Lower Motor Neuron Syndrome
A rare genetic motor neuron disease characterized by slowly progressive, predominantly proximal muscular weakness and atrophy At the onset, symptoms include muscle cramps, fasciculations, decreased or absent deep tendon reflexes, hand tremor, and elevated serum creatine kinase, is later associated with reduced walking ability and impaired vibration sensation. Lower motor neuron (LMN) syndromes cause muscle wasting and weakness and can be caused by pathology affecting the distal motor nerve up to the anterior horn cell. Introduction Damage to the cell bodies or peripheral axons of lower motor neurons results in paralysis (loss of movement) or paresis (weakness) of the affected muscles. Aside from paralysis and/or paresis, the lower motor neuron syndrome includes a loss of reflexes (areflexia) caused by an interruption of the efferent (motor) limb of the sensory motor reflex arcs. Muscle tone is lost when lower motor neurons are damaged because