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generalized seizures, widespread myoclonus, ataxia, and sometimes visual disturbances due to progressive chorioretinitis As the disease advances, rigidity, hyperactive re exes, Babinski signs, progressive unresponsiveness, and signs of autonomic dysfunction appear In the nal stage the child lies insensate, virtually decorticated The course is usually steadily progressive, death occurring within 1 to 3 years In about 10 percent of cases the course is more prolonged, with one or more remissions In a similar number the course is fulminant, leading to death within months of onset Exceptionally, the disease occurs in young adults In two reported cases in pregnant women, blurred vision and weakness of limbs developed before they became akinetic and mute, without a trace of myoclonus or cerebellar ataxia Nevertheless, the progressive ataxic-myoclonic chronic dementia in a child is so typical that bedside diagnosis is usually possible The EEG shows a characteristic abnormality consisting of periodic (every 5 to 8 s) bursts of 2- to 3-per-second high-voltage waves, followed by a relatively at pattern The CSF contains few or no cells, but the protein is increased, particularly the gamma globulin fraction, and agarose gel electrophoresis discloses oligoclonal bands of IgG These have been shown to represent measles virus speci c antibody (Mehta et al) Both the serum and CSF contain high concentrations of neutralizing antibody to measles (rubeola) virus, but the virus has been recovered from the brain tissue only with dif culty The MRI changes begin in the subcortical white matter and spread to the periventricular region (Anlar et al) Histologically the lesions involve the cerebral cortex and white matter of both hemispheres and the brainstem The cerebellum is usually spared Destruction of nerve cells, neuronophagia, and perivenous cuf ng by lymphocytes and mononuclear cells indicate the viral nature of the infection In the white matter there is degeneration of medullated bers (myelin and axis cylinders), accompanied by perivascular cuf ng with mononuclear cells and brous gliosis (hence the term sclerosing encephalitis) Eosinophilic inclusions, the histopathologic hallmark of the disease, are found in the cytoplasm and nuclei of neurons and glial cells Virions, thought to be measles nucleocapsids, have been observed in the inclusion-bearing cells examined electron microscopically How a ubiquitous and transient viral infection in a seemingly normal young child allows the development, many years later, of a rare encephalitis is a matter of speculation Sever believes that there is a delay in the development of immune responses during the initial infection and a later inadequate immune responses that are incapable of clearing the suppressed infection An alternative hypothesis is that certain brain cells fail to synthesize a so-called M protein, which is essential for the assembly of the viral membrane, and that this limitation of host-cell capability is related to the extent of viral seeding of the brain during the initial infection (Hall et al) The differential diagnosis includes the childhood and adolescent dementing diseases such as lipid storage diseases (Chap 37) and Schilder demyelinative disease (page 782) In presumptive clinical cases of SSPE, the ndings of periodic complexes in the EEG, elevated gamma globulin and oligoclonal bands in the CSF, and elevated measles antibody titers in the serum and CSF are suf cient to make the diagnosis No effective treatment is available The administration of amantadine and inosiplex was found by some investigators to lead to improvement and prolonged survival but others could not corroborate these effects The therapeutic value of intrathecal administration of alpha interferon is being investigated.

The boot process described previously is known as normal mode: it is how Windows 9x will boot when things are running normally However, in some cases, you might want to alter the boot process so that some or most of the normal steps are skipped For example, if Windows 9x will not load properly, you can use an alternate boot method to at least get to a command prompt so that you can determine and troubleshoot the cause of the problem There are several boot modes for Windows 9x To select an alternate boot mode, press F8 at startup when you see the message Starting Windows On some Windows 98 systems, you might be required to press CTRL rather than F8 The two most commonly used alternate boot modes, Safe mode and MS-DOS mode, are described in the subsections that follow

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