ALS and SMA Quiz

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Questions and Answers

What is the incidence rate of Guillain-Barré Syndrome?

1 to 2 per 100,000 people (correct)

1 per 1,000 people

1 per 100 people

1 per 10,000 people

What are the common complications of Guillain-Barré Syndrome?

Skin rash, dizziness, nausea

Respiratory failure, pneumonia, cardiac arrest, autonomic dysfunction (correct)

Vision problems, hearing loss, cognitive impairment

Muscle cramps, joint pain, headache

What is the mortality rate of Guillain-Barré Syndrome during the acute phase?

0.3-1%

3-10% (correct)

50-60%

20-30%

What percentage of patients with Guillain-Barré Syndrome experience near to complete recovery, including walking?

75% Signup and view all the answers

Which condition is characterized by a selective targeting of motor neurons in both the central and peripheral nervous system?

Amyotrophic Lateral Sclerosis (ALS) Signup and view all the answers

Which variant of Guillain-Barré Syndrome primarily affects cranial nerves and brainstem, causing abnormal muscle coordination and paralysis of eye muscles?

Miller Fisher variant Signup and view all the answers

Which factor is responsible for approximately 5-10% of Amyotrophic Lateral Sclerosis (ALS) cases?

Genetic mutation Signup and view all the answers

Which disease is characterized by antibodies that attach to both C-jejuni and peripheral nerves, leading to cross-reactivity and initiation of attacks on nerves?

Guillain-Barré Syndrome (GBS) Signup and view all the answers

What is the etiology of Huntington's disease?

Mutation in a single gene Signup and view all the answers

What is the typical onset age for Huntington's disease?

30-50 years Signup and view all the answers

What is the inheritance pattern of Huntington's disease?

Autosomal dominant Signup and view all the answers

What is the impact of Huntington's disease on the brain?

Decrease in caudate size, cerebellum, and cerebrum Signup and view all the answers

Which disease is characterized by symptoms such as rigidity, bradykinesia, micrographia, masked face, postural abnormalities, and resting tremor?

Parkinson's disease Signup and view all the answers

What is a factor that can increase susceptibility to developing Multiple Sclerosis?

High consumption of dairy products Signup and view all the answers

What is a common neuromuscular condition that can be confused with Multiple Sclerosis?

Stroke Signup and view all the answers

Which type of SMA is associated with the most severe symptoms and early onset?

Acute SMA (Type 1) Signup and view all the answers

Which motor neurons does ALS affect?

Both lower and upper motor neurons Signup and view all the answers

What is the inheritance pattern of SMA?

Autosomal recessive Signup and view all the answers

Study Notes

Amyotrophic Lateral Sclerosis (ALS) and Spinal Muscular Atrophy (SMA) Overview

SOD gene mutations may lead to misfolded proteins, causing poor anti-oxidation and mitochondrial dysfunction
ALS can affect both lower and upper motor neurons, leading to varied impairments such as muscle weakness, hypertonia, and hyporeflexia
Patients with ALS may experience musculoskeletal pain, muscle cramps, and non-motor symptoms like cognitive impairment and emotional lability
ALS can present with bulbar system onset (cranial nerve dysfunction) or limb onset (weakness in the limbs)
Conditions that may be considered for differential diagnosis or share similarities with ALS include frontal temporal dementia, spinal muscular atrophy, and peripheral neuropathies
SMA is a rare hereditary neuromuscular disorder characterized by the loss of motor neurons, leading to muscle wasting and weakness
SMA is the most common genetic cause of death in infants, impacting every race and gender equally
The survival motor neuron 1 gene (SMN1) on chromosome 5q produces the survival motor neuron protein, which maintains the health and normal function of motor neurons
SMA is inherited as an autosomal recessive disease, requiring two mutated genes for manifestation
There are different types of SMA, varying in severity and age of onset, such as acute SMA (Type 1), intermediate SMA (Type 2), mild SMA (Type 3), and later onset SMA (Type 4)
SMA is associated with progressive weakness of voluntary muscles, respiratory impairments, and other associated problems like difficulties swallowing and joint contractures
SMA primarily affects trunk, leg, and arm muscles more than hands and feet, and is characterized by degeneration of anterior horn cells and muscle weakness.

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Description

Test your knowledge of Amyotrophic Lateral Sclerosis (ALS) and Spinal Muscular Atrophy (SMA) with this informative quiz. Explore key concepts such as SOD gene mutations, motor neuron impairments, differential diagnoses, genetic factors, disease types, and associated symptoms. Whether you're a healthcare professional or interested in neurodegenerative disorders, this quiz will enhance your understanding of ALS and SMA.