Subacute Sclerosing Panencephalitis with Fulminating Course: Follow-Up Magnetic Resonance Spectroscopy (MRS) Findings

2004 ◽  
Vol 19 (3) ◽  
pp. 233-233 ◽  
Keyword(s):  
Magnetic Resonance ◽  
Magnetic Resonance Spectroscopy ◽  
Subacute Sclerosing Panencephalitis ◽  
Clinical Stage ◽  
Clinical Status ◽  
Neuronal Loss ◽  
Rare Condition ◽  
Clinical Staging ◽  
Resonance Spectroscopy

The fulminating form of subacute sclerosing panencephalitis is an extremely rare condition. Imaging findings are usually not correlated with clinical staging. We describe a 4-year-old girl with severe neuronal loss, demyelination, and gliosis in subcortical white matter by magnetic resonance spectroscopic examination even though she was diagnosed as clinical stage II. In 2 months’ time, her clinical status worsened significantly. Follow-up magnetic resonance spectroscopy revealed findings that were consistent with clinical status. It is our opinion that magnetic resonance spectroscopy could demonstrate a rapidly progressive fulminating course of subacute sclerosing panencephalitis even in the early clinical stages.

Brain Phenylalanine Concentrations in Phenylketonuria: Research and Treatment of Adults

PEDIATRICS ◽  
2003 ◽  
Vol 112 (Supplement_4) ◽  
pp. 1575-1579
Author(s):  
Rex A. Moats ◽  
Kathryn D. Moseley ◽  
Richard Koch ◽  
Marvin Nelson
Keyword(s):  
Amino Acids ◽  
Amino Acid ◽  
Magnetic Resonance ◽  
Magnetic Resonance Spectroscopy ◽  
Outpatient Treatment ◽  
Dietary Supplementation ◽  
Resonance Spectroscopy ◽  
A Prospective Study ◽  
The Brain

Objective. To assess the effects of 2 pharmacologic interventions (amino acid supplements) on the brain levels of phenylalanine (Phe) in adults with phenylketonuria (PKU). Methods. A prospective study was conducted in an outpatient treatment and follow-up setting. The volunteers who were recruited for the first intervention included 4 subjects with classic PKU. The second intervention included 3 adults with classic PKU. The first intervention consisted of dietary supplementation during 1 day with Phlexy 10. Two individuals were given a dose of 0.5 g/kg/d, and 2 were given 1.0 g/kg/d. The second intervention consisted of dietary supplementation with PreKUnil at 0.4 g kg/d over a period of 6 months. Brain Phe was measured by magnetic resonance spectroscopy. The number of the patients involved precluded analysis for significance. Results. The first, shorter intervention resulted in a decrease in brain Phe. The second intervention resulted in a 20% decrease in brain Phe, which was maintained after 6 months of treatment. Conclusion. Dietary supplementation of large neutral amino acids seems to lower the brain Phe in adults who have PKU and have difficulty following their diet.

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