Alternative names: Moersch-Woltmann Syndrome, Stiff Person Syndrome or SPS.
Stiff Man Syndrome is a neuromuscular condition in which a hyperactive startle reflex results in the contraction of the muscles, thus causing violent spasms. These spasms are capable of slamming the victim into walls and furniture. The disorder's cause is unknown, although evidence is pointing increasingly to the fact that it is an autoimmune disorder.
Stiff person syndrome is a rare progressive neurological disorder. It is rather unique among neurologic diagnoses because of its lack of significant similarity to any other neurologic disease. Although rare, once observed it is quite unforgettable. Possibly the closest related disease is tetanus because both conditions affect peripheral inhibition via central mechanisms and both conditions inhibit central gamma-aminobutyric acid (GABA) systems.
Although they appear completely normal, those afflicted with Stiff Man Syndrome must avoid any situation where they might be exposed to sudden sounds or stimulation. Often, they remain isolated in their home and excluded from normal living – orphaned from society by this rare and ruthless disorder.
One variation of the disease known as stiff limb syndrome is observed more frequently in patients with diabetes mellitus. In this variation, the axial involvement is less marked, and one or (rarely) more extremities are affected.
Researchers theorize that stiff-person syndrome may be an autoimmune disorder. Other autoimmune disorders such as diabetes, pernicious anemia (a chronic, progressive blood disorder), and thyroiditis (inflammation of the thyroid gland) may occur more frequently in patients with stiff-person syndrome.
SPS is characterized by constant painful contractions and spasms of voluntary muscles, particularly the muscles of the back and upper legs.
In general, increased muscle tension, which is more marked proximally than distally, is present. Less frequently, lower extremities are most affected. More rarely, upper and lower extremities are affected. In people with diabetes, one limb may be affected, sparing other muscle groups. In most if not all patients, opposing muscle groups are noted to be tense, and tonic contraction with long relaxation times may be noted following percussion of the muscle. In most patients, the neurologic examination findings are otherwise normal.
Symptoms may occur gradually, spreading from the back and legs to involve the arms and neck. Symptoms may worsen when the affected individual is anxious or exposed to sudden motion or noise. Affected muscles may become twisted and contracted, resulting in bone fractures in the most severe cases.
Individuals with stiff-person syndrome may have difficulty making sudden movements and may have a stiff-legged, unsteady gait. Sleep usually suppresses frequency of contractions. Stiffness may increase and patients may develop a hunched posture (kyphosis) or a swayback (lordosis).
Although there is no cure for stiff-person syndrome, the drug diazepam, which relaxes the muscles, provides improvement in most cases. Baclofen, phenytoin, clonidine, or tizanidine may provide additional benefit. Physical and rehabilitation therapy may also be needed.
The long-term prognosis for individuals with stiff-person syndrome is uncertain. Management of the disorder with drug therapy may provide significant improvements and relief of symptoms.
Musculoskeletal complications are common, particularly in later stages of the disease. Joint deformity, joint dislocation, joint contracture, skeletal fracture, and muscle rupture have been reported.
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