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Stiff Man syndrome

What are the symptoms?

This condition is characterised by progressive stiffness and painful spasms in the back and lower limbs which are often triggered by touch, noise or anxiety. The unpredictable onset and nature of the symptoms associated with SMS may be experienced as frightening. Where the limbs are predominately involved, the condition is also known as Stiff Limb syndrome.

This is a progressive condition, and stiffness may increase to the extent that the individual becomes a wheelchair user. Both the rigidity and the frequency of spasms may be relieved by sleep, general anaesthesia, nerve block and peripheral nerve block. About forty per cent of individuals also have Type 1 diabetes (see entry, Diabetes Mellitus) which is an autoimmune disease.

Three types of Stiff Man syndrome have been described:

  • Classical Stiff Man syndrome
    Painful spasms and rigidity occur around the back, stomach and sometimes thighs and neck. As the condition progresses, classical curvature of the lower back can occur. Classical Stiff Man syndrome is commonly associated with Type 1 Diabetes.
  • Stiff Limb syndrome
    The legs, including the feet, are affected by painful spasms and occasionally fixed rigidity. More rarely, the hands can be affected
  • Jerking Stiff Person syndrome
    Otherwise known as progressive encephalomyelitis with rigidity, this is the rarest form of SMS. It is a more aggressive form of SMS and can lead to progressive disability over a number of years.

Some individuals with Stiff Man syndrome/Stiff Limb syndrome show an immune response to an enzyme called glutamic acid decarboxylase (GAD). Individuals with classic Type 1 Diabetes show a similar immune response. GAD is an important enzyme in the formation of a chemical messenger in the brain and spinal cord and also in the transmission of insulin. When a patient is developing SMS or Type 1 Diabetes, antibodies to GAD are produced which leads to its destruction, thus interrupting transmission.

View Background Background  |  How is it diagnosed? View How is it diagnosed?

Medical text written July 2004 by Contact a Family. Approved July 2004 by Professor P Brown, Professor of Neurology, Institute of Neurology, London, UK.

 

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