There are many differential diagnoses of SSPE of which we have excluded some important ones including brain tumors, encephalitis, Wilson's disease, Huntington's disease, and cerebral venous thrombosis. (HPO) . SSPE is a chronic and debilitating slow-virus infection with an incidence around 1/1 000 000 . The authors describe five cases of subacute sclerosing panencephalitis (SSPE) identified through the California Encephalitis Project that emphasize the importance of considering SSPE in the differential diagnosis of encephalitis, particularly among pediatric patients. Measles virus infection can be clinical or subclinical. c. Diagnosis: A presumptive diagnosis is based on clinical and epidemiological grounds. Importantly, this shows that the risk of SSPE is much higher than previously thought. Cureus | A Rare Case of Subacute Sclerosing ... We agree, but would like to add that the diagnosis of SSPE may even be more difficult in adults, particularly if they present with a chronic progressive . Differential Diagnosis List Subacute sclerosing panencephalitis (SSPE) Motor regression is eventually seen in 100% of individuals with SSPE, cognitive decline in 86%, myoclonus in 74%, generalized seizures in 16%, and focal seizures in 10%.16, Some individuals may present with early visual symptoms that predate cortical signs by up to 2 years, with deficits in visuospatial orientation and early macular damage reported.5, 19 The appearance of ‘periodic dystonic myoclonus’ of abrupt onset as also been reported.40 Atypical presentations account for 10% of cases and run a fulminant course (defined as neurological deficit of 66% in the first 3 months or death within 6 months of disease onset). Malignancy enters into the differential diagnosis in a variety of ways: rapidly progressive brain primary neoplasm, cerebral secondaries, intravascular lymphoma and non-metastatic encephalopathy. The typical periodic EEG complexes in our patients led to the diagnosis of SSPE. If you need medical advice, you can look for doctors or other healthcare professionals who have experience with this disease. Usually two major criteria plus one minor criterion are required; the more atypical the SSPE, the more criteria 5 and/or 6 are needed. MRI abnormalities followed a consistent pattern. Reprinted from Ekmekci Ö, Karasoy H, Gökçay A, Ülkü A. Atypical EEG findings in subacute sclerosing panencephalitis. , Brain imaging is not needed for the diagnosis nor has an impact on the outcome, but can show the evolution/extent of the disease, and for differential diagnosis. 013 Differential diagnosis of rapidly progressive dementia ... . Signs and symptoms of the condition primarily affect the central nervous system and often develop approximately 7 to 10 years after a person recovers from the measles. The disease may develop due to reactivation of the measles virus or an inappropriate immune response to the measles virus. Although initially uncertain, the diagnosis of SSPE in both our cases became evident when they developed rapidly progressive cognitive deterioration and myoclonic jerks, with the characteristic EEG changes of periodic complexes, elevated anti-measles serology, and positive CSF oligoclonal bands. The aim of this study was to ascertain diagnostic errors and their possible causes. Mills and Sternberg's Diagnostic Surgical Pathology: . Initially, the patient presents with mild cognitive deterioration characterized by poor . Subacute sclerosing panencephalitis (SSPE) is a chronic encephalitis of childhood and young adolescence due to persistant measles virus infection of the central nervous system. Acute Rheumatic Fever and Rheumatic Heart Disease is a concise, yet comprehensive, clinical resource highlighting must-know information on rheumatic heart disease and acute rheumatic fever from a global perspective.
A key resource for FRCPath and MRCP trainees, mapped to the current curriculum, using over 300 exam-style Q&A. This site needs JavaScript to work properly. Differential Diagnosis - Volume 341, Part 1994 - Page 796 1. Lack of clinical suspicion has led to late diagnosis. Subacute sclerosing panencephalitis.
It affects children and young adults. 2012;5(7):359-61. doi: 10.4066/AMJ.2012.1262. We remove all identifying information when posting a question to protect your privacy. . Crash Course Neurology Updated Edition - E-Book - Page 308 Found insideSubacute sclerosing panencephalitis inthe differential diagnosis of encephalitis. Neurology. 2004;63:1489â1493. 154. SingerC,LangAE, Suchowersky O.Adultonset subacutesclerosing panencephalitis: case reportsandreviewofthe literature. See EXANTHEMS—DIFFERENTIAL DIAGNOSIS (Appendix A). Epub 2010 Jun 15. Given the variable presentation of SSPE and the clinical findings it is associated with, there are many other differential diagnoses to think of. A multidisciplinary study of eight cases, Implication of sensorimotor integration in the generation of periodic dystonic myoclonus in subacute sclerosing panencephalitis (SSPE), Long-term follow-up of patients with subacute sclerosing panencephalitis treated with inosiplex, Serodiagnosis of subacute sclerosing panencephalitis by enzyme linked immunosorbent assay, Increased levels of beta 2-microglobulin, soluble interleukin-2 receptor, and soluble CD8 in patients with subacute sclerosing panencephalitis, Electroencephalographic and imaging profile in a subacute sclerosing panencephalitis (SSPE) cohort: a correlative study, Improvement of SSPE by intrathecal infusion of alpha-IFN, Atypical EEG findings in subacute sclerosing panencephalitis, Serial magnetic resonance imaging in subacute sclerosing panencephalitis, Long-term follow-up of a patient with subacute sclerosing panencephalitis successfully treated with intrathecal interferon alpha, MR imaging, diffusion-weighted imaging and MR spectroscopy findings in acute rapidly progressive subacute sclerosing panencephalitis, The variable natural history of subacute sclerosing panencephalitis: a study of 118 cases from the Middle East, Combined oral isoprinosine-intraventricular alpha-interferon therapy for subacute sclerosing panencephalitis, Intraventricular interferon and oral inosiplex in the treatment of subacute sclerosing panencephalitis, High-dose intravenous ribavirin therapy for subacute sclerosing panencephalitis, Retrospective evaluation of interferon-beta treatment in subacute sclerosing panencephalitis, The cooperative effect of interferon-alpha and ribavirin on subacute sclerosing panencephalitis (SSPE) virus infections, in vitro and in vivo, Combination therapy with intraventricular interferon-alpha and ribavirin for subacute sclerosing panencephalitis and monitoring measles virus RNA by quantitative PCR assay, [Subacute sclerosing panencephalitis: combined treatment with interferon alpha and intraventricular ribavirin], Inhibition of measles virus and subacute sclerosing panencephalitis virus by RNA interference, Rituximab in subacute sclerosing panencephalitis, Current developments in the management of subacute sclerosing panencephalitis, Carbamazepine and myoclonus in SSPE subacute sclerosing panencephalitis, Successful treatment of encephalopathy and myoclonus with levetiracetam in a case of subacute sclerosing panencephalitis, https://doi.org/10.1111/j.1469-8749.2010.03717.x, Personality changes, failure in school, strange behaviour, Massive, repetitive, and frequent myoclonic jerks, seizures and dementia, Rigidity, extrapyramidal symptoms, and progressive unresponsiveness, Coma, vegetative state, autonomic failure, and akinetic mutism, 1. Early in the disease, oedema appears to be the predominant finding.6 Evidence of DNA and ribonucleic acid oxidative damage has been found in infected cells during the first few years of the disease, with lipid peroxidation found in areas of early demyelination.34, During the acute inflammatory phase, nucleocapsids are found in oligodendrocytes, and neurons and nuclear bodies with granulofilamentous inclusions are found in astrocytes.5, 36 Cortical and subcortical perivascular infiltration of inflammatory cells, spongiosis, and demyelination are also reported in the acute phase, followed by neuronal loss as the disease evolves.5-7 The inflammatory changes initially tend to be more pronounced in the posterior areas of the brain, with marked involvement of the medial thalamus and deep structures followed by spread to anterior areas,7 and with relative sparing of the cerebellum.34, The age at presentation is usually 8 to 11 years,19, 37, 38 with onset usually occurring 6 years after measles infection.6, 19 Affected individuals present with poor school performance and progressive intellectual deterioration, personality changes, and behaviour abnormalities; this is followed by steady motor decline, myoclonus, focal paralysis, seizures, autonomic failure, and rigidity, finally leading to death with akinetic mutism.5, 16, 19, 38, 39 These changes are characterized in four stages (see Table I). 2018 Jun 26;9:67-71. doi: 10.2147/PHMT.S126293. Found inside â Page 367TERMINOLOGY Abbreviations ⢠Subacute sclerosing panencephalitis (SSPE) Synonyms ⢠Dawson encephalitis Definitions ... 367 Subacute Sclerosing Panencephalitis TERMINOLOGY IMAGING DIFFERENTIAL DIAGNOSIS PATHOLOGY CLINICAL ISSUES ... It is widely accepted that SSPE cannot occur in the absence of direct measles virus infection.13, 14, 18 Viruses recovered from individuals with SSPE are highly mutated, and no vaccine strains have been recovered from tissue specimens.6, 9, 19 These results argue against any etiological relationship between the measles vaccine and SSPE.
Neurology. 8600 Rockville Pike Research helps us better understand diseases and can lead to advances in diagnosis and treatment. Although SSPE generally occurs in childhood, it can rarely occur in adults and should not be dismissed if the patient is a pregnant woman.
A volume in the Handbook of Clinical Neurology series, which has an unparalleled reputation as the world's most comprehensive source of information in neurology International list of contributors including the leading workers in the field ... The authors point out that subacute sclerosing panencephalitis (SSPE) is rare in the US so many clinicians do not consider this diagnosis in some cases of acute encephalopathy in children. Early Brismar et al.
Subacute sclerosing panencephalitis (SSPE) is a progressive neurological disorder of childhood and early adolescence. ocular finding in SSPE. MEASLES Fen Hua Chen, M.D.,PhD. Found inside â Page 473See Subacute sclerosing panencephalitis (SSPE) Stance, in neurologic examination, 39 Status epilepticus, 196-197 conditions associated with, 196 described, 196 diagnosis, 196 differential diagnosis, 196 epidemiology of, 196 etiology, ... Subacute sclerosing panencephalitis: a preventable disease that needs to be prevented. Subacute sclerosis panencephalitis (SSPE) is a persistent and chronic encephalitis secondary to measles virus infection that causes widespread demyelination of the central nervous system (CNS).1 SSPE was described first by Dawson2 in 1934, in an individual with rapidly progressive encephalitis.
differential diagnosis of focal MRI lesions and of the Neuroimaging can be . 3. "Subacute sclerosing panencephalitis (SSPE) still occurs in many countries, particularly among people in overcrowded communities and those with insufficient measles vaccination coverage. The diagnosis is usually established based upon the characteristic clinical picture, abnormal EEG findings and elevated titer of IgG measles antibody in the CSF and serum. This antibody-mediated immunity is characterized by the production of Th2 lymphocytes and the release of predominantly IL-4, with lower levels of INFα and IL-2.22, 23. Subsequently, myoclonic seizures usually lead to a final stage of akinetic mutism. 2).46, 47 Their duration ranges from 1 to 3 seconds and the interval between complexes varies from 2 to 20 seconds, although in the early phases they can recur every 5 minutes.47 These periodic complexes can occur during sleep and, in the early stages, can be elicited by external stimuli.40, 48 It is thought that the periodic complex is secondary to widespread neuronal excitability, pathological hypersynchronization, and rhythmic triggering by a pacemaker, potentially in the brainstem or perithalamic area. SSPE is diagnosed based on the clinical assessment, EEG findings and elevated serum and CSF antimeasles antibody titers. The disease is so called because it typically develops over a period of less than 9 months (subacute),4 because of the nature of the pathological lesions (sclerosis), and from the fact that the whole brain is affected (i.e.
Emerg Infect Dis. Differential Diagnosis. Epilepsy A to Z: A Concise Encyclopedia - Page 326 Early diagnosis may help in medical interventions and counseling. 2013;112:1183-9. doi: 10.1016/B978-0-444-52910-7.00039-8. panencephalitis (SSPE) is extremely rare. A typical clinical picture: personality and behavioral changes, worsening school performance, followed by myoclonic seizures, paresis, dyspraxias, memory impairment, language difficulties . Subacute Sclerosing Panencephalitis in a Child with ...
Use the HPO ID to access more in-depth information about a symptom. They can also disappear with high body temperatures, lose their rhythmicity with variable intervals between them, or be preceded by bisynchronous occipital spikes. Get the latest public health information from CDC: https://www.coronavirus.gov (link is external) You can find more tips in our guide, How to Find a Disease Specialist. PDF r n a l of Psy o uJ Journal of Psychiatry Bhat et al., J ... Epub 2012 Jul 31. Subacute sclerosing panencephalitis: an update - GUTIERREZ ... Found inside â Page 169It is characterized by seizures and progressive deterioration of cognitive and motor functions, followed by death within 2 years.16 SSPE most TABLE 14-1 Differential Diagnosis of Measles Diagnosis Rubella Roseola Dengue Scarlet fever ... panencephalitis (SSPE) is extremely rare. Interferons are believed to increase IL-1 antagonism, enhance the cellular immune response, and activate natural killer lymphocytes.9, 49, 54 The adverse effects include flu-like symptoms and the production of INF antibodies that decrease biological functions, leading to clinical relapses.49 Longer-term INF administration seems to be better than short-term administration to achieve maximal clinical efficacy.55, Ribavirin is a nucleotide analogue that depletes the cell of nucleotides and blocks ribonucleic acid (RNA) polymerase.56 It has been used alone in high doses to treat SSPE with somewhat unconvincing results.54 Ribavirin in combination with INF has been found to produce better results than monotherapy. ), which may also affect immunocompetent individuals. The prognosis is good in uncomplicated cases. This section provides resources to help you learn about medical research and ways to get involved. Tropical Dermatology E-Book - Page 169 We read Honarmand et al.'s article1 with interest. Inclusion on this list is not an endorsement by GARD. Magnetic Resonance Imaging of the Brain and Spine Pediatric Neurology, Part II Diagnosis of SSPE is based on clinical manifestations, typical EEG, and laboratory findings . 2. Diagnosis of Subacute Panencephalitis: The Value and ... Clinical Presentation: Subacute sclerosing panencephalitis (SSPE) is a chronic neuroinfection caused by a mutant measles virus (1,3) that usually occurs in children. We want to hear from you. is updated regularly. Neurology. The 5-minute Neurology Consult - Page 473 Measles - Knowledge @ AMBOSS Psychiatric Manifestation of SSPE | The Journal of ... The MRI findings do not correspond to the clinical stages, and sometimes progress despite some clinical stabilization;6, 49 the changes mainly depend on disease duration. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. In the USA, the incidence in 1963 was 0.61 per million population, compared with the current rate of 4 to 5 cases per year (an incidence of approximately 0.01 per million).7 For comparison, current reported incidence rates elsewhere are 21 cases per million population in India,9 0.461 per million in Turkey,8 11 per million in Japan,9 and 0.06 per million in Canada.10 Overall, 4 to 11 cases of SSPE are expected for every 100 000 cases of measles, but the incidence is higher among children aged less than 5 years (18/100 000, compared with 1.1/100 000 after 5 years of age).9 The highest incidence of SSPE relative to the rate of measles is reported in the Middle East, where the rate is 360/100 000 in individuals infected before 1 year of age.11 The incidence varies dramatically depending on the age at which the measles infection is acquired and vaccination status.8, 11-13, The disease is more prevalent in males than in females;9, 14-16 in addition, the latency period tends to be longer, and onset of symptoms later in females.14 It has been suggested that the prevalence of SSPE is higher in Hispanics and Asians and the prevalence of the disease is lower in African-Americans; however, confounding factors such as socio-economic status make this assumption questionable.9 Risk factors associated with SSPE include younger age at measles onset, living in a rural area, poverty, overcrowding, low level of parental education, an older mother, living in a country with few cultural events, a higher number of siblings, and a higher birth order (i.e. Specials: molecular diagnostic test to identify measles virus mutated genome. PDF Basal ganglia lesions in subacute sclerosing panencephalitis Neuroimaging does not have an obligatory role in the diagnosis but gives important clues in the clinical assessment and differential diagnosis.
Infections of the Central Nervous System Ophthalmology - Page 792 Atypical Subacute Sclerosing Panencephalitis With Short ... In developed countries, subacute sclerosing panencephalitis (SSPE) is considered a rare disease. (PDF) MRI follow-up of basal ganglia involvement in ... Infections must be considered, including HIV-associated dementia, PML, SSPE, Whipples disease and prion diseases. One is particularly impressed by the wide acceptance on the part of medical students of the concept of the primary physician. Dr. John S. 8 measles - SlideShare March 21, 2016 - Case of the Week | American Journal of ... Enhancement of the volitional neuronal pathways is thought to be responsible for both the periodic complexes and the myoclonus.40. He presented with macular retinopathy, followed by progressive myoclonus and encephalopathy. By continuing to browse this site you are agreeing to our use of cookies. Children with SSPE had experienced natural infection with the rubeola virus at an early age, half . Found inside â Page 364... FORMULAS FOR EVALUATING BETWEEN-GROUP DIFFERENCES) DISCUSSION Taken globaly the single formulas do not appear to be able to give reliable and useful indications for the differential diagnosis between SSPE , Neurosyphilis and MS. For most diseases, symptoms will vary from person to person. If you have problems viewing PDF files, download the latest version of Adobe Reader, For language access assistance, contact the NCATS Public Information Officer, Genetic and Rare Diseases Information Center (GARD) - PO Box 8126, Gaithersburg, MD 20898-8126 - Toll-free: 1-888-205-2311. Found inside â Page 308125 Strabismus 67 Stroke aetiology 202 definition 201 differential diagnosis 202f haemorrhagic see Intracranial ... cord (SCDC) 165â166 Subacute-onset headache 50 causes 51f Subacute sclerosing panencephalitis (SSPE) 241 Subarachnoid ... The diagnosis of SSPE is often not considered by clinicians because of its rarity in the United States and the nonspecific clinical manifestations at onset. SSPE was not suspected in the differential diagnosis of three of the cases until results of measles testing were known. The diagnosis of SSPE is based on characteristic clinical and EEG findings and demonstration of elevated antibody titres against measles in cerebrospinal fluid. Living with a genetic or rare disease can impact the daily lives of patients and families. . A 29-year-old pregnant woman with worsening left hemiparesis, encephalopathy, and hemodynamic instability: a case report of subacute sclerosing panencephalitis. Trials have shown that, at best, 30 to 35% of individuals benefit from therapy, depending on the study design.37, 41 Benefit is defined as either slower progression, temporal stabilization of disease progression, prolonged survival, or, less likely, clinical improvement.4, 37 The benefit afforded, although relatively modest, is much better than the 5% spontaneous remission rate reported in the literature.41, 51 So far, the best results have been achieved with a combination of weekly intrathecal INF-α and daily oral isoprinosine, which was found to be of benefit in 35% of participants; however, this treatment was found to offer no clear advantage compared with isoprinosine monotherapy.37, 41, Despite this, the combination is still recommended because of theoretical synergistic effects. It affects children and young adults. As mentioned above, the best way to avoid SSPE is to vaccinate against measles. Try our interactive tool for help finding information, services, experts, financial aid, and more!
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