The condition primarily affects children, teens, and young adults. It has been estimated that about 1 in 10,000 people who get measles will eventually develop sspe. Subacute sclerosing panencephalitis sspe is a slowly progressive brain disorder caused by mutant measles virus. The first signs are usually behavioral changes such as failing schoolwork, memory loss, andor irritability. Subacute sclerosing panencephalitis resembling rasmussens. Subacute sclerosing panencephalitis sspe is a rare degen. Variable clinical presentations may lead to diagnostic dilemma and unnecessary investigations especially in. Brain biopsies or postmortem histopathological examination show evidence of astrogliosis, neuronal loss, degeneration of dendrites, demyelination, neurofibrillary tangles, and infiltration of inflammatory cells. Subacute sclerosing panencephalitis sspe is a progressive inflammatory disease of the central nervous system with poor prognosis and high mortality, caused by the persistent infection with measles virus mv.
In total, 92 mr examinations were performed in 44 patientsand67ctexaminationswereperformedin42 patients. As with other complications of measles, the risk of death is highest among young children and adults. The disease has been related to a persistent and aberrant measles virus infection and no. Measles infection and encephalitis the encephalitis society.
Subacute sclerosing panencephalitis sspe is a rare progressive neuroinfectious disease due to a late complication of the measles virus. Subacute sclerosing panencephalitis postgraduate medical. Diagnosis involves electroencephalography, ct or mri, cerebrospinal fluid examination, and measles serologic testing. Sspe is a degenerative neurological condition which progressively destroys. Early and latestate subacute sclerosing panencephalitis. Subacute sclerosing panencephalitis information page. Intrathecal interferon in subacute sclerosing panencephalitis. Diagnosis involves electroencephalography, ct or mri, cerebrospinal fluid examination, and. Subacute sclerosing panencephalitis sspealso known as dawson diseaseis a rare form of chronic progressive brain inflammation caused by slow infection with certain defective strains of hypermutated measles virus. The interictal eeg can be useful for the diagnosis in the antinmdar encephalitis.
See the major article by wendorf et al on pages 22632. Subacute sclerosing panencephalitis in an americanborn. Pdf subacute sclerosing panencephalitis researchgate. Immunology and vaccinepreventable diseases pink book.
Motor incoordination and impairment of speech and sight subsequently develop. Six patients with subacute sclerosing panencephalitis were treated with the antiviral agent inosiplex. The prevalence of the disease varies depending on uptake of measles vaccination, with the virus disproportionally affecting regions with low vaccination rates. Subacute sclerosing panencephalitis sspe generally develops approximately seven to ten years after a person recovers from the measles. We present a case of a fully vaccinated 3yearold boy who was diagnosed with and treated for autoimmune encephalitis before arriving at a diagnosis of sspe. Subacute sclerosing panencephalitis genetic and rare. Subacute sclerosing panencephalitis pathology britannica. Subacute sclerosing panencephalitis sspe is a chronic encephalitis occurring after infection with measles virus. The hallmark clinical features of this disease include. Five patients with clinically advanced subacute sclerosing panencephalitis sspe were given human leukocyte interferon ifn by the lumbar route, 1 million iu every other day for a total of 30 days. Subacute sclerosing panencephalitis sspe is a rare, predominantly childhoodonset neurologic illness that probably results from a latent or mutant measles rubella virus infection of neurons. Subacute sclerosing panencephalitis, is a progressive fatal neuroinfection caused by persistent mutated measles virus. Clinical and magnetic resonance imaging evaluation of 36 patients. Subacute sclerosing panencephalitis sspe is a preventable condition reported in 6.
Subacute sclerosing panencephalitis clinical infectious. Subacute sclerosing panencephalitis sspe is a progressive inflammatory disorder of the central nervous system with both poor prognosis and high mortality. Pdf subacute sclerosing panencephalitis sspe in iraq. Subacute sclerosing panencephalitis sspe is a progressive neurodegenerative disease with a high morbidity and mortality which is caused by measles virus 1. Measles virus and subacute sclerosing panencephalitis. Subacute sclerosing panencephalitis presenting as acute. Subacute sclerosing panencephalitis sspe, also known as dawson disease, is a rare chronic, progressive and fatal encephalitis that affects primarily children and young adults, caused by a persistent infection of immune resistant measles virus. This is a rare condition that can develop some years after natural measles infection. Subacute sclerosing panencephalitis sspe is a slowly progressive inflammatory disease of the central nervous system caused by a persistent measles virus usually affecting the childhood and. Differential diagnosis an overview sciencedirect topics. There are wide varieties of presentations of sspe described in the literatures. Subacute sclerosing panencephalitis sspe is a progressive and fatal central nervous system disorder that results from a persistent sspe virus infection. The most common causes of death are pneumonia in children and acute encephalitis in adults. Magnetic resonance mr imaging is the modality of choice for determining such changes in white matter.
Subacute sclerosing panencephalitis sspe is a chronic progressive encephalitis of childhood and young adults due to persistent measles virus infection. Subacute sclerosing panencephalitis sspe is a fatal complication of measles infection. Subacute sclerosing panencephalitis is an extremely rare but extremely serious and potentially fatal disorder of the brain which is basically caused by an abnormal reaction of the immune system of the body to the measles virus. Subacute sclerosing panencephalitis sspe is a progressive neurological disorder of children and young adults that affects the central nervous system cns. The final stages of stupor, dementia, blindness, and death occur within six to nine months. Subacute sclerosing panencephalitis radiology reference. Subacute sclerosing panencephalitis sspe is a brain disorder.
Who subacute sclerosing panencephalitis and measles. Subacute sclerosing panencephalitis is a progressive neurological disorder of children and young adults caused by a measles virus that became defective by persisting in the host. Subacute sclerosing panencephalitis sspe is a progressive neurological disorder of childhood and early adolescence. Subacute sclerosing panencephalitis sspe pediatrics. Methods fiftytwo patients with sspe were examined, 44 with mr imaging and 42 with ct of the brain on one or more occasions. A measles outbreak that occurred in the united states in 19891991, however, placed a new generation at risk for sspe. Early signs and symptoms of the condition can include behavioral changes and mild mental deterioration affecting. Subacute sclerosing panencephalitis is a progressive, usually fatal brain disorder occurring months to usually years after an attack of measles. Our aim was to determine the metabolic abnormalities of brain in early and latestage sspe by using mr spectroscopy and to assess areas of involvement in the early stages when mr imaging findings were normal. It is a slow, but persistent, viral infection caused by defective measles virus. Intrathecal ifn produced a meningeal inflammatory reaction in all patients and was associated with transient hemiparesis in 1. Subacute sclerosing panencephalitis sspe is a rare, progressive, inflammatory neurodegenerative disease. Purpose to evaluate the progression of ct and mr changes of the brain in subacute sclerosing panencephalitis sspe as a basis for assessing the effects of different types of therapy.
Subacute sclerosing panencephalitis sspe is so rare in developed countries with measles immunization programs that national active surveillance is now needed to capture sufficient number of cases for meaningful analysis of data. Measles is also the cause of a disease called subacute sclerosing panencephalitis sspe. Know the causes, symptoms, treatment and prognosis of subacute sclerosing panencephalitis. The deliberations were considerably helped by a commissioned report presented by experts from the health protection agency hpa of the united kingdom. Subacute sclerosing panencephalitis is a rare neurological disease of childhood or young adulthood. We discuss the challenges of diagnosing sspe in developed countries. The clinical condition of four children continued to deteriorate in spite of the treatment, the condition of one remained unchanged, and that of another improved minimally. It causes mental deterioration, myoclonic jerks, and seizures. Subacute sclerosing panencephalitis and chorioretinitis.
Because of widespread measles immunization, subacute sclerosing panencephalitis sspe is now an exceptionally rare disease in the united states. It is caused by persistent defective measles virus. Subacute sclerosing panencephalitis definition is a usually fatal neurological disease of children and young adults caused by infection of the brain by a previously latent measles virus that is marked especially by behavioral changes, myoclonic seizures, progressive deterioration of motor and mental functioning, and coma. Long term survival in subacute sclerosing panencephalitis. Subacute sclerosing panencephalitis sspe is a rare inflammatory and neurodegenerative disease caused by persistent cerebral infection with the measles virus occurring in a delayed manner after acute measles infection garg, 2008. Its incidence rates in developed countries have been reported as 711 cases of sspe100,000 cases of measles. Pharmacokinetics and effects of ribavirin following. Sspe virus strains are measles viruses in which the viral. Subacute sclerosing panencephalitis sspe is a progressive neurologic disorder caused by measles virus that silently develops for several years after the primary measles virus infection and is inevitably fatal. Subacute sclerosing panencephalitis sspe is a rare, slowly progressing but invariably fatal disease that is related to a prior measles virus infection and most commonly affects paediatric patients.
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