A few studies have reported that the incidence of the syndrome varies between three and six per 100,000 per year.Nine out of 10 SS patients are women. In addition to prevalence in women, having a first-degree relative with an autoimmune disease and previous pregnancies have been identified as epidemiological risk factors. Despite the lower risk for men, primary SS in men tends to represent a more severe form of the disease. The role of race and ethnicity in the prevalence of the disease is unknown.Although Sjögrens syndrome occurs in all age groups, the average age of onset is between ages 40 and 60, although as many as half of all cases may be left undiagnosed or unreported. The prevalence of SS generally increases with age.Sjögrens syndrome is reported in 30-50% of people with rheumatoid arthritis and in 10-25% with systemic lupus erythematosus. History
Jan Mikulicz-Radecki (1850–1905) is generally credited with the first description of SS. In 1892, he described a 42-year-old man with enlargement of the parotid and lacrimal glands associated with a round-cell infiltrate and acinar atrophy. However, the criteria that Mikulicz established for diagnosis often led to misdiagnosis of Mikuliczs syndrome. Many conditions, such as tuberculosis, infections, sarcoidosis and lymphoma present with similar conditions to those ascribed to Mikuliczs syndrome. Nevertheless, the term "Mikulicz’s syndrome" is still used occasionally to describe the appearance of lymphocytic infiltrates on salivary-gland biopsies.In 1930, Henrik Sjögren (1899–1986), an ophthalmologist in Jönköping, Sweden, observed a patient with low secretions from the lacrimal and salivary glands.
Paroxysmal Nonkinesigenic Dyskinesia (PNKD) is an episodic movement disorder first described by Mount and Reback in 1940 under the name "Familial paroxysmal choreoathetosis". It is a rare hereditary disease that affects various muscular and nervous systems in the body, passing to roughly fifty percent of the offspring. Signs and symptoms
The condition manifests itself as attacks lasting from a few minutes to several hours. Episodes only happen when the individual is awake, and they remain conscious throughout the attack. Symptoms are most severe in youth and lessen with age. Sufferers can have multiple attacks on a daily basis or may have periods of weeks or months between attacks. Symptoms experienced during attacks can vary and include dystonia, chorea, athetosis, ballismus, or a combination. Genetics
It has been mapped to chromosome 2q31-36.It has been associated with PNKD. Risk triggers
While not the same in all people, there are several common triggers that can precipitate an attack:
Moderate to high consumption of stimulants, such as alcohol, caffeine, or nicotine. Low amounts of energy due to hunger, lack of sleep, illness, or physical fatigue. Moderate to high presence of stress. Menstruation and ovulation. Diagnosis
Treatment
Most pharmacological treatments work poorly, but the best treatment is a low dosage of clonazepam, a muscle relaxant. Patients may also benefit from other benzodiazepines, phenobarbital, and other anticonvulsants such as valproic acid. Affected individuals have reported garlic to be effective for softening the attacks, but no studies have been done on this. References


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