IP Journal of Otorhinolaryngology and Allied Science

Print ISSN: 2582-4147

Online ISSN: 2582-421X

CODEN : IJOABK

IP Journal of Otorhinolaryngology and Allied Science (JOAS) open access, peer-reviewed quarterly journal publishing since 2018 and is published under the Khyati Education and Research Foundation (KERF), is registered as a non-profit society (under the society registration act, 1860), Government of India with the vision of various accredited vocational courses in healthcare, education, paramedical, yoga, publication, teaching and research activity, with the aim of faster and better dissemination of knowledge, we will be publishing the article more...

  • Article highlights
  • Article tables
  • Article images

Article statistics

Viewed: 282

PDF Downloaded: 97


Get Permission Shabnam, Madhusudhanan, Udumbassery, and Bhagavan: Navigating early speech and swallowing challenges in progressive supranuclear palsy: A case study


Background

Progressive supranuclear palsy (PSP) is a multisystem neurodegenerative disease of unknown etiology. PSP is a rare neurological condition that results in severe and persistent issues with control of gait and balance.1 The disorder is brought on by a progressive decline in the brain cells that regulate coordination and movement, as well as by exposure to an unidentified dietary ingredient and random genetic mutation. The symptoms of PSP are cognitive change, impaired balance, early falls, blurry or double vision, sleep difficulties, dysphagia, drooling, urinary urgency or incontinence, constipation, depression or anxiety, hyperphagia and change in food preferences, and weight loss. Common sign of PSP include akinetic rigidity, slow saccades and verticle saccades, vertical supranuclear gaze palsy, frontalis overactivity, reduced blink, staring expression, uncontrolled decent into a chair, dystonia, apraxia, emotional lability, reduced verbal fluency and dysarthrophonia. 2 The gradual reduction of voluntary movements is one of the hallmarks of progressive supranuclear palsy. Dysarthria and dysphagia were the most prevalent speech and language traits connected to PSP. 3 Josephs et al. 4 have discussed a clinical variant of PSP, i.e. progressive supranuclear palsy (PSP) with predominant speech/ language disorder (PSP-SL), where individuals of this variant reported to have progressive apraxia of speech, or agrammatism. Most of these individuals present symptoms such as Bradykinesia, rigidity, postiural instability, ocular motor impairment and ideomotor apraxia.5, 6 However, there is very little knowledge about the dysphagia aspect in these individuals. The present case study discusses speech and swallowing problems in a patient at early stage of PSP, speech and swallowing assessment and rehabilitation.

Case Presentation

A 61-year-old man who had been experiencing balance issues, nasal regurgitation, and swallowing issues for 1.5 years brought his concerns to the hospital. The progression began gradually and was excessive. The neurological evaluation revealed abnormalities with speaking and swallowing, regurgitation episodes, bradykinesia, balance problems, saliva dribbling, hypophonia, hyponemia, and emotional vulnerabilty. The patient was given the diagnosis of Progressive Supranuclear Palsy P-type, and the neurologist recommended T Syndopa - plus (1mg 1-1-1/day). Following a neurological assessment, the patient was recommended for physiotherapy and a speech-language & swallowing evaluation. Bradykinesia and balance problems were found during the physiotherapy evaluation.

At the department of speech-language pathology, assessments of swallowing and speech were performed. The patient presented the complaint of nasal regurgitation, swallowing issues, and some speech slurring. The patient had a history of acidity for three months and pneumonia six months prior. After the detailed case history, the speech-motor evaluation, the Frenchay dysarthria assessment (FDA) 7 and the Manipal manual of swallowing assessment (MMSA) 8 were carried out. The purpose of the speech motor examination was to evaluate the structure, appearance and function of the oral structures, including the lips, tongue, jaw, velum, and teeth. The examination results revealed that the lateralization and elevation of tongue were affected. For lips, retraction, protrusion and alternative movements were affected. Table 1 provides an illustration of the test's outcomes for speech motor function.

Table 1

Results of speech motor examination

Oral structure

Appearance

Function

Lips

Normal

Retraction, protrusion- slow; Alternative movements- restricted

Tongue

Normal

Retraction, protrusion- normal Lateralization and elevation- restricted

Jaw

Normal

Normal

Velum

Normal

Normal

Teeth

Missing teeth

-

The FDA findings are shown in Figure 1. The FDA results revealed poor respiratory control both at rest and during speech. Reduced lip alternating movements, inadequate lip seal, and restricted lip spreading were noted. Palatal movements were fairly normal. The range of pitch and volume was diminished. There were limitations on tongue protrusion, elevation, lateral movements, and alternating movements. Speech intelligibility was marginally compromised. Hence, the diagnosis made was dysarthria. Further, MPD and s/z ratio was assessed for the patient. The maximum phonation duration was /a/= 8seconds, /i/ =9seconds, /o/ = 9seconds. The s/z ratio was 0.5 Both MPD and s/z ratio revealed poor respiratory control.

Figure 1

FDA results of the patient (initial assessment)

https://s3-us-west-2.amazonaws.com/typeset-prod-media-server/4778bd8b-d2c9-452a-82b9-437a7482fe30image1.jpeg

MMSA was used to evaluate swallowing, and Table 2 provides a summary of the findings. The results revealed that the oral and pharyngeal phases of swallowing were affected. Although the oral preparatory stage was normal, occasionally aspiration was present during swallowing. More difficulty was observed for liquids than solids. Hence, the results revealed the presence of oropharyngeal dysphagia.

Table 2

MMSA results of the patient

Subscales

Patient score

MMSA score for individuals with dysphagia 8

Mean

SD

Assessment of function

Sensory assessment

0

2.5

1.91

Motor assessment

38

34

8.76

Assessment of phases of swallowing

Dry swallow, thick liquid, thin liquid, solid

17

18

14.14

Total score

55

52.50

31.8

   Therefore, oropharyngeal dysphagia with dysarthria secondary to Progressive Supranuclear Palsy was the diagnosis made at the speech-language pathology department. The patient was given advice on diet modification, safe swallow strategies, and speech therapy. The patient was suggested to intake soft and mashed food, and to drink small sips of water frequently. Following safe swallow strategies were suggested to the patient: (i) Consume your food at a slow and attentive pace, giving your full attention to your meal, and avoid distractions like watching television or engaging in conversation during your eating time; (ii) Maintain an upright sitting posture when you dine, refraining from eating while reclining or lying down; (iii) Opt for a teaspoon when consuming solid foods. If you choose to use a straw, sip your drink one at a time, and if you're using a cup, take a small sip, tilting your chin downward before swallowing; (iv) Keep the food or beverage in your mouth and take a moment to consider swallowing before actually doing so; (v) Ensure that you've completely swallowed all the food in your mouth before taking another bite; and (vi) Don't rush through your meal; instead, savor it at a slow pace. In addition, oro-motor exercises to improve the strength and mobility of tongue and lip were demonstrated to the patient.

The individual was referred back to the neurology department, where they received instructions to take one tablet of T Syndopa plus (1mg) three times a day and were advised to come back for a follow-up appointment in a month. The patient came in for follow-up at the speech-language pathology department a month later. The patient's swallowing abilities significantly improved (Table 3), and there was no aspiration noted. Additionally, speaking clarity had increased as depicted in Figure 1. The FDA results at one month follow up indicted that there was a significant improvement across different domains of FDA.

Figure 2

FDA results of the patient (at first follow-up)

https://s3-us-west-2.amazonaws.com/typeset-prod-media-server/4778bd8b-d2c9-452a-82b9-437a7482fe30image2.png
Table 3

MMSA results of the patient (at first follow-up)

Subscales

Patient score (at first follow-up)

Patient score (initial assessment)

Assessment of function

Sensory assessment

0

0

Motor assessment

22

38

Assessment of phases of swallowing

Dry swallow, thick liquid, thin liquid, solid

09

17

Total score

32

55

The initial visit's interventions, which included dietary adjustments, safe swallowing techniques, oro-motor exercises, and medication, yielded a favorable outcome for the patient. The patient received advice from the speech-language pathology department to visit for follow-up appointments every six months and to contact the department right away if their speech intelligibility or swallowing difficulties worsen. The speech-language pathologist recommended that the patient schedule follow-up visits every six months and emphasized the importance of promptly reaching out if there is any deterioration in speech clarity or swallowing issues. Also, the patient was advised to continue the dietary adjustments, safe swallowing techniques, and oro-motor exercises.

Discussion

PSP, or Progressive Supranuclear Palsy, is a degenerative disease that impacts multiple systems, including the extrapyramidal system. It primarily affects the frontal cortex and its connections to the basal ganglia and brainstem. 9 Individuals with PSP may experience deficits in both the oral and pharyngeal phases of swallowing. While extensive research has been conducted to understand various aspects of PSP, such as its neurological signs and symptoms, and medical treatments, there is a noticeable lack of literature focusing on the speech and swallowing challenges faced by individuals with PSP, particularly in the early stages of the disease. The objective of the current study is to provide insights into a case of early-stage PSP and how the associated speech and swallowing symptoms were effectively managed.

This case report focuses on a 61-year-old male in the early stages of PSP who experienced aspiration, hypophonia, and swallowing difficulties. After assessment, he was diagnosed with Dysarthria and oropharyngeal dysphagia. Treatment included T Syndopa Plus medication, diet modification, oro-motor exercises and safe swallowing strategies, resulting in significant improvement in speech and swallowing skills within a month. This suggests that early intervention with medication and therapeutic strategies can be effective in managing PSP-related symptoms. These results are supported by previous researchers who suggest that combining specific exercises with compensatory strategies can yield better prognosis in individuals with dysphagia.10, 11

Conclusion

This case report describes a patient with progressive supranuclear palsy exhibiting typical symptoms. Treatment with T Syndopa Plus, along with dietary changes and safe swallowing techniques, led to significant improvement in speech and swallowing within a month, indicating the effectiveness of early intervention. The study highlights the significant impact of early intervention in neurodegenerative cases, particularly in improving the quality of life for patients with conditions like progressive supranuclear palsy. Early intervention encompasses both medical treatment, and rehabilitation strategies. To ensure the generalizability and reliability of these findings, further studies with larger sample sizes are necessary. A larger population-based study can provide more robust evidence regarding the effectiveness of early intervention strategies in improving the lives of individuals with neurodegenerative diseases. It can also help identify common patterns and variations in responses to treatment, ultimately leading to better guidelines for managing these conditions.

Source of Funding

No external funding was received for the research reported in the paper.

Competing Interests

We declare no conflict of interest in the paper titled “Navigating Early Speech and Swallowing Challenges in Progressive Supranuclear Palsy: A Case Study”.

A written consent from the participants was taken, where information regarding clinical procedure and approximate duration of the procedure was mentioned in the form. Permission was taken from the patient to publish the inform collected from the clinical procedure.

SM, NU, AB: ideation, formulation of the study design, data acquisition, inferring articles and drafting the manuscript; SS: ideation, inferring articles, and revision of manuscript.

Acknowledgements

Authors would like to acknowledge the participant of the study for consenting to participate in the study

.

References

1 

JR Duffy Motor speech disorders : substrates, differential diagnosis, and management2ndElsevier Health Sciences2019578

2 

JB Rowe N Holland T Rittman Progressive supranuclear palsy: diagnosis and managementPractical Neurol202121537683

3 

DB Freed Motor speech disorders: diagnosis and treatmentPlural Publishing2018381

4 

KA Josephs JR Duffy EA Strand MM Machulda ML Senjem JL Gunter The evolution of primary progressive apraxia of speechBrain201413710278395

5 

ZI Seckin JR Duffy EA Strand M Clark RL Utianski MM Machulda The evolution of parkinsonism in primary progressive apraxia of speech: A 6-year longitudinal studyParkinsonism Relat Dis2020813440

6 

KA Tetzloff JR Duffy EA Strand MM Machulda SM Boland RL Utianski Clinical and imaging progression over 10 years in a patient with primary progressive apraxia of speech and autopsy-confirmed corticobasal degenerationNeurocase201824211131

7 

P Enderby Frenchay dysarthria assessmentBrit J Dis Commun198015316573

8 

RK Balasubramanium JS Bhat Manipal Manual for Swallowing Assessment1stManipal Universal Press201288

9 

A Pakalnis M E Drake S Huber G Paulson B Phillips Central conduction time in progressive supranuclear palsy. Electro Clin Neurophysiol199232413

10 

ML Huckabee CA Pelletier Management of adult neurogenic dysphagiaSingular Publishing GroupSan Diego, CA1999350

11 

ME Groher MA Crary Dysphagia3rdElsevier Health Sciences2020400



jats-html.xsl


This is an Open Access (OA) journal, and articles are distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as appropriate credit is given and the new creations are licensed under the identical terms.

Article type

Case Report


Article page

45-48


Authors Details

Srushti Shabnam, Sneha Madhusudhanan, Nandana Udumbassery, Anushree Bhagavan


Article History

Received : 10-04-2024

Accepted :


Article Metrics


View Article As

 


Downlaod Files