STIFF-PERSON SYNDROME

Stiff-person syndrome (SPS), also known as stiff-man syndrome (SMS), is a rare neurologic disorder of unclear cause characterized by progressive rigidity and stiffness. The stiffness primarily affects the truncal muscles and is superimposed by spasms, resulting in postural deformities. Chronic pain, impaired mobility, and lumbar hyperlordosis are common symptoms.

SPS occurs in about one in a million people and is most commonly found in middle-aged people. A small minority of patients have the paraneoplastic variety of the condition. Variants of the condition, such as stiff-limb syndrome which primarily affects a specific limb, are often seen.

SPS was first described in 1956. Diagnostic criteria were proposed in the 1960s and refined two decades later. In the 1990s and 2000s the roles of antibodies in the condition became clearer. SPS patients generally have glutamic acid decarboxylase (GAD) antibodies, which seldom occur in the general population. In addition to blood tests for GAD, electromyography tests can help confirm the condition's presence.

Benzodiazepine-class drugs are the most common treatment; they are used for symptom relief from stiffness. Other common treatments include baclofen, intravenous immunoglobin and rituximab. There is a limited but encouraging therapeutic experience of haematopoietic stem cell transplantation for SPS.

SIGNS AND SYMPTOMS

Stiff-person syndrome (SPS) is often separated into several subtypes, based on the cause and progression of the disease.

There are three clinical classifications of SPS

Classic SPS, associated with other autoimmune conditions and usually GAD-positive;

Partial SPS variants

Progressive encephalomyelitis with rigidity and myoclonus (PERM).[4]

Around 70% of those with SPS have the "classic" form of the disease. People with classic SPS typically first experience intermittent tightness or aching in the muscles of the trunk. These muscles repeatedly and involuntarily contract, causing them to grow and rigidify. Eventually, rigidified muscles reduce the affected person's range of motion, slow their voluntary movements, and may cause them to have abnormal posture, particularly lumbar hyperlordosis (a distinctive curve in the lower back). Rigid trunk muscles can also prevent the chest and abdomen from expanding, causing shortness of breath and early satiety. In many people with SPS, muscle rigidity eventually progresses from the trunk to the limbs – first affecting muscles closest to the trunk, then further. Stiffened limbs can affect a person's balance and gait, causing awkward 'statue-like' falls where the affected person cannot put out their arms to soften the impact. Alongside growing stiffness, many with SPS develop bouts of muscle spasms that are triggered by sudden movements as well as feeling upset or startled. Spasms are sometimes accompanied by elevated blood pressure, heart rate, body temperature, and sweating. Some experience chronic muscle pain.

The muscle stiffness initially fluctuates, sometimes for days or weeks, but eventually begins to consistently impair mobility. As the disease progresses, patients sometimes become unable to walk or bend. Chronic pain is common and worsens over time, but sometimes acute pain occurs as well. Stress, cold weather, and infections lead to an increase in symptoms, and sleep decreases them.

SPS patients experience superimposed spasms and extreme sensitivity to touch and sound. These spasms primarily occur in the proximal limb and axial muscles. Spasms usually last for minutes and can recur over hours. Attacks of spasms are unpredictable and are often caused by fast movements, emotional distress, or sudden sounds or touches. In rare cases, facial muscles, hands, feet, and the chest can be affected and unusual eye movements and vertigo occur. There are brisk stretch reflexes and clonus occurs in patients. Late in the disease's progression, hypnagogic myoclonus can occur.

In addition to their physical symptoms, many with SPS experience neurological and psychiatric disorders. Some with SPS have various neurological disorders that affect physical reflexes, and the movement of the eyes. Some also experience anxiety, depression, alcohol use disorders, and phobias – particularly agoraphobia. Most patients are psychologically normal and respond reasonably to their situations.

A minority of people with SPS experience "partial" SPS, also called "stiff limb syndrome", where the muscle contractions and stiffness are limited to the limbs, or sometimes a single limb. This syndrome develops into full SPS about 25% of the time.[16] The stiffness begins in one limb and remains most prominent there. Sphincter and brainstem issues often occur with stiff-limb syndrome.

Progressive encephalomyelitis with rigidity and myoclonus, another variant of the condition, includes symptoms of SPS with brainstem issues, autonomic disturbances, and myoclonus. In some cases, the limbic system is affected as well. Most patients have upper motor neuron issues and autonomic disturbances.

Around 5% of those with SPS experience the symptoms as a paraneoplastic syndrome – a result of a tumor elsewhere in the body releasing bioactive molecules. Paraneoplastic SPS can affect either a single limb, or the trunk and limbs together.

HOMOEOPATHIC APPROACH

Homoeopathy today is a rapidly growing system and is being practiced all over the world. It strength lies in its evident effectiveness as it takes a holistic approach towards the sick individual through promotion of inner balance at mental, emotional, spiritual and physical levels. When is concerned there are many effective medicines available in Homoeopathy, but the selection depends upon the individuality of the patient, considering mental and physical symptoms

Few homoeopathic medicine can be thought of in the management of Stiff-person syndrome are:

cuprum met., tarentula, secale cor., plumbum met., etc