The rare neurological disease causes muscle stiffness and sometimes very painful spasms. It mainly affects women aged 45 and over. There is no definitive cure, but some medications relieve symptoms
The stiff man syndrome (in English stiff-person syndrome) from which Celine Dion suffers one rare and disabling neurological pathology which originates in central nervous system. The disease characterized by muscle stiffnessfocused on the abdomen and from muscle spasmsoften painfulwith a tendency to jump abnormally and with the presence of ankylosing deformity, such as fixed lumbar posture in hyperlordosis. The disease typically progresses slowly over time causing stiffness throughout the body.
Progressive muscle stiffness immobilizes the Chest not alsol’gait becomes stiff and awkward. Concomitant spontaneous or reflex-induced painful muscle spasms can cause disastrous falls. The continuous contraction of agonist and antagonist muscles is caused by the involuntary activity of motor neurons at rest. The pathology is often secondary and connected to other pathologies already present.
The prevalence estimated at 1/1.000.000. About the 2/3 of the patients are women. The onset occurs around 45 years and symptoms develop over months to years.
The cause of stiff person syndrome unknown but often related to other pathologies. Stiff person syndrome autoimmune type more frequently present in sufferers of type 1 diabetes oh you pernicious anemia with antibodies to specific proteins. Stiff person syndrome is more rare paraneoplastic type (only 2% of those suffering from this pathology) and related to pathologies such as Hodgkin’s lymphoma, breast, colo, thyroid tumors.
The diagnosis is not immediate. In fact, the neurological objective examination is generally normal except for one overall muscle stiffness. Computed tomography and nuclear magnetic resonance imaging of the brain are normal. only theelectromyography reveals the activation of continuous potentials in the motor units of the muscles involved.
Stiff-person syndrome is clinically elusive but potentially treatable and should be suspected in patients with unexplained muscle stiffness and spasms. There is still no definitive cureHowever, sedative medications are often prescribed which have a soothing and relaxing effect to relieve muscle stiffness.
December 8, 2022 (change December 8, 2022 | 18:33)
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