FDG-PET assessment and metabolic patterns in Lafora disease.

AIM
To describe the pattern of brain glucose metabolism assessed by 18F-fluorodeoxyglucose positron emission tomography (FDG-PET) in LaFora disease (LD), a rare, deadly form of progressive myoclonus epilepsy is caused by biallelic mutations in EPM2A or NHLRC1.


We retrospectively included patients with confirmed LD genetic underwent FDG-PET scan called three Italian epilepsy center. FDG-PET images were evaluated both visually and using SPM12 software. subgroup analysis done on the basis of genetic and clinical features using SPM. In addition, we perform a systematic literature review of the DA case patients underwent FDG-PET assessment.

Eight Italy (3M / 5F, 3 EPM2A / 5 NHLRC1) underwent FDG-PET examination after an average of 6 years from the onset of illness (range 1- 12 year). All patients showed bilateral hypometabolic area, more widespread and pronounced in advanced disease stages. Most often, the area hypometabolic is temporal (8/8), parietal (7/8), and the frontal lobe (7/8), and thalamus (6/8). In three cases, FDG-PET was repeated after an average of 17 months (range 7-36 months) showed metabolic deteriorated compared with the initial assessment. SPM subgroup analysis found no significant difference based on genetics, but showed a more significant temporoparietal hypometabolism in patients with visual symptoms compared with those who did not.

In nine additional cases were identified from eight publications, FDG-PET showed a heterogeneous findings, ranging from diffuse decrease in brain glucose metabolism for mediocre examination in two cases.FDG-PET appears to be very sensitive to evaluate LD at each stage and can be correlated with the development of disease. Area glucose metabolism decreased in LD broad, often involving several cortical and subcortical regions, the thalamus, temporal, frontal, and parietal lobes of the most severely affected. Longitudinal prospective collaborative study is needed to validate our findings.

 FDG-PET assessment and metabolic patterns in Lafora disease.
FDG-PET assessment and metabolic patterns in Lafora disease.

Glia-Derived Reactive Neuroinflammation: Novel Hallmark in LaFora Progressive myoclonus epilepsy That Progress with the Age.

LaFora disease (LD) is a rare form, fatal progressive myoclonus epilepsy. The molecular basis of this devastating disease is still poorly understood, and no treatment is available yet, which leads to the death of a patient about 10 years from the onset of the first symptoms. The hallmark of LD is the accumulation of glycogen as late inclusion in the brain and peripheral tissues, as a consequence of altered glycogen homeostasis.

In addition, other determining factors in the pathophysiology of LD have been proposed, such as proteostasis disorders, with reduced autophagy, and oxidative stress, among others. In order to get a general idea of ​​the genes involved in the pathophysiology of LD, in this work, we have performed RNA-Seq transcriptome analysis of tissue around the brain of two mice models independent of the disease, namely Epm2a – / – and Epm2b – / – mice, the different time ages.

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Description: A sandwich ELISA for quantitative measurement of Goat (IgM) in samples from blood, plasma, serum, cell culture supernatant and other biological fluids. This is a high quality ELISA kit developped for optimal performance with samples from the particular species.

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Description: A sandwich ELISA for quantitative measurement of Human (IgM) in samples from blood, plasma, serum, cell culture supernatant and other biological fluids. This is a high quality ELISA kit developped for optimal performance with samples from the particular species.

Human (IgM) ELISA kit

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  • Kit contents: 1. MICROTITER PLATE * 1 2. ENZYME CONJUGATE*1 vial 3. STANDARD A*1 vial 4. STANDARD B*1 vial 5. STANDARD C*1 vial 6. STANDARD D*1 vial 7. STANDARD E*1 vial 8. STANDARD F*1 vial 9. SUBSTRATE A*1 vial 10. SUBSTRATE B*1 vial 11. STOP SOLUT
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Description: A sandwich ELISA for quantitative measurement of Human (IgM) in samples from blood, plasma, serum, cell culture supernatant and other biological fluids. This is a high quality ELISA kit developped for optimal performance with samples from the particular species.

Human (IgM) ELISA kit

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  • Kit contents: 1. MICROTITER PLATE * 1 2. ENZYME CONJUGATE*1 vial 3. STANDARD A*1 vial 4. STANDARD B*1 vial 5. STANDARD C*1 vial 6. STANDARD D*1 vial 7. STANDARD E*1 vial 8. STANDARD F*1 vial 9. SUBSTRATE A*1 vial 10. SUBSTRATE B*1 vial 11. STOP SOLUT
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Description: A sandwich ELISA for quantitative measurement of Human (IgM) in samples from blood, plasma, serum, cell culture supernatant and other biological fluids. This is a high quality ELISA kit developped for optimal performance with samples from the particular species.

Mouse (IgM) ELISA kit

E03A1876-192T 192 tests
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Description: A sandwich ELISA for quantitative measurement of Mouse (IgM) in samples from blood, plasma, serum, cell culture supernatant and other biological fluids. This is a high quality ELISA kit developped for optimal performance with samples from the particular species.

Mouse (IgM) ELISA kit

E03A1876-48 1 plate of 48 wells
EUR 520.00
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Description: A sandwich ELISA for quantitative measurement of Mouse (IgM) in samples from blood, plasma, serum, cell culture supernatant and other biological fluids. This is a high quality ELISA kit developped for optimal performance with samples from the particular species.

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E03A1876-96 1 plate of 96 wells
EUR 685.00
  • Kit contents: 1. MICROTITER PLATE * 1 2. ENZYME CONJUGATE*1 vial 3. STANDARD A*1 vial 4. STANDARD B*1 vial 5. STANDARD C*1 vial 6. STANDARD D*1 vial 7. STANDARD E*1 vial 8. STANDARD F*1 vial 9. SUBSTRATE A*1 vial 10. SUBSTRATE B*1 vial 11. STOP SOLUT
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Description: A sandwich ELISA for quantitative measurement of Mouse (IgM) in samples from blood, plasma, serum, cell culture supernatant and other biological fluids. This is a high quality ELISA kit developped for optimal performance with samples from the particular species.

Rabbit (IgM) ELISA kit

E04A1876-192T 192 tests
EUR 1270.00
  • Kit contents: 1. MICROTITER PLATE * 1 2. ENZYME CONJUGATE*1 vial 3. STANDARD A*1 vial 4. STANDARD B*1 vial 5. STANDARD C*1 vial 6. STANDARD D*1 vial 7. STANDARD E*1 vial 8. STANDARD F*1 vial 9. SUBSTRATE A*1 vial 10. SUBSTRATE B*1 vial 11. STOP SOLUT
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Description: A sandwich ELISA for quantitative measurement of Rabbit (IgM) in samples from blood, plasma, serum, cell culture supernatant and other biological fluids. This is a high quality ELISA kit developped for optimal performance with samples from the particular species.

Rabbit (IgM) ELISA kit

E04A1876-48 1 plate of 48 wells
EUR 520.00
  • Kit contents: 1. MICROTITER PLATE * 1 2. ENZYME CONJUGATE*1 vial 3. STANDARD A*1 vial 4. STANDARD B*1 vial 5. STANDARD C*1 vial 6. STANDARD D*1 vial 7. STANDARD E*1 vial 8. STANDARD F*1 vial 9. SUBSTRATE A*1 vial 10. SUBSTRATE B*1 vial 11. STOP SOLUT
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Description: A sandwich ELISA for quantitative measurement of Rabbit (IgM) in samples from blood, plasma, serum, cell culture supernatant and other biological fluids. This is a high quality ELISA kit developped for optimal performance with samples from the particular species.

Rabbit (IgM) ELISA kit

E04A1876-96 1 plate of 96 wells
EUR 685.00
  • Kit contents: 1. MICROTITER PLATE * 1 2. ENZYME CONJUGATE*1 vial 3. STANDARD A*1 vial 4. STANDARD B*1 vial 5. STANDARD C*1 vial 6. STANDARD D*1 vial 7. STANDARD E*1 vial 8. STANDARD F*1 vial 9. SUBSTRATE A*1 vial 10. SUBSTRATE B*1 vial 11. STOP SOLUT
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Description: A sandwich ELISA for quantitative measurement of Rabbit (IgM) in samples from blood, plasma, serum, cell culture supernatant and other biological fluids. This is a high quality ELISA kit developped for optimal performance with samples from the particular species.

Rat (IgM) ELISA kit

E02A1876-192T 192 tests
EUR 1270.00
  • Kit contents: 1. MICROTITER PLATE * 1 2. ENZYME CONJUGATE*1 vial 3. STANDARD A*1 vial 4. STANDARD B*1 vial 5. STANDARD C*1 vial 6. STANDARD D*1 vial 7. STANDARD E*1 vial 8. STANDARD F*1 vial 9. SUBSTRATE A*1 vial 10. SUBSTRATE B*1 vial 11. STOP SOLUT
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Description: A sandwich ELISA for quantitative measurement of Rat (IgM) in samples from blood, plasma, serum, cell culture supernatant and other biological fluids. This is a high quality ELISA kit developped for optimal performance with samples from the particular species.

Rat (IgM) ELISA kit

E02A1876-48 1 plate of 48 wells
EUR 520.00
  • Kit contents: 1. MICROTITER PLATE * 1 2. ENZYME CONJUGATE*1 vial 3. STANDARD A*1 vial 4. STANDARD B*1 vial 5. STANDARD C*1 vial 6. STANDARD D*1 vial 7. STANDARD E*1 vial 8. STANDARD F*1 vial 9. SUBSTRATE A*1 vial 10. SUBSTRATE B*1 vial 11. STOP SOLUT
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Description: A sandwich ELISA for quantitative measurement of Rat (IgM) in samples from blood, plasma, serum, cell culture supernatant and other biological fluids. This is a high quality ELISA kit developped for optimal performance with samples from the particular species.

Rat (IgM) ELISA kit

E02A1876-96 1 plate of 96 wells
EUR 685.00
  • Kit contents: 1. MICROTITER PLATE * 1 2. ENZYME CONJUGATE*1 vial 3. STANDARD A*1 vial 4. STANDARD B*1 vial 5. STANDARD C*1 vial 6. STANDARD D*1 vial 7. STANDARD E*1 vial 8. STANDARD F*1 vial 9. SUBSTRATE A*1 vial 10. SUBSTRATE B*1 vial 11. STOP SOLUT
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Description: A sandwich ELISA for quantitative measurement of Rat (IgM) in samples from blood, plasma, serum, cell culture supernatant and other biological fluids. This is a high quality ELISA kit developped for optimal performance with samples from the particular species.

Pig (IgM) ELISA kit

E07A1876-192T 192 tests
EUR 1270.00
  • Kit contents: 1. MICROTITER PLATE * 1 2. ENZYME CONJUGATE*1 vial 3. STANDARD A*1 vial 4. STANDARD B*1 vial 5. STANDARD C*1 vial 6. STANDARD D*1 vial 7. STANDARD E*1 vial 8. STANDARD F*1 vial 9. SUBSTRATE A*1 vial 10. SUBSTRATE B*1 vial 11. STOP SOLUT
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Description: A sandwich ELISA for quantitative measurement of Porcine (IgM) in samples from blood, plasma, serum, cell culture supernatant and other biological fluids. This is a high quality ELISA kit developped for optimal performance with samples from the particular species.

Pig (IgM) ELISA kit

E07A1876-48 1 plate of 48 wells
EUR 520.00
  • Kit contents: 1. MICROTITER PLATE * 1 2. ENZYME CONJUGATE*1 vial 3. STANDARD A*1 vial 4. STANDARD B*1 vial 5. STANDARD C*1 vial 6. STANDARD D*1 vial 7. STANDARD E*1 vial 8. STANDARD F*1 vial 9. SUBSTRATE A*1 vial 10. SUBSTRATE B*1 vial 11. STOP SOLUT
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Description: A sandwich ELISA for quantitative measurement of Porcine (IgM) in samples from blood, plasma, serum, cell culture supernatant and other biological fluids. This is a high quality ELISA kit developped for optimal performance with samples from the particular species.

Pig (IgM) ELISA kit

E07A1876-96 1 plate of 96 wells
EUR 685.00
  • Kit contents: 1. MICROTITER PLATE * 1 2. ENZYME CONJUGATE*1 vial 3. STANDARD A*1 vial 4. STANDARD B*1 vial 5. STANDARD C*1 vial 6. STANDARD D*1 vial 7. STANDARD E*1 vial 8. STANDARD F*1 vial 9. SUBSTRATE A*1 vial 10. SUBSTRATE B*1 vial 11. STOP SOLUT
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Description: A sandwich ELISA for quantitative measurement of Porcine (IgM) in samples from blood, plasma, serum, cell culture supernatant and other biological fluids. This is a high quality ELISA kit developped for optimal performance with samples from the particular species.

Monkey (IgM) ELISA kit

E09A1876-192T 192 tests
EUR 1270.00
  • Kit contents: 1. MICROTITER PLATE * 1 2. ENZYME CONJUGATE*1 vial 3. STANDARD A*1 vial 4. STANDARD B*1 vial 5. STANDARD C*1 vial 6. STANDARD D*1 vial 7. STANDARD E*1 vial 8. STANDARD F*1 vial 9. SUBSTRATE A*1 vial 10. SUBSTRATE B*1 vial 11. STOP SOLUT
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Description: A sandwich ELISA for quantitative measurement of Monkey (IgM) in samples from blood, plasma, serum, cell culture supernatant and other biological fluids. This is a high quality ELISA kit developped for optimal performance with samples from the particular species.

Monkey (IgM) ELISA kit

E09A1876-48 1 plate of 48 wells
EUR 520.00
  • Kit contents: 1. MICROTITER PLATE * 1 2. ENZYME CONJUGATE*1 vial 3. STANDARD A*1 vial 4. STANDARD B*1 vial 5. STANDARD C*1 vial 6. STANDARD D*1 vial 7. STANDARD E*1 vial 8. STANDARD F*1 vial 9. SUBSTRATE A*1 vial 10. SUBSTRATE B*1 vial 11. STOP SOLUT
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Description: A sandwich ELISA for quantitative measurement of Monkey (IgM) in samples from blood, plasma, serum, cell culture supernatant and other biological fluids. This is a high quality ELISA kit developped for optimal performance with samples from the particular species.

Monkey (IgM) ELISA kit

E09A1876-96 1 plate of 96 wells
EUR 685.00
  • Kit contents: 1. MICROTITER PLATE * 1 2. ENZYME CONJUGATE*1 vial 3. STANDARD A*1 vial 4. STANDARD B*1 vial 5. STANDARD C*1 vial 6. STANDARD D*1 vial 7. STANDARD E*1 vial 8. STANDARD F*1 vial 9. SUBSTRATE A*1 vial 10. SUBSTRATE B*1 vial 11. STOP SOLUT
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Description: A sandwich ELISA for quantitative measurement of Monkey (IgM) in samples from blood, plasma, serum, cell culture supernatant and other biological fluids. This is a high quality ELISA kit developped for optimal performance with samples from the particular species.

Human IgM ELISA Kit

EHI0045 96Tests
EUR 521.00

Human IgM ELISA Kit

EHI0104 96Tests
EUR 521.00

Human IgM ELISA Kit

ELA-E0543h 96 Tests
EUR 824.00

Goat IgM ELISA Kit

EGTI0045 96Tests
EUR 521.00

Goat IgM ELISA Kit

EGTI0104 96Tests
EUR 521.00

Dog (IgM) ELISA kit

E08A1876-192T 192 tests
EUR 1270.00
  • Kit contents: 1. MICROTITER PLATE * 1 2. ENZYME CONJUGATE*1 vial 3. STANDARD A*1 vial 4. STANDARD B*1 vial 5. STANDARD C*1 vial 6. STANDARD D*1 vial 7. STANDARD E*1 vial 8. STANDARD F*1 vial 9. SUBSTRATE A*1 vial 10. SUBSTRATE B*1 vial 11. STOP SOLUT
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Description: A sandwich ELISA for quantitative measurement of Canine (IgM) in samples from blood, plasma, serum, cell culture supernatant and other biological fluids. This is a high quality ELISA kit developped for optimal performance with samples from the particular species.

Dog (IgM) ELISA kit

E08A1876-48 1 plate of 48 wells
EUR 520.00
  • Kit contents: 1. MICROTITER PLATE * 1 2. ENZYME CONJUGATE*1 vial 3. STANDARD A*1 vial 4. STANDARD B*1 vial 5. STANDARD C*1 vial 6. STANDARD D*1 vial 7. STANDARD E*1 vial 8. STANDARD F*1 vial 9. SUBSTRATE A*1 vial 10. SUBSTRATE B*1 vial 11. STOP SOLUT
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Description: A sandwich ELISA for quantitative measurement of Canine (IgM) in samples from blood, plasma, serum, cell culture supernatant and other biological fluids. This is a high quality ELISA kit developped for optimal performance with samples from the particular species.

Dog (IgM) ELISA kit

E08A1876-96 1 plate of 96 wells
EUR 685.00
  • Kit contents: 1. MICROTITER PLATE * 1 2. ENZYME CONJUGATE*1 vial 3. STANDARD A*1 vial 4. STANDARD B*1 vial 5. STANDARD C*1 vial 6. STANDARD D*1 vial 7. STANDARD E*1 vial 8. STANDARD F*1 vial 9. SUBSTRATE A*1 vial 10. SUBSTRATE B*1 vial 11. STOP SOLUT
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Description: A sandwich ELISA for quantitative measurement of Canine (IgM) in samples from blood, plasma, serum, cell culture supernatant and other biological fluids. This is a high quality ELISA kit developped for optimal performance with samples from the particular species.

Bovine IgM ELISA Kit

EBI0045 96Tests
EUR 521.00

Bovine IgM ELISA Kit

EBI0104 96Tests
EUR 521.00

Canine IgM ELISA Kit

ECI0045 96Tests
EUR 521.00

Canine IgM ELISA Kit

ECI0104 96Tests
EUR 521.00

Chicken IgM ELISA Kit

ECKI0045 96Tests
EUR 521.00

Cardiolipin IgM ELISA Kit

E4661-100
EUR 539.00

Rubella IgM ELISA Kit

E4667-100
EUR 539.00

Mumps IgM ELISA Kit

E4668-100
EUR 675.00

Toxoplasma IgM ELISA Kit

E4674-100
EUR 539.00

Brucella IgM ELISA Kit

E4710-100
EUR 675.00

Anserini IgM ELISA Kit

EAI0045 96Tests
EUR 521.00

Anserine IgM ELISA Kit

EAI0104 96Tests
EUR 521.00

IgM ELISA KIT|Human

EF000297 96 Tests
EUR 689.00

Mouse IgM ELISA kit

F090 1 kit (96 wells plate)
EUR 880.00
  • Product category: Enzyme linked immunosorbent assay (ELISA) kit.
Description: Mouse IgM ELISA kit by Cygnus Technologies is available in Europe via Gentaur.

Human IgM ELISA kit

F170 1 kit (96 wells plate)
EUR 880.00
  • Product category: Enzyme linked immunosorbent assay (ELISA) kit.
Description: Human IgM ELISA kit by Cygnus Technologies is available in Europe via Gentaur.

Porcine IgM ELISA Kit

EPI0045 96Tests
EUR 521.00

Porcine IgM ELISA Kit

EPI0104 96Tests
EUR 521.00

Rat IgM ELISA Kit

ERI0045 96Tests
EUR 521.00

Rat IgM ELISA Kit

ERI0104 96Tests
EUR 521.00

Rabbit IgM ELISA Kit

ERTI0045 96Tests
EUR 521.00

Rabbit IgM ELISA Kit

ERTI0104 96Tests
EUR 521.00

Sheep IgM ELISA Kit

ESI0045 96Tests
EUR 521.00

Mouse IgM ELISA Kit

EMI0045 96Tests
EUR 521.00

Mouse IgM ELISA Kit

EMI0104 96Tests
EUR 521.00

Monkey IgM ELISA Kit

EMKI0045 96Tests
EUR 521.00

Rat IgM ELISA kit

55R-1919 96 tests
EUR 482.00
Description: ELISA Kit for detection of IgM in the research laboratory

Human IgM ELISA kit

55R-1920 96 tests
EUR 482.00
Description: ELISA Kit for detection of IgM in the research laboratory

Our results provide strong evidence for three main facts: first, whether the model LD, we found a set of deregulated genes, most of them encoding mediators of the inflammatory response; secondly, there is progress with age in the appearance of inflammatory markers, ranging from the age of 3 months; and third, reactive glia are responsible for the expression of inflammatory genes. These results clearly indicate that the nerve inflammation is one of the most important features that should be considered in order to fully understand the pathophysiology of LD, and defines a reactive glia as a new therapeutic target in the disease.