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Rasmussen's encephalitis

Rasmussen's encephalitis is a rare, progressive neurological disorder, characterized by frequent and severe seizures, loss of motor skills and speech, hemiparesis (paralysis on one side of the body), encephalitis (inflammation of the brain), dementia, and mental deterioration. more...

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The disorder, which affects a single cerebral hemisphere, generally occurs in children under the age of 10.


When seizures have not spontaneously remitted by the time hemiplegia and aphasia are complete, the standard treatment for Rasmussen's encephalitis is surgery to remove or disconnect the affected part of the brain (hemispherectomy). Although anti-epileptic drugs may be prescribed initially, they are usually not effective in controlling the seizures. Alternative treatments may include plasmapheresis (the removal and reinfusion of blood plasma), ketogenic diet (high fat, low carbohydrate), and steroids.


Prognosis for individuals with Rasmussen's encephalitis varies. Untreated, the disorder may lead to severe neurological deficits including mental retardation and paralysis. In some patients surgery decreases seizures. However, most patients are left with some paralysis and speech deficits.


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Annual Review of Neuroscience, Vol. 22, 1999
From American Journal of Pharmaceutical Education, 10/1/99 by Hine, Bromfield

W MAXWELL COWAN, ERIC M. SHOOTER, CHARLES E STEVENS AND RICHARD E THOMPSON. Annual Review of Neuroscience, Vol. 22,1999. Palo Alto CA: Annual Reviews, Inc., 1999. ix + 652 pp., 71 figs., 7 tbls., $60.00.

No field encompasses a broader range of information than neuroscience, and this diversity certainly is reflected in this year's Annual Review topics. Twenty-one articles are included, ranging from those focusing on molecular mechanisms or techniques, to a treatise on common mechanisms and themes underlying birdsong and human speech.

The variety of topics defies easy classification, except to say that this volume emphasizes various aspects of developmental (five articles) and systems (receptor, autonomic; sensory-motor) neuroscience (six articles). Other papers focus on anatomical structures or features (bloodbrain barrier cell biology; role of microglia in pathology; stress and hippocampal plasticity), degenerative processes (Parkinson's disease; inflammation, autoimmune processes in Myasthenia gravis, LambertEaton syndrome, neuromyotonia, and Rasmussen's encephalitis) and techniques (fluorescent membrane dyes; computational neuroimaging). The content of some of these articles is summarized in order to give a flavor of the variety of information provided in the reviews.

With respect to developmental/systems neuroscience, various receptors and signal transduction factors influencing and guiding initial connections between neurons, via growth cones, is explicitly detailed by B.K. Mueller, with the anticipation that such information will contribute to the understanding of basic developmental processes. Development of visual processing is discussed with respect to how coordinated activity in the immature retina (waves) helps determine connection patterns prior to sensory stimulation (R. Wong) and new information about molecular processes governing scanning eye movements and their cortical regulation (J.D. Schall and K.G. Thompson). Cellular and molecular events controlling identified stages of sympathetic nervous system development is the subject of a review by N.J. Francis and S.C. Landis, emphasizing the role of neurotrophic factors and the interaction between sympathetic cholinergic neurons and effectors (sweat glands). L.L. Rubin and J.M. Staddon provide a detailed molecular analysis of the blood-brain-barrier, emphasizing recent findings on properties of junctions between adjacent capillary endothelial cells and how intracellular signalling systems control the passage of molecules across these junctions. B.S. McEwen reviews how steroids and excitatory amino acids mediate both plasticity and the destructive effects of stress on the hippocampus. E. Jones provides a historical perspective, detailed in its description of the development and use of neuroanatomical tracing techniques over a 40-year period, of the contributions of Thomas Powell in elucidating the synaptic circuitry and organization of the vertebrate forebrain. The review on developmental similarities in birdsong and human speech provides tantalizing suggestions that aspects of the fonner may provide valuable insights into mechanisms relevant to the latter. The role of innate predispositions, critical developmental periods, vocal learning, the importance of forebrain structures, and the role of auditory feedback and experience in lateralization of peripheral structures, as these factors influence central processing, are discussed with respect to similarities (many) and differences between species. This treatment provides a context for fi-aming hypotheses about the critical factors in human speech development.

As interesting and informative as these topics are, a smaller number of reviews may be perceived as more relevant to pharmaceutical scientists, especially basic and clinical pharmacologists and toxicologists. For example, A. MacDermott et al. provide a detailed tutorial on mechanisms of presynaptic regulation of neurotransmitter release and discuss the molecular biology and pharmacology of nicotinic cholinergic, GABA, glutamate and other candidate receptors controlling presynaptic ion channels. Two reviews discuss different aspects of Parkinson's disease (PD). C.W Olanow and WG. Tatton present a review of what is known about genetic and environmental factors contributing to PD etiology and discuss mechanisms likely to contribute to pathology, including oxidative stress and antioxidant deficits, mitochondrial dysfunction, excitotoxicity, immune modulators from glial cells and neuronal apoptosis. Neuroprotective strategies relevant to these mechanisms are proposed. J.C. Shih et al. discuss the progress that has been made in determining the structure and finction of MAO-A and MAO-13 genes and how this information is related to the participation of these two enzyme isoforms in PD, drug abuse, aggression and stress-related disorders. Future research directions are emphasized based on recent studies using MAO-A and MAO-B knockout mice. E Gonzalez-Scarano and G. Baltuch review dual actions of microglia in the CNS (amplifying the effects of inflammation and mediating cellular degeneration) that could contribute to pathology, The authors discuss microglia as sources of potential neurotoxic factors, and they review the events that can stimulate this activity within the context of three diseases: HIV infection, multiple sclerosis and Alzheimer's disease.

This Annual Review issue contains articles that are of greatest interest to developmental neuroscientists and biologists, receptor and CNS pharmacologists and clinicians working in the area of (or having an interest in) cellular and molecular approaches to neurodegenerative and immune-mediated diseases. But beyond this specificity, the treatment of the topics covered in this issue illustrate how the increasing wealth of detail provided by these approaches are relevant to therapeutic breakthroughs and success. Individuals with the ability to integrate such information with existing data and to extract its relevance to normal and pathophysiological processes will be essential to realize the promise of the knowledge that is gained.

Bromfield Hine

University of Puerto Rico

Copyright American Association of Colleges of Pharmacy Fall 1999
Provided by ProQuest Information and Learning Company. All rights Reserved

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