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

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

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.

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.

Consent

Written informed consent has been obtained from the patient.