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suppressed adults (rarely after allogeneic hematopoietic cell transplantation) with impaired cell-mediated immunity, especially AIDS patients Symptoms reflect the diverse areas of CNS involvement (including aphasia, ataxia, cranial nerve deficits, hemiplegia, visual field disturbances), although frank seizures are reported in only about 18% In February 2005, natalizumab, a monoclonal antibody against integrins (the alpha 4 beta 1 integrin), with potential therapeutic roles in multiple sclerosis and inflammatory bowel disease, was withdrawn from the market after case reports of progressive multifocal leukoencephalopathy in patients receiving this medication The currently reported incidence is 1:1000 after 18 months of therapy The cause of all progressive multifocal leukoencephalopathy is reactivation of JC virus (JCV), a widespread papovavirus that initially infects children, leading to latent infection in the kidneys and lymphoid organs JCV targets myelinating oligodendrocytes of the CNS Genotyping of the virus shows that type 1 is more common among healthy individuals, type 2 among the immunocompromised PCR of the cerebrospinal fluid for JCV is used for diagnosis in patients with compatible clinical and radiologic findings Diffusion-weighted imaging on magnetic resonance scans increases the specificity of diagnosis HAART for HIV infection is effective when CD4 counts improve to > 100 cells/mcL in improving survival as well as the clinical and radiographic features associated with this disease, although a small number of cases appear to worsen with immune restoration Other agents used include cidofovir, not routinely recommended, and topotecan (a topoisomerase inhibitor), which appears to be of some benefit A single case report of response to risperidone (the JCV requires the serotonin 2A receptor for entry and risperidone is a serotonin-2A antagonist) needs further confirmation

Aksamit AJ Review of progressive multifocal leukoencephalopathy and natalizumab Neurologist 2006 Nov;12(6):293 8 [PMID: 17122725] Berenguer J et al; GESIDA 11/99 Study Group Clinical course and prognostic factors of progressive multifocal leukoencephalopathy in patients treated with highly active antiretroviral therapy Clin Infect Dis 2003 Apr 15;36(8):1047 52 [PMID: 12684918] Cinque P et al The effect of highly active antiretroviral therapyinduced immune reconstitution on development and outcome of progressive multifocal leukoencephalopathy: study of 43 cases with review of the literature J Neurovirol 2003;9 Suppl 1:73 80 [PMID: 12709876] Lima MA et al Seizures and their outcome in progressive multifocal leukoencephalopathy Neurology 2006 Jan 24;66 (2):262 4 [PMID: 16434670] Schaffer K et al JC virus in the Irish population: significant increase of genotype 2 in immunocompromised individuals J Neurovirol 2006 Feb;12(1):39 46 [PMID: 16595373] Yousry TA et al Evaluation of patients treated with natalizumab for progressive multifocal leukoencephalopathy N Engl J Med 2006 Mar 2;354(9):924 33 [PMID: 16510746]

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We perceive the world to be composed of things. Look at your computer. You do not see various bits of plastic and glass amorphously merging with the surrounding environment. You naturally and inevitably see distinct things: a computer, a keyboard, a monitor, speakers, pens, paper. Things. More importantly, even before you decide to do it, you ve categorized these things. You immediately classify the computer on your desk as a specific instance of a type of thing: this computer is one of type computer. This particular pen in your pocket is an instance of a more general type of thing, pens. It is so natural you can t avoid it, and yet the process is so subtle, it s difficult to articulate. When I see my dog Milo, I can t help also seeing him as a dog, not just as an individual entity.

female sex, codon heterozygosity of the prion protein (129), the presence in the cerebrospinal fluid of protein 14-3-3, and the 2a prion protein type Trials with antiprion antibodies and heterocyclic small molecule compounds are underway New vCJD was originally described in an outbreak from Great Britain that spread to involve several countries in Western Europe Three cases in the United States all reported former residence in either the United Kingdom or Saudi Arabia Compared with patients who have cCJD, patients with vCJD are younger, the duration of disease is longer, the clinical symptoms are unique (psychiatric and sensory symptoms and cerebellar signs are more common), and the MRI characteristically demonstrates hyperintensity of the posterior thalamus ( pulvinar sign ) All patients with vCJD have been homozygous for methionine at codon 129 Tonsillar biopsy can assist with diagnosis vCJD probably results from ingestion of beefsteak contaminated with neural tissue (brain, spinal cord) from livestock infected with bovine spongiform encephalopathy (BSE) ( mad cow disease ) Most cases of BSE to date are reported from Europe, particularly United Kingdom Over the past 12 years two cases of BSE occurred in Canada and two were recently reported from Texas and Washington State BSE is a product of livestock contaminated by ingesting material rendered from infected ruminants (deer, cattle, bison, sheep, and goats) Such feeding practices are now forbidden in many countries including the United States and Canada There is no animal-to-animal spread of BSE and milk and its derived products are not infected The risk for vCJD associated with the ingestion of beef products in the United States and Canada is deemed very small Nonetheless, the US Food and Drug Administration (FDA) is currently embarking on a set of restrictive measures that will prevent the use of potentially infected beef and other ruminant offal in food and cosmetic products Three cases of vCJD attributed to blood transfusion are now reported from the United Kingdom Patients with vCJD studied to date show a unique PrP phenotype.

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