Mixed Dysarthria

Question 1

Mixed dysarthrias are common because…

Answer 1

the damage that causes dysarthia often affects more than one area or system.

Question 2

T/F

Mixed dysarthria is a combination of just 2 disarthrias

Answer 2

False

Can be 2 OR MORE (typically no more than 3 types)

Question 3

How is the mixed dysarthria named?

Answer 3

The component that dominates is the first name given, such as flaccid-spastic

Question 4

Why is it important to determine the types of mixed dysarthrias, the components,

Answer 4

because it may help rule out or confirm neurological diagnoses
e.g. flaccid-spastic usually = ALS

Question 5

What is the most common type of dysarthria?

Answer 5

Mixed (Figure 1-3 shows it to be the most frequently occurring dysarthria)

Question 6

Etiologies:

Answer 6

• 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)

Question 7

ALS is usually…

Answer 7

flaccid-spastic

Question 8

Types of mixed dysarthrias and percentages in Duffy’s practice:

Answer 8

• Flaccid-spastic – 42%
• Hyperkinetic-hypokinetic – 35%
• Ataxic-spastic – 23%
• Other mixes – 19%
• Hypokinetic-spastic - 7%
• Ataxic-flaccid-spastic – 6%

Question 9

Which is the most common type of mixed dysarthria?

Answer 9

Flaccid-spastic (42%)

Question 10

Which is the least common type of mixed dysarthria?

Answer 10

Ataxic-flaccid-spastic (6%)

Question 11

ETIOLOGIES: Specific diseases associated with mixed dysarthrias (6):

Answer 11

1. Degenerative diseases
2. Toxic - metabolic conditions
3. Vascular disorders
4. Trauma
5. Tumor
6. Infections and autoimmune diseases

Question 12

Degenerative diseases and mixed dysarthria:

Answer 12

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.

Question 13

Most common type of motor neuron degenerative disease that causes mixed dysarthria:

Answer 13

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.

Question 14

Onset of ALS between ages ___ to __ years old

Answer 14

40-70

Question 15

Patients with ALS usually die between __ and __ years after diagnosis

Answer 15

1-5

Question 16

Death with ALS is usually due to:

Answer 16

respiratory failure

Question 17

2 types of ALS:

Answer 17

Bulbar or spinal

Question 18

Patients with bulbar ALS initially have troubles with (2):

Answer 18

speech and swallowing problems very early on

Question 19

Patients with spinal ALS initially have troubles with (1):

Answer 19

problems with limbs first

Question 20

Motor neuron degenerative disease that causes mixed dysarthria (4):

Answer 20

1. ALS
2. Primary lateral sclerosis (PLS)
3. Spinal muscle atrophies
4. Progressive Bulbar Palsy

Question 21

Primary lateral sclerosis (PLS) (a motor neuron degenerative disease) and mixed dysarthria:

Answer 21

– affects UMN only
– may be hard to distinguish from ALS
- It typically produces spastic dysarthria but is hard to differentiate from ALS

Question 22

Spinal muscle atrophies (a motor neuron degenerative disease) and mixed dysarthria:

Answer 22

– 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

Question 23

Progressive Bulbar Palsy (a motor neuron degenerative disease) and mixed dysarthria:

Answer 23

– 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.

Question 24

Multiple Sclerosis and mixed dysarthria:

Answer 24

– 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.

Question 25

ALS affects more (men or women)

Answer 25

men

Question 26

MS affects more (men or women)

Answer 26

women

Question 27

What is the most common CNS disease affecting young and middle age adults?

Answer 27

MS

Question 28

Age of onset for MS

Answer 28

onset between 20 to 40 years of age

Question 29

Courses of disease for MS (4):

Answer 29

1. Benign – only few episodes, then remission 2. Relapsing-remitting – comes and goes 3. Remitting-progressing - comes and goes but progressively gets worse 4. Progressive – no remission

Question 30

Type of mixed dysarthria with MS:

Answer 30

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

Question 31

Frederich's Ataxia and mixed dysarthria:

Answer 31

- Is associated with cerebellar dysfunction but can also be associated with mixed spastic/ataxic dysarthria

Question 32

Progressive Supranuclear Palsy and mixed dysarthria:

Answer 32

- 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

Question 33

Multiple Systems Atrophy and mixed dysarthria:

Answer 33

- 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

Question 34

Shy-Drager syndrome (type of Multiplle Systems Atrophy) and mixed dysarthrai:

Answer 34

- 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

Question 35

What characteristics distinguish ALS from the others (2)?

Answer 35

1. Wet/gurgle voice | 2. Vocal Flutter

Question 36

Corticobasal degeneration and mixed dysarthria:

Answer 36

- 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.)

Question 37

Degenerative diseases that cause mixed dysarthria (6):

Answer 37

1. Motor Neuron Diseases (ALS, PLS, Spinal Muscle atrophies, Progressive Bulbar Palsy) 2. Multiple Sclerosis 3. Frederich's Ataxia 4. Progressive Supranuclear Palsy 5. Multiple Systems Atrophy e. g. Shy-Drager syndrome 6. Corticobasal degeneration

Question 38

Toxic - metabolic conditions that cause mixed dysarthria (2):

Answer 38

* Effects tend to be diffuse and damage can impair several components, thus resulting in a mixed dysarthria. 1. Wilson's disease 2. Hypoxic encephalopathy

Question 39

Wilson's disease (a toxic - metabolic condition that causes mixed dysarthria):

Answer 39

- 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

Question 40

Hypoxic encephalopathy (a toxic - metabolic condition that causes mixed dysarthria):

Answer 40

- 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

Question 41

Vascular disorders and mixed dysarthria:

Answer 41

- 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

Question 42

Trauma and mixed dysarthria:

Answer 42

- 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

Question 43

Tumors and mixed dysarthrias:

Answer 43

- Can affect various components of neurological system and produce various mixed dysarthrias

Question 44

Infections and autoimmune diseases and mixed dysarthria:

Answer 44

- 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

Question 45

SPEECH PATHOLOGY and ALS

Answer 45

* 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.

Question 46

Other symptoms that occur with ALS that aren't seen with single dysarthrias (2):

Answer 46

 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