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Alexander disease

Alexander disease is a slowly progressing fatal neurodegenerative disease. more...

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Synonyms

  • Dysmyelogenic Leukodystrophy
  • Dysmyelogenic Leukodystrophy-Megalobare
  • Fibrinoid Degeneration of Astrocytes
  • Fibrinoid Leukodystrophy
  • Hyaline Panneuropathy
  • Leukodystrophy with Rosenthal Fibers
  • Megalencephaly with Hyaline Inclusion
  • Megalencephaly with Hyaline Panneuropathy

Clinical features

Delays in development of some physical, psychical and behavioral skills, progressive enlargement of the head (macrocephaly), seizures, spasticity, in some cases also hydrocephalus, dementia, clumsy movements.

Pathology

This genetically based condition, affecting the central nervous system (mid brain and cerebellum)is caused by mutations in the gene for glial fibrillary acidic protein (GFAP) that maps to chromosome 17q21. It is inherited in an autosomal dominant manner. Alexander disease belongs to leukodystrophies, a group of diseases which affect growth or development of the myelin sheath. The destruction of white matter in the brain is accompanied by the formation of fibrous, eosinophilic deposits known as Rosenthal fibers.

CT shows:

  • decreased density of white matter
  • frontal lobe predominance
  • +/- dilated lateral ventricles

Etiology

Unknown.

Occurrence and prevalence

Very rare, occurs mostly in males. The infantile form (80% of all cases) starts usually at the age of six months or within the first two years. The average duration of the infantile form of the illness is usually about 3 years. Onset of the juvenile form (14% of all cases) presents usually between four to ten years of age. Duration of this form is in most cases about 8 years. In younger patients, seizures, megalencephaly, developmental delay, and spasticity are usually present. Neonatal onset is also reported. Onset in adults is least frequent. In older patients, bulbar or pseudobulbar symptoms and spasticity predominate. Symptoms of the adult form may also resemble multiple sclerosis. There are no more than 300 cases reported.

Treatment

There is neither cure nor standard treatment for Alexander disease. All treatment is symptomatic and supportive, for example antibiotics for intercurrent infection and anticonvulsants for seizure control are usually used.

Prognosis

The prognosis is generally poor. With early onset, death usually occurs within 10 years after the onset of symptoms. Usually, the later the disease occurs, the slower its course is.

Read more at Wikipedia.org


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Landscape transformation and disease emergence
From Emerging Infectious Diseases, 5/1/05 by Polyxeni Potter

During his long ocean passage to Australia in 1852, Eugene von Guerard reported that conditions were far from ideal, and "meals were late and bad" (1). He had boarded a sailing ship at Gravesend, England, to seek his fortune in Victoria. At the gold fields of Ballarat, he described mining as "arduous at first" and causing "much backache and blistering of hands" (1). His gold mining efforts in Ballarat and environs were unsuccessful, and after a year, he abandoned the venture. Even so, he had struck gold. His illustrated diaries chronicled the history of the region, the harsh life of the gold digger, and the scarring of landscape from gold mining. He opened a studio in Melbourne, soon to become the most important Australian artist of his day.

The son of an artist and court painter, von Guerard was born in Vienna, Austria. He toured Italy with his father and lived in Rome for a while, where he became familiar with the work of famed French landscapists Claude Lorraine and Nicholas Poussin. Later, he studied landscape painting at the Dusseldorf Academy, where he was influenced by German romanticism--a movement that also dwelled on the visual aspects of nature. During his 30 years in Australia, he became a renowned landscape painter, as well as teacher and honorary curator at the National Gallery in Victoria. He died in London, where he had settled near the end of his life (2).

The 1850s gold rush that lured von Guerard to Victoria coincided with a revived interest in landscape painting, particularly in Australia and the United States (the Hudson River School). In the midst of 19th-century urbanization grew longing to connect with nature. Travelers sought areas of untouched wilderness, and artists labored to bring exotic freshness to the homebound. From his studio in Melbourne, von Guerard traveled to and explored many regions, among them timbered Illawarra and Tasmania, seeking the picturesque and generating drawings for his monumental landscape paintings (3).

At Illawarra, von Guerard was able to capture, in minute detail, the character of local flora: cabbage palms, ferns, fig trees, and multiple varieties of vines within the dark green tones of the dense Australian forest. Meticulous geographic, geologic, and ecosystem markers lend his artistic work historical importance as backdrop to subsequent transformation of the landscape by widespread mining and population growth.

In a letter to Melbourne newspaper The Argus in 1870, von Guerard explained that he painted "with the greatest desire to imitate nature" and sought to capture not only her details but also her "poetical feelings" (4). Descriptive and emotional, his elaborate artistic observations imbued the physical world with inner life quite apart from human society. Like many of his contemporaries (e.g., American painter Frederic Church), von Guerard was influenced by prominent German naturalist Alexander yon Humboldt (1769-1859), who advocated a "mutual reinforcement of art and science." In this context, topographic detail was acceptable in paintings only if motivated and sustained by emotional connection and personal relevance. Landscape painting was a way to express love of nature. And nature was constantly changing, driven by forces that shaped it throughout the eons (5).

Ferntree Gully in the Dandenong Ranges on this month's cover was hailed a masterpiece in its day. A lush mountain panorama, this painting excels in its faithful depiction of both the forest and the trees. Balanced and lyrical, the work is a romantic rendering of pure, un-adulterated nature. The scene is carefully structured: background fully outlined, center well-lit, and foreground intentionally shaded to frame and enhance view of the cloistered center. Each leaf is described in detail.

A broad path leads inward for a better look at the botanical life nearby as well as the treed horizon afar. A couple of lyrebirds walk the brush, their ancient silhouettes outlined against the grounds sheltering their fare of insects, myriapods, and snails. Curving fern tops and tree branches create a circular feeling as the eye moves from dark to light, from mountaintop to forest floor, from live greenery to fallen tree limbs and skeletal trunks, recounting a natural cycle of death and regeneration (6).

Von Guerard provided a respectful glimpse at unspoiled wilderness. His artistic eye scanned the exotic flora and through the bucolic stillness saw the real Arcadia, a goldmine of natural elements in constant change.

The semitropical rainforest idyll witnessed by von Guerard in Illawarra repeats itself in tropical and subtropical regions around the globe. Under a canopy of green, away from direct light, rain, and wind, moisture seeps down or hangs in mid-air, creating a fertile environment for propagation and growth. Along with tern spores, wildlife and microbial life are beneficiaries of the gullies' hothouse. Tiny creatures of the forest and blood-sucking insects, nature's fine detail, populate the underbrush-among them, mosquitoes, which feed on wild animals and thrive in this habitat.

As human development encroaches on the forest and urbanization transforms the native environment, mosquitoes become able to travel to all global destinations. These adaptable insects, some of them vectors of dengue viruses, have become anthropophilic, domesticated, and dangerous to humans. Environmental change affects nature's cycle, once more frustrating efforts to disrupt the persistent reemergence of tropical diseases like dengue (7).

References

(1.) Butters P. A painting miner--Eugene von Guerard. [cited 2005 Mar]. Available from http://www.ballarathistory.org/artsvong.html

(2.) Clark J, Whitelaw B. Golden summers: Heidelberg and beyond. Sydney: International Cultural Corporation of Australia; 1986.

(3.) The garden of New South Wales. [cited 2005 Mar]. Available from http://www.michaelorgan.org.au/illart1.htm

(4.) Eugene von Guerard. [cited 2005 Mar]. Available from http://www.ngv.vic.gov.au/collection/australian/painting/g/vonguerard_e/ education_kit.html

(5.) New worlds from old: 19th century Australian and American landscapes. [cited 2005 Mar]. Available from http://www.tfaoi.com/newsmu/nmus153a.htm

(6.) Thomas D. Australian art. In: The encyclopedia of visual art. Danbury (CT): Grolier Educational Corporation; 1983.

(7.) Calisher CH. Persistent reemergence of dengue. Emerg Infect Dis. 2005;11:738-9.

Address for correspondence: Polyxeni Potter, EID Journal, Centers for Disease Control and Prevention, 1600 Clifton Rd NE, Mailstop D61, Atlanta, GA 30333, USA; fax: 404-371-5449; email: PMP1@cdc.gov

* Centers for Disease Control and Prevention, Atlanta, Georgia, USA

COPYRIGHT 2005 U.S. National Center for Infectious Diseases
COPYRIGHT 2005 Gale Group

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