Huntington's Disease and Dystonia Flashcards
(18 cards)
What’s the difference between hyperkinetic and hypo-kinetic?
Hyperk- excessive movement
Hypok- paucity of movement
Define chorea
- ‘group of dances’
- involuntary and rapid movements which look like jerky dancing
- Symptom of huntington’s, drugs and some infections
What causes HD?
- Defective Gene (mutated Huntingtin protein)
- Chromosome 4
- Leads to death of proteins
Typical onset age for HD
- 35-55
- Juvenile HD= <20
HD Symptoms
- Communication difficulties (dysarthria/cognitive/etc)
- Dysphagia
- Motor control changes (chorea)
- Mood changes
Expand on speech and language difficulties
- Articulation difficulties at start, progressively unintelligible to the point of being non-verbal
- Impaired breathing, strained voice quality, inappropriate rate, rhythm and pitch
- Language, struggling to; begin convos, put thoughts into words, access vocab
Medical management for Chorea
- Anti-psychotic meds (suppression of involuntary movements)
- Drugs reducing dopamine reaching cells
Dystonia occurs from dysfunction in what areas?
- Basal ganglia
- Cerebellum
- Supplementary motor areas
- Sensorimotor cortex
Dystonia Symptoms
- Uncontrollable, excessive muscle spasms and contractions
Diagnosing Dystonia
- MRI scan
- Genetic test
- Urine and blood tests
Focal Dystonia
- Affects single region of the body
- Possibly task specific
Segmental Dystonia
- Affects 2+ connected regions
Multifocal Dystonia
- Affects 2+ unconnected regions
Generalised Dystonia
- Affects trunk and 2+ parts of the body
Hemidystonia
- Entirely affects 1 side of body
Surgical Management for dystonia
- Pallidotomy and Thalamotomy
- DBS
Medical Management for dystonia
- Medication (anticholinergics, muscle relaxants, baclofen)
- Botulinum Toxin (injected into affected area to reduce spasms)
Concerning issues for SLTs
- Laryngeal dystonia (laryngeal muscles spasm)
- Oromandibular dystonia (affects lower facial muscles, tongue or jaw) (potential swallowing difficulties)
