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Glossary

Glossary

Optic Neuritis

Optic neuritis is an inflammation of the optic nerve, the bundle of nerve fibers that transmit visual information to your brain from your eye. Pain and temporary vision loss are common symptoms of optic neuritis.

Optic neuritis usually develops in association with an autoimmune disorder that may be triggered by an infection. In some people, signs and symptoms of optic neuritis may be an indication of multiple sclerosis, a condition resulting in inflammation and damage to nerves in your brain and spinal cord.

Most people who experience a single episode of optic neuritis eventually recover their vision. Treatment with steroid medications may speed up vision recovery after optic neuritis.

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Ormond’s Disease

Ormond’s Disease or Retroperitoneal Fibrosis is a disease featuring the proliferation of fibrous tissue in the retroperitoneum, the compartment of the body containing the kidneys,aorta, renal tract, and various other structures. It may present with lower back pain, renal failure, hypertension, deep vein thrombosis, and other obstructive symptoms. It is named after John Kelso Ormond, who rediscovered the condition in 1948.

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Ovarian

Ovarian cysts are fluid-filled sacs or pockets within or on the surface of an ovary. Women have two ovaries – each about the size and shape of an almond – located on each side of the uterus. Eggs (ova) develop and mature in the ovaries and are released in monthly cycles during your childbearing years.

Many women have ovarian cysts at some time during their lives. Most ovarian cysts present little or no discomfort and are harmless. The majority of ovarian cysts disappear without treatment within a few months.

However, ovarian cysts – especially those that have ruptured – sometimes produce serious symptoms. The best ways to protect your health are to know the symptoms that may signal a more significant problem, and to schedule regular pelvic examinations.

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Palindromic Rheumatism (PR)

Palindromic Rheumatism (PR) consists of sudden and rapidly developing attacks ofarthritis. There is acute pain, redness, swelling, and disability of one (usually) or multiple joints. The interval between recurrent attacks is extremely variable and how long the attack lasts is also variable. There is no joint damage after attacks.

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Pancreatitis

Pancreatitis is inflammation in the pancreas. The pancreas is a long, flat gland that sits tucked behind the stomach in the upper abdomen. The pancreas produces enzymes that help digestion and hormones that help regulate the way your body processes sugar (glucose).

Pancreatitis can occur as acute pancreatitis – meaning it appears suddenly and lasts for days. Or pancreatitis can occur as chronic pancreatitis, which describes pancreatitis that occurs over many years.

Mild cases of pancreatitis may go away without treatment, but severe cases can cause life-threatening complications.

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PANDAS (Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcus)

PANDAS is an acronym for Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal infections. This term describes a hypothesis that there exists a subset of children with rapid onset of obsessive-compulsive disorder (OCD) and/ortic disorders and these symptoms are caused by group A beta-hemolytic streptococcal (GABHS) infections. The proposed link between infection and these disorders is that an initial autoimmune reaction to a GABHS infection produces antibodies that continues to interfere with basal ganglia function, causing symptom exacerbations.

The PANDAS hypothesis was based on observations in clinical case studies at the USNational Institute of Health and in subsequent clinical trials where children appeared to have dramatic and sudden OCD exacerbations and tic disorders following infections. There is supportive evidence for the link between streptococcus infection and onset in some cases of OCD and tics, but proof of causality has remained elusive. The PANDAS hypothesis is controversial; whether it is a distinct entity differing from other cases of Tourette syndrome (TS)/OCD is debated.

PANDAS has not been validated as a disease entity; it is not listed as a diagnosis by the International Statistical Classification of Diseases and Related Health Problems (ICD) or the Diagnostic and Statistical Manual of Mental Disorders (DSM). Pediatric acute-onset neuropsychiatric syndrome (PANS) is a 2012 proposal describing another subset of acute-onset OCD cases including “not only disorders potentially associated with a preceding infection, but also acute-onset neuropsychiatric disorders without an apparent environmental precipitant or immune dysfunction”.

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Paraneoplastic Cerebellar Degeneration

Paraneoplastic cerebellar degeneration (PCD) is a paraneoplastic syndromeassociated with lung, ovarian, breast, Hodgkin’s lymphoma and other cancers. PCD is a rare condition that occurs in less than 1% of cancer patients and usually occurs in middle-aged women.

As is the case with other paraneoplastic syndromes, PCD is believed to be due to anautoimmune reaction targeted against components of the central nervous system (in PCD, this is specifically Purkinje cells in the cerebellum, sometimes accompanied by a proliferation of Berg- mann astrocytes and microglia in the molecular layer of the cerebellum and a loss of granule cells). It is thought to be triggered when tumor cells (in PCD, most commonly ovarian or breast cancer) express a protein normally expressed in the brain (in PCD, this is the Purkinje neuronal protein termed cdr2). This is believed to trigger an anti-tumor immune response that may be clinically significant, but also an anti-neuronal immune response. PCD patients harbor an anti-neuronal antibody known as anti-Yo (named after the first two letters of the index patient). PCD may be associated with onconeural antibodies directed against other intracellular antigens or against cell surface and with other tumors. When associated with small cell lung cancer, it is antibody termed “anti-Hu” (more commonly associated with paraneoplastic subacute sensory neuropathy and/or limbic encephalitis). The immune cells cross the blood–brain barrier, resulting in an autoimmune attack of Purkinje cells throughout the cerebellar cortex. Radiologic imaging occasionally reveals cerebellar atrophy. Other paraneoplastic antibodies may be associated with PCD symptoms, including anti-Tr and antibodies to glutamate receptor. Occasionally myoclonia and opsoclonus may occur.

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Parkinson Disease

Parkinson’s disease is a progressive disorder of the nervous system that affects your movement. It develops gradually, sometimes starting with a barely noticeable tremor in just one hand. But while tremor may be the most well-known sign of Parkinson’s disease, the disorder also commonly causes stiffness or slowing of movement.

In early stages of Parkinson’s disease, your face may show little or no expression, or your arms may not swing when you walk. Your speech may become soft or slurred. Parkinson’s disease symptoms worsen as your condition progresses over time.

Although Parkinson’s disease can’t be cured, medications may markedly improve your symptoms. In occasional cases, your doctor may suggest surgery to regulate certain regions of your brain and improve your symptoms.

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Paroxysmal Nocturnal Hemoglobinuria (PNH)

Paroxysmal nocturnal hemoglobinuria (PNH), sometimes referred to as Marchiafava-Micheli syndrome, is a rare, acquired, potentially life-threatening disease of the blood characterised by complement-induced intravascular hemolytic anemia (anemia due to destruction of red blood cells in the bloodstream), red urine (due to the appearance ofhemoglobin in the urine) and thrombosis. PNH is the only hemolytic anemia caused by anacquired (rather than inherited) intrinsic defect in the cell membrane (deficiency ofglycophosphatidylinositol leading to absence of protective proteins on the membrane). It may develop on its own (“primary PNH”) or in the context of other bone marrow disorders such as aplastic anemia (“secondary PNH”). Only a minority have the telltale red urine in the morning.

Allogeneic bone marrow transplantation is the only curative therapy, although themonoclonal antibody eculizumab (Soliris) is effective at reducing the need for blood transfusions, improving quality of life, and reducing the risk of thrombosis.

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Parry Romberg Syndrome

Parry–Romberg Syndrome (also known as progressive hemifacial atrophy) is a rareneurocutaneous syndrome characterized by progressive shrinkage and degeneration of thetissues beneath the skin, usually on only one side of the face (hemifacial atrophy) but occasionally extending to other parts of the body. An autoimmune mechanism is suspected, and the syndrome may be a variant of localized scleroderma, but the preciseetiology and pathogenesis of this acquired disorder remains unknown. The syndrome has a higher prevalence in females and typically appears between 5 – 15 years of age.

In addition to the connective tissue disease, the condition is often accompanied by significant neurological, ocular and oral signs and symptoms. The range and severity of associated symptoms and findings are highly variable.

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Pemphigus

Pemphigus is a group of rare skin disorders that cause blisters of your skin or mucous membranes, such as in your mouth or on your genitals.

There are two main types: pemphigus vulgaris and pemphigus foliaceus. Pemphigus vulgaris is the most common form. Pemphigus can occur at any age, but often strikes people in middle age or older.

Usually a chronic condition, pemphigus is best controlled by early diagnosis and treatment, which may include medications or treatments similar to those used for severe burns.

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Pemphigus Neuropathy

Peripheral neuropathy, a result of nerve damage, often causes numbness and pain in your hands and feet. People typically describe the pain of peripheral neuropathy as tingling or burning, while they may compare the loss of sensation to the feeling of wearing a thin stocking or glove.

Peripheral neuropathy can result from problems such as traumatic injuries, infections, metabolic problems and exposure to toxins. One of the most common causes is diabetes.

In many cases, peripheral neuropathy symptoms improve with time — especially if the condition is caused by an underlying condition that can be treated. A number of medications often are used to reduce the painful symptoms of peripheral neuropathy.

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Pernicious Anemia

Pernicious Anemia is a condition in which you don’t have enough healthy red blood cells to carry adequate oxygen to your tissues. Having anemia may make you feel exhausted.

There are many forms of pernicious anemia, each with its own cause. Pernicious Anemia can be temporary or long term, and it can range from mild to severe.

See your doctor if you suspect you have anemia, because pernicious anemia can be a sign of serious illnesses. Treatments for pernicious anemia range from taking supplements to undergoing medical procedures. You may be able to prevent some types of pernicious anemia by eating a healthy, varied diet.

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POEMS Syndrome

POEMS syndrome is a rare disorder of the blood that affects many of your body systems and causes damage to your nerves. POEMS syndrome may occur because of the growth of certain bone marrow plasma cells (clonal plasma cells) that produce an abnormal amount of blood proteins, which damage body organs.

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Polmyalgia Rheumatica

Polymyalgia rheumatica is an inflammatory disorder that causes muscle pain and stiffness. The pain and stiffness often occur in your shoulders, neck, upper arms and hips. Symptoms of polymyalgia rheumatica (pol-ee-my-AL-juh roo-MA-ti-kah) usually begin quickly, within two weeks.

Most people who develop polymyalgia rheumatica are older than 65. It rarely affects people younger than 50.

Anti-inflammatory drugs called corticosteroids improve the symptoms of polymyalgia rheumatica, but these drugs require careful monitoring for serious side effects.

Polymyalgia rheumatica is related to another inflammatory disorder called giant cell arteritis, which can cause headaches, vision difficulties, jaw pain and other symptoms. It’s possible to have both of these conditions together.

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