Primary Lateral Sclerosis Care at| Arunalaya Healthcare

Best Primary Lateral Sclerosis Treatment in Delhi

What is Primary Lateral Sclerosis ?

Primary Lateral Sclerosis (PLS) is a rare, chronic, and slowly progressive neurodegenerative disease that primarily affects the upper motor neurons (nerve cells in the brain and spinal cord that control voluntary muscle movement). Unlike Amyotrophic Lateral Sclerosis (ALS), PLS typically spares the lower motor neurons, which accounts for its slower progression and generally non-fatal nature.

Causes of Primary Lateral Sclerosis

The exact cause of adult-onset PLS is largely unknown.

However, there is a known genetic cause for juvenile PLS, which is a rare form that begins in childhood. This form is caused by a change in the ALS2 gene, which is responsible for creating a protein called alsin. In juvenile PLS, this protein becomes unstable and does not function properly. Juvenile PLS is inherited in an autosomal recessive pattern, meaning a child must inherit a copy of the changed gene from both parents to develop the condition.

Symptoms of Primary Lateral Sclerosis

PLS symptoms usually develop gradually over years, or even decades, and can vary from person to person. They generally begin in the legs and can progress to the arms, hands, and eventually the bulbar muscles (those controlling speech, swallowing, and chewing).

Common symptoms include:-
- Muscle stiffness (spasticity): This is a hallmark symptom, often starting in the legs and leading to slow, stiff movements.
- Weakness: Progressive weakness in the affected muscles.
- Balance difficulties and clumsiness: Leading to tripping and falls.
- Slowed movement: Due to muscle stiffness and weakness.
Muscle spasms or painful cramps.
- Hand clumsiness.
Bulbar symptoms:
- Dysarthria: Slowed, slurred, or hoarse speech.
- Dysphagia: Difficulty chewing and swallowing.
Drooling.
- Emotional lability (pseudobulbar affect): Frequent, rapid, and intense shifts in emotions, such as sudden and uncontrollable crying or laughing that may not be reflective of actual feelings.
- Urinary urgency or incontinence.
- Fatigue.
- Rarely, breathing problems: May occur in very advanced stages of the disease, but are much less common and severe than in ALS.

Advanced Physiotherapy for Primary Lateral Sclerosis

Assessment
- Thorough evaluation of muscle strength, spasticity, range of motion, balance, gait, posture, and functional abilities (e.g., transfers, activities of daily living).
- Spasticity Management
- Stretching and Range of Motion Exercises: Regular stretching to prevent muscle shortening and contractures, maintaining flexibility.
- Positioning and Splinting: Using proper positioning and custom-made splints or orthoses to reduce spasticity and maintain joint integrity, especially at night.
- Modalities: Techniques like heat, ice, or electrical stimulation may be used as adjuncts to reduce spasticity and pain.
- Functional Electrical Stimulation (FES): May be used to stimulate weak muscles and improve motor control, potentially reducing spasticity.
Strengthening and Endurance Training:
- Strengthening Exercises: Focusing on maintaining and improving strength in unaffected or less affected muscle groups to compensate for weakness.
- Aerobic Training: Low-impact aerobic activities (e.g., stationary cycling, swimming) to maintain cardiovascular fitness and endurance, while being mindful of fatigue.
Balance and Gait Training:
- Balance Exercises: Static and dynamic balance activities, often incorporating eyes open/closed challenges and varied surfaces.
- Gait Re-education: Training to improve walking patterns, reduce tripping hazards, and optimize walking efficiency.
Mobility and Functional Training:-
- Transfer Training: Teaching safe and efficient techniques for transfers (e.g., bed to chair, chair to floor).
- Bed Mobility: Strategies for rolling, sitting up, and repositioning in bed.
- Activities of Daily Living (ADLs) Training: Working on practical skills like dressing, bathing, and eating, often in conjunction with occupational therapy.
- Facial and Oral Motor Exercises: To maintain strength and coordination of muscles involved in speech and swallowing.
- Breathing Exercises: To improve respiratory support, especially if breathing muscles are mildly affected.

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