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Topic: Epilepsy mental deterioration Finnish type

	E-epilepsy - Molecular genetics of the epilepsies
		A second useful distinction is between the 'symptomatic' epilepsies, in which recurrent seizures are one component of a multifaceted neurological phenotype, and the 'idiopathic' epilepsies, in which recurrent seizures occur in individuals who are otherwise neurologically and cognitively intact and who have no detectable anatomical or metabolic abnormality.
		Epilepsy forms part of the phenotype of a number of Mendelian diseases including tuberous sclerosis (TS), fragile X syndrome (FRAX), neurofibromatosis (NF1), and an array of metabolic disorders, all of which are individually rare.
		Progressive myoclonic epilepsy of the Unverricht-Lundborg type (ULD, locus symbol, EPM1) is an autosomal recessive disorder which is enriched in the Finnish population with an incidence of one in 20,000 births.
	www.e-epilepsy.org.uk /pages/articles/show_article.cfm?id=44 (4260 words)

	Mental retardation in Finland - Finnish Information Center on Mental Retardation
		It is a form of childhood epilepsy occurring in northern Finland.
		Finnish Disease Heritage I: characteristics, causes, background, Norio R, Hum Genet.
		Finnish Disease Heritage II: population prehistory and genetic roots of Finns, Norio R, Hum Genet.
	www.saunalahti.fi /kup/engl/finmr.htm (1517 words)

	Rare Pediatric Disease Database
		Batten Disease, or neuronal ceroid lipofuscinosis (NCL), is a rare condition characterized by progressive mental deterioration (dementia), epilepsy and vision loss.
		The disorder is most common in Finland, where 1% of the population is thought to be carriers of the defective gene.
		There are four types of NCL that tend to appear at different ages and have different degrees of severity.
	www.madisonsfoundation.org /content/3/1/display.asp?did=423 (1181 words)

	ORPHANET - About rare diseases - About orphan drugs
		Epilepsy-mental deterioration, Finnish type, is a very rare form of childhood epilepsy occurring in northern Finland.
		The patients are normal at birth and develop normally until school age.
		Mental development, which is originally normal, begins to deteriorate 2 to 5 years after the onset of epilepsy, and the deterioration continues during adulthood in spite of good epilepsy control, leading to mental retardation by middle age.
	www.orpha.net /consor/cgi-bin/OC_Exp.php?Lng=GB&Expert=1947 (160 words)

	eMedicine - Infantile Spasm (West Syndrome) : Article by Tracy A Glauser, MD (Site not responding. Last check: 2007-09-01)
		This severe epilepsy syndrome is an age-dependent expression of a damaged brain.
		Mixed spasms are the most common type, consisting of flexion of the neck and arms with extension of the legs, or flexion of the legs with extension of the arms.
		Mental retardation is severe in 70% of patients, often with psychiatric problems such as autistic features or hyperactivity.
	www.emedicine.com /NEURO/topic171.htm (5576 words)

	BDSRA - Batten Disease Support and Research Association
		In 1931, the Swedish psychiatrist and geneticist, Torben Sjogren, presented 115 cases with extensive clinical and genetic documentation and came to the conclusion that the disease which we now call the Spielmeyer-Sjogren (juvenile) type is genetically separate from Tay Sachs.
		There are four main types of NCL, including two forms that begin earlier in childhood and a very rare form that strikes adults.
		The typical early signs are loss of muscle coordination (ataxia) and seizures along with progressive mental deterioration..
	www.bdsra.org /batten.htm (2175 words)

	Santavuori Disease
		They may then begin to exhibit a delay in the acquisition of skills that require the coordination of mental and muscular activity (psychomotor retardation).
		Affected infants may then experience a variety of symptoms including episodes of uncontrolled electrical disturbances in the brain (seizures), impaired ability to coordinate voluntary movements (cerebellar ataxia), abnormally diminished muscle tone (hypotonia), and repeated, brief, shock-like muscle spasms of the arms, legs, or entire body (myoclonic seizures).
		Affected infants also experience progressive visual impairment due to deterioration of the nerves of the eyes (optic nerves) that transmit impulses from the nerve-rich membranes lining the eyes (retina) to the brain (optic atrophy).
	www.peacehealth.org /kbase/nord/nord1090.htm (595 words)

	Health Library -
		In most cases, infants with Santavuori Disease appear to develop normally until approximately nine to 19 months of age.
		In addition, affected infants begin to lose previously acquired physical and mental abilities (developmental regression).
		Neurological impairment continues to progress and may be characterized by an inability to move voluntarily (immobility); sudden involuntary muscle spasms (spasticity); and lack of response to stimuli in the environment.
	www.memorialhermann.org /library/healthguide/en-us/illnessconditions/topic.asp?hwid=nord1090 (607 words)

	ScienceDaily: 'Brick Wall' Helps Explain How Corrosion Spreads Through Alloy
		They've developed a statistical model of the deterioration and simulated it on computer, using what may seem like an unlikely analogy: a cracking brick wall.
		What they've found could one day help scientists better understand this kind of corrosion, and also explain corrosion in other types of alloys.
		Although the alloy, called 2024-T3, is strong and resistant to corrosion in general, it is vulnerable to intergranular corrosion -- when tiny pits on the surface grow into crack-like fissures that snake down into a part, weakening the structure, explained Gerald Frankel, professor of materials science and engineering at Ohio State.
	www.sciencedaily.com /releases/2004/11/041123205611.htm (2219 words)

	Ataxia: Dominant
		DRPLA: Myoclonus and Epilepsy (Onset < 20 years); Choreoathetosis, Dementia, Psychosis (Onset > 20 years); OPCA
		Type I: Earliest onset (5 to 30 yo)
		Type II: Intermediate onset (36 yo) and clinical features
	www.neuro.wustl.edu /neuromuscular/ataxia/domatax.html (2319 words)

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