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What is the most common cause of hypokinetic dysarthria?
Damage to the dopaminergic nigrostriatal pathway
What are the neurodegenerative disorders of hypokinetic dysarthria?
Parkinson’s Disease, Progressive Supranuclear Palsy, and Multisystem atrophy-Parkinsonian type (MSA-P)
What are the vascular conditions of hypokinetic dysarthria?
midbrain or thalamic stroke
What is Parkinson’s Disease caused by? How is it treated?
-a progressive disease that affects the dopamine producing cells of substantia nigra
-Typically treated by neurology team
What is Parkinsonism/atypical Parkinsons?
a disease that presents like PD but doesn’t respond to typical medication (because the receptors are damaged)
What is progressive supranuclear palsy (PSP)? What systems are affected?
-progressive disorder that affects multiple systems (basal ganglia, midbrain, and brainstem), but has strong parkinsonian features; dysarthria and dysphagia often present quite early
What is multisystem atrophy, Parkinsonian type (MSA-P); what is the prognosis?
-progressive disease that involves multiple areas of the brain
-typically presents in early 50s and prognosis is about 9 years
-can develop into multiple types of dysarthria (due to the multisystem involvement)
What are the vascular conditions resulting in hypokinetic dysarthria? What are the symptoms?
-Strokes that affect: basal ganglia, midbrain, thalamus (bilaterally), and frontal lobe white matter
-tends to present more with UMN-type symptoms (pseduobulbar affect, pathological reflexes, spastic dysarthria) along with parkinsonism
What is drug-induced parkinsonism? When do symptoms start/stop?
-medications that block dopamine receptors (dopamine antagonists)
-symptoms usually develop within 2 months of starting medication and symptoms stop soon after going off medications
What can traumatic brain injuries cause?
hypokinetic dysarthria because repeated head injuries can cause damage to deep brain structures, including substantia nigra
What are the some of the most common symptoms for hypokinetic dysarthria?
bradykinesia (slow to initiate/move), festination, pill-rolling tremor (resting tremor),
What are the perceptual characteristics of hypokinetic dysarthria?
Respiration: shallow breathes, reduced loudness
Phonation: monopitch, breathy, vocal flutter
Resonance: hypernasility
Articulation: consistently imprecise from undershooting
Prosody: abnormal speech rate (either fast or slow), faster AMRs than SMRs (however, they are likely imprecise)
What are the general therapy goals for hypokinetic dysarthria?
compensation and adjustment; many rely on cognitive effort to speak more intelligibility (Speak with Intent, LoudCrowd); may also focus on more social aspect (to help prevent social withdrawal and increase patient engagement)
Why does hyperkinetic lead to excess movements?
not enough inhibition, so extra movements “spill out” due to extra firing from thalamus
What can cause the imbalance of excitatory vs. inhibitory for hyperkinetic dysarthria?
-Indirect (inhibitory) circuit parts can be damaged (especially the subthalamic nuleus)
-Some cerebellar damage can generate hyperkinesia
-Neurotransmitters can be unbalanced (too much dopamine, too much glutamate, not enough GABA)
In hypokinetic dysarthria, the intended movements are what?
diminished
What are dyskinesias?
abnormal movements
What is chorea?
rapid, purposeless and random type movements
What is dystonia?
abnormal postures due to inappropriate co-contraction of agonists and antagonists- can be constant or very slow
What are the perceptual characteristics you would expect for hyperkinetic dysarthria?
-Respiration: variable loudness
-Pitch: abnormal pitch and vocal quality
-Resonance: both hypo and hyper
-Articulation: irregular articulatory breakdowns (both under and overshoot)
-Prosody: slow speech rate
What are the degenerative diseases that cause hyperkinetic dysarthria?
-Huntington’s Chorea
-Essential tremor
What are the congenital disorders that cause hyperkinetic dysarthria?
-dyskinetic (athetoid) cerebral palsy
-Tourette’s Syndrome
What is Huntington’s Disease/Chorea? What is the onset and prognosis?
-loss of neurons in striatum
-typically presents ages 40-50, though there is also juvenile onset
-prognosis 10-20 years
What are the 2 main types of tremors?
resting and action tremor
What are the types of action tremors?
postural, kinetic, and terminal (intention)
What are postural tremors?
observed when actively sustaining a posture
What are kinetic tremors?
triggered by a movement and observed throughout the movement
What are terminal (intention) tremors?
triggered by movement, but specifically prominent when approaching the target
What is essential tremor?
is observed during postures and active movements
What is a palatal tremor (myoclonus)?
observed specifically in the palate, pharynx, and larynx
What is dyskinetic (athetoid) cerebral palsy? What is it associated with? What are the different presentations?
-subtype of CP that results in dyskinesia and/or dystonia
-Associated with thalamic/basal ganglia damage
-Different presentations: chorea-type (random, rapid), dystonia type (slower), and athetotic type (slow/writhing-neurologists lump this with dystonia)
What is Gilles de la Tourette Syndrome?
repetitive/unwanted sounds that can’t be easily controlled?
What are the 2 main types of tics?
-Simple tics: use a small set of muscles
-Complex tics: more complex, can be “full action”
What can tics be further divided into?
-Vocal tics and Motor tics
What is tardive dyskinesia? How is it treated?
-affects people who take antipsychotic medications, especially when taken long-term
-typically managed by adjusting medication dose, but some people have persistent symptoms
What is spasmodic dysphonia? When does it typically present? What is it caused by?
-a type of focal dystonia
-typically presents in 40s/50s, but can appear anywhere in adulthood
-causes: often after upper respiratory infection or similar; can be exacerbated by stress and induced by action
What are the two types of spasmodic dysphonia?
-abductor (ABSD) and adductor (ADSD)
What is abductor (ABSD) dysphonia?
irregular periods of breathy or whispered voice
What is adductor (ADSD) dysphonia?
irregular periods of strained, pressed, or squeezed voice
When testing for spasmodic dysphonia, what do we typically rely on?
-mostly percept of vocal quality changing
What do we mainly rely on when looking at EVT?
-pitch and/or loudness changing (higher pitches increases amplitude and frequency of tremor)
For dyskinesias, what can we do in therapy?
can do behavior modification for spasmodic dysphonia & sensory tricks for dystonia; however, they are often treated surgically or pharmacologically
What does ataxia result from?
damage to any part of the cerebellar circuit
What are some of the more common symptoms of ataxia?
-Typically the symptoms of someone who is drunk (difficulty with gait- balance and walking), postural sway, intention tremor, dysdiadochokinesia (difficulty with rapid alternating movements)
What are the degenerative disorders of ataxia?
spinocerebellar ataxia, Friedreich’s ataxia, mulitsytem atrophy, cerebellar type (MSA-C), demyelinating disease (e.g., MS), and ataxia telangiectasia
What is spinocerebellar ataxia (SCA)?
-neurodegenerative disease that affects the cerebellum; a variety of subtypes and a range of prognoses
What is Friedreich’s Ataxia? When is is typical onset and prognosis?
-affects both cerebellum and input/output tracts > also impacts other areas like peripheral nerves, brainstem, heart, etc.
-can start in childhood through early adulthood and about a 20 year prognosis from onset of symptoms
What is multisystem atrophy, cerebellar type (MSA-C)?
-multiple brain structures impacted and the dominant symptoms are ataxic in natre
What is ataxia telangiectasia (Louis-Bar syndrome)
-ataxia + dilation of small blood vessels
When does ataxia telangiectasia (Louis-Bar syndrome begin? What are its features?
-emerges in childhood (usually before age 5)
-affects nervous system, vascular system, and immune system
-also has hyperkinetic features (myoclonus and/or dystonia)
What are the types of cerebral palsy that result in ataxia?
ataxic and hypotonic
What is cerebellitis?
sudden onset of ataxia that follows viral infection; can manifest in both children and adults
What are cerebellar strokes? What are it’s features?
-blockages/hemorrhages of arteries supplying the cerebellum
-immediate ataxic symptoms (e.g., vertigo, nausea, loss of balance, dysmetria, dysarthria)
-follows same pattern as other strokes: sudden onset of symptoms, spontaneous recovery (to some level), and slow improvement/plateau
What are the different types of alcohol-related ataxia?
-acute: when actively intoxicated
-chronic/alcoholism-related: permanent condition; may improve with cessation of alcohol intake but not always
-fetal alcohol spectrum disorder (FASD): permanent condition caused by in-utero exposure to alcohol
What are the perceptual characteristics of ataxia?
-Respiration: inconsistent (sometimes explosive)
-Phonation: variable vocal quality, wandering pitch
-Resonance: typically normal, but occasionally hypernasal
-Articulation: inconsistent, imprecise articulation (AMRs are harder than SMRs)
-Prosody: typically slow, but inconsistent; telescoping syllables
Why is ataxia especially hard to work on in therapy?
because cerebellar damage makes it harder to motor plan
What do we typically focus on for individuals with ataxia in therapy?
-compensation
-taking slower and putting effort into speaking clearly