PLS Primary Lateral Sclerosis; progressive motor neuron disease, affects only upper motor neurons

Описание: Primary Lateral Sclerosis (PLS) is a rare motor neuron disease that affects only the upper motor neurons in the brain, leading to progressive muscle stiffness, weakness, and spasticity. Unlike ALS, PLS progresses more slowly, is not usually fatal, and does not shorten life expectancy, though it can significantly impact mobility and quality of life.

🧠 What is PLS?
Definition: A progressive neuromuscular disorder caused by degeneration of upper motor neurons (UMN) in the brain.

Difference from ALS:

PLS → affects only UMN → stiffness, spasticity, slow progression.

ALS → affects both UMN and lower motor neurons (LMN) → muscle wasting, faster progression, shortened lifespan.

Rarity: Much less common than ALS.

⚠️ Symptoms
Symptoms develop slowly, often over years:

Early signs:

Muscle stiffness and spasms (spasticity), usually starting in the legs

Weakness in legs → difficulty walking, balance problems, frequent falls

Clumsiness, dropping objects

Progressive signs:

Weakness and stiffness spreading to arms, hands, tongue, and jaw

Slurred speech (dysarthria), hoarse voice, drooling

Difficulty chewing and swallowing (dysphagia)

Emotional changes (rapid mood shifts, pseudobulbar affect)

Rarely, bladder issues or breathing difficulties late in disease

🧬 Causes
Adult-onset PLS: Cause unknown; not usually inherited.

Juvenile PLS: Caused by mutations in the ALS2 gene (autosomal recessive). Both parents must carry the gene for a child to develop juvenile PLS.

🧪 Diagnosis
Clinical diagnosis after ruling out ALS and other conditions.

Tests may include:

Electromyography (EMG) → checks lower motor neuron involvement (normal in PLS)

MRI scans → rule out multiple sclerosis or spinal cord disease

Blood tests & CSF analysis → exclude mimicking conditions

💊 Treatment & Management
There is no cure, but treatment focuses on symptom management:

Medications:

Baclofen, tizanidine, diazepam → reduce spasticity

Quinine → relieve muscle cramps

Therapies:

Physical therapy → maintain flexibility, reduce stiffness

Speech therapy → improve communication

Nutritional support → manage swallowing difficulties



#Sclerosis