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Research & Reviews: A Journal of Neuroscience
ISSN: 2277-6427 (Online), ISSN: 2348-7925 (Print)
Volume 10, Issue 3
www.stmjournals.com

Dysarthria and Dysphagia in Amyotrophic Lateral


Sclerosis: A Case Study
Theaja Kuriakose1,*, Girish K.S.2
1
Reader, Department of Speech Language Pathology, JSS Institute of Speech and Hearing, Kelageri,
Dharwad, India
2
Lecturer, Department of Speech Language Pathology, JSS Institute of Speech and Hearing, Kelageri,
Dharwad, India

Abstract
Amyotrophic lateral sclerosis (ALS) is the most common motor neuron disease affecting the
bulbar, limb and respiratory muscles. ALS is generally characterized by progressive
degeneration of both upper motor neuron (UMN) and lower motor neuron (LMN) dysfunction
leading to a ‘mixed’ spastic-flaccid dysarthria. To date, only a few studies have been
published regarding the nature of speech characteristics and dysphagia during the
developmental course of ALS. The present study highlighted the nature of speech impairment
and swallowing difficulties in a 36-year-old male who was diagnosed as having ALS with
bulbar signs. Speech evaluation indicated the presence of flaccid spastic mixed dysarthria.
His speech was characterized by hypernasality, imprecise consonants, distorted vowels,
harshness, short phrases, mono-pitch, mono-loudness, silent pauses, prolonged phonemes,
slow rate of speech, leading to poor speech intelligibility. Also, the swallowing assessment
revealed a moderate degree of dysphagia because of the “bulbar onset” of ALS. Knowledge of
speech, language and swallowing characteristics in individuals with ALS is crucial, as it helps
the professionals and the caregivers to give a better service. This knowledge is often used for
rehabilitation, employment, participation in the community, and to inform the role of family
members in the betterment of the patient.

Keywords: amyotrophic lateral sclerosis (ALS), degeneration, dysarthria, dysphagia, motor


neurons

*Author for Correspondence E-mail: thejakuriakose@gmail.com

INTRODUCTION sporadic disease and 45–55 years for familial


Amyotrophic lateral sclerosis (ALS) is a motor disease [5]. Though it can also affect young
neuron disease. It is an idiopathic, fatal disease adults, ALS is rare before the age of 40 years
of insidious onset, characterized by [6] and very few studies reported young
progressive degeneration of motor units in the individuals with ALS [7]. ALS impacts males
human nervous system. ALS is also known as and females differently where males are
‘Lou Gehrig’s disease’, ‘Charcot disease’ and affected more than females with a ratio of 3:2
‘Lou Gehrig disease’ [1]. Though uncommon, [8].
ALS is not a rare disease and is seen among all
ethnic and socioeconomic groups around the ALS is generally characterized by progressive
world [2]. Worldwide, ALS has an incidence degeneration of motor nerve cells in the brain
of 2.08, 1.80 and 0.60; with a prevalence of (upper motor neurons or UMN) and spinal
5.40, 3.40 and 2.34 in 100,000 European, cord (lower motor neurons or LMN).When the
American, and Asian populations, respectively motor neurons can no longer send impulses to
[3]. However, the frequency of ALS cases in the muscles, the muscles begin to waste away
India is 5 in 100,000 population [4]. (atrophy), causing motor weakness. UMN
damage leads to spasticity, weakness, brisk
The peak incidence of ALS is observed among gag reflex and brisk deep tendon reflexes in
individuals in the age range of 55–63 years for the limbs. Whereas LMN damage leads to

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Dysarthria and Dysphagia in Amyotrophic Lateral Sclerosis: A Case Study Kuriakose and
Girish

fasciculation, weak cough, absent gag reflex mono-pitch, short phrases and distorted
and weakness in the limbs, and later dysphagia vowels in their study.
[7, 9–11]. Prosodic abnormalities are also reported in an
individual with ALS. Motor speech analysis in
Despite the motor weakness, ALS does not individuals with ALS revealed a significantly
show any sensory abnormalities [12] that is, lower rate of speech (2.2 syllables/second
vision, hearing, sense of taste, smell, and touch diadochokinetic rate [3.1 ± 218.1]) [19].
stay intact. Language and cognition are also Individuals with ALS having mixed flaccid
considered to be intact in ALS, but studies spastic dysarthria exhibited prolonged
have reported that 1–3% of sporadic ALS intervals between phonemes, prolonged
involve overt dementia as a feature [13]. On phonemes and inappropriate silences [9, 10].
the other hand, 80% of the individuals with These prosodic problems can be due to the
ALS commonly possess complex speech weakness of the subsystems of speech
impairments which significantly hamper their production. Recently, the tongue and jaw
daily communication [14]. movements were investigated in a longitudinal
study on 33 English speaking individuals with
Over the last three decades, very few studies ALS (mean age: 62.65±6.87 years) and the
have discussed the speech characteristics in kinematic measurements revealed a reduction
ALS. The phonetic impairments underlying in the tongue movement size and speed [21].
reduced speech intelligibility in 25 English
speaking individuals with ALS was studied. Besides speech problems, one of the most
The results revealed that the most disrupted common problems of ALS is dysphagia or
phonetic features involved phonatory function swallowing difficulty [22]. Swallowing is a
(voicing contrast), velopharyngeal valving, complex function, which requires coordination
place and manner of articulation for lingual between various phases and any dysfunction in
consonants and regulation of tongue height for any of the phases of swallowing would lead to
vowels [15]. Similar studies were conducted dysphagia or disordered swallowing. Studies
by various authors on English speaking reported that most of the individuals with ALS
individuals with ALS and the results revealed suffer from mild or moderate dysphagia within
a significant deterioration in speech the first two years after the incidence [23, 24].
intelligibility, diadochokinetic rate, pulmonary The symptoms include inability to form and
capacity, speaking rate, and highly variable hold bolus in the mouth, difficulty in
phonatory measures such as jitter, shimmer mastication, bolus residue in the oral cavity,
and signal to noise ratio [16–18]. Increase in delayed swallow reflex, nasal penetration,
fundamental frequency (only in males) was cough before or during the swallow, reduced
also reported in individuals with ALS [19]. laryngeal elevation, throat clearing, gurgled
Also, the variability of frequency stability voice quality after the swallow and multiple
during sustained vowel sound prolongation swallow for a single bolus [25].
and a standard deviation of F0 were
significantly increased in individuals with Knowledge of speech, language and
ALS. swallowing characteristics in individuals with
ALS is very important and it helps the
Darley [20] reported that individuals with ALS professionals and the caregivers to give a
have a defective articulation of both consonant better service to ALS patients. This knowledge
and vowels, often rendering the speech is often used for rehabilitation, employment,
unintelligible, extremely slow production of participation in the community and to inform
words in short phrases, marked hypernasality the role of family members in the betterment
coupled with severe harshness and strained of the patient. In India, very few studies have
voice. In a study of 30 individuals with ALS, been carried out regarding the nature of speech
Orsini et al. & Ingre et al. [5, 6] reported that impairment and swallowing problems in
these individuals had imprecise consonants, people with ALS. A review of the literature
hypernasality, harsh voice quality, slow rate, indicates that ALS in young adults is rare [6].

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Research & Reviews: A Journal of Neuroscience
Volume 10, Issue 3
ISSN: 2277-6427 (Online), ISSN: 2348-7925 (Print)

Hence the present study was aimed to signs of both UMN and LMN damage such as
investigate the nature of speech impairment left lower limb weakness, weak cough, facial
and dysphagia in a young adult diagnosed with and tongue weakness with fasciculation and
ALS. dysphagia. The patient was diagnosed with
METHOD ALS by that neurologist.
A 36-year-old male working as a mobile shop
owner was referred to the Department of The patient reported that he had not
Speech Language Pathology, JSS Institute of experienced any speech difficulties prior to
Speech and Hearing, Dharwad, India from a this incidence. Speech and language
neurologist in July 2020 with the complaint of evaluation revealed no indication of language
unclear speech and difficulty in swallowing. impairment, but he exhibited severe
Detailed history revealed that, in June 2019, impairment in all the speech subsystems
the patient reported a high-grade fever for 8 indicating presence of severe dysarthria. He
days; later his friends informed him that they did not have any mask like face, rigidity of
are observing a change in his speech. speech musculature and involuntary
However, it was very subtle, and he neglected movements of upper or lower limbs. Frenchay
it. These speech problems gradually Dysarthria Assessment (FDA) test was carried
progressed. After two months of the above out to rate and examine the speech
symptoms, he also developed difficulty in subsystems. Henningsson’s four-point rating
swallowing both solid and liquid food. These scale [26] was used to assess the nasality,
symptoms also gradually progressed. The speech understandability and acceptability.
patient consulted a neurologist on 2019 Oct 1, Praat software (version 5.3) was used to
where nerve conduction study (NCS) was extract fundamental frequency and formant
done and diagnosed a case of anterior horn cell frequencies. Sydney Swallow Questionnaire
disease involving bulbar segments. His was used to assess the severity of swallowing
computed tomography (CT) scan reports problem.
revealed a few small T2/FLAIR hyper-intense
foci in bilateral fronto-parietal white matter, RESULTS
few enlarged upper jugular lymph nodes and The results of FDA revealed apparent masseter
no abnormal mass lesions. Later he consulted muscle weakness. Figure 1 shows the FDA
another neurologist and the reports revealed results.

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Dysarthria and Dysphagia in Amyotrophic Lateral Sclerosis: A Case Study Kuriakose and
Girish

Fig. 1: Results of Components of FDA.

Fig. 2: Formants of vowels /a/, /i/ and /u/.

The patient took frequent breath intake examination revealed severe weakness. He
indicating poor respiratory control. He could was unable to attempt tongue elevation and
sustain ‘ah’ for about 8–9 sec. His cough was lateral movements. His tongue protrusion was
weak. His lips were slightly drooping, and the found to be limited and unable to manage
movement was consistently poor—there was a more than two movements. Alternating motion
reduction in speed and range during retraction rate (AMR) and sequential motion rate (SMR)
and pursing of the lips. showed reduced speed and accuracy of
movements. The rate obtained for AMR and
At rest, the jaw appeared to be relaxed in a SMR were 1.75 and 0.36, respectively.
normal position; however, in speech task
minimal deviation was noticed. Tongue

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Research & Reviews: A Journal of Neuroscience
Volume 10, Issue 3
ISSN: 2277-6427 (Online), ISSN: 2348-7925 (Print)

The palatal movement revealed minimal Swallowing assessment revealed that the
movements over repetitions of ‘ah-ah-ah’. patient experienced chocking occasionally and
There were significant hypernasality and nasal reported drooling. He was intaking a pureed
air emission. Henningsson’s four-point rating diet at the time of diagnosis. Sydney swallow
scale of nasality [26] was administered and the questionnaire was used to assess the severity
patient had a score of four indicating severe of the swallowing problem. The results
hypernasality. indicated that he was having moderate
difficulty in swallowing soft, hard and dry
The articulatory assessment revealed the food. He always needed to swallow more than
presence of imprecise consonant production once for the food to go down. The impression
and vowel distortions. All classes of sounds obtained from the evaluation showed the
were affected. There were severe distortions presence of dysphagia. However, we could not
for lingua-palatal and velar sounds followed carry out an instrumental evaluation.
by bilabials and labiodentals. Glottal sounds
and inter-dental sounds were least affected. Speech evaluation indicated that the patient
Vowel articulation revealed mild distortion for demonstrated some of the speech
the vowel /a/. The vowel /u/ was more affected characteristics of more than one type of
followed by the vowel /i/. Acoustic analysis dysarthria indicating it to be a mixed type of
revealed higher F1, F2 and F3 and breakdown dysarthria. He had severe hypernasality, nasal
of formant structure. Figure 2 and Table 1 air emission (a characteristic feature of flaccid
shows the formant frequencies of vowels /a/, dysarthria), strained and harsh voice quality,
/i/ and /u/. short phrase, slow rate of speech (spastic
component), and imprecise consonant,
Table 1: Formant Frequencies of Vowels /a/, monopitch and monoloudness (characteristic
/i/ and /u/. features that are common to both flaccid and
Vowels Formant 1 Formant 2 Formant 3 spastic dysarthria). Hence the provisional
/a/ 804 Hz 1259 Hz 2771 Hz diagnosis of the patient was flaccid-spastic
/i/ 514 Hz 1643 Hz 2806 Hz mixed dysarthria.
/u/ 569 Hz 1108 Hz 2756 Hz
DISCUSSION
Henningsson’s four-point rating scale [26] was Motor neuron diseases are a group of disorders
used to assess the speech understandability that involve the degeneration of motor
and acceptability. The patient obtained a score neurons. ALS is the most common motor
of four in speech understandability which neuron disease. ALS can affect bulbar, limb
indicated that the speech was hard to and respiratory muscles. ALS is a disease of
understand most or all the time. His speech both UMNs and LMNs, but its initial
acceptability indicated deviation of speech to a manifestation may be confined to the LMNs.
severe degree. Voice evaluation revealed Thus, ALS may produce flaccid dysarthria
hypernasality, breathiness and reduction in secondary to cranial nerve involvement [27].
loudness. His fundamental frequency was About 80% of the affected individuals with
found to be 148 Hz. ALS develop dysarthria at some point during
the disease course [28]. The type of dysarthria
Prosodic aspects of speech were also found to that appears in individuals with ALS depends
be affected. The perceptual evaluation on which motor neurons are affected by the
indicated that he had monopitch and disorder. In the study of 30 subjects with ALS,
monoloudness in connected speech. Speech Darley et al. [9, 10] found that the subjects’
examination also revealed short phrases, speech errors reflected the combined
inappropriate silences, prolonged phonemes characteristics of flaccid and spastic
and prolonged intervals between phonemes. dysarthria.
His rate of speech was found to be 1–2 The present study also showed clusters of
syllables per second indicating a slow rate of abnormal speech characteristics of both flaccid
speech. and spastic dysarthria. He had severe

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Dysarthria and Dysphagia in Amyotrophic Lateral Sclerosis: A Case Study Kuriakose and
Girish

hypernasality, nasal air emission and poor intelligibility. The presence of resonatory
respiratory control (a characteristic feature of incompetence, which was characterized by the
flaccid dysarthria), strained and harsh voice presence of hypernasality, nasal air emissions,
quality, slow rate of speech and distorted short phrases, and imprecise consonants in our
vowels (spastic component) and imprecise case, indicated the presence of velopharyngeal
consonants, short phrases, monopitch and valve weakness, leading to excessive nasal
monoloudness (characteristic features that are resonance.
common to both flaccid and spastic
dysarthria). These findings agreed with the Voice problems are reported for individuals
previous findings [9, 10, 20]. Darley et al. [9, with ALS [16–18]. In specific, studies have
10] also reported that ALS with mixed flaccid– reported an increased fundamental frequency
spastic dysarthria exhibit three features that in their case [19]. In the present study, the
are not found in pure flaccid or spastic patient also obtained a higher fundamental
dysarthria. They were prolonged intervals, frequency of about 148 Hz and increased
prolonged phonemes and inappropriate formant frequencies. Other voice problems
silences. These three features were also identified in the present case were harsh and
evident in our case indicating that our case had strained voice quality. Researchers also
flaccid–spastic mixed dysarthria. Results of reported the presence of harsh voice quality in
the intelligibility rating scale revealed poor an individual with mixed flaccid–spastic
speech intelligibility, which can also be seen dysarthria due to ALS [9, 10]. The probable
in flaccid–spastic mixed dysarthria [9, 10, 20]. reason what they reported in their cases was
due to turbulence during the speech from
Several studies [15, 16, 29, 30] shed light on saliva that has accumulated in the pyriform
the articulatory abnormalities that contribute to sinuses and on the vocal folds because of
reduced intelligibility in ALS. The most
reduced frequency of swallowing or
prominent abnormalities are related to
inadequate clearing of secretions. The same
velopharyngeal function, lingual functions,
may be the reason in the present case as well
syllable shapes, voicing contrasts and
because on examination it was noted that the
regulation of tongue height for vowels. In the
present case, there were imprecise consonants present case also experienced moderate
and vowel distortions. Linguapalatal and velar difficulty in swallowing.
sounds were severely affected followed by
bilabials and labiodentals. Glottal sounds and Prosodic abnormalities were evident in our
interdental sounds were least affected. The case. Speech evaluation revealed the presence
reason for this articulatory deficit can be a of prolonged intervals, prolonged phonemes,
weakness in the speech mechanism and inappropriate silences, reduced rate of speech,
reduced range and speed of movements of short phrases and reduced variation in pitch
articulators. and intensity. These findings were supported
by the findings of Darley et al. [9] who
Individuals with ALS can have weaknesses in reported individual with ALS exhibiting three
limbs and oral speech mechanisms [7, 9–11]. features such as prolonged intervals,
The neurological findings of the patient prolonged phonemes and inappropriate
revealed left lower limb weakness, facial and silences which are not found in pure flaccid or
tongue weakness with fasciculation which was spastic dysarthria. Researchers found that the
in agreement with the previous literature. individuals with ALS experience a slow rate of
There were marked reduction in the range and speech and reduction in DDK rate [19, 21].
speed of movements of the structures such as The probable reasons for these prosodic
lips, tongue and soft palate indicating abnormalities are weakness and reduction in
weaknesses in the oral peripheral mechanism. the speed and range of articulators. Short
These abnormalities directly correlate with phrases in the present case reflect the effect of
measures of speech function including the air wastage through the velopharyngeal

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Research & Reviews: A Journal of Neuroscience
Volume 10, Issue 3
ISSN: 2277-6427 (Online), ISSN: 2348-7925 (Print)

port. Another reason for short phrases could be


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ISSN: 2277-6427 (Online), ISSN: 2348-7925 (Print)

Cite this Article


Theaja Kuriakose, Girish K.S. Dysarthria
and Dysphagia in Amyotrophic Lateral
Sclerosis: A Case Study. Research &
Reviews: A Journal of Neuroscience. 2020;
10(3): 12–19p.

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