Subacute sclerosing panencephalitis: Difference between revisions

Jump to navigation Jump to search
VisualWikitext
No edit summary
No edit summary
Line 1: Line 1:
'''For patient information, click [[Subacute sclerosing panencephalitis (patient information)|here]]'''
'''For patient information, click [[Subacute sclerosing panencephalitis (patient information)|here]]'''
{{Infobox_Disease |
{{Infobox_Disease |
   Name          = Subacute sclerosing panencephalitis |
   Name          = Subacute sclerosing panencephalitis |
Line 15: Line 16:
   MeshNumber    = C02.182.500.300.600 |
   MeshNumber    = C02.182.500.300.600 |
}}
}}
{{SI}}
{{Subacute sclerosing panencephalitis}}
{{CMG}}
{{CMG}}


{{SK}} SSPE, Dawson Disease, Dawson encephalitis and measles encephalitis
{{SK}} SSPE, Dawson Disease, Dawson encephalitis and measles encephalitis


==Overview==
==[[Subacute sclerosing panencephalitis overview|Overview]]==
'''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]].


==Symptoms==
==[[Subacute sclerosing panencephalitis classification|Classification]]==
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]].


===Progression===
==[[Subacute sclerosing panencephalitis historical perspective|Historical Perspective]]==
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.


==Diagnosis==
==[[Subacute sclerosing panencephalitis pathophysiology|Pathophysiology]]==
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.
==[[Subacute sclerosing panencephalitis epidemiology and demographics|Epidemiology & Demographics]]==
 
==[[Subacute sclerosing panencephalitis epidemiology and demographics|Risk Factors]]==
 
==[[Subacute sclerosing panencephalitis screening|Screening]]==
 
==[[Subacute sclerosing panencephalitis causes|Causes]]==


==Prognosis==
==[[Subacute sclerosing panencephalitis differential diagnosis|Differentiating Subacute sclerosing panencephalitis from other Diseases]]==
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==
==[[Subacute sclerosing panencephalitis natural history|Natural History, Complications & Prognosis]]==


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.
==Diagnosis==
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.
[[Subacute sclerosing panencephalitis history and symptoms|History & Symptoms]] | [[Subacute sclerosing panencephalitis physical examination|Physical Examination]] | [[Subacute sclerosing panencephalitis staging|Staging]] | [[Subacute sclerosing panencephalitis laboratory tests|Lab Tests]] | [[Subacute sclerosing panencephalitis electrocardiogram|Electrocardiogram]] | [[Subacute sclerosing panencephalitis chest x ray|Chest X Ray]] | [[Subacute sclerosing panencephalitis CT|CT]] | [[Subacute sclerosing panencephalitis MRI|MRI]] | [[Subacute sclerosing panencephalitis echocardiography or ultrasound|Echocardiography or Ultrasound]] | [[Subacute sclerosing panencephalitis other imaging findings|Other Imaging Findings]] | [[Subacute sclerosing panencephalitis other diagnostic studies#EEG|EEG]] | [[Subacute sclerosing panencephalitis other diagnostic studies#CSF|CSF]]
Combinations of treatment for SSPE include:
* 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==
==Treatment==
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.
[[Subacute sclerosing panencephalitis medical therapy|Medical Therapy]] | [[Subacute sclerosing panencephalitis surgery|Surgery]] | [[Subacute sclerosing panencephalitis primary prevention|Primary Prevention]] | [[Subacute sclerosing panencephalitis secondary prevention|Secondary Prevention]]


{{Viral diseases}}
{{Viral diseases}}
{{Symptoms and signs}}
{{Symptoms and signs}}
{{SIB}}
 


[[es:panencefalitis esclerosante subaguda]]
[[es:panencefalitis esclerosante subaguda]]
Line 59: Line 56:
[[fi:Subakuutti sklerosoiva panenkefaliitti]]
[[fi:Subakuutti sklerosoiva panenkefaliitti]]
[[zh:稍尖的硬化全脑炎]]
[[zh:稍尖的硬化全脑炎]]
{{WikiDoc Help Menu}}
{{WikiDoc Help Menu}}
{{WikiDoc Sources}}
{{WikiDoc Sources}}
[[Category:Disease]]
[[Category:Infectious disease]]
[[Category:Neurology]]


[[Category:Neurological disorders]]
[[Category:Neurological disorders]]
[[Category:Viral diseases]]
[[Category:Viral diseases]]
[[Category:Inflammations]]
[[Category:Inflammations]]
[[Category:Infectious disease]]
[[Category:Neurology]]
[[Category:Overview complete]]
[[Category:Overview complete]]

Revision as of 17:51, 2 February 2012

For patient information, click here

Subacute sclerosing panencephalitis
Brain: Subacute Sclerosing Panencephalitis: Micro high mag H&E inflammatory reaction (well depicted).
Image courtesy of Professor Peter Anderson DVM PhD and published with permission © PEIR, University of Alabama at Birmingham, Department of Pathology
ICD-10 A81.1
ICD-9 046.2
OMIM 260470
DiseasesDB 12597
MedlinePlus 001419
MeSH C02.182.500.300.600

Subacute sclerosing panencephalitis Microchapters

Home

Patient Information

Overview

Historical Perspective

Pathophysiology

Causes

Differentiating Subacute sclerosing panencephalitis from other Diseases

Epidemiology and Demographics

Risk Factors

Natural History, Complications and Prognosis

Diagnosis

History and Symptoms

Physical Examination

Laboratory Findings

CT

MRI

Other Imaging Findings

Other Diagnostic Studies

Treatment

Medical Therapy

Primary Prevention

Cost-Effectiveness of Therapy

Future or Investigational Therapies

Case Studies

Case #1

Subacute sclerosing panencephalitis On the Web

Most recent articles

Most cited articles

Review articles

CME Programs

Powerpoint slides

Images

American Roentgen Ray Society Images of Subacute sclerosing panencephalitis

All Images
X-rays
Echo & Ultrasound
CT Images
MRI

Ongoing Trials at Clinical Trials.gov

US National Guidelines Clearinghouse

NICE Guidance

FDA on Subacute sclerosing panencephalitis

CDC on Subacute sclerosing panencephalitis

Subacute sclerosing panencephalitis in the news

Blogs on Subacute sclerosing panencephalitis

Directions to Hospitals Treating Subacute sclerosing panencephalitis

Risk calculators and risk factors for Subacute sclerosing panencephalitis

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Synonyms and keywords: SSPE, Dawson Disease, Dawson encephalitis and measles encephalitis

Overview

Classification

Historical Perspective

Pathophysiology

Epidemiology & Demographics

Risk Factors

Screening

Causes

Differentiating Subacute sclerosing panencephalitis from other Diseases

Natural History, Complications & Prognosis

Diagnosis

History & Symptoms | Physical Examination | Staging | Lab Tests | Electrocardiogram | Chest X Ray | CT | MRI | Echocardiography or Ultrasound | Other Imaging Findings | EEG | CSF

Treatment

Medical Therapy | Surgery | Primary Prevention | Secondary Prevention

Template:Viral diseases

Template:Skin and subcutaneous tissue symptoms and signs Template:Nervous and musculoskeletal system symptoms and signs Template:Urinary system symptoms and signs Template:Cognition, perception, emotional state and behaviour symptoms and signs Template:Speech and voice symptoms and signs Template:General symptoms and signs

fi:Subakuutti sklerosoiva panenkefaliitti


Template:WikiDoc Sources

Retrieved from "https://www.wikidoc.org/index.php?title=Subacute_sclerosing_panencephalitis&oldid=629706"