Case Report: Comprehensive management of Bulbar Motor Neuron disease in an elderly male: A Multidisciplinary case report approaching the treatment

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This case report delves into the clinical presentation and management of an 85-year-old male patient with typical symptoms, such as left-sided weakness, dysphagia, and dysarthria, along with a history of aphasia and hypertension, underlining the complexities associated with diagnosing and managing this condition. Methods: The patient underwent a thorough clinical examination, diagnostic investigations, and imaging, leading to a confirmed diagnosis of bulbar MND. Multidisciplinary interventions were employed to addressed the diverse symptoms, including compensation therapy, swallowing function training, physiotherapy, bolus modification, behavioral adjustments, oromotor exercises, postural corrections, and sensory and neurophysiologic stimulation to enhance swallowing safety and comfort. Additionally, interventions for dysarthria and left-sided weakness encompassed speech rate control, strength training, and the use of augmentative communication aids. Results: Over a 30-day follow-up period, the patient showed improvements in dysphagia, dysarthria, and activities of daily living. The treatment strategies effectively addressed the specific challenges associated with bulbar MND, focusing on enhancing the patient’s quality of life and functional abilities. Conclusions: This case report underscores the need for a comprehensive, multidisciplinary approach to manage bulbar MND. It highlights the complexities of diagnosing and addressing the diverse symptoms and difficulties that patients with this condition encounter, emphasizing the importance of a holistic and individualized therapeutic strategy." } { "@context": "http://schema.org", "@type": "BreadcrumbList", "itemListElement": [ { "@type": "ListItem", "position": "1", "item": { "@id": "https://f1000research.com/", "name": "Home" } }, { "@type": "ListItem", "position": "2", "item": { "@id": "https://f1000research.com/browse/articles", "name": "Browse" } }, { "@type": "ListItem", "position": "3", "item": { "@id": "https://f1000research.com/articles/13-117", "name": "Case Report: Comprehensive management of Bulbar Motor Neuron disease..." } } ] } Home Browse Case Report: Comprehensive management of Bulbar Motor Neuron disease... ALL Metrics - Views Downloads Get PDF Get XML Cite How to cite this article Hullumani V S and Mudey R. Case Report: Comprehensive management of Bulbar Motor Neuron disease in an elderly male: A Multidisciplinary case report approaching the treatment [version 1; peer review: awaiting peer review] . F1000Research 2024, 13 :117 ( https://doi.org/10.12688/f1000research.144134.1 ) NOTE: If applicable, it is important to ensure the information in square brackets after the title is included in all citations of this article. Close Copy Citation Details Export Export Citation Sciwheel EndNote Ref. Manager Bibtex ProCite Sente EXPORT Select a format first Track Share ▬ ✚ Case Report Case Report: Comprehensive management of Bulbar Motor Neuron disease in an elderly male: A Multidisciplinary case report approaching the treatment [version 1; peer review: awaiting peer review] Sharath Hullumani V https://orcid.org/0009-0000-2926-5609 1 , Rajas Mudey 1 Sharath Hullumani V https://orcid.org/0009-0000-2926-5609 1 , Rajas Mudey 1 PUBLISHED 19 Feb 2024 Author details Author details 1 Department of Paediatrics, Ravi Nair Physiotherapy, College, Datta Meghe Institute of Higher Education and Research, Wardha, Maharasthra, 442001, India Sharath Hullumani V Roles: Conceptualization, Data Curation, Formal Analysis, Funding Acquisition, Investigation, Methodology, Project Administration, Resources, Software, Supervision, Validation, Visualization, Writing – Original Draft Preparation, Writing – Review & Editing Rajas Mudey Roles: Conceptualization, Data Curation, Formal Analysis, Investigation, Methodology, Project Administration, Resources, Software, Validation, Visualization, Writing – Original Draft Preparation, Writing – Review & Editing OPEN PEER REVIEW REVIEWER STATUS AWAITING PEER REVIEW This article is included in the Datta Meghe Institute of Higher Education and Research collection. Abstract Abstract* Bulbar Motor Neuron Disease (MND), a specific form of amyotrophic lateral sclerosis (ALS) primarily affecting the upper and lower motor neurons in the brainstem’s bulbar region, presents a significant challenge in clinical management. This case report delves into the clinical presentation and management of an 85-year-old male patient with typical symptoms, such as left-sided weakness, dysphagia, and dysarthria, along with a history of aphasia and hypertension, underlining the complexities associated with diagnosing and managing this condition. Methods: The patient underwent a thorough clinical examination, diagnostic investigations, and imaging, leading to a confirmed diagnosis of bulbar MND. Multidisciplinary interventions were employed to addressed the diverse symptoms, including compensation therapy, swallowing function training, physiotherapy, bolus modification, behavioral adjustments, oromotor exercises, postural corrections, and sensory and neurophysiologic stimulation to enhance swallowing safety and comfort. Additionally, interventions for dysarthria and left-sided weakness encompassed speech rate control, strength training, and the use of augmentative communication aids. Results: Over a 30-day follow-up period, the patient showed improvements in dysphagia, dysarthria, and activities of daily living. The treatment strategies effectively addressed the specific challenges associated with bulbar MND, focusing on enhancing the patient’s quality of life and functional abilities. Conclusions: This case report underscores the need for a comprehensive, multidisciplinary approach to manage bulbar MND. It highlights the complexities of diagnosing and addressing the diverse symptoms and difficulties that patients with this condition encounter, emphasizing the importance of a holistic and individualized therapeutic strategy. READ ALL READ LESS Keywords bulbar motor neuron disease; dysphagia; dysarthria, physiotherapy, geriatric Corresponding Author(s) Sharath Hullumani V ( [email protected] ) Close Corresponding author: Sharath Hullumani V Competing interests: No competing interests were disclosed. Grant information: The author(s) declared that no grants were involved in supporting this work. Copyright: © 2024 Hullumani V S and Mudey R. This is an open access article distributed under the terms of the Creative Commons Attribution License , which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. How to cite: Hullumani V S and Mudey R. Case Report: Comprehensive management of Bulbar Motor Neuron disease in an elderly male: A Multidisciplinary case report approaching the treatment [version 1; peer review: awaiting peer review] . F1000Research 2024, 13 :117 ( https://doi.org/10.12688/f1000research.144134.1 ) First published: 19 Feb 2024, 13 :117 ( https://doi.org/10.12688/f1000research.144134.1 ) Latest published: 19 Feb 2024, 13 :117 ( https://doi.org/10.12688/f1000research.144134.1 ) Introduction Bulbar motor neuron disease, sometimes called bulbar onset motor neuron disease or bulbar ALS (amyotrophic lateral sclerosis), is a devastating neurodegenerative disorder primarily targeting the motor neurons within the brainstem’s bulbar region. 1 The motor cortex’s higher groups and the brain stem and spinal cord’s lower groups are the two categories into which motor neurons can be separated. 2 Lower motor neuron loss causes muscle atrophy, cramping, and weakness. Rapid responses and functional limitations result from the loss of higher motor neurons. 3 MND encompass a range of disorders that impact the voluntary motor system, which involves components such as frontal cortex motor neurons, anterior horn cells, cranial motor nerve nuclei, and the corticospinal and corticobulbar tracts. These conditions can have variable effects on this essential neural network, depending on the specific type of MND. MND can either be acquired or inherited. In adults, the most common MND is Amyotrophic Lateral Sclerosis (ALS), which can manifest spontaneously or be inherited and affects both upper motor neurons (UMN) and lower motor neurons (LMN). Meanwhile, in children, the prevalent MND is spinal muscular atrophy (SMA), a condition driven by genetic factors leading to the degeneration of LMNs. 4 Motor neuron diseases like ALS are marked by the gradual deterioration and depletion of motor neurons, the nerve cells that govern voluntary muscle actions. 1 Motor neuron disease often affects middle-aged and elderly people and first manifests in the 6 th and 7 th decades of life. Familial motor neurone disease, which makes up around 5% of cases, is more frequently encountered in younger age groups. 5 The exact cause of this condition remains elusive, but it is believed to arise from a complex interplay of genetic and environmental factors. Numerous genetic mutations, including those in the C9 or f72 gene, SOD1 gene, and other genes, have been identified as contributing to the aetiology of the illness. 6 Slurred speech [dysarthria], trouble swallowing [dysphagia], and weakness or muscular atrophy in the face muscles, tongue, and throat are the characteristic signs of bulbar motor neuron disease. It can eventually result in severe impairment and, in most cases, respiratory failure, the main cause of death in ALS patients, as the illness advances and affects the limbs and other muscle groups. 7 A thorough clinical evaluation, electromyography [EMG], nerve conduction investigations, and frequent genetic testing to find suspected causal mutations are commonly used to diagnose bulbar motor neuron illness. 8 The physiotherapy management of Bulbar Motor Neuron Disease [MND] presents unique challenges due to this neurodegenerative condition’s progressive and debilitating nature. Despite extensive study, how amyotrophic lateral sclerosis is currently treated, from diagnosis to prognosis, is still not optimal. 9 Motor neuron diseases (MND), including the bulbar variant, encompass the degeneration of both upper and lower motor neurons, leading to muscle weakness, spasticity, and eventual paralysis. In the case of bulbar MND, these manifestations primarily impact the muscles responsible for speech, swallowing, and facial mobility. 9 Case presentation An 85-year-old male came to neurology out patient department, complaining of left-side weakness and reduced strength associated with difficulty swallowing solid food and mild discomfort with liquid food with difficulty in speech articulation. He has been aphasic for 1.5 years and has been a known hypertensive case for 15 years. Also, the patient showed a history of discontinuing the antihypertensive medications for the last 2 months. The patient’s history indicated a gradual progression of these symptoms over several months, accompanied by slurred speech, recurrent choking during meals, and weakness in facial muscles. Various diagnostic investigations were conducted, including CT scan [Computed Tomography] of the brain and blood tests. These investigations led to the diagnosis of bulbar motor neuron disease. Clinical findings The first examination was done following the patient’s and family’s consent. The patient was conscious, cooperative and well-oriented to time, place and person. The patient was examined in a supine lying position with the head end elevated to 30° and shoulder slightly abducted, elbow flexed, wrist in the neutral position and the hip externally rotated, knee extended, and ankle in plantarflexion. On Higher mental function evaluation, the GCS Score was E4V1M6. Ryles tube was attached to the patient. Notably, loss of reflexes such as the gag reflex and jaw jerk reflex were observed during examination. On sensory examination, sensations were intact, and on motor examination, there was reduced strength on the left upper and lower limbs mentioned in Table 1 . Table 1. MMT of joints. Joint Left Right Temporomandibular 2/5 2/5 Cervical 3/5 4/5 Shoulder 3/5 4/5 Elbow 3/5 4/5 Wrist 3/5 4/5 Hip 3/5 4/5 Knee 3/5 4/5 Ankle 3/5 4/5 Intervention Dysphagia ( Table 2 ) Table 2. Treatment for dysphagia is mentioned. Intervention Specification Compensation Therapy This involves adjusting one's eating posture, modifying food characteristics, and using specialized utensils to enhance swallowing comfort and safety. Swallowing Function Training Swallowing function training encompasses exercises that aim to strengthen and improve the coordination of the muscles involved in swallowing, typically under the guidance of speech therapists. Bolus Modification Adjustments to the consistency of food and liquids are sometimes necessary, such as thickening liquids to make them safer to swallow. Behavioural Adjustments The patient learned various techniques and behaviour, including taking smaller bites and thoroughly chewing their food, to improve their ability to swallow safely. Oromotor Exercises Special exercises are performed to strengthen or enhance the coordination of the oromotor muscles involved in swallowing. Postural Adjustments Changing the positioning of the head and body during meals helps prevent aspiration, which occurs when food or liquids enter the airway. Swallowing Manoeuvre Patient has been taught specific manoeuvres, like the Mendelsohn manoeuvre, to aid in more effective and safe swallowing. Sensory and Neurophysiologic Stimulation Techniques that stimulate the sensory and neurophysiologic aspects of swallowing, such as sensory input or biofeedback, incorporated into therapy to improve swallowing function. Dysarthria ( Table 3 ) Table 3. Treatment for dysarthria is mentioned. Intervention Specification Activity Level To address difficulties with speech, interventions have been focused on controlling speech rate and utilizing augmentative or alternative communication aids. This involves the use of tools such as alphabet charts to facilitate communication. Participation Level At the participation level, interventions aim to support social interactions and overall communicative participation, helping individuals with dysarthria engage more effectively in social communication. Left-sided weakness ( Table 4 ) Table 4. Treatment for left-sided weakness is mentioned. Intervention Specification Strength training To address left-side weakness, strength training is essential. It involves resistance exercises targeting various muscle groups on the left side, including the arm, leg, and core. Manual resistance and resistance bands are used to progressively increase resistance levels. Emotional incontinence ( Table 5 ) Table 5. Treatment for emotional incontinence is mentioned. Intervention Specification Medication The FDA has approved a fixed-dose combination of dextromethorphan and quinidine medication. This medication reduces the frequency and severity of involuntary laughing and crying episodes associated with pseudobulbar affect. Informed patient consent was taken. I hereby give my consent for images or other clinical information concerning my case to be reported in publication ( Figure 1 ). Figure 1. Shows treatment of the patient. A – Facial exercise [raising eyebrows], B – Facial exercise [blowing cheeks], C – Positioning for dysphagia, D – Active assisted Range of Motion exercises of upper limb, E – Active assisted Range of Motion exercises of lower limb, F – Mendelsohn manoeuvre, G – strengthening exercises of the upper limb with manual resistance, H – strengthening exercises of the lower limb with manual resistance, I – use of tools to facilitate communication. Outcome measures Dysphagia [dysphagia outcome severity scale] 10 Follow up 1 st – 10 th day 11 th – 20 th day 21 st – 30 th day levels Level 2/7 Level 4/7 Level 5/7 Dysarthria [Dysarthria rating of severity] 11 Follow up 1 st – 10 th day 11 th – 20 th day 21 st – 30 th day levels Level 5/5 Level 4/5 Level 3/5 Activities of daily living [Barthel index] 12 Follow up 1 st – 10 th day 11 th – 20 th day 21 st – 30 th day Score 25/100 45/100 60/100 Discussion The presented case report focuses on the clinical presentation and management of an 85-year-old male with symptoms indicative of bulbar motor neuron disease [MND], a specific form of amyotrophic lateral sclerosis [ALS]. This case is of particular significance as it underscores the challenges and complexities associated with the diagnosis and management of bulbar MND, which primarily affects the upper and lower motor neurons controlling muscles in the brainstem’s bulbar region, resulting in characteristic symptoms such as dysarthria, dysphagia, and muscle weakness. 1 This patient’s clinical presentation included left-sided weakness, difficulty in swallowing, and impaired speech articulation, all of which align with the typical symptoms of bulbar MND. These symptoms and the patient’s history of aphasia and known hypertension raised strong suspicions of this neurodegenerative condition ( Figure 2 ). 7 Figure 2. CT brain. Impression CT brain reveals: Subdural haemorrhage with mass effect. Acute infarct in right corona radiata. Age-related cerebral atrophic changes. The management of bulbar MND is multi-faceted and necessitates a comprehensive approach to address the myriad symptoms and difficulties patients encounter. In the case of dysphagia, various interventions were employed, including compensation therapy, swallowing function training, physiotherapy, alternative therapies, bolus modification, behavioral adjustments, oromotor exercises, postural adjustments, swallowing manoeuvres, and sensory and neurophysiologic stimulation. These interventions aim to enhance the safety and comfort of swallowing, thereby preventing aspiration and improving overall quality of life. 13 Bulbar MND leads to the progressive loss of upper and lower motor neurons, resulting in muscle weakness and atrophy. Physiotherapy interventions, such as targeted exercises and strength training, are crucial in preserving muscle function and delaying the onset of muscle wasting. These exercises help stimulate the remaining motor neurons and maintain muscle strength, essential for mobility and daily activities. 14 Muscle weakness and immobility can lead to joint contractures and pain. Physiotherapists help prevent contractures by employing stretching exercises and ensuring proper positioning to maintain joint mobility. 15 Staying active and engaging in physiotherapy can boost mood, combat depression and anxiety, and enhance social interaction, which are crucial aspects of living well with MND. 16 As muscle weakness advances in MND, individuals may face mobility and daily activities challenges. Physiotherapists work on improving mobility through gait training, balance exercises, and mobility aids to enhance functional independence. Additionally, they can offer guidance on adaptive equipment, such as wheelchairs or walkers, to facilitate safe and efficient movement. 8 In summary, this case report provides a comprehensive overview of the challenges presented by Bulbar MND, emphasizing the need for a multidisciplinary approach to address the wide array of symptoms and difficulties patients encounter. Patient perspective The patient, in their own perspective, experienced a significant improvement in their quality of life and functional abilities during the 30-day follow-up period. He stated that the strategies and treatments given for left-sided weakness, dysarthria, and dysphagia were effective in addressing the specific difficulties presented due to bulbar MND. The patient reported improvements in their ability to speak more clearly, swallow safely, and regain strength on their left side of the body. These changes had a good effect on their general mental condition in addition to improving their physical health. Consent Written informed consent has been obtained from the patient. Data availability All data underlying the results are available as part of the article and no additional source data are required. Figshare: CARE Checklist for ‘Comprehensive management of Bulbar Motor Neuron disease in an elderly male: A Multidisciplinary case report approaching the treatment’ https://doi.org/10.6084/m9.figshare.25040702.v1 References 1. Rowland LP, Shneider NA: Amyotrophic lateral sclerosis. N. Engl. J. Med. May 2001; 344 (22): 1688–1700. Publisher Full Text 2. Arora RD, Khan YS: Motor Neuron Disease. StatPearls. Treasure Island (FL): StatPearls Publishing; 2023. Accessed: Oct. 16, 2023. 3. Zhang X, Yang K, Le W: Autophagy and Motor Neuron Diseases. Adv. Exp. Med. Biol. 2020; 1207 : 53–74. Publisher Full Text 4. Kang X, Quan D: Electrodiagnostic Assessment of Motor Neuron Disease. Neurol. Clin. Nov. 2021; 39 (4): 1071–1081. PubMed Abstract | Publisher Full Text 5. McDermott CJ, Shaw PJ: Diagnosis and management of motor neurone disease. BMJ. Mar. 2008; 336 (7645): 658–662. PubMed Abstract | Publisher Full Text | Free Full Text 6. Al-Chalabi A, Hardiman O: The epidemiology of ALS: a conspiracy of genes, environment and time. Nat. Rev. Neurol. Nov. 2013; 9 (11): 617–628. PubMed Abstract | Publisher Full Text 7. Hardiman O, van den Berg LH , Kiernan MC: Clinical diagnosis and management of amyotrophic lateral sclerosis. Nat. Rev. Neurol. Oct. 2011; 7 (11): 639–649. Publisher Full Text 8. Brown RH, Al-Chalabi A: Amyotrophic Lateral Sclerosis. N. Engl. J. Med. Jul. 2017; 377 (2): 162–172. Publisher Full Text 9. Feldman EL, et al. : Amyotrophic lateral sclerosis. Lancet. Oct. 2022; 400 (10360): 1363–1380. PubMed Abstract | Publisher Full Text | Free Full Text 10. O’Neil KH, Purdy M, Falk J, et al. : The Dysphagia Outcome and Severity Scale. Dysphagia. 1999; 14 (3): 139–145. Publisher Full Text 11. Gupta S, Yadav R, Malhotra AK: Assessment of physical disability using Barthel index among elderly of rural areas of district Jhansi (U.P), India. J. Family Med. Prim. Care. 2016; 5 (4): 853–857. PubMed Abstract | Publisher Full Text | Free Full Text 12. Saleem F, Munakomi S: Pseudobulbar Palsy. StatPearls. Treasure Island (FL): StatPearls Publishing; 2023. Accessed: Oct. 12, 2023. 13. Silva ST, et al. : Physical therapy for the management of motor symptoms in amyotrophic lateral sclerosis: protocol for a systematic review. BMJ Open. Nov. 2022; 12 (11): e063689. PubMed Abstract | Publisher Full Text | Free Full Text 14. Ng L, Khan F, Young CA, et al. : Symptomatic treatments for amyotrophic lateral sclerosis/motor neuron disease. Cochrane Database Syst. Rev. Jan. 2017; 2017 (1): CD011776. PubMed Abstract | Publisher Full Text | Free Full Text 15. Feinstein A, Freeman J, Lo AC: Treatment of progressive multiple sclerosis: what works, what does not, and what is needed. Lancet Neurol. Feb. 2015; 14 (2): 194–207. Publisher Full Text 16. Francis K, Bach JR, DeLisa JA: Evaluation and rehabilitation of patients with adult motor neuron disease. Arch. Phys. Med. Rehabil. Aug. 1999; 80 (8): 951–963. PubMed Abstract | Publisher Full Text Comments on this article Comments (0) Version 1 VERSION 1 PUBLISHED 19 Feb 2024 ADD YOUR COMMENT Comment Author details Author details 1 Department of Paediatrics, Ravi Nair Physiotherapy, College, Datta Meghe Institute of Higher Education and Research, Wardha, Maharasthra, 442001, India Sharath Hullumani V Roles: Conceptualization, Data Curation, Formal Analysis, Funding Acquisition, Investigation, Methodology, Project Administration, Resources, Software, Supervision, Validation, Visualization, Writing – Original Draft Preparation, Writing – Review & Editing Rajas Mudey Roles: Conceptualization, Data Curation, Formal Analysis, Investigation, Methodology, Project Administration, Resources, Software, Validation, Visualization, Writing – Original Draft Preparation, Writing – Review & Editing Competing interests No competing interests were disclosed. Grant information The author(s) declared that no grants were involved in supporting this work. 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