Subacute sclerosing panencephalitis: Difference between revisions

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	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

VisualWikitext

Latest revision as of 18:52, 18 September 2017

For patient information, click here

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

Synonyms and keywords: SSPE; Dawson disease; Dawson encephalitis; measles encephalitis

Overview

Historical Perspective

Pathophysiology

Causes

Differentiating Subacute sclerosing panencephalitis from other Diseases

Epidemiology and Demographics

Risk Factors

Natural History, Complications and Prognosis

Diagnosis

Treatment

Medical Therapy | Primary Prevention | Cost-Effectiveness of Therapy | Future or Investigational Therapies

Case Studies

Case #1

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

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+__NOTOC__
+'''For patient information, click [[Subacute sclerosing panencephalitis (patient information)|here]]'''
 {{Infobox_Disease |
    Name           = Subacute sclerosing panencephalitis |
    Image          = Subacute sclerosing panencephalitis 1.jpg|
    Caption        = Brain: Subacute Sclerosing Panencephalitis: Micro high mag H&E inflammatory reaction (well depicted). <br> <small> [http://www.peir.net Image courtesy of Professor Peter Anderson DVM PhD and published with permission © PEIR, University of Alabama at Birmingham, Department of Pathology] </small>|
-  DiseasesDB     = 12597 |
-  ICD10          = {{ICD10|A|81|1|a|80}} |
-  ICD9           = {{ICD9|046.2}} |
-  ICDO           = |
-  OMIM           = 260470 |
-  MedlinePlus    = 001419 |
-  eMedicineSubj  = |
-  eMedicineTopic = |
-  MeshName       = Subacute+Sclerosing+Panencephalitis |
-  MeshNumber     = C02.182.500.300.600 |
 }}
-{{SI}}
+{{Subacute sclerosing panencephalitis}}
 {{CMG}}
-{{Editor Help}}
+{{SK}} SSPE; Dawson disease; Dawson encephalitis; measles encephalitis
+==[[Subacute sclerosing panencephalitis overview|Overview]]==
+==[[Subacute sclerosing panencephalitis historical perspective|Historical Perspective]]==
-{{SK}} SSPE, Dawson Disease, Dawson encephalitis and measles encephalitis
+==[[Subacute sclerosing panencephalitis pathophysiology|Pathophysiology]]==
-==Overview==
+==[[Subacute sclerosing panencephalitis causes|Causes]]==
-'''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 differential diagnosis|Differentiating Subacute sclerosing panencephalitis from other Diseases]]==
-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 epidemiology and demographics|Epidemiology and Demographics]]==
-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.
+==[[Subacute sclerosing panencephalitis risk factors|Risk Factors]]==
-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.
+==[[Subacute sclerosing panencephalitis natural history|Natural History, Complications and Prognosis]]==
 ==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.
-==Prognosis==
+[[Subacute sclerosing panencephalitis history and symptoms|History and Symptoms]] | [[Subacute sclerosing panencephalitis physical examination|Physical Examination]] | [[Subacute sclerosing panencephalitis laboratory tests|Laboratory Findings]] | [[Subacute sclerosing panencephalitis CT|CT]] | [[Subacute sclerosing panencephalitis MRI|MRI]] | [[Subacute sclerosing panencephalitis other imaging findings|Other Imaging Findings]] | [[Subacute sclerosing panencephalitis other diagnostic studies|Other Diagnostic Studies]]
-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.
+[[Subacute sclerosing panencephalitis medical therapy|Medical Therapy]] | [[Subacute sclerosing panencephalitis primary prevention|Primary Prevention]] | [[Subacute sclerosing panencephalitis cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[Subacute sclerosing panencephalitis future or investigational therapies|Future or Investigational Therapies]]
-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:
-* 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==
+==Case Studies==
-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 case study one|Case #1]]
-==External links==
-* {{MerckHome|23|273|l}}
-* {{cite journal |author=Bonthius D, Stanek N, Grose C |title=Subacute sclerosing panencephalitis, a measles complication, in an internationally adopted child |journal=Emerg Infect Dis |volume=6 |issue=4 |pages=377-81 |year=2000  |pmid=10905971 |url=http://www.cdc.gov/ncidod/eid/vol6no4/bonthius.htm}}
-* {{NINDS|subacute_panencephalitis}}
-* {{WhoNamedIt|synd|1889}}
 {{Viral diseases}}
 {{Symptoms and signs}}
-{{SIB}}
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+[[Category:Disease]]
+[[Category:Neurology]]
 [[Category:Neurological disorders]]
 [[Category:Viral diseases]]
 [[Category:Inflammations]]
-[[Category:Infectious disease]]
-[[Category:Neurology]]

v t e Symptoms and signs: circulatory (R00–R03, 785)
Cardiovascular	Template:Navbox subgroup
Myeloid/blood	Template:Navbox subgroup

v t e Symptoms and signs: respiratory system (R04–R07, 786)
Hemorrhage	Epistaxis Hemoptysis
Abnormalities of breathing	Respiratory sounds Stridor Wheeze Crackles Rhonchi Hamman's sign Apnea Dyspnea Hyperventilation/Hypoventilation Hyperpnea/Tachypnea/Hypopnea/Bradypnea Orthopnea/Platypnea Trepopnea Biot's respiration Cheyne-Stokes respiration Kussmaul breathing Hiccup Mouth breathing/Snoring Breath-holding
Other	Asphyxia Cough Pleurisy Sputum Respiratory arrest Hypercapnia/Hypocapnia Pectoriloquy: Whispered pectoriloquy Egophony Bronchophony Pleural friction rub Fremitus Silhouette sign
Chest, general	Chest pain Precordial catch syndrome

v t e Symptoms and signs: digestive system and abdomen (R10–R19, 787,789)
GI tract	Template:Navbox subgroup
Accessory	Hepatosplenomegaly/Hepatomegaly Jaundice
Abdominopelvic	Ascites
Abdominal – general	Abdominal pain (Acute abdomen, Colic, Baby colic) Splenomegaly Abdominal guarding · Abdominal mass · Rebound tenderness Shifting dullness · Bulging flanks · Puddle sign · Fluid wave test

v t e Symptoms and signs: Symptoms concerning nutrition, metabolism and development (R62–R64, 783)
Ingestion/Weight	decrease: Anorexia • Weight loss/Cachexia/Underweight increase: Polyphagia • Polydipsia • Orexigenia • Weight gain
Growth	Delayed milestone • Failure to thrive • Short stature (e.g., Idiopathic)


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

Subacute sclerosing panencephalitis: Difference between revisions

Latest revision as of 18:52, 18 September 2017

Diagnosis

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