Straight Man Syndrome

Straight Man Syndrome(Stiff-man syndrome)

The stiff-man syndrome is a disorder of the central nervous system that causes muscle stiffness and progressive spasms.

What is Stiff-Person Syndrome?

Stiff-person syndrome (SPS) is a rare neurological problem with the features of an autoimmune issue.

Here are some signs of Stiff person syndrome for identifying the disease:

Stiff-Person syndrome is characterized by fluctuating muscle rigidity in the trunk and limbs and increased sensitivity to stimuli such as noise, touch, and emotional distress, which can trigger muscle spasms. Abnormal postures, often leaning on stiffness, are characteristic of the disease. People with the stiff-person syndrome may be too disabled to walk or move, or they are afraid to leave the house because of street noise, such as the sound of a transport and vehicle.

The stiff person syndrome (formerly called the stiff man syndrome) affects the CNS but includes neuromuscular manifestations.

Most patients with stiff man syndrome have antibodies against glutamic acid decarboxylase (GAD), the enzyme involved in the production of the inhibitory neurotransmitter GABA (gamma-amino butyric acid). However, the stiff man syndrome can be

  • Autoimmune
  • paraneoplastic
  • idiopathic

The autoimmune type often occurs with type 1 diabetes, as well as other autoimmune disorders, including thyroiditis, vitiligo, and pernicious anemia. Auto-Ac directed against several affected proteins in GABA glycinergic synapses are present in the autoimmune type, mainly affecting the inhibitory neurons, which originate in the ventral horn of the spinal cord.

In the paraneoplastic type, anti-amphiphysin antibodies are often present; anti-GAD and anti-Ri antibodies may be present. This type is usually associated with breast cancer, but it can also occur in cases of lung cancer, kidney cancer, thyroid cancer, colon cancer or lymphoma.

The clinical manifestations are similar in all types. Stiffness, stiffness and muscle spasms develop insidiously in the trunk, abdomen and, to a lesser degree, in the legs and arms. The patients are otherwise normal and the examination aims only at hypertrophy and muscular stiffness. Electromyography (EMG) only shows the electrical activity of a normal contraction.

The diagnosis of the stiff-man syndrome is based on the recognition of symptoms and is supported by antibody tests, a response to diazepam and EMG results, which indicate continuous motor unit activity in agonist muscles and antagonists.

Who is concerned with, and what are the causes of Stiff-Person Syndrome?

Stiff-Person syndrome affects women twice as many as men. It is frequently associated with other autoimmune diseases such as diabetes, thyroiditis, vitiligo, and pernicious anaemia. Scientists still do not understand what causes Stiff-Person Syndrome, but research indicates that it is the result of an autoimmune reaction that went wrong in the spinal cord and brain.

How is Stiff-Person syndrome diagnosed?

The disorder is often misdiagnosed as Parkinson’s disease, multiple sclerosis, fibromyalgia, psychosomatic illnesses, or anxiety and phobia. A complete solution can be made with a blood test which is used to measures the level of glutamic acid decarboxylase (GAD) antibodies in the blood. People with the stiff-person syndrome have high levels of GAD, an antibody that acts against an enzyme involved in the synthesis of a major neurotransmitter in the brain.

Is it a treatment for Stiff-Person Syndrome?

People with stiff person syndrome respond to high doses of diazepam and several anticonvulsants, gabapentin, and tiagabine. A recent study funded by the NINDS demonstrated the efficacy of intravenous immunoglobulin (IVIg) therapy by reducing stiffness and lowering sensitivity to noise, touch, and stress in people with the stiff-nosed syndrome.

What is the prognosis for Stiff-Person syndrome?

Treatment with IVIG, anti-anxiety medications, muscle relaxants, anticonvulsants, and pain relievers will improve symptoms of Stiff-Person Syndrome, but do not cure the disease. Most people with Stiff-Person Syndrome have frequent falls and because they do not have the normal defense reflexes; injuries can be serious. With proper treatment, the symptoms are usually well controlled.

Treatment

  • Diazepam or baclofen
  • Possibly IgIV

Only symptomatic treatment is indicated. Diazepam is the only drug of choice; which effectively decreases muscle hypertonia. If diazepam is ineffective, baclofen, poor intrathecally, may be considered.

Corticosteroids are considered effective but have many long-term adverse effects.

Plasma exchange results are inconsistent, but IV immunoglobulins (IVIg) appear to lead to lasting improvement up to one year.

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