Autopsy always teach and tell: neurodegeneration with brain iron accumulation: a case report.

Autopsy always teach and tell: neurodegeneration with brain iron accumulation: a case report. Research Abstract Details 

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Autopsy always teach and tell: neurodegeneration with brain iron accumulation: a case report. Abstract Text:

Ruchika Gupta,Ashok Kumar,M C Sharma,Chitra Sarkar,Vinay Goyal,Madhuri Bihari,

Neurodegeneration with brain iron accumulation (NBIA), or Hallervorden- Spatz disease, is an extremely rare autosomal recessive disorder with cysteine-iron complex accumulation in globus pallidus, seen histopathologically. Magnetic resonance imaging offers an opportunity for diagnosis; however, therapeutic options are still ineffective. We report a case of 13-year-old girl, symptomatic since the age of three years with dystonia, poor scholastic performance and speech disturbances. She was admitted with aspiration pneumonia, and died before she could be investigated. Examination of brain at autopsy revealed iron deposition in bilateral globus pallidi, confirmed by special stains and elemental dispersion analysis by spectrometry and a diagnosis of Hallervorden- Spatz disease or NBIA was made. This report highlights the importance of autopsy and scanning electron microscopic examination in unsuspected cases where cause of death is not known.

Autopsy always teach and tell: neurodegeneration with brain iron accumulation: a case report. Publishing Authors By Initials

R Gupta,A Kumar,MC Sharma,C Sarkar,V Goyal,M Bihari,

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Autopsy always teach and tell: neurodegeneration with brain iron accumulation: a case report. Journal Published:

PUBLICATION TYPE: Journal Article

Journal: Indian journal of pathology & microbiology

VOLUME: 50

Page Numbers: 792-4

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ISSN: 0377-4929

DAY: 29

MONTH: Oct

YEAR: 2007

Autopsy always teach and tell: neurodegeneration with brain iron accumulation: a case report. Information

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LANGUAGE: eng

NlmUniqueID: 7605904

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AFFILIATION: Department of Pathology, All India Institute of Medical Sciences, New Delhi.

Country: India

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MEDLINETA: Indian J Pathol Microbiol

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