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What is ALS?
What is affected?
What is usually the initial symptom?
Gender? Age? Symptom onset?
- Rapidly progressing degenerative disease
- UMN and LMN
- Men more than women, 40-70, slow but progressive
How to diagnose ALS?
Criteria for dx?
- EMG, clinical history, nerve conduction studies, diff dx
- El Escorial: Clinically definite (in bulbar region), probable (in 2 regions w/ UMN signs above LMN), or possible (UMN/LMN signs in 1 region)
LMN (spinal) onset
- speech problems earlier
- speech fine initially
- respiratory & swallowing problems
Dysarthria in ALS
Bulbar onset worse relative to speech
What symptoms are first noted?
- Mixed flaccid-spastic'
- Laryngeal (others may show too)
Mixed Spastic-Flaccid Dysarthria
Characteristics of spastic component?
- Weakness, increased muscle tone, hyperreflexia, extensor plantar reflex, pseudo bulbar palsy (exaggerated emotional response, hyperreflexia)
- Muscle weakness, atrophy
- Reduced vital capacity and maximum phonation time (bulbar symptoms)
- Elevated laryngeal resistance d/t spasticity (UMN)
- strained voice
- flaccid=weak vocal folds=breathy voice, reduced loudness
- Hypernasality, nasal emission
- reduced speed, strength, ROM, lingual movements, reduced ability to vary tongue at height
- May be affected
Overall speech characteristics (dysarthria in ALS)
slow rate, strained quality, imprecise articulation, hyper nasality, mono pitch, mono loudness, short phrases
Dysarthria in ALS
Acoustic evidence for: (4)
Typical changes in functional communication?
- Lengthened segment durations, lengthened vowels, reduced vowel space, voice onset timing differences
- reduced speaking rate, reduced length of breath groups
What is intelligibility compared to comprehensibility?
Issues to consider relative to comprehensibility?
- Intelligibility is actual acoustic signal
- Comprehensibility takes into account context; signal independent information
- semantic context, gestures, orthographic cues
Considerations with ALS and dysarthria?
- Discussion of communication disorder and information to give
- stay on top of symptom progression
- bset strategies for individual based on their needs (access to services, transportation, etc.)
- Treatment considerations: reduce environment distractions, repeat utterances, reduce length, complexity of utterances, writing key words, alphabet supplementation