Stiff person syndrome
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Clinic
- SPS also known as stiff-man syndrome (SMS)
- It is a rare neurologic disorder of unclear cause characterized by progressive rigidity and stiffness.
- The stiffness primarily affects the truncal muscles and is superimposed by spasms, resulting in postural deformities.
- Chronic pain, impaired mobility, and lumbar hyperlordosis are common symptoms.
3 clinical classifications
- Classic SPS, associated with other autoimmune conditions and usually GAD-positive
- Partial SPS variants
- Progressive encephalomyelitis with rigidity and myoclonus (PERM)
Classic SPS
- Around 70%
- Intermittent tightness or aching in the muscles of the trunk.
- Muscles Repeatedly / Involuntarily contract, causing them to grow and rigidify.
- Rigidified muscles Reduce the range of motion, Slow their voluntary movements, Abnormal posture, particularly Lumbar hyperlordosis / Shortness of breath
- Imbalance and gait abnormality, causing Awkward 'statue-like' falls
- Bouts of muscle spasms/ Sudden movements or startled.
- Spasms are sometimes accompanied by Elevated blood pressure / Heart rate / Body temperature / Sweating.
- Some experience chronic muscle pain
- Extreme sensitivity to touch and sound
- Unusual eye movements and vertigo occur
- Brisk stretch reflexes and clonus occurs in patients
- Hypnagogic myoclonus
Partial SPS
- A minority of people with SPS experience "partial" SPS
- Also called "stiff limb syndrome"
- This syndrome develops into full SPS about 25% of the time.
- The stiffness begins in one limb and remains most prominent there.
- Sphincter and brainstem issues often occur with stiff-limb syndrome.
PERM
- Encephalomyelitis
- Rigidity
- Myoclonus
- Brainstem issues
- Autonomic disturbances
- Limbic system is affected
- Most patients have upper motor neuron issues
Around 5% of those with SPS are experiencing the symptoms as a paraneoplastic syndrome – a result of a tumor elsewhere in the body releasing bioactive molecules. Paraneoplastic SPS can affect either a single limb, or the trunk and limbs together.
Causes
High glutamic acid decarboxylase (GAD) antibody levels in their blood.(80%)