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Encyclopedia > Subacute sclerosing panencephalitis
Subacute sclerosing panencephalitis
Classification & external resources
ICD-10 A81.1
ICD-9 046.2
OMIM 260470
DiseasesDB 12597
MedlinePlus 001419
MeSH C02.182.500.300.600

Subacute sclerosing panencephalitis (SSPE) is a rare chronic, progressive encephalitis that affects primarily children and young adults, caused by defective measles virus (which can be a result of a mutation of the virus itself). 1 in 100,000 people infected with measles are at risk. SSPE is 'incurable' but the condition can be managed by medication if treatment is started at an early stage. Much of the work on SSPE has been completed by the NINDS. The International Statistical Classification of Diseases and Related Health Problems (most commonly known by the abbreviation ICD) provides codes to classify diseases and a wide variety of signs, symptoms, abnormal findings, complaints, social circumstances and external causes of injury or disease. ... The following codes are used with International Statistical Classification of Diseases and Related Health Problems. ... // A00-A79 - Bacterial infections, and other intestinal infectious diseases, and STDs (A00-A09) Intestinal infectious diseases (A00) Cholera (A01) Typhoid and paratyphoid fevers (A010) Typhoid fever (A02) Other Salmonella infections (A03) Shigellosis (A04) Other bacterial intestinal infections (A040) Enteropathogenic Escherichia coli infection (A045) Campylobacter enteritis (A046) Enteritis due to Yersinia... The International Statistical Classification of Diseases and Related Health Problems (most commonly known by the abbreviation ICD) provides codes to classify diseases and a wide variety of signs, symptoms, abnormal findings, complaints, social circumstances and external causes of injury or disease. ... The following is a list of codes for International Statistical Classification of Diseases and Related Health Problems. ... The Mendelian Inheritance in Man project is a database that catalogues all the known diseases with a genetic component, and - when possible - links them to the relevant genes in the human genome. ... The Disease Bold textDatabase is a free website that provides information about the relationships between medical conditions, symptoms, and medications. ... MedlinePlus (medlineplus. ... Medical Subject Headings (MeSH) is a huge controlled vocabulary (or metadata system) for the purpose of indexing journal articles and books in the life sciences. ... Encephalitis is an acute inflammation of the brain, commonly caused by a viral infection. ... Measles, also known as rubeola, is a common disease caused by a virus of the genus Morbillivirus. ... The National Institute of Neurological Disorders and Stroke is a part of the U.S. National Institutes of Health. ...


SSPE is also known as Dawson Disease, Dawson encephalitis and measles encephalitis.

Contents

Symptoms

Characterized by a history of primary measles infection before the age of 2 years, followed by several asymptomatic years (6–15 on average), and then gradual, progressive psychoneurological deterioration, consisting of personality change, seizures, myoclonus, ataxia, photosensitivity, ocular abnormalities, spasticity, and coma. The 2nd episode of 1st season of House (TV series) is a story about a patient with this disease. This article is about the medical condition. ... Myoclonus is brief, involuntary twitching of a muscle or a group of muscles. ... Ataxia (from Greek ataxiā, meaning failure to put in order) is unsteady and clumsy motion of the limbs or torso due to a failure of the gross coordination of muscle movements. ... Photosensitivity is the amount to which an object reacts upon receiving photons of light. ... In medicine, a coma (from the Greek koma, meaning deep sleep) is a profound state of unconsciousness. ... House, also known as House M.D., is a critically-acclaimed American medical drama television series created by David Shore and executive produced by film director Bryan Singer. ...


Progression

The progression of symptoms begins with stage 1 — in this stage the behaviour of person become more abnormal and erratic, the person can be irritable and personality alterations can occur. This is often accompanied by memory loss and mental deterioration characterised by intellectual difficulty. As the nervous system begins to lose control of movement, the person develops myoclonic spasms/jerks (these being involuntary motions and spasms in extremities). The myoclonic spasm is a key warning sign of SSPE as these spasms are only normally seen as the body drifts off to sleep — breathing rate decreases, heart rate slows and body temperature lowers causing the brain to think the body is dying and so a myoclonic spasm ensues to try and rouse the body — and so if myclonic spasms are seen when the person isn't tired or during the day then this is indicative of a potentially serious problem (such as SSPE). As the disease progresses towards stage 2, the intensity of the spasms and the mental deterioration increases. The spasms can grow to such an extent that loss of the ability to walk can be a common sign. Also, the person will suffer speech impairment and increasingly deteriorated comprehension coupled with dysphagia. At this point the infection is at stage 2. The final, advanced stages of SSPE include the steady decline in body function with increased intensity of the stage 2 symptoms/signs and also blindness. At the end of the final stages the person is likely to be mute and/or comatose. Dysphagia () is a medical term defined as difficulty swallowing. ...


Diagnosis

Characteristic periodic activity is seen on EEG (this activity showing widespread cortical dysfunction); pathologically, the white matter of both the hemispheres and brainstem are affected, as well as the cerebral cortex, and eosinophilic inclusion bodies are present in the cytoplasm nuclei of neurons and glial cells. Diagnosis of SSPE is often difficult due to a normal CSF profile — noted changes in the CSF profile only include a marked elevation in CSF immunoglobulin. EEG can mean: Electroencephalography - the method and science of recording and interpreting traces of brain electrical activity as recorded from the skull surface or the device used to record such traces Emperor Entertainment Group - A Hong Kong entertainment company. ... White matter is one of the two main solid components of the central nervous system. ... The brain stem is the stalk of the brain below the cerebral hemispheres. ...


Prognosis

Death usually occurs within 3 years. If the diagnosis is made during stage 1 of the SSPE infection then it is possible to cure the disease. However, once SSPE progresses to stage 2 then it is universally fatal in all occurrences. The standard rate of decline spans anywhere between 1–3 years after the onset of the infection. The progression of each stage is unique to the sufferer and cannot be predicted although the pattern or symptoms/signs can be. Although the prognosis is bleak for SSPE past stage 1, it should be noted that there is a 5% remission rate — this may be either a full remission or an improvement in condition giving a longer progression period or at least a longer period with the less severe symptoms. Regardless of the stage that the infection is at, treatment with isonine pranobex combined with interferon can give up to a 50% remission/improvement rate.


Treatment

Should the viral progression be diagnosed during stage 1 (even during late stage 1 when stage 2 symptoms start to manifest themselves) then treatment to combat the infection can be administered successfully — there is no cure for SSPE but if it is caught early enough then the sufferer can respond to the treatment and prevent symptom recurrence by taking the medication for the rest of their life. The treatment for the SSPE infection is the immunomodulator interferon and specific antiviral medication — ribavirin and inosine pranobex are specifically used to greater effect than antivirals such as Amantadine. For those who have progressed to stage 2 or beyond then the disease is incurable. For patients in the terminal phase of the disease there is a palliative care and treatment scheme — this involves anticonvulsant therapy (to help with the body's progressive loss of control of the nervous system causing gradually more intensive spasms/convulsions) alongside supportive measures to help maintain vital functioning. It is fairly standard as the infection's spread and symptoms intensify that feeding tubes need to be inserted to keep a nutritional balance. As the disease progresses to its most advanced phase, the patient will need constant nursing as normal bodily function declines to the complete collapse of the nervous system. Combinations of treatment for SSPE include: To meet Wikipedias quality standards, this article or section may require cleanup. ... Palliative care (from Latin palliare, to cloak) is any form of medical care or treatment that concentrates on reducing the severity of disease symptoms or slowing the diseases progress, rather than providing a cure. ...

  • Oral inosine pranobex (oral isoprinosine) combined with intrathecal (injection through a lumbar puncture into the spinal fluid) or intraventricular interferon alpha.
  • Oral inosine pranobex (oral isoprinosine) combined with interferon beta.
  • Intrathecal interferon alpha combined with intravenous ribavirin.

Global Patterns of Infection

SSPE is an incredibly rare condition although there is still relatively high incidence in Asia and the Middle East. However, the number of reported cases is declining since the introduction of the measles vaccine — eradication of the measles virus prevents the SSPE mutation and therefore the progression of the disease or even the initial infection itself.


External links


  Results from FactBites:
 
SUBACUTE SCLEROSING PANENCEPHALITIS DAWSON’S ENCEPHALITIS REVISITED PEDIATRIC ONCALL (806 words)
A differential diagnosis of subacute sclerosing panencephalitis, MERRF, Ramsay Hunt disease and Lafora’s disease was considered.
Subacute Sclerosing Panencephalitis (SSPE) is rare chronic encephalitis caused by persistent measles virus infection of the central nervous system.
SSPE should be distinguished from subacute measles encephalitis with measles inclusion body encephalitis (MIBE) which occurs in children with immunocompromised cellular immunity.
Subacute Sclerosing Panencephalitis: Viral Infections: Merck Manual Home Edition (374 words)
Subacute sclerosing panencephalitis, a progressive and usually fatal disorder, is a rare complication of measles that appears months or years later and produces mental deterioration, muscle jerks, and seizures.
Subacute sclerosing panencephalitis results from a long-term brain infection with the measles virus.
Subacute sclerosing panencephalitis occurs because the measles virus reactivates; in the United States for reasons that are not known, the disorder occurs in about 1 or 2 people per 1 million who previously had measles.
  More results at FactBites »

 
 

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