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Rigid spine syndrome, also known as congenital muscular dystrophy with rigidity of the spine (CMARS), is a rare and often debilitating neuromuscular disorder.It is characterized by progressive muscle stiffness and rigidity, particularly in the spine, which can severely limit mobility and impact quality of life.
In many people with SPS, muscle rigidity eventually progresses from the trunk to the limbs — first affecting muscles closest to the trunk, then further. [6] Stiffened limbs can affect a person's balance and gait , causing awkward 'statue-like' falls, where the affected person cannot put out their arms to soften the impact. [ 6 ]
Spastic hypertonia involves uncontrollable muscle spasms, stiffening or straightening out of muscles, shock-like contractions of all or part of a group of muscles, and abnormal muscle tone. It is seen in disorders such as cerebral palsy, stroke, and spinal cord injury. Rigidity is a severe state of hypertonia where muscle resistance occurs ...
Hypokinesia is characterized by a partial or complete loss of muscle movement due to a disruption in the basal ganglia. [citation needed] Hypokinesia is a symptom of Parkinson's disease shown as muscle rigidity and an inability to produce movement. It is also associated with mental health disorders and prolonged inactivity due to illness ...
In those unable to control their secretions, or who have muscle spams of the respiratory muscles, mechanical ventilation may be needed. [7] The best pharmacological treatment is still unclear. Dantrolene has been used when needed to reduce muscle rigidity, and dopamine pathway medications such as bromocriptine have shown benefit. [44]
“SPS is characterized by fluctuating muscle rigidity in the trunk and limbs and a heightened sensitivity to stimuli such as noise, touch, and emotional distress, which can set off muscle spasms ...