GB and ALS
Guillain-Barré Syndrome
Overview of Guillain-Barré Syndrome
Rare disease affecting the body's immune system.
The immune system attacks the peripheral nervous system (PNS).
This condition can eventually cause paralysis.
Cause of Guillain-Barré Syndrome
The exact cause of Guillain-Barré syndrome is unknown.
Classified as an autoimmune disease.
It is believed to be triggered by an infection.
There is speculation about vaccinations potentially serving as a trigger.
Symptoms and Signs (S/S)
In addition to muscle weakness, patients may experience:
Difficulty with eye muscles and vision.
Difficulty swallowing, speaking, or chewing.
Pricking or pins-and-needles sensations in the hands and feet.
Severe pain, particularly at night.
Coordination problems and unsteadiness.
Abnormal heart rate or blood pressure.
Problems with digestion and/or bladder control.
Pathophysiology
Normal nerve structure contrasted with affected nerve due to Guillain-Barré syndrome:
Exposed fibers and damaged myelin sheath which lead to impaired nerve function.
Prognosis
Recovery outcomes:
Approximately 70% of patients recover fully.
Around 30% experience slow or incomplete recovery.
15% of patients may have long-term weakness, necessitating assistive devices (e.g., wheelchair, walker, ankle support orthoses).
Mortality rate in this syndrome is about 3-5% of cases, often due to complications such as:
Sepsis.
Acute Respiratory Distress Syndrome (ARDS).
Pneumonia.
Pulmonary Embolism (PE).
Treatment Options
Treatment approaches include:
Medications and medical interventions aimed at addressing immediate paralysis.
Physical therapy to aid in recovery and rehabilitation.
Occupational therapy to support the patient’s daily living activities.
Amyotrophic Lateral Sclerosis (ALS)
Overview of ALS
Also known as Lou Gehrig’s Disease.
It is classified as a progressive neurodegenerative disease affecting nerve cells in the brain and spinal cord.
The term "amyotrophic" can be broken down etymologically:
"A" indicates absence.
"Myo" refers to muscle.
"Trophic" implies nourishment.
"Lateral" indicates the specific area of the spinal cord primarily affected.
"Sclerosis" refers to scarring.
Pathophysiology of ALS
ALS leads to degeneration of motor neurons, ultimately resulting in their death.
Motor neurons are crucial as they stimulate muscle activity.
The disease causes progressive muscular atrophy and weakness.
The precise cause of ALS remains unknown.
Definition of ALS
ALS can be defined as: Amyotrophic Lateral Sclerosis | noun:
A progressive neurodegenerative disease that affects nerve cells in the brain and spinal cord.
Healthy motor neurons stimulate muscles to contract, but ALS causes these neurons to die, leading to muscle weakening and eventual paralysis.
Forms of ALS
There are two primary forms of ALS:
Sporadic ALS:
The most common form, accounting for approximately 90% of cases.
Familial ALS:
This form is hereditary, making up about 10% of all cases.
Onset and Diagnosis
The onset of ALS typically occurs between the ages of 40 to 70.
Military veterans, especially those who served in the Gulf War, are at twice the risk of developing ALS.
Incidence is approximately 20% higher in men compared to women.
Prognosis of ALS
Prognostic outcomes include:
Around half of all individuals diagnosed with ALS survive at least three years post-diagnosis.
Approximately 20% of affected individuals live five years or more.
Up to 10% of patients may survive for more than ten years.
Treatment Options for ALS
Available treatment approaches consist of:
Medications to manage symptoms and progression.
Physical therapy to maintain mobility and function.
Occupational therapy to assist with daily living skills.
Respiratory therapy to support breathing function.
Patient Story
Notable case: Hilde Heard, an ALS patient who exemplifies the experiences of those affected by this disease.