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Topic: Encephalopathy basal ganglia calcification


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In the News (Sat 2 Jun 12)

  
  Neuroradiological features of six kindreds with MELAS tRNALeu A3243G point mutation: implications for pathogenesis -- ...
basal ganglia calcification, and cerebral and cerebellar atrophy.
Focal lesions in the parietal and occipital lobes in the presence of cerebral and cerebellar atrophy and basal ganglia calcification
Calcification of the basal ganglia: computerized tomography and clinical correlation.
jnnp.bmjjournals.com /cgi/content/full/65/2/233   (3820 words)

  
 Encyclopedia   (Site not responding. Last check: 2007-10-24)
Patients with renal disease are particularly prone to develop toxic complications of drugs normally excreted in the urine, as for example a peripheral neuropathy due to nitrofurantoin, labyrinthine damage due to streptomycin, or optic atrophy due to ethambutol.
Calcification in basal ganglia on skull radiograph or CT scan may be evident.
Rarely, basal ganglia calcification may be associated with extrapyramidal disorders.
www.eamg-med.com /members/encyclopedia/24/24_19.shtml   (5450 words)

  
 Early Diagnosis of Basal Ganglionic Germinoma by MRI   (Site not responding. Last check: 2007-10-24)
The first sign seen on CT scans has been reported to be slightly high density in the basal ganglia and/or mild asymmetry of the anterior horns of the lateral ventricles [7,8,14].
Therefore, early diagnosis of basal ganglia germinomas is of paramount importance.
CT and MR germinoma in the basal ganglia.
www.tsgh.ndmctsgh.edu.tw /radm/cv/juanCJ/Germinoma.htm   (1856 words)

  
 [No title]
(Leukodystrophy with calcification of the basal ganglia and lymphocytosis of cerebrospinal fluid): This is a severe, early onset encephalopathy with characteristic calcification in the basal nuclei and chronic lymphocytosis in the CSF.
The salient features are calcification of the basal ganglia and lymphocytosis of the CSF with elevated interferon-alpha level.
Imaging discloses calcification of the basal ganglia, possibly the thalamus and, occasionally, cerebellum on imaging.
moon.ouhsc.edu /kfung/JTY1/NeuroHelp/ZNE2IE03.htm   (244 words)

  
 [No title]
The syndrome is characterised by acquired microcephaly, basal ganglia calcifications, white matter abnormalities, chronic cerebrospinal fluid (CSF) lymphocytosis and raised interferon-alpha (INF-alpha) in the CSF.
AGS is diagnosed on the basis of a clinical picture characterised by microcephaly and by the onset of encephalopathy associated with severe psychomotor delay, spasticity and extrapyramidal signs.
The syndrome is characterised by acquired (or sometimes congenital) microcephaly, basal ganglia calcifications, white matter abnormalities, chronic cerebrospinal fluid (CSF) lymphocytosis and raised interferon-alpha (INF-alpha) in the CSF.
www.functionalneurology.it /materiale_cic/13_XVIII_2/57_aicardi-goutieres   (3078 words)

  
 [No title]   (Site not responding. Last check: 2007-10-24)
Fahr's syndrome is a rare neurological disorder characterized by abnormal deposits of calcium in certain of areas of the brain including the basal ganglia and the cerebral cortex.
Encephalopathy with calcifications of the basal ganglia in children.
Geschwind, D. H.; Loginov, M.; Stern, J. Identification of a locus on chromosome 14q for idiopathic basal ganglia calcification (Fahr disease).
www.noranclinic.com /mi_fahrs.htm   (345 words)

  
 LEARNING ABOUT AICARDI-GOUTIERES SYNDROME
Aicardi Goutières syndrome (AGS) is an early-onset (in the first year of life) progressive encephalopathy that shows an autosomal recessive pattern of inheritance and is characterised by acquired microcephaly, basal ganglia calcification, white matter alterations, chronic cerebrospinal fluid (CSF) lymphocytosis and raised levels of interferon-a in the CSF.
A distinction must be drawn between AGS and other early-onset pathologies in which there is basal ganglia calcification, in particular, the static encephalopathies caused by prenatal infections (cytomegalovirus, rubella, HIV, etc..), and other progressive disorders that have a metabolic and/or mitochondrial underlying cause.
It is only by establishing a clear distinction between the clinical picture of AGS and those of similar encephalopathies that the right approach to the problems of these children can be found, and accurate information given to parents on the risk of recurrence of the syndrome in subsequent pregnancies.
www.aicardi-goutieres.com /berichte/learning.htm   (1193 words)

  
 Chapter I   (Site not responding. Last check: 2007-10-24)
Wernick's encephalopathy can cause confusional state with ophthalmoplegia and ataxia (not a hemiparesis state).Keep in mind that metabolic causes of encephalopathy (like hypo or hyperglycemia, hepatic, uremicط·آ£ط¢آ¢ط£آ¢أ¢â‚¬ع‘ط¢آ¬ط·آ¢ط¢آ¦etc) can cause focal or multifocal neurological signs that are usually fluctuating or ALTERNATING between the right and left sides of the body.
Encephalopathy may begin to clear within 24 hours after the first vitamin B12 injection, but full neurological recovery, when it occurs, may take several months.
NB: hypertensive encephalopathy is a diagnosis of exclusion.
www.neurology4mrcp.50megs.com /about.html   (7722 words)

  
 AIDS: calcification of the basal ganglia in infants and children.
AIDS: calcification of the basal ganglia in infants and children.
CT or postmortem examination demonstrated calcification of the basal ganglia in eight infants and children with acquired immune deficiency syndrome.
Postmortem examination of four children revealed variable degrees of calcific vasopathy of the basal ganglia, involving predominantly the putamen and globus pallidus.
www.aegis.com /aidsline/1986/dec/M86C0198.html   (315 words)

  
 Medical Dictionary: Encephalopathy, familial infantile, with calcification of basal ganglia and chronic cerebrospinal ...
Dictionary » Encephalopathy, familial infantile, with calcification of basal ganglia and chronic cerebrospinal fluid lymphocytosis
Encephalopathy, familial infantile, with calcification of basal ganglia and chronic cerebrospinal fluid lymphocytosis is listed as a "rare disease" by the Office of Rare Diseases (ORD) of the National Institutes of Health (NIH).
This means that Encephalopathy, familial infantile, with calcification of basal ganglia and chronic cerebrospinal fluid lymphocytosis, or a subtype of Encephalopathy, familial infantile, with calcification of basal ganglia and chronic cerebrospinal fluid lymphocytosis, affects less than 200,000 people in the US population.
www.wrongdiagnosis.com /medical/encephalopathy_familial_infantile_with_calcification_of_basal_ganglia_and_chronic_cerebrospinal_fluid_lymphocytosis.htm   (254 words)

  
 Complex consanguinity associated with short rib-polydactyly syndrome III and congenital infection-like syndrome: a ...
Note the calcification in the periventricular white matter which can be distinguished from the bleeding, mainly on the left side (viewer's right).
The basal ganglia were not recognisable because of the haemorrhage
Aicardi J, Goutieres F. A progressive familial encephalopathy in infancy with calcifications of the basal ganglia and chronic cerebrospinal fluid lymphocytosis.
jmg.bmjjournals.com /cgi/content/full/36/6/461   (2780 words)

  
 PATHOLOGY part1
Spongiform encephalopathy is a special form of encephalitis that is quite topical because of mad cow disease in the United Kingdom.
Spongiform encephalopathy in humans was characterised in cannibals from the Niuginian highlands, who showed rapidly developing dementia and sponge-like vacuoles in the grey matter of their brains.
Pathologically, HIV encephalopathy is characterized by diffuse myelin damage (spongy myelinopathy, gliosis), neuron loss, vascular damage, microglial nodules, lymphocytic infiltrates and multinucleated giant cells.
members.fortunecity.com /danilhammoudimd/neuro1/id20.htm   (5833 words)

  
 Calcifying arteriopathy in the basal ganglia in human immunodeficiency virus infection.
Calcifying arteriopathy in the basal ganglia in human immunodeficiency virus infection.
Echogenic stripes in the basal ganglia were identified sonographically in a 4-month-old infant with human immunodeficiency virus (HIV) infection.
At 3 years and 5 months, CT examination performed after three seizures showed calcification in the area of the sonographically affected arteries but no signs of progressive encephalopathy.
www.aegis.com /aidsline/1995/sep/M9590874.html   (326 words)

  
 A3243G - Abstract - Neuroradiological features of six kindreds with MELAS tRNA(Leu) A2343G point mutation: implications ...
Basal ganglia calcification was progressive, symmetric, and asymptomatic.
Histologically, basal ganglia calcification in one patient was found to be in the pericapillary regions of the globus pallidus, with no neuronal involvement.
CONCLUSIONS: These radiological findings, when considered in the context of the clinical and pathological findings, seem to reflect two major disease processes: an intermittent abrupt loss of function associated with cell injury from which there is at least partial recovery and a slowly progressive degenerative process causing basal ganglia calcification, and cerebral and cerebellar atrophy.
www.a3243g.com /abstract_pm_db_view.asp?ID=9703178   (324 words)

  
 AIDS: calcification of the basal ganglia in infants and children -- Belman et al. 36 (9): 1192 -- Neurology
AIDS: calcification of the basal ganglia in infants and children -- Belman et al.
CT or postmortem examination demonstrated calcification of the basal
calcific vasopathy of the basal ganglia, involving predominantly the
www.neurology.org /cgi/content/abstract/36/9/1192   (252 words)

  
 A3243G - Abstract - Heteroplasmic mitochondrial DNA mutation in a patient with mitochondrial myopathy, encephalopathy, ...
A3243G - Abstract - Heteroplasmic mitochondrial DNA mutation in a patient with mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes.
Heteroplasmic mitochondrial DNA mutation in a patient with mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes.
Starting from the age of 14 years, she experienced recurrent episodes of headaches, with vomiting, seizures, transient right hemiparesis and decreased visual acuity.
www.a3243g.com /abstract_pm_db_view.asp?ID=7613232   (220 words)

  
 CNS INFECTIONS, SPONGIFORM ENCEPHALOPATHY AND DEMYELINATING DISEASES   (Site not responding. Last check: 2007-10-24)
Calcification can be detected in some neonates infected in utero with encephalitis-producing agents like CMV and HIV viruses.
The pathology due to different viruses is similar, although the severity and area affected (cortex or basal ganglia) varies.
Human transmissible spongiform encephalopathies (including Creutzfeldt-Jakob (C-J) disease and kuru) have been transmitted to primates and to other animals through cell-free injections of infected brain tissue.
ampat.amu.edu.pl /guzyuno/CNS_INFE.HTM   (3184 words)

  
 Indian Pediatrics - Editorial
HIV encephalopathy is especially common in HIV-infected children who present in early infancy and have rapid downhill course(3).
The risk of HIV encephalopathy is also correlated directly with the severity of HIV-related symptoms, depression of CD4+ counts and p24 antigen levels in the mother(3).
The cumulative incidence of HIV-1-related encephalopathy at 7 years post- infection was noted to be 16% in children and the incidence of encephalopathy was 9.9% in the first year of life, 4.2% in the second year and less than 1% per year thereafter(4).
www.indianpediatrics.net /mar2003/mar-230-234.htm   (1736 words)

  
 Cerebellar Vermian Atrophy after Neonatal Hypoxic-Ischemic Encephalopathy -- Sargent et al. 25 (6): 1008 -- American ...
and the cortex and basal ganglia were assessed for atrophy,
lobes, the thalami, basal ganglia, hippocampi, cerebellar hemispheres,
Neonatal asphyxia: vulnerability of basal ganglia, thalamus, and brainstem.
www.ajnr.org /cgi/content/full/25/6/1008   (4103 words)

  
 THE MERCK MANUAL, Sec. 2, Ch. 12, Water, Electrolyte, Mineral, And Acid-Base Metabolism
Associated abnormalities include short stature, round facies, mental retardation with calcification of the basal ganglia, shortened metacarpal and metatarsal bones, as well as mild hypothyroidism and other subtle endocrine abnormalities.
The presence of hypoparathyroidism is often suggested by the clinical manifestations of the underlying condition (eg, cataracts, basal ganglia calcification, and chronic candidiasis in idiopathic hypoparathyroidism).
Slowly developing, insidious hypocalcemia may produce mild, diffuse encephalopathy and should be suspected in any patient with unexplained dementia, depression, or psychosis.
www.merck.com /pubs/mmanual/section2/chapter12/12d.htm   (7071 words)

  
 Aicardi-Goutieres syndrome   (Site not responding. Last check: 2007-10-24)
Aicardi-Goutières syndrome (AGS) is an autosomal recessive progressive encephalopathy associated with basal ganglia calcification, myelin abnormality, cerebro-spinal fluid (CSF) lymphocytosis and elevated CSF interferon (IFN)-alpha.
Transgenic mice with chronic overexpression of IFN-alpha in astrocytes develop a phenotype similar to AGS with calcifications of the basal ganglia and leukodystrophy, supporting the hypothesis that abnormal secretion of IFN-alpha may be crucial in the syndrome pathogenesis.
The syndrome displays genetic heterogeneity, and linkage analysis showed that 40-50% of AGS families map to the locus AGS1 on chromosome 3p21.
www.orpha.net /static/GB/aicardigoutieres_syndrome.html   (222 words)

  
 MedPix™ 3638: Hypoxic/ischemic encephalopathy - Medical Image Database and Atlas
Hypoxic encephalopathy results from a global hypoperfusion or oxygenation defect rather than from infarction in a specific vascular territory.
The basal ganglia and the borders of the ACA, PCA, and MCA distributions are therefore more likely to be injured.
Most often, cerebral edema with sparing of the cerebellum is followed by calcific cortical necrosis and atrophic changes.
rad.usuhs.mil /medpix/medpix.html?mode=single&comebackto=mode=geo_browse&recnum=3638   (425 words)

  
 Rapid Brain Calcification after Ischemic Stroke -- Wityk et al. 119 (6): 490 -- Annals of Internal Medicine
and the calcification involved both white and gray matter.
Brain computed tomographic scan showing extensive calcification of the right frontal lobe in the anterior and middle cerebral artery territories.
Midroni G, Willinsky R. Rapid postanaoxic calcification of the basal ganglia.
www.annals.org /cgi/content/full/119/6/490   (771 words)

  
 Brain Case Report 1
Predominant locations of lymphoma deposits include the deeper parts of the frontal and parietal lobes, basal ganglia, corpus callosum, paraventricular region, and thalamus.
Hemorrhage, calcification, necrosis, and cyst formation are uncommon imaging findings for primary CNS lymphoma.
Greater than half of patients with HIV present with a supratentorial parenchymal mass with frequent involvement of the corpus callosum, basal ganglia, and other deep cerebral nuclei.
www.med.nyu.edu /mri/brain/case01.html   (1099 words)

  
 Aicardi-Goutieres Syndrome - United Leukodystrophy Foundation
Intracerebral calcification: presence of calcium deposits on a particular area of the brain
You may also hear Aicardi-Goutieres Syndrome called familial infantile encephalopathy, with calcification of basal ganglia and chronic cerebrospinal fluid lymphocytosis; this is simply a more medically descriptive name for the disease.
Two other diseases (Cree encephalopathy and microcephaly, intracranial calcification syndrome (MICS)) share many common features with Aicardi-Goutieres, and therefore may be related.
www.ulf.org /types/Aicardi.html   (610 words)

  
 2. Clinical Manifestations (Pediatric HIV Infection)
Progressive HIV encephalopathy, which may include developmental delay or regression, spastic weakness of the extremities, microcephaly, seizures and, by computerized tomography, cerebral atrophy and basal ganglia calcification, occurs less commonly.
It is hypothesized that activation of these cells by HIV can result in overproduction of certain cytokines, arachidonic acid metabolites and quinolinic acid, which, in turn, may produce some of the neuropathological changes observed.
Elevated serum concentrations of tumor necrosis factor (TNF) have been associated with progressive encephalopathy in children with HIV infection, and TNF can produce white matter destruction similar to that observed in children with progressive encephalopathy.
bayloraids.org /resources/pedaids/manifestations.shtml   (1442 words)

  
 eMedicine - MELAS Syndrome : Article Excerpt by: Fernando Scaglia, MD
Background: Mitochondrial myopathy, encephalopathy, lactacidosis, stroke (MELAS) is a progressive neurodegenerative disorder.
Other abnormalities that may be observed are ventricular dilatation, cortical atrophy, and basal ganglia calcification.
The encephalopathy may progress to dementia, and eventually the patient may become quite cachectic and die.
www.emedicine.com /ped/byname/melas-syndrome.htm   (571 words)

  
 Basal ganglia infarction associated with HHV-6 infection -- Webb et al. 76 (4): 362 -- Archives of Disease in Childhood
Basal ganglia infarction associated with HHV-6 infection -- Webb et al.
The right putamen is small (arrowhead) and shows focal hyperintensity which could be due to methaemoglobin or early calcification.
Gloning H, Stettner-Gloning R, Pontz BF, Emmrich P. [Exanthum subitum, encephalopathy, and hepatitis caused by human herpes virus type 6 in a 10 month old infant].
adc.bmjjournals.com /cgi/content/full/76/4/362   (1012 words)

  
 Cree encephalitis is allelic with Aicardi-Goutieres syndrome: implications for the pathogenesis of disorders of ...
encephalopathy characterised by calcification of the basal ganglia,
Cerebral calcification in a patient with systemic lupus erythematosus and a monoclonal IgG reactive with glial fibrillary acidic protein.
Systemic lupus erythematosus causing cerebral infarction and basal ganglia calcification: a case report.
jmg.bmjjournals.com /cgi/content/full/40/3/183   (2226 words)

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