Mixed Dysarthria Flashcards
Mixed dysarthrias are common because…
the damage that causes dysarthia often affects more than one area or system.
T/F
Mixed dysarthria is a combination of just 2 disarthrias
False
Can be 2 OR MORE (typically no more than 3 types)
How is the mixed dysarthria named?
The component that dominates is the first name given, such as flaccid-spastic
Why is it important to determine the types of mixed dysarthrias, the components,
because it may help rule out or confirm neurological diagnoses
e.g. flaccid-spastic usually = ALS
What is the most common type of dysarthria?
Mixed (Figure 1-3 shows it to be the most frequently occurring dysarthria)
Etiologies:
- Commonly caused by more than one event, i.e. multiple strokes, combination of neurological or degenerative diseases such as PD and stroke, chorea and stroke.
- Often seen in degenerative diseases (e.g. ALS)
ALS is usually…
flaccid-spastic
Types of mixed dysarthrias and percentages in Duffy’s practice:
- Flaccid-spastic – 42%
- Hyperkinetic-hypokinetic – 35%
- Ataxic-spastic – 23%
- Other mixes – 19%
- Hypokinetic-spastic - 7%
- Ataxic-flaccid-spastic – 6%
Which is the most common type of mixed dysarthria?
Flaccid-spastic (42%)
Which is the least common type of mixed dysarthria?
Ataxic-flaccid-spastic (6%)
ETIOLOGIES: Specific diseases associated with mixed dysarthrias (6):
- Degenerative diseases
- Toxic - metabolic conditions
- Vascular disorders
- Trauma
- Tumor
- Infections and autoimmune diseases
Degenerative diseases and mixed dysarthria:
These often affect several parts of the motor system thus produce mixed dysarthrias
This is the most common etiology of mixed dysarthrias.
e.g. Motor neuron disease - primarily affects upper or lower motor neurons or both.
Most common type of motor neuron degenerative disease that causes mixed dysarthria:
Amyotrophic lateral sclerosis (ALS)
- Has both UMN and LMN involvement
- Commonly has mixed spastic-flaccid or flaccid-spastic components
- ALS occurs in 1% to 5 % per 100,000
- More men affected than women. 5% is familial
- Onset for most is between 40 to 70 years
- Often hard to diagnose - Diagnosis confirmed by EMG findings of weakness
- Nearly always fatal. Most die between 1 and 5 years after diagnosis. Some few live more than 12 years
- Death usually due to respiratory failure – but person can be put on ventilator.
Onset of ALS between ages ___ to __ years old
40-70
Patients with ALS usually die between __ and __ years after diagnosis
1-5
Death with ALS is usually due to:
respiratory failure
2 types of ALS:
Bulbar or spinal
Patients with bulbar ALS initially have troubles with (2):
speech and swallowing problems very early on
Patients with spinal ALS initially have troubles with (1):
problems with limbs first
Motor neuron degenerative disease that causes mixed dysarthria (4):
- ALS
- Primary lateral sclerosis (PLS)
- Spinal muscle atrophies
- Progressive Bulbar Palsy
Primary lateral sclerosis (PLS) (a motor neuron degenerative disease) and mixed dysarthria:
– affects UMN only
– may be hard to distinguish from ALS
- It typically produces spastic dysarthria but is hard to differentiate from ALS
Spinal muscle atrophies (a motor neuron degenerative disease) and mixed dysarthria:
– affects LMN only
– progressive limb wasting and weakness
– may have some cranial nerve weakness as well
- It is a motor neuron disease but typically produces only flaccid dysarthria, not mixed
Progressive Bulbar Palsy (a motor neuron degenerative disease) and mixed dysarthria:
– primarily has LMN weakness of cranial nerve muscles. - Dysarthria and dysphagia predominate
- Similar to ALS without limb involvement
- It is usually associated with flaccid dysarthria, not mixed.
Multiple Sclerosis and mixed dysarthria:
– it is a demyelinating disease
– the myelination is attacked but the axon itself is not
- Cause is unknown – may be related to autoimmune cause triggered by environmental and genetic interactions
- Diagnosis is difficult – MRI often used for diagnosis.
- Patient often exhibits problems with vision and other sensory difficulties and some cognitive deficits.
- Dysphagia is not common.
ALS affects more (men or women)
men
MS affects more (men or women)
women
What is the most common CNS disease affecting young and middle age adults?
MS
Age of onset for MS
onset between 20 to 40 years of age
Courses of disease for MS (4):
- Benign – only few episodes, then remission
- Relapsing-remitting – comes and goes
- Remitting-progressing - comes and goes but progressively gets worse
- Progressive – no remission
Type of mixed dysarthria with MS:
Most common type of mixed dysarthria is ataxic-spastic but any combination can occur
The type of dysarthria is more unpredictable than with other diseases
Frederich’s Ataxia and mixed dysarthria:
- Is associated with cerebellar dysfunction but can also be associated with mixed spastic/ataxic dysarthria
Progressive Supranuclear Palsy and mixed dysarthria:
- neurodegenerative disease associated with multisystem involvement
- May be confused with PD because it has similar symptoms such as bradykinesia
- Dysphagia and dysarthria are common and are evidenced early on in the disease
- Mixed dysarthrias seen in this disease are combinations of hypokinetic, spastic and ataxic
Multiple Systems Atrophy and mixed dysarthria:
- similar to PSP and PD - but doesn’t respond well to L-Dopa
- Shy Drager Syndrome falls under this category of diseases
- Types of mixed dysarthrias include hypokinetic, hyperkinetic, ataxic and spastic depending on the underlying pathologies
Shy-Drager syndrome (type of Multiplle Systems Atrophy) and mixed dysarthrai:
- progressive neurologic disease
– unknown etiology
- First signs are usually blood pressure problems when standing, incontinence and decreased respiration and impotence
- May also have problems with gait, dysarthria and dysphagia
- Most common types of dysarthria associated with SDS are hypokinetic, ataxic, spastic and flaccid
What characteristics distinguish ALS from the others (2)?
- Wet/gurgle voice
2. Vocal Flutter
Corticobasal degeneration and mixed dysarthria:
- It is rare and involved impairment of cortex and basal ganglia
- presents most often with spastic-hypokinetic dysarthria, but can have ataxic and hyperkinetic components.
(Primary progressive aphasia is sometimes put under this category.)
Degenerative diseases that cause mixed dysarthria (6):
- Motor Neuron Diseases (ALS, PLS, Spinal Muscle atrophies, Progressive Bulbar Palsy)
- Multiple Sclerosis
- Frederich’s Ataxia
- Progressive Supranuclear Palsy
- Multiple Systems Atrophy
e. g. Shy-Drager syndrome - Corticobasal degeneration
Toxic - metabolic conditions that cause mixed dysarthria (2):
- Effects tend to be diffuse and damage can impair several components, thus resulting in a mixed dysarthria.
1. Wilson’s disease
2. Hypoxic encephalopathy
Wilson’s disease (a toxic - metabolic condition that causes mixed dysarthria):
- It is a metabolic disorder leading to too much copper in brain, eyes and liver
- It can be fatal
- It often demonstrates brown rings around cornea of the eyes
- Dysarthria is a predominant symptom - and typically involves two or more of these: hypokinetic, ataxic, and spastic
Hypoxic encephalopathy (a toxic - metabolic condition that causes mixed dysarthria):
- Due to lack of oxygen to brain because of heart and lung problems
- With loss of oxygen, brain damage occurs
- Mixed dysarthrias often occur involving hypokinetic, hyperkinetic and ataxic dysarthrias
Vascular disorders and mixed dysarthria:
- Multiple strokes often affect multiple systems and produce mixed dysarthrias of any combination
- If strokes affect brainstem, dysarthria may include flaccid, spastic and ataxic due to close proximity of pyramidal and extrapyramidal fibers
Trauma and mixed dysarthria:
- TBI can produce any combination of dysarthrias
- Mixed dysarthia is more common with TBI than single dysarthrias due to the diffuse injuries associated with TBI
Tumors and mixed dysarthrias:
- Can affect various components of neurological system and produce various mixed dysarthrias
Infections and autoimmune diseases and mixed dysarthria:
- AIDS is a good example and can produce various mixed dysarthrias
- The type of dysarthias depend on the condition that is presenting with AIDs as various conditions can result
SPEECH PATHOLOGY and ALS
- Dysarthria is often the first sign.
- The majority are unintelligble by the time death occurs.
- Most need AAC intervention within approximately 3 years of diagnosis and use them about 2 years before death.
- Dysarthria may not be mixed throughout the entire disease.
- Dysarthria has components of both flaccid and spastic when it is mixed and either of those can predominate.
- Symptoms of each of those types can occur, i.e. with spasticity - pathological reflexes and pseudobulbar effect can occur; with flaccidity - fasiculations and atrophy can occur.
- Intelligibility can be severely affected due to hypernasality, reduced ability to build up intraoral air pressure and weakness of tongue.
Other symptoms that occur with ALS that aren’t seen with single dysarthrias (2):
tremor or “flutter” in voice is commonly seen, especially in vowel prolongation. (The cause is not known.)
wet or gurgly voice - due to turbulence in voice from saliva that builds up in pyriform sinuses and on vocal folds due to reduced swallowing and clearing of secretions