Huntingtons Disease Flashcards
(20 cards)
What is the pathology of Huntington’s
A loss of neuron’s in the basal ganglia and cortex of the brain which can lead to cortical thinning, lateral ventricle dilation and a loss of white matter
What neurotransmitters are decreased in Huntington’s?
GABA
Acetylcholine
GAD-GABA
What are the three main signs of Huntington’s?
Chorea
Dementia
Behavioral changes
What is the peak age group for Huntington’s?
35-45y/o
Chorea is a sign of what pathway being affected?
Indirect pathway of the basal ganglia
What is another sign associated with chorea?
Cicadic eye movements
List some symptoms of Huntington’s:
Mood disorders
Unsteady gait
Cognitive thinking disturbance
Rigidity and seizures
What is the cause of death in 9% of patients with Huntington’s
Suicide
What class of drugs are given for symptomatic management?
Dopamine depleting agents
Dopamine Receptor antagonists
What is the significant difference between Huntington’s dementia and Alzheimer’s?
Patients with Huntington’s can still remember places and people even at their advanced stage
Where in the brain will show significant degeneration in Huntington’s?
Caudate nucleus
How many repeats of glutamine will result in full penetrance?
> 38
What chromosome is the gene for Huntington’s located on?
Chromosome 4
What is the trinucleotide that repeats in Huntington’s?
CAG
What medication is used first line in the treatment of Huntington’s chorea
Tetrabenazine
When might tetrabenazine be contraindicated?
In patients with underlying psychiatric conditions
What medication can be used second line therapy for Huntington’s chorea?
Sulpiride
What other medications can be added on to the treatment regime in Huntington’s as third line options
Anti-psychotics
both typical and atypicals
Why should you reduce the dose of tetrabenazine in alter stages of disease?
As the disease progresses the chorea tends to reduce and the patient begins to suffer more from bradykinesia.
Tetrabenazine is specifically for chorea so will be of little benefit later in the disease.
In Huntington’s where is cortical atrophy most common?
Frontal lobe
