Laboratory Features Elevation of sarcoplasmic enzymes in serum (creatine phosphokinase, aldolase, transaminases and lactic dehydrogenase) is valuable both for diagnosis and for following the clinic...

Polymyositis and dermatomyositis (first of two parts)

Forty years after effective vaccines were licensed, measles continues to cause death and severe disease in children worldwide. Complications from measles can occur in almost every organ system. Pneumonia, croup, and encephalitis are common causes of death; encephalitis is the most common cause of long-term sequelae. Measles remains a common cause of blindness in developing countries. Complication rates are higher in those 20 years old, although croup and otitis media are more common in those <2 years old and encephalitis in older children and adults. Complication rates are increased by immune deficiency disorders, malnutrition, vitamin A deficiency, intense exposures to measles, and lack of previous measles vaccination. Case-fatality rates have decreased with improvements in socioeconomic status in many countries but remain high in developing countries.

/pdf/the-clinical-significance-of-measles-a-review-3pnyq7ma1j.pdf

The Clinical Significance of Measles: A Review

This report is a compendium of all current recommendations for the prevention of measles, rubella, congenital rubella syndrome (CRS), and mumps The report presents the recent revisions adopted by the Advisory Committee on Immunization Practices (ACIP) on October 24, 2012, and also summarizes all existing ACIP recommendations that have been published previously during 1998-2011 (CDC Measles, mumps, and rubella--vaccine use and strategies for elimination of measles, rubella, and congenital rubella syndrome and control of mumps: recommendations of the Advisory Committee on Immunization Practices [ACIP] MMWR 1998;47[No RR-8]; CDC Revised ACIP recommendation for avoiding pregnancy after receiving a rubellacontaining vaccine MMWR 2001;50:1117; CDC Updated recommendations of the Advisory Committee on Immunization Practices [ACIP] for the control and elimination of mumps MMWR 2006;55:629-30; and, CDC Immunization of healthcare personnel: recommendations of the Advisory Committee on Immunization Practices (ACIP) MMWR 2011;60[No RR-7]) Currently, ACIP recommends 2 doses of MMR vaccine routinely for children with the first dose administered at age 12 through 15 months and the second dose administered at age 4 through 6 years before school entry Two doses are recommended for adults at high risk for exposure and transmission (eg, students attending colleges or other post-high school educational institutions, healthcare personnel, and international travelers) and 1 dose for other adults aged ≥18 years For prevention of rubella, 1 dose of MMR vaccine is recommended for persons aged ≥12 months At the October 24, 2012 meeting, ACIP adopted the following revisions, which are published here for the first time These included: • For acceptable evidence of immunity, removing documentation of physician diagnosed disease as an acceptable criterion for evidence of immunity for measles and mumps, and including laboratory confirmation of disease as a criterion for acceptable evidence of immunity for measles, rubella, and mumps • For persons with human immunodeficiency virus (HIV) infection, expanding recommendations for vaccination to all persons aged ≥12 months with HIV infection who do not have evidence of current severe immunosuppression; recommending revaccination of persons with perinatal HIV infection who were vaccinated before establishment of effective antiretroviral therapy (ART) with 2 appropriately spaced doses of MMR vaccine once effective ART has been established; and changing the recommended timing of the 2 doses of MMR vaccine for HIV-infected persons to age 12 through 15 months and 4 through 6 years • For measles postexposure prophylaxis, expanding recommendations for use of immune globulin administered intramuscularly (IGIM) to include infants aged birth to 6 months exposed to measles; increasing the recommended dose of IGIM for immunocompetent persons; and recommending use of immune globulin administered intravenously (IGIV) for severely immunocompromised persons and pregnant women without evidence of measles immunity who are exposed to measles As a compendium of all current recommendations for the prevention of measles, rubella, congenital rubella syndrome (CRS), and mumps, the information in this report is intended for use by clinicians as baseline guidance for scheduling of vaccinations for these conditions and considerations regarding vaccination of special populations ACIP recommendations are reviewed periodically and are revised as indicated when new information becomes available

Prevention of measles, rubella, congenital rubella syndrome, and mumps, 2013: summary recommendations of the Advisory Committee on Immunization Practices (ACIP).

Subacute sclerosing panencephalitis (SSPE) is a progressive neurological disorder of childhood and early adolescence. It is caused by persistent defective measles virus. Brain biopsies or postmortem histopathological examination show evidence of astrogliosis, neuronal loss, degeneration of dendrites, demyelination, neurofibrillary tangles, and infiltration of inflammatory cells. Patients usually have behavioral changes, myoclonus, dementia, visual disturbances, and pyramidal and extrapyramidal signs. The disease has a gradual progressive course leading to death within 1-3 years. The diagnosis is based upon characteristic clinical manifestations, the presence of characteristic periodic EEG discharges, and demonstration of raised antibody titre against measles in the plasma and cerebrospinal fluid. Treatment for SSPE is still undetermined. A combination of oral isoprinosine (Inosiplex) and intraventricular interferon alfa appears to be the best effective treatment. Patients responding to treatment need to receive it life long. Effective immunisation against measles is the only solution presently available to the problem of this dreaded disease.

https://pmj.bmj.com/content/postgradmedj/78/916/63.full.pdf

Subacute sclerosing panencephalitis

Stroke has enormous clinical, social, and economic implications, and demands a significant effort from both basic and clinical science in the search for successful therapies. Atherosclerosis, the pathologic process underlying most coronary artery disease and the majority of ischemic stroke in humans, is an inflammatory process. Complex interactions occur between the classic risk factors for atherosclerosis and its clinical consequences. These interactions appear to involve inflammatory mechanisms both in the periphery and in the CNS. Central nervous system inflammation is important in the pathophysiologic processes occurring after the onset of cerebral ischemia in ischemic stroke, subarachnoid hemorrhage, and head injury. In addition, inflammation in the CNS or in the periphery may be a risk factor for the initial development of cerebral ischemia. Peripheral infection and inflammatory processes are likely to be important in this respect. Thus, it appears that inflammation may be important both before, in predisposing to a stroke, and afterwards, where it is important in the mechanisms of cerebral injury and repair. Inflammation is mediated by both molecular components, including cytokines, and cellular components, such as leukocytes and microglia, many of which possess pro- and/or antiinflammatory properties, with harmful or beneficial effects. Classic acute-phase reactants and body temperature are also modified in stroke, and may be useful in the prediction of events, outcome, and as therapeutic targets. New imaging techniques are important clinically because they facilitate dynamic evaluation of tissue damage in relation to outcome. Inflammatory conditions such as giant cell arteritis and systemic lupus erythematosus predispose to stroke, as do a range of acute and chronic infections, principally respiratory. Diverse mechanisms have been proposed to account for inflammation and infection-associated stroke, ranging from classic risk factors to disturbances of the immune and coagulation systems. Considerable opportunities therefore exist for the development of novel therapies. It seems likely that drugs currently used in the treatment of stroke, such as aspirin, statins, and modulators of the renin-angiotensin-aldosterone system, act at least partly via antiinflammatory mechanisms. Newer approaches have included antimicrobial and antileukocyte strategies. One of the most promising avenues may be the use of cytokine antagonism, for example, interleukin-1 receptor antagonist.

Inflammation and Infection in Clinical Stroke

Clinical, electroencephalographic, and other laboratory features and subacute sclerosing panencephalitis (SSPE) were studied in eight patients by the same group of investigators. The relatively high frequency of the disease within a small geographical area in a short period of time suggests that SSPE may be unusually common in the vicinity of Memphis. The delineation of four clinical stages of the disease, markedly elevated measles antibody titers in serum and cerebrospinal fluid, ultrastructural observation of intranuclear and intracytoplasmic inclusions in neurons and neuroglia in three patients, and alteration of serum and CSF immunoglobulins indicate that the measles myxovirus may be the etiological agent in these patients.

Subacute sclerosing panencephalitis. A multidisciplinary study of eight cases

• Twelve cases of large- and mediumsized cerebral artery stenosis and/or occlusion associated with bacterial meningitis occurred. Neurological complication due to arterial involvement developed in seven patients on the third and fourth days of illness; in one patient, it developed on the fifth day, and in another it developed on the 14th day. In three cases, this could not be determined. Arterial stenosis is considered primarily to result from arterial spasm due to humoral factors that may be elaborated within the CSF or arterial wall, as in the cases of ruptured aneurysm; and secondarily, from to inflammatory involvement of major vessels at the base of the brain and from irritation by angiographic contrast material.

Cerebral arteritis and bacterial meningitis.

In this report of the Subacute Sclerosing Panencephalitis (SSPE) Registry during 1960 through 1970, the findings indicate a 50% relationship of SSPE to early (under 2 years of age) measles infection. The frequency of SSPE is one per million, and a majority of the patients reside in the southeastern part of the United States.

Epidemiology of subacute sclerosing panencephalitis (SSPE). A report of the SSPE registry.

Epidemiology of subacute sclerosing panencephalitis

Fifty-two persons with subacute sclerosing panencephalitis (SSPE) were compared with playmate and hospital controls matched for age, sex, and race. Persons with SSPE were more likely to have had measles than their age-matched controls. The age at measles infection for children with SSPE was significantly younger than that for controls who had had measles. Persons with SSPE were less likely to have received measles vaccine than were playmate or hospital controls. There were no differences with regard to the average age at vaccination, having received more than one measles vaccination, or having received measles vaccine after natural measles. Although measles vaccine may rarely predispose a child to develop SSPE, the overall impact of vaccination has been to prevent SSPE by preventing natural measles. No significant differences were observed between cases and controls for infections other than measles, or for vaccines other than measles vaccine. Previous epidemiologic studies have noted significant geographic clustering of SSPE and higher rates in children living in rural areas. These findings suggest that environmental factors other than measles are important in the pathogenesis of SSPE. In this study, children with SSPE were more likely to have suffered a serious head injury and to have come from larger families and more crowded homes than control children. Persons with SSPE were significantly more likely to have close exposure to birds (p less than 0.001) and to swine (p less than 0.05) than were control persons. No differences between cases and controls were found for exposure to other animals. These data suggest that some infectious agent(s), transmitted from birds to man, may have contributed to the development of SSPE in predisposed individuals. A variety of other factors were investigated and found not to correlate with SSPE. These included birth weight, breastfeeding, maternal age at birth, nutritional status, source of drinking water, development, and allergic or atopic disorders.

J. T. Jabbour

Papers

Subacute sclerosing panencephalitis. A multidisciplinary study of eight cases

Cerebral arteritis and bacterial meningitis.

Epidemiology of subacute sclerosing panencephalitis (SSPE). A report of the SSPE registry.

Epidemiology of subacute sclerosing panencephalitis

Risk factors in subacute sclerosing panencephalitis: a case-control study