Stiff-person syndrome is a CNS disorder that causes progressive muscle stiffness and spasms.
(See also summary of disorders of the peripheral nervous system)
Stiff-person syndrome is a CNS disorder that causes progressive muscle stiffness and spasms. (See also overview of disorders of the peripheral nervous system) (formerly called stiff-man syndrome) The Stiff-person syndrome affects the central nervous system, but has neuromuscular manifestations. Most patients with stiff person syndrome have antibodies to glutamic acid decarboxylase (GAD), the enzyme responsible for the production of the inhibitory neurotransmitter GABA (gamma-aminobutyric acid) is involved. However, the stiff-person syndrome may be autoimmune Paraneoplastic Idiopathic The autoimmune type often occurs with type 1 diabetes and other autoimmune diseases, incl. Thyroiditis, vitiligo and pernicious anemia. When autoimmune type autoantibodies found against various proteins belonging to GABA synapses. These preferably harm inhibitory neurons originating in the ventral horn of the spinal cord. Beimparaneoplastischen type antibodies against Amphiphysin are often present; anti-GAD and anti-Ri antibodies may be present. This type is often associated in the context of breast cancer, but can also occur in patients with lung, kidney, thyroid, or colon cancer or lymphoma. The clinical manifestations are similar for all types. Muscle stiffness, rigidity and spasms proceed slowly in the trunk and abdomen ahead and, to a lesser extent, also in the legs and arms. Patients are otherwise unremarkable, and in the investigation there are only muscle hypertrophy and stiffness. Electromyography (EMG) shows only the electrical activity in normal contraction. The diagnosis of stiff person syndrome is based on recognition of symptoms and is supported by antibody test, reaction to diazepam and the results of EMG studies showing in agonists and antagonists continuous motor unit activity. Therapy diazepam or baclofen may intravenous immunoglobulin (IVIG) There are only symptomatic therapy. Diazepam is the drug of choice; it reduces most resistant muscle stiffness. If diazepam is ineffective, baclofen, oral or intrathecal, can be considered. Corticosteroids are reportedly effective, but have many harmful long-term effects. The results for plasma exchange are inconsistent, but IVIG appears to lead to an improvement with a duration of up to one year.