Amyotrophic Lateral Sclerosis (ALS): A case report

Anuradha. S

Clinical Pharmacist, Kauvery Hospital, Hosur, Tamil Nadu

Introduction

Amyotrophic lateral sclerosis (ALS), is a nervous system disease that affects nerve cells in the brain and spinal cord. ALS causes loss of muscle control. ALS is often called lou gehrig’s disease. It is characterised by rapidly worsening muscular paralysis brought by motor neurone degeneration, which results in muscle atrophy, spasticity, and vulnerability. The exact cause of the disease is still not known. A small number of cases are inherited. ALS often begins with muscle twitching and weakness in an arm or leg, trouble swallowing or slurred speech. Eventually ALS affects control of the muscles needed to move, speak, eat and breathe. There is no cure for this fatal disease. Amyotrophic lateral sclerosis can manifest clinically in a variety of ways. The diagnosis is crucial for both the prognosis and the course of treatment. ALS is typically indicated by both upper motor neurone and lower motor neurone sings as well as electrodiagnostic tests.

Clinical manifestations on ALS

Symptoms

ALS vary from person to person. Symptoms depend on which nerve cells are affected. ALS generally begins with muscle weakness that spreads and gets worse over time. Symptoms might include: trouble walking or doing usual daily activities, muscle twitching, tripping and falling, weakness in the legs, feet or ankles, hand weakness or clumsiness, slurred speech or trouble swallowing, weakness associated with muscle cramps and twitching in the arms, shoulders and tongue, untimely crying, laughing or yawning, thinking or behavioral changes.

Cause

It also usually doesn’t affect the senses, including the ability to taste, smell, touch and hear.ALS affects the nerve cells that control voluntary muscle movements. These nerve cells are called motor neurons.

There are two groups of motor neurons:

The first group extends from the brain to the brain stem and spinal cord. They’re referred to as upper motor neurons.
The second group extends from the brain stem and spinal cord to muscles throughout the body. They’re referred to as lower motor neurons. ALS causes both groups of motor neurons to gradually deteriorate and then die.

Complications on ALS

As the disease progresses, ALS causes complications, such as:

Breathing problems, speaking problems, eating problems, dementia
The most common cause of death for people with ALS is breathing failure. Half of people with ALS die within 14 to 18 months of diagnosis. However, some people with ALS live 10 years or longer.
Some people with ALS have problems with language and decision-making.
Some are eventually diagnosed with a form of dementia called frontotemporal dementia.

Case Presentation

Patient Mr. 44y/male, came to opd with above mentioned complaint and shifted to ward for further management. he was evaluated and found to have Amyotrophic lateral sclerosis. strict vitALS & i/o chart were monitored. patient was managed with iv Inj.edaravone, Inj.methylprednisolone and other supportive measures. patient symptomatically better and hence discharged with following advice.

Subjective

Current Complaints (Cc)

Patient came to opd with c/o difficulty using both upper limbs: 1 years
C/o walking difficulty: 2-3 months’ progressive
Started as difficulty holding object with left hand which progressed to difficulty raising left arm followed by right upper limb. Over past 6 months, difficulty in buttoning. Over 3 months, couldn’t mix and eat food with right hand.
H/o twitching of left arm muscles
H/o / difficulty raising left leg especially on getting up from sitting. Needs to hold railings to climb stairs
Feels left leg stiff
Past Medical History: No Comorbidities
Past Medication History: Nil

On Examination

Conscious, Oriented
Cranial Nerves – Normal Except Tongue Fibrillations
Muscle Fasciculations Present
All Dtrs Brisk/Exaggeratted Polyminimyoclonus Was Present
Touch, Pain, Vibration – Normal
Power – Small Muscles of Hand Weak
Elbow Grade 4
Shoulders Grade 3 To 4
Proximal Lower Limbs Grade 4 – On Left
Grade 4 On Right
Ankle Clonus (+) Right
Plantar – Extensor On Right

Objective

Physical Examination
BP	130/80 mmhg
Pulse rate	98/Min
Respiratory rate	22/Min
Temperature	97.20f
Spo2	97% On Room Air
Other Systemic Examination	Normal

Vital Signs

Vital/Date	Day 1	Day 2
Temp (F)	97.20f	97.20f
Pulse /Min	98/Min	98/Min
Bp (Mm/Hg)	130/80 Mmhg	130/80 Mmhg

Laboratory Investigation

Department of Hematology

Constituents	Status	Detected Values	Normal Values	Unit
WBC Count	Normal	7710	4000 - 10000	Cells/Cu Mm
Monocytes	High	9.7	1-8	%

Department of Biochemistry

Constituents	Status	Detected Values	Normal Values	Unit
Potassium	Normal	3.6	3.5-5	Mmol/L
Urea Serum	Normal	12.8	10 -50	Mg/Dl
Sodium	Normal	139	136-145	Mmol/L
Creatinine	Normal	0.6	0.5-1.4	Mg/Dl

Drug-Drug Intractions: Nil

Advice on discharge

Drug	Dose	Frequency	Duration	Af/Bf
Cap.Riluzole	50 Mg	BD	1 Month	After Food
Injection. Edaravone 60mg As Infusion Over 1 Hour Od X 12 Days

Assessment of therapeutic response of Edaravone and Riluzole combination therapy in Amyotrophic Lateral Sclerosis patients.

Discussion

Edaravone and Riluzole are both FDA-approved ALS drugs, but they work differently: Riluzole extends survival slightly by reducing glutamate, while Edaravone slows functional decline by fighting oxidative stress, particularly effective early on. Combination therapy, often using both, shows promise, with Edaravone potentially offering better functional maintenance using both drugs targets different pathways (glutamate & oxidative stress) for potentially greater benefit gives synergistic effect. Efficacy of Edaravone provides better functional preservation (ALSfrs-r) when used with riluzole compared to riluzole alone. The association between riluzole and edaravone with liver damage and gastrointestinal discomfort underscores the need for enhanced safety monitoring in patients with hepatic dysfunction. Despite these side effects, riluzole, edaravone, remain essential in ALS management. These medications can slow disease progression and extend survival, making them invaluable in the absence of a cure. However, clinical practice must prioritize personalized treatment approaches that balance therapeutic efficacy with potential risks to ensure patient safety and quality of life. Future research should focus on the long-term impact of these side effects and strengthen drug safety monitoring to optimize treatment strategies and advance personalized ALS therapies.

Conclusion

Combined with riluzole, edaravone slows disease progression and is safe, but the effect is short-term. Bulbar symptoms respond better to combination therapy. The serum creatinine is helpful in monitoring disease progression. Survival rate of real-world studies show lower mortality risk with edaravone (often combined with riluzole). Offers broader protection, with edaravone potentially enhancing riluzole’s effects, though safety monitoring remains important.

Amyotrophic Lateral Sclerosis (ALS): A case report

Amyotrophic Lateral Sclerosis (ALS): A case report

Anuradha. S

Nightingale Journals