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Schnitzler syndrome

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He began work at Vienna's General Hospital German: Allgemeines Krankenhaus der Stadt Wien , but ultimately abandoned the practice of medicine in favour of writing. On 26 August , Schnitzler married Olga Gussmann — , a year-old aspiring actress and singer who came from a Jewish middle-class family.

They had a son, Heinrich — , born on 9 August In they had a daughter, Lili, who committed suicide in The Schnitzlers separated in Schnitzler died on 21 October , in Vienna, of a brain hemorrhage. In , following the Anschluss , his son Heinrich went to the United States and did not return to Austria until ; he is the father of the Austrian musician and conservationist Michael Schnitzler , born in in Berkeley, California, who moved to Vienna with his parents in Schnitzler's works were often controversial, both for their frank description of sexuality in a letter to Schnitzler Sigmund Freud confessed "I have gained the impression that you have learned through intuition — although actually as a result of sensitive introspection — everything that I have had to unearth by laborious work on other persons" [4] and for their strong stand against anti-Semitism , represented by works such as his play Professor Bernhardi and his novel Der Weg ins Freie.

However, although Schnitzler was himself Jewish, Professor Bernhardi and Fräulein Else are among the few clearly identified Jewish protagonists in his work. Schnitzler was branded as a pornographer after the release of his play Reigen , in which ten pairs of characters are shown before and after the sexual act, leading and ending with a prostitute.

The furore after this play was couched in the strongest anti-semitic terms. The film achieved considerable success in the English-speaking world, with the result that Schnitzler's play is better known there under its French title. More recently, in Fernando Meirelles ' film , Schnitzler's play was provided with a new version, as has been the case with many other TV and film productions.

In the novella Fräulein Else Schnitzler may be rebutting a contentious critique of the Jewish character by Otto Weininger by positioning the sexuality of the young female Jewish protagonist.

What other subjects are there? Professor Bernhardi , a play about a Jewish doctor who turns away a Catholic priest in order to spare a patient the realization that she is on the point of death, is his only major dramatic work without a sexual theme. A member of the avant-garde group Young Vienna Jung Wien , Schnitzler toyed with formal as well as social conventions.

With his novella Lieutenant Gustl , he was the first to write German fiction in stream-of-consciousness narration. The story is an unflattering portrait of its protagonist and of the army's obsessive code of formal honour.

It caused Schnitzler to be stripped of his commission as a reserve officer in the medical corps — something that should be seen against the rising tide of anti-semitism of the time.

He specialized in shorter works like novellas and one-act plays. And in his short stories like "The Green Tie" "Die grüne Krawatte" he showed himself to be one of the early masters of microfiction. However he also wrote two full-length novels: In addition to his plays and fiction, Schnitzler meticulously kept a diary from the age of 17 until two days before his death.

The manuscript, which runs to almost 8, pages, is most notable for Schnitzler's casual descriptions of sexual conquests — he was often in relationships with several women at once, and for a period of some years he kept a record of every orgasm. Most of the reported cases of Schnitzler syndrome have been from Europe, particularly France, but cases from Australia, Japan and the United States have been reported too. Symptoms of the following disorders can be similar to those of Schnitzler syndrome.

Comparisons may be useful for a differential diagnosis. Autoinflammatory syndromes are a group of disorder characterized by recurrent episodes of inflammation due to an abnormality of the innate immune system. Symptoms of the syndromes often include periodic fevers, rash, abdominal pain, joint pain, bone pain and other characteristic findings associated with chronic inflammation. For more information on this disorder, choose the specific disorder name as your search term in the Rare Disease Database.

Autoimmune disorders are a group of disorders in which the abnormalities affecting the adaptive immune system, which consists of cells and proteins antibodies that are suppose to protect the body from infection. These antibodies mistakenly attack healthy tissue and may be referred to as autoantibodies. Symptoms common to many autoimmune disorders include repeated episodes of fever, rash, abdominal pain, joint pain and other symptoms associated with chronic inflammation.

POEMS syndrome is an extremely rare multisystem disorder. POEMS is an acronym that stands for P olyneuropathy, disease affecting many nerves; O rganomegaly, abnormal enlargement of an organ; E ndocrinoapthy, disease affecting certain hormone-producing glands that help to regulate the rate of growth, sexual development, and certain metabolic functions endocrine system ; M onoclonal gammopathy or M proteins; and S kin defects.

Endocrine abnormalities such as failure of the ovaries and testes gonads to function properly primary gonadal failure and diabetes mellitus type I may be present. Other important features of the disease include swelling around the optic nerve papilledema , abnormal fluid retention, which may occur in the ankles edema , the abdominal cavity ascites , or around the lungs pleural effusions , painless scars on bone x-ray osteosclerosis , and an elevated platelet count a blood cell responsible for clotting.

POEMS syndrome is associated with a group of disorders known as monoclonal gammopathies or plasma cell dyscrasias. These disorders are characterized the uncontrolled growth of a single clone monoclonal of plasma cells, which results in the abnormal accumulation of M-proteins IgM in the blood.

Waldenström macroglobulinemia WMG is a slow-growing indolent malignant cancerous disorder of the blood, closely related to lymphoma and characterized by the presence of abnormally large numbers of a particular kind of white blood cell known as B lymphocytes. As these cells accumulate in the body, excessive quantities of an antibody known as a monoclonal immunoglobulin M IgM are produced.

This causes the blood to become thick hyperviscosity and affects the flow of blood through the smaller blood vessels, leading to some of symptoms of the disorder.

Accumulation of B lymphocytes in the bone marrow can inhibit bone marrow production of new blood cells pancytopenia.

Affected individuals may have low levels of red blood cells anemia , white blood cells leukopenia and platelets thrombocytopenia. Consequently, affected individuals may experience fatigue, pallor, nose bleeds, and susceptibility to infection.

Some individuals with Schnitzler syndrome have developed Waldenström macroglobulinemia later in life. The exact relationship between these two disorders is not fully understood.

The exact cause of Waldenström macroglobulinemia is not known. A diagnosis of Schnitzler syndrome is based upon a thorough clinical evaluation, a detailed patient history, exclusion of other disorders, and identification of characteristic findings, specifically a urticarial rash, an M protein and at least two of the following findings — fever, joint pain or inflammation, bone pain, palpable lymph nodes, enlargement of the liver or spleen, elevated numbers of white blood cells leukocytosis , elevated red blood cell erythrocyte sedimentation rate or abnormalities on bone morphological study, which can reveal increased bone density osteosclerosis.

Sedimentation rate measures how long it takes red blood cells to settle in a test tube over a given period. Many individuals with Schnitzler syndrome have an elevated sedimentation rate, which is an indication of inflammation. But this is often not sufficient. In more severe cases, the standard treatment is with therapy to inhibit the cytokine IL Patients with Schnitzler syndrome are successfully treated with anakinra, an interleukin-1 receptor antagonist.

Anakinra is a drug that blocks the activity of interleukin-1, which some researchers believe plays a key role in the development of Schnitzler syndrome. There have also been at least 2 studies showing the efficacy of the interleukin-1 beta antibody canakinumab. High-dose regimens of corticosteroids have temporarily improved symptoms in some cases, but usually must be stopped due to side effects. In a small percentage of cases, colchicine a medication used to suppress inflammation in acute gout and dapsone were effective in treating some individuals with Schnitzler syndrome.

Interleukin-6 is a cytokine that can be induced by interleukin-1; , anti-interleukin-6 therapy was also recently tried in three patients with Schnitzler syndrome, in which it was effective. At least three individuals with Schnitzler syndrome have been successfully treated with thalidomide, a drug that affects how the immune system works immunomodulatory drugs.

Thalidomide induced a complete resolution of the rash and dramatic improvement of other symptoms in three individuals who received the drug as a therapy for Schnitzler syndrome. However, thalidomide is often associated with significant side effects including pain, numbness and a tingling sensation in the hands and feet peripheral neuropathy. Two of the three patients had to stop thalidomide therapy because of side effects. In addition, two additional individuals with Schnitzler syndrome did not improve after treatment with thalidomide.

More research is necessary to determine the long-term safety, effectiveness and role, if any, of thalidomide in treating individuals with Schnitzler syndrome. A small study investigated the effectiveness of the antibiotic drug, pefloxacine, for the treatment of Schnitzler syndrome. Eleven affected individuals received pefloxacine, which caused rapid and dramatic improvement of both the rash and systemic symptoms associated with the disorder.

More research is necessary to determine the long-term safety and effectiveness of pefloxacine in the treatment of individuals with Schnitzler syndrome. Schnitzler syndrome does not affect lifespan in most cases, but requires periodic follow up because of the increased risk of developing cancer.

Information on current clinical trials is posted on the Internet at www. All studies receiving U. Some current clinical trials also are posted on the following page on the NORD website: For information about clinical trials sponsored by private sources, contact: For information about clinical trials conducted in Europe, contact: Researchers in the department of General Internal Medicine at the Radboud University Medical Center Nijmegen, The Netherlands, have created a website to provide information and support for individuals with Schnitzler syndrome.

The researchers have an active interest in this rare disorder and have started an international registry on Schnitzler syndrome. The registry is a database that catalogues information on affected individuals to increase knowledge of this disorder. The Web site is located at: Molecular genetic investigation, clinical features, and response to treatment in 21 patients with Schnitzler syndrome. Blood Mar 1; 9: Efficacy and safety of canakinumab in Schnitzler syndrome: A multicenter randomized placebo-controlled study.

J Allergy Clin Immunol. Complete remission in 3 of 3 anti-IL-6 treated patients with Schnitzler syndrome. Successful canakinumab treatment identifies IL-1beta as a pivotal mediator in Schnitzler syndrome. Chronic urticaria and monoclonal IgM gammopathy Schnitzler syndrome. Report of 11 cases treated with pefloxacin. Worm M, Kolde G.

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It is however exceedingly rare to be free of rash for a period longer than a month in untreated patients. Anakinra is a drug that blocks the activity of interleukin-1, which some researchers believe plays a key role in the development of Schnitzler syndrome.

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