Motor Neuron Disease Overview

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Questions and Answers

Which type of motor neuron disease primarily affects the lower motor neurons?

Amyotrophic lateral sclerosis

Progressive muscular atrophy (correct)

Spastic quadriplegia

Spastic paraparesis

What is a common characteristic of the clinical picture in motor neuron disease?

Acute onset of symptoms

Unilateral space-occupying lesions

Bilateral and symmetrical muscle affection (correct)

Cardiovascular symptoms

Which investigative method is typically used to assess motor neuron disease?

Electromyography (EMG) (correct)

Magnetic resonance imaging (MRI)

CT scan of the brain

Serum biochemical tests

Which of the following is NOT included in the treatment for motor neuron disease?

Chemotherapy Signup and view all the answers

What is the age of onset commonly associated with motor neuron disease?

Middle or old age Signup and view all the answers

Which condition presents with symptoms of spastic paraparesis or quadriplegia related to motor neuron disease?

Combined UMN and LMN disorder Signup and view all the answers

Which of the following reflects a sign of upper motor neuron involvement?

Hypereflexia Signup and view all the answers

Which symptom is associated with pure lower motor neuron affection?

Fasciculations of the tongue Signup and view all the answers

Motor neuron disease primarily affects the sensory nervous system.

False Signup and view all the answers

The age of onset for motor neuron disease typically occurs in young adults.

False Signup and view all the answers

Spinal muscle atrophy is an example of lower motor neuron affection.

True Signup and view all the answers

Riluzole is a treatment used for motor neuron disease.

True Signup and view all the answers

Pseudo bulbar palsy is commonly associated with multiple sclerosis.

False Signup and view all the answers

Upper motor neuron lesions can cause hypertonia and hypereflexia.

True Signup and view all the answers

The clinical picture of motor neuron disease can include bilateral and asymmetrical motor affection.

False Signup and view all the answers

Motor neuron disease can have a purely upper motor neuron affection presentation.

True Signup and view all the answers

Study Notes

Motor Neuron Disease (MND)

MND is a degenerative disease affecting only the motor system, including upper or lower motor neurons, or both.
The disease onset typically occurs in middle or old age.
In terms of sex, males are more commonly affected compared to females.
The course is progressive, with a gradual onset.
Etiologies include idiopathic, degenerative disease, para-neoplastic, and genetic factors.
Clinical presentation often involves bilateral and symmetrical affection of upper motor neurons (UMN), lower motor neurons (LMN), or both.
UMN affection alone can present as spastic paraparesis.
LMN affection alone may present as spinal muscle atrophy.
Combined UMN and LMN affection is characteristic of amyotrophic lateral sclerosis (ALS).

Clinical Picture (con't)

Pure UMN affection: can affect the spinal cord (spastic paraplegia, quadriplegia) or cerebral/brain stem (pseudobulbar palsy, exaggerated reflexes, emotional liability).
Pure LMN affection: typically presents as progressive muscular atrophy, affecting lower cervical regions, with weakness in upper limbs and lower motor neuron signs. This can involve cranial nerve affection (true bulbar palsy), presenting as nasal tonation, nasal regurgitation, loss of palatal and pharyngeal reflexes, and absent emotional liability; tongue wasting and fasciculations may be present.
Combined UMN and LMN affection: (ALS) characterized by wasting, fasciculation, weakness (LMN signs), hypertonia, exaggerated reflexes (UMN signs). It often includes a tonic atrophy component and the signs in the lower limbs are mainly those of UMN pathology with only minimal fasciculation.

Investigation

Electromyography (EMG) and nerve conduction studies (NCS) on upper and lower limbs.
Workup for para-neoplastic causes is necessary.

Treatment

Physiotherapy is frequently involved.
Care of bedridden patients and respiratory care are vital aspects.
Multivitamins and Riluzole (a medication) are potential treatment options.

Additional Clinical Presentations

Pseudobulbar palsy: Possible causes include vascular events (bilateral stroke), infections (encephalitis), neoplasms (midline brain stem tumors), demyelinating diseases (MS), and degenerative diseases (MND).
True bulbar palsy: Potential origins include vascular disease (vertebrobasilar insufficiency), infection (diphtheria), neoplasms (brain stem tumors), and degenerative conditions (MND).
Wasting of small hand muscles (differential diagnosis): possible causes include spinal cord segment issues (like poliomyelitis, transverse myelitis), root issues (like spinal spondylosis, tumors, fractures, Pott's disease), brachial plexus problems (Klumpke's paralysis, thoracic outlet syndrome), peripheral nerve problems (carpal tunnel syndrome, peripheral neuropathy), muscle issues (distal myopathy of Gower), or other (rheumatoid arthritis).

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Description

This quiz explores the complexities of Motor Neuron Disease (MND), including its types, symptoms, and progression. It highlights the differences between upper motor neuron and lower motor neuron affections, as well as the clinical presentation of conditions like amyotrophic lateral sclerosis (ALS). Test your understanding of this degenerative disease affecting the motor system.