - Uncontrollable, aggressive, fidgety, jerky movements - causing rigidity, writhing and abnormal posture. (Striatum atrophy)
2) Personality Change - Irritability, lack of emotions, mood swings, difficulty concentrating. (Frontal cortex atrophy)
3) Psychosocial - Depression, apathy, self-neglect, develop dementia/schizophrenia/OCD. (Atrophy of cortex)
4) Seizures, dysarthria, dysphagia, abnormal eye movements + death within 15 years of diagnosis.
Diagnosing Huntington’s?
1) Diagnosis mainly clinical
2) CT/MRI initially normal, but shows caudate nucleus atrophy and increased size of frontal horns of lateral ventricles.
3) Genetic testing and counselling.
How do you treat Huntington’s?
1) SSRI - citalpram/amitryptaline - antidepressant.
2) GABA-agonist - Carbamazepine/Gabapentin - anticonvulsant + nerve pain
3) Risperidone - antipsychotic and suppresses chorea.
4) Benzodiazepines - for seizures.
5) Tetrabenzine - Dopamine depleting agent.
