Lower Motor Neuron Disease
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A lower motor neuron lesion is a
lesion A lesion is any damage or abnormal change in the tissue of an organism, usually caused by injury or diseases. The term ''Lesion'' is derived from the Latin meaning "injury". Lesions may occur in both plants and animals. Types There is no de ...
which affects nerve fibers traveling from the
lower motor neuron Lower motor neurons (LMNs) are motor neurons located in either the anterior grey column, anterior nerve roots (spinal lower motor neurons) or the cranial nerve nuclei of the brainstem and cranial nerves with motor function (cranial nerve lower ...
(s) in the anterior horn/
anterior grey column The grey columns are three regions of the somewhat ridge-shaped mass of grey matter in the spinal cord. These regions present as three columns: the anterior grey column, the posterior grey column, and the lateral grey column, all of which are ...
of the
spinal cord The spinal cord is a long, thin, tubular structure made up of nervous tissue that extends from the medulla oblongata in the lower brainstem to the lumbar region of the vertebral column (backbone) of vertebrate animals. The center of the spinal c ...
, or in the
motor nuclei A cranial nerve nucleus is a collection of neuron cell bodies (gray matter) in the brain stem that is associated with one or more of the cranial nerves. Axons carrying information to and from the cranial nerves form a synapse first at these nucl ...
of the
cranial nerves Cranial nerves are the nerves that emerge directly from the brain (including the brainstem), of which there are conventionally considered twelve pairs. Cranial nerves relay information between the brain and parts of the body, primarily to and f ...
, to the relevant
muscle Muscle is a soft tissue, one of the four basic types of animal tissue. There are three types of muscle tissue in vertebrates: skeletal muscle, cardiac muscle, and smooth muscle. Muscle tissue gives skeletal muscles the ability to muscle contra ...
(s). One major characteristic used to identify a lower motor neuron lesion is
flaccid paralysis Flaccid paralysis is a neurological condition characterized by weakness or paralysis and reduced muscle tone without other obvious cause (e.g., trauma). This abnormal condition may be caused by disease or by trauma affecting the nerves associ ...
– paralysis accompanied by loss of muscle tone. This is in contrast to an
upper motor neuron lesion An upper motor neuron lesion (also known as pyramidal insufficiency) Is an injury or abnormality that occurs in the neural pathway above the anterior horn cell of the spinal cord or motor nuclei of the cranial nerves. Conversely, a lower motor ...
, which often presents with
spastic paralysis Spasticity () is a feature of altered skeletal muscle performance with a combination of paralysis, increased tendon reflex activity, and hypertonia. It is also colloquially referred to as an unusual "tightness", stiffness, or "pull" of muscles ...
– paralysis accompanied by severe
hypertonia Hypertonia is a term sometimes used synonymously with ''spasticity'' and ''rigidity'' in the literature surrounding damage to the central nervous system, namely upper motor neuron lesions. Impaired ability of damaged motor neurons to regulate ...
.


Signs and symptoms

* Muscle
paresis In medicine, paresis (), compound word from Greek , (πᾰρᾰ- “beside” + ἵημι “let go, release”), is a condition typified by a weakness of voluntary movement, or by partial loss of voluntary movement or by impaired movement. Whe ...
or
paralysis Paralysis (: paralyses; also known as plegia) is a loss of Motor skill, motor function in one or more Skeletal muscle, muscles. Paralysis can also be accompanied by a loss of feeling (sensory loss) in the affected area if there is sensory d ...
* Fibrillations *
Fasciculations A fasciculation, or muscle twitch, is a spontaneous, involuntary muscle contraction and relaxation, involving fine muscle fibers. They are common, with as many as 70% of people experiencing them. They can be benign, or associated with more seriou ...
– caused by increased receptor concentration on muscles to compensate for lack of innervation. *
Hypotonia Hypotonia is a state of low muscle tone (the amount of tension or resistance to stretch in a muscle), often involving reduced muscle strength. Hypotonia is not a specific medical disorder, but it is a potential manifestation of many different dis ...
or
atonia This glossary of medical terms is a list of definitions about medicine, its sub-disciplines, and related fields. A * Aarskog–Scott syndrome – (AAS) A rare, inherited (X-linked) disease characterized by short stature, facial abnormalities, ...
– Tone is not velocity dependent. *
Hyporeflexia Hyporeflexia is the reduction or absence of normal bodily reflexes. It can be detected through the use of a reflex hammer and is the opposite of hyperreflexia. Hyporeflexia is generally associated with a deficit in the lower motor neurons (at ...
- Along with deep reflexes even cutaneous reflexes are also decreased or absent. *
Strength Strength may refer to: Personal trait *Physical strength, as in people or animals *Character strengths like those listed in the Values in Action Inventory *The exercise of willpower Physics * Mechanical strength, the ability to withstand ...
– weakness is limited to segmental or focal pattern, Root innervated pattern The extensor
plantar reflex The plantar reflex is a reflex elicited when the sole of the foot is stimulated with a blunt instrument. The reflex can take one of two forms. In healthy adults, the plantar reflex causes a downward response of the hallux (flexion). An upward ...
(
Babinski sign The plantar reflex is a reflex elicited when the sole of the foot is stimulated with a blunt instrument. The reflex can take one of two forms. In healthy adults, the plantar reflex causes a downward response of the hallux (flexion). An upward ...
) is usually absent. Muscle paresis/paralysis, hypotonia/atonia, and hyporeflexia/areflexia are usually seen immediately following an insult.
Muscle wasting Muscle atrophy is the loss of skeletal muscle mass. It can be caused by immobility, aging, malnutrition, medications, or a wide range of injuries or diseases that impact the musculoskeletal or nervous system. Muscle atrophy leads to muscle weakne ...
, fasciculations and fibrillations are typically signs of end-stage muscle
denervation Denervation is any loss of nerve supply regardless of the cause. If the nerves lost to denervation are part of neural communication to an organ system or for a specific tissue function, alterations to or compromise of physiological functioning ca ...
and are seen over a longer time period. Another feature is the segmentation of symptoms – only muscles
innervated A nerve is an enclosed, cable-like bundle of nerve fibers (called axons). Nerves have historically been considered the basic units of the peripheral nervous system. A nerve provides a common pathway for the electrochemical nerve impulses called ...
by the damaged nerves will be symptomatic.


Causes

The most common causes of lower motor neuron injuries are trauma to peripheral nerves that serve the axons, and viruses that selectively attack ventral horn cells. Disuse atrophy of the muscle occurs i.e., shrinkage of muscle fibre finally replaced by fibrous tissue (fibrous muscle) Other causes include
Guillain–Barré syndrome Guillain–Barré syndrome (GBS) is a rapid-onset Paralysis, muscle weakness caused by the immune system damaging the peripheral nervous system. Typically, both sides of the body are involved, and the initial symptoms are changes in sensation ...
,
West Nile fever West Nile fever is an infection by the West Nile virus, which is typically spread by mosquitoes. In about 80% of infections people have few or no symptoms. About 20% of people develop a fever, headache, vomiting, or a rash. In less than 1% of ...
, ''C. botulism'',
polio Poliomyelitis ( ), commonly shortened to polio, is an infectious disease caused by the poliovirus. Approximately 75% of cases are asymptomatic; mild symptoms which can occur include sore throat and fever; in a proportion of cases more severe ...
, and cauda equina syndrome; another common cause of lower motor neuron degeneration is
amyotrophic lateral sclerosis Amyotrophic lateral sclerosis (ALS), also known as motor neuron disease (MND) or—in the United States—Lou Gehrig's disease (LGD), is a rare, Terminal illness, terminal neurodegenerative disease, neurodegenerative disorder that results i ...
.


Diagnosis


Differential diagnosis

*
Myasthenia gravis Myasthenia gravis (MG) is a long-term neuromuscular junction disease that leads to varying degrees of skeletal muscle weakness. The most commonly affected muscles are those of the eyes, face, and swallowing. It can result in double vision, ...
– synaptic transmission at motor end-plate is impaired *
Amyotrophic lateral sclerosis Amyotrophic lateral sclerosis (ALS), also known as motor neuron disease (MND) or—in the United States—Lou Gehrig's disease (LGD), is a rare, Terminal illness, terminal neurodegenerative disease, neurodegenerative disorder that results i ...
– causes death of motor neurons, although exact cause is unknown it has been suggested that abnormal build-up of proteins proves toxic for the neurons.


See also

*
Lower motor neuron Lower motor neurons (LMNs) are motor neurons located in either the anterior grey column, anterior nerve roots (spinal lower motor neurons) or the cranial nerve nuclei of the brainstem and cranial nerves with motor function (cranial nerve lower ...
*
Upper motor neuron Upper motor neurons (UMNs) is a term introduced by William Gowers in 1886. They are found in the cerebral cortex and brainstem and carry information down to activate interneurons and lower motor neurons, which in turn directly signal muscles ...
*
Upper motor neuron lesion An upper motor neuron lesion (also known as pyramidal insufficiency) Is an injury or abnormality that occurs in the neural pathway above the anterior horn cell of the spinal cord or motor nuclei of the cranial nerves. Conversely, a lower motor ...


References


External links

* http://library.med.utah.edu/neurologicexam/html/motor_anatomy.html#06 {{Movement and gait symptoms and signs Neurology