Lecture 5 Flashcards
What parts of the brain are first degenerated in HD
Caudate and putamen
Can HD affect other areas of the brain besides CP
Yes as it progresses
Is HD hypo or hyper kinetic
Hyper
Juvenile onset age HD
before 20
Juvenile onset prevalence HD
10%
When do most people develop motor symptoms of HD
In their 40s or 50s
What percent of people have motor symptoms of HD after age 60
10%
Adult onset age of HD
After age 20
Major stages of adult onset HD progression
- Prodrome
2. Manifest
Prodrome
Presymptomatic stage. Often includes a host of subtle yet detectable symptoms occurring up to 15 years before HD diagnosis
Manifest
Stages of disease progression follow a clinical diagnosis
Substages of manifest
- Early stage
- Middle stage
- Late stage
Length of survival after clinical diagnosis of HD
10-20 years
Why is HD considered a 3 disorder disease
Has 3 major disorders involved (movement, cognitive, and psychiatric)
Involuntary movements
Chorea
When does chorea typically increase in HD
Middle stages
When does chorea decline in HD
As rigidity increases in the later stages
What movements are imapired in HD
Voluntary
Other movement issues in HD
Reduced manual dexterity, slurred speech, swallowing difficulties, problems with balance, and falls
Exectutive efficacy in HD
Reduction of speed amd flexibility in mental processing
What cognitive skills decline in HD
Learning, memory, and language
Perception issues in HD
Trouble with perception of emotion, time, and space
What is the first sense to be lost in neurological diseases
Smell
Are HD patients aware of their actions and feelings
No
What percent of HD patients have impaired awareness
1/3
Impulsivity in HD
Irritabilitym temper outburts, acting without thinking
Do HD patients have normal declarative memory
Yes, but they have trouble accessing it at normal speed (can answer multiple choice but not opened ended questions)
What types of memories are affected in HD
Coordinated skills and movements (procedural)
Why do HD patients have trouble chewing and swallowing
Requires muscle memory
Hypofrontal or dysexecutive syndrome
Dpression, mania, apathy, irritability, hallicunations, impulsivity, and obsessionality
Suicide in HD
Common
Sexual behavior in HD
Lack of interest
Sleep and HD
Insomina
Is CAG more common in spermatogenesis or oogenesis
Spermatogenesis (more likely to get it from your father)
Why do juveniles get HD
More CAG repeats (early onset, faster progression, more severe symptoms)
Common symptoms in juvenile HD
Seizures and rigidity (not seen in adults)
What symptom of HD is common in adults but not in juveniles
Chorea
How many CAG repeats do juveniles usually have
Usually above 55, sometimes over 100
Signs of onset of juvenile HD
Decline in school performance, behavioral problems, movement difficulties, personality changes
Symptoms of juvenile HD
Rigidity, seizures, dementia, speech, and swallowing problems
Treatments for juvenile HD
Anticonvulsant (seizure) drugs and physiotherapy
Affected areas of the juvinile HD brain
CP, GP, and cerebellum
Length of HD in juviniles
Less than 10 years
Inheritance of juvinile HD
50/50 chance of passing it to children
Is there a cure or treatment to strop progression or reverse HD
No
What do HD medications do
reduce the burden of symptoms
Chorea drug
Tetrabenazine and antipsychotics (risperidone)
Side effects of tetrabenazine
Worsening or triggering depression or other psychiatric conditions
Can antidepressants treat HD
Yes
Therapies for HD
Psycho, speech, physical, occupational