Stiff Man Syndrome
Stiff Man Syndrome Causes
Since many patients with SPS have circulating antibodies to the enzyme glutamic acid decarboxylase (GAD), an autoimmune cause of the disease has been theorized. A mutation in GLRA1 (glycine receptor) is responsible for a few cases of stiff person syndrome.
Stiff Man Syndrome Definition
Stiff man syndrome (or Stiff Person syndrome) is a rare neurological disorder with features of an autoimmune disease. SPS is marked 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. Abnormal postures, often hunched over and stiffened, are common signs of the disorder.
Stiff Man Syndrome Diagnosis
The symptoms of SPS suggest the diagnosis.
Stiff Man Syndrome Symptoms and Signs
Those affected with the illness experience progressive, fluctuating tonic muscle contractions, particularly the axial musculature. Depression and anxiety are often seen although this may be a result of discomfort due to stiffness, rather than underlying neurochemical abnormalities. MRI detection of GABA in the brain have demonstrated decreased levels in stiff-person syndrome
Stiff Man Syndrome Treatment
Treatment is usually palliative with muscle relaxants which enhance GABA production, such as benzodiazepines. These treatments gradually lose their effectiveness as the illness progresses.