Neurology 2 Flashcards
Parkinson’s disease is a progressive neurodegenerative condition caused bydegeneration of dopaminergic neurons in ……….
the substantia nigra
The symptoms of Parkinson’s disease are characteristically ………..
asymmetrical
The most common psychiatric problem is ……. in Parkinson’s disease
Depression
Drug-induced parkinsonism has slightly different features to Parkinson’s disease:
motor symptoms are generally rapid onset and bilateral rigidity and rest tremor are uncommon
Causes of Parkinsonism
Parkinson’s disease
drug-induced e.g.antipsychotics,metoclopramide*
progressive supranuclear palsy
multiple system atrophy
Wilson’s disease
post-encephalitis
dementia pugilistica (secondary to chronic head trauma e.g. boxing)
toxins: carbon monoxide, MPTP
Domperidone does not cause extra-pyramidal side-effects because it …..
does not cross the blood-brain barrier
Essential tremor improved by ….
alcohol and rest
What is most common cause of titubation (head tremor)?
Essential tremor
Management of Essential tremor
propranolol is first-line
primidone is sometimes used
(5) Causes and associations of Restless legs syndrome
- Idiopathic ( +ve FH)
- IDA
- Uremia
- DM
- Pregnancy
Management of Restless legs syndrome
simple measures: walking, stretching, massaging affected limbs
treat any iron deficiency
dopamine agonists are first-line treatment (e.g. Pramipexole,ropinirole)
benzodiazepines
gabapentin
Chorea is caused by damage to …….
the basal ganglia, especially the caudate nucleus
Causes of chorea
Huntington’s disease,Wilson’s disease, ataxic telangiectasia
SLE, anti-phospholipid syndrome
rheumatic fever: Sydenham’s chorea
drugs: oral contraceptive pill, L-dopa, antipsychotics
neuroacanthocytosis
pregnancy: chorea gravidarum
thyrotoxicosis
polycythaemia rubra vera
carbon monoxide poisoning
cerebrovascular disease
Huntington’s disease is
Autosomal dominant
trinucleotide repeat disorder: repeat expansion of CAG
Huntington’s disease results in degeneration of cholinergic and GABAergic neurons in ……1….
- due to defect in huntingtin gene on chromosome ……
- the striatum of the basal ganglia
- chromosome 4
Features of Huntington’s disease
Chorea
Dystonia
saccadic eye movements
personality changes (e.g. irritability, apathy, depression) and intellectual impairment
Transient global amnesia is characterized by the acute onset of ……… amnesia
episodes are self-limited and resolve within ……..
- anterograde amnesia
- 24 hours
Avoid drug holiday of parkinson’s medications due to the high risk of ….
Acute akinesia or neuroleptic malignant syndrome
Impulse control disorders in parkinson’s
Common with
dopamine agonist therapy
a history of previous impulsive behaviours
a history of alcohol consumption and/or smoking
If orthostatic hypotension develops in parkinson’s
medication review looking at potential causes should be done.
If symptoms persist then midodrine (acts on peripheral alpha-adrenergic receptors to increase arterial resistance) can be considered.
excessive daytime sleepiness develops in parkinson’s
patients should not drive.
Medication should be adjusted to control symptoms.
Modafinil can be considered if alternative strategies fail.
Consider …………………. to manage drooling of saliva in people with Parkinson’s disease.
glycopyrronium bromide
prion diseases
CJD
kuru
fatal familial insomnia
Gerstmann Straussler-Scheinker disease
Features of Creutzfeldt-Jakob disease
Rapid onset of dementia
Myoclonus
Ataxia telangiectasia is an autosomal …… disorder caused by a defect in the ………. gene which encodes for……….
autosomal recessive disorder
caused by a defect in the ATM gene
encodes forDNA repair enzymes.
Ataxia telangiectasiais …….. immunodeficiency disorders.
combined ( B & T cell) immunodeficiency disorders.
cerebellar ataxia
telangiectasia (spider angiomas)
IgA deficiency resulting in recurrent chest infections
10% risk of developing malignancy, lymphoma or leukaemia, but also non-lymphoid tumours
Features of
Ataxia telangiectasia
What are the most common presenting features of Friedreich’s ataxia?
Gait ataxia and kyphoscoliosis