LOWER MOTOR NEURON SYNDROMES

Conf. dr. Rodica Blaa

 LOWER MOTOR NEURON I
Lower motor neuron (LMN).
LMN is an afferent neuron which has its body located in the
anterior gray column of spinal cord or in motor brain stem nuclei
and its axon passing by peripheral nerves or cranial nerves to the
skeletal muscles (final common pathway).
LMNs are the neurons bringing the nerve impulses from the
upper motor neurons to the muscles.
Glutamate released from upper motor neurons triggers
depolarization in the LMNs which in turn causes an action
potential to propagate the length of the axon to the muscles.
 LMN II
LMNs are classified based on the type of muscle fiber they
innervate:
Alfa motor neurons innervate extrafusal muscle fibers, the
most numerous type of muscle fiber and the one involved in
muscles contraction.
Gamma motor neurons innervate intrafusal muscle fibers,
which together with sensory afferents compose muscle spindles
(part of system serving body position = proprioception).
A motor unit is made of a large anterior horn cell, its motor
axon and the skeletal muscle fibers innervated by that axon (the
number of muscles fibers within each unit can vary within
particular muscle for instance, thigh muscles can have a
thousand fibers in each unit and eye muscles might have ten).
 LOWER MOTOR NEURON
SYNDROMES (LMNS) I
LMNS = result from lesions of LMN somewhere along its
course: a) cell bodies in brain stem or/and anterior horn of
spinal cord; b) axons in brain stem, cranial nerves, spinal
cord roots and peripheral nerves.
Impairment of active mobility = motor deficit.
Paresis/paralysis.
Paralysis include all voluntary and reflex movements
dependent on affected LMN.
Motor deficit of the muscles is proportional to number of
affected motor units.
Motor deficit can be limited (e.g.: mononeuropathy) or
extended (e.g.: polyneuropathy, polyradiculoneuropathy).
 LMNS II
Impairment of passive motility (muscle tonus).
Hypotonia.
Hypotonia is on the same muscle with weakness.
Diminution/disappearance of muscle aspect.
Reduction of muscle tonus.
Exaggeration of passive movements of the affected limbs.
Impairment of automatic mobility.
Decreased or abolished on paretic muscle (e.g.: blinking,
balancing of upper limb during walking).
Impairment of muscular trophicity (muscular atrophy).
Appears after some weeks from LMN lesion.
Appears more rapidly after cutting than compression of LMN.
In progressive lesion, muscular atrophy appear before motor
deficit.
 LMNS III
Impairment of deep tendon reflexes (DTRs).
DTRs are diminished or abolished.
Present only on the territory of affected LMN.
Grade of diminution of DTRs is dependent on number of
affected motor units.
If the LMN lesion progress, the diminished DTRs become
abolished.
Involuntary movements.
Fasciculations (Fs).
Fs = visible twitchings of small groups of muscle fibers in the
early stage of wasting.
Fs appear only after slowly irritative lesions of LMN at the
level of nerve cell body.
Fs arise from spontaneous discharge of motor neurons with
activation of motor units.
 LMNS IV
Fibrillations (Fb).
Fb = minute muscle contractions, detectable only by needle
electromyography (EMG).
Impairment of muscle contraction and structure.
Muscular contraction is in normal limits until muscle atrophy
do not reach an important degree.
Needle EMG and muscular biopsy are typical of LMNS.
Topographic diagnosis.
LMNS at the spinal cord anterior horn level.
Paralyses have a radicular distribution.
Fasciculations are present.
In acquired diseases, the paralyses are asymmetrical.
In hereditary diseases, the paralyses are symmetrical.
Etiology: a) chronic poliomyelitis; b) progressive spinal
muscular atrophy.
 LMNS V
LMNS at the anterior spinal roots level.
Paralyses have a radicular distribution (radiculitis).
Fasciculations are not present.
Frequent associated with signs of posterior spinal roots
lesion.
Etiology: a) Pott disease; b) spinal trauma; c) spinal tumors;
d) spinal disc herniation; e) polyradiculoneuropathy
(e.g.: Guillain-Barr syndrome).
LMNS at the plexuses level.
Paralyses have an intermediate distribution between
radiculitis and neuritis.
Constant associated with sensitive and autonomic signs.
Etiology: a) polyradiculoneuropathy; b) plexopathy.
 LMNS VI
LMNS at peripheral nerves level.
Paralyses have a neuritis distribution (e.g.: polyneuritis;
b) mononeuritis.
Characteristics of polyneuritis: a) symmetrical; b) predo-
minant distal; c) predominant at lower limbs level.
Characteristics of mononeuritis: a) only a peripheral nerve
affected.
Constant associated with sensitive and autonomic signs.
Only motor signs: a) if nerves has only motor fibers (e.g.:
hypoglossal, accesory); B) if the pathogen agents has a moror
nerve tropism (e.g.: lead).
Etiology: a) mononeuritis (e.g.: traumatic); b) mononeuritis
multiplex (e.g.: immunological) ; c) polyneuritis (e.g.: diabetes
mellitus, toxic, immunological, hereditary, vitamin deficiency).