Movement Disorders Flashcards
hypokinesia definition
Slowed movements with increased tone (Parkinsonism)
Hyperkinesia definition
Excessive Involuntary movements
types of Parkinsonism
Idiopathic Parkinson’s disease
Parkinson’s plus syndromes (MSA, CBD, PSP)
Drug induced (anti-psychotics, anti-emetics, methyldopa, lithium)
Other pathology (tumour, post-encephalitis)
What is Parkinsonism?
presence of bradykinesia and one of the following three symptoms:
- Resting tremor
- postural instability
- Rigidity
What is Parkinson’s disease?
progressive neurodegenerative condition caused by degeneration of dopaminergic neurons in the substantia nigra
What causes tremor and rigidity in Parkinson’s?
Imbalance between acetylcholine and dopamine acting on the basal ganglia
Clinical features of Parkinson’s disease
T - tremor (resting ‘pill rolling tremor’), improves with movement
R - Rigidity (‘cog wheel’ rigidity in the arm, ‘lead pipe’ rigidity in the leg)
A - akinesia (bradykinesia), asymmetrical
P - postural instability (shuffling gait with reduced arm swing)
Other features:
Mask-like face
Depression, psychosis
Fatiguing and decrement in rapid continuous movement
Micrographia
Postural hypotension
features of drug induced Parkinsonism
features of Parkinsonism BUT
motor symptoms are generally rapid onset bilateral
rigidity and resting tremors are uncommon
Features signifying possible Parkinson plus syndromes
Symmetrical presentation
Absence of tremor
Levodopa unresponsiveness
Early falls (within 1st year)
Additional neurological features
Features of Multiple System Atrophy (MSA)
Parkinsonism
Characteristically rapid progressive autonomic dysfunction with severe postural hypotension and urogenital dysfunction
Alpha-synuclein + glial cytoplasmic inclusions are found in the basal ganglia
No response to levodopa
Features of progressive supra nuclear palsy (PSP)
Parkinsonism
Postural instability with early falls
vertical Supra nuclear gaze palsy
Pseudobular palsy and dementia
Tau deposition in the substantia nigra, sub thalamic nucleus and midbrain
Features of corticobasal degeneration (CBD)
Alien limb phenomena
Dementia
myoclonus
Types of hyperkinesia
Chorea
Tics
Myoclonus
Dystonia
Tremor
What is chorea?
Excessive, irregular movements flitting from one body part to another
Myoclonus
Brief electric shock like jerks
Dystonia
Sustained muscle spasms causing twisting movements and abdominal positions
Features of an essential tremor
Autosomal dominant inheritance
Bilateral, fast, low amplitude, mainly upper limb
Postural: holding glass or cutlery, worse if arm stretched out
Better with alcohol and rest
Huntington’s disease mode of inheritance
Autosomal dominant
What is Huntington’s disease?
Trinucleotide repeat disorder: increased number of cystine-adenosine-guanine (CAG) trinucleotide repeats on the Huntington gene on chromosome 4
Pathophysiological result of the genetics of Huntington’s disease
Degeneration of cholinergic and GABAergic neurones in the striatum of the basal ganglia
Clinical features of Huntingtons
Huntington’s Triad:
Dementia
Personality changes (Depression, apathy, irritability) and intellectual impairment
Chorea (irregular, excessive movements from one body part to another)
Saccadic eye movements
Investigation for Huntington’s
PCR analysis and gel/capillary electrophoresis