MovDisordV3, Main, Exploration, bibRecord, 003156

Tau‐predominant–associated pathology in a sporadic late‐onset Hallervorden–Spatz syndrome

Identifieur interne : 003156 ( Main/Exploration ); précédent : 003155; suivant : 003157

Tau‐predominant–associated pathology in a sporadic late‐onset Hallervorden–Spatz syndrome

Auteurs : Juan J. Zarranz [Espagne] ; Juan C. G Mez-Esteban [Espagne] ; Bego A Atarés [Espagne] ; Elena Lezcano [Espagne] ; Maribel Forcadas [Espagne]

Source :

Movement Disorders [ 0885-3185 ] ; 2006-01.

RBID : ISTEX:43EE5DF3A401082679D24ACC932619FF4F6336D9

Descripteurs français

Pascal (Inist)
- Anatomopathologie, Sporadique, Système nerveux pathologie.

English descriptors

KwdEn :
- Anatomic pathology, Axons (pathology), Basal Ganglia (pathology), Brain (pathology), Brain Stem (pathology), Diagnosis, Differential, Hallervorden‐Spatz, Humans, Inclusion Bodies (pathology), Iron (analysis), Lewy Bodies (pathology), Male, Middle Aged, Myelin Sheath (pathology), Nervous system diseases, Neurodegenerative Diseases (pathology), Neurofibrillary Tangles (pathology), Pantothenate Kinase-Associated Neurodegeneration (genetics), Pantothenate Kinase-Associated Neurodegeneration (pathology), Protein Folding, Spheroids, Cellular (pathology), Sporadic, TAV, Tauopathies (genetics), Tauopathies (pathology), Thalamic Nuclei (pathology), alpha-Synuclein (analysis), tau Proteins (analysis), α‐synuclein.
MESH :
- chemical , analysis : Iron, alpha-Synuclein, tau Proteins.
- genetics : Pantothenate Kinase-Associated Neurodegeneration, Tauopathies.
- pathology : Axons, Basal Ganglia, Brain, Brain Stem, Inclusion Bodies, Lewy Bodies, Myelin Sheath, Neurodegenerative Diseases, Neurofibrillary Tangles, Pantothenate Kinase-Associated Neurodegeneration, Spheroids, Cellular, Tauopathies, Thalamic Nuclei.
- Diagnosis, Differential, Humans, Male, Middle Aged, Protein Folding.

Abstract

Hallervorden–Spatz syndrome (HSS) is a heterogeneous clinicopathological disorder currently included within the broader title of neurodegeneration with brain iron accumulation (NBIA). The classic histological hallmarks of HSS are axonal spheroids and excessive iron‐containing granules accompanied by neuronal loss and gliosis in the globus pallidus and substantia nigra reticulata. In the modern literature, attention has been drawn to the co‐occurrence of two other histological markers: Lewy bodies mainly composed of abnormal α‐synuclein, and neurofibrillary tangles due to hyperphosphorilated tau aggregation. Discrepancies exist regarding the importance of these molecular changes and its relevance for the nosology of HSS. Most authors have emphasized the importance of the Lewy body–like pathology, favoring the inclusion of HSS within the α‐synucleinopathies. We report on a case of late‐onset HSS, with the typical histological findings restricted to the basal ganglia and cerebellum in which tau pathology was exceedingly more abundant than α‐synuclein pathology. This case contributes to the increasing evidence about the heterogeneity of HSS. We favor the view that the molecular changes and the protein misfolding underlying the Lewy body and tangle formation in HSS/NBIA are secondary to the main pathological process and should not be taken as the basis for its nosological classification. © 2005 Movement Disorder Society

Url:

https://api.istex.fr/document/43EE5DF3A401082679D24ACC932619FF4F6336D9/fulltext/pdf

DOI: 10.1002/mds.20661

Affiliations:

Espagne

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<front><div type="abstract" xml:lang="en">Hallervorden–Spatz syndrome (HSS) is a heterogeneous clinicopathological disorder currently included within the broader title of neurodegeneration with brain iron accumulation (NBIA). The classic histological hallmarks of HSS are axonal spheroids and excessive iron‐containing granules accompanied by neuronal loss and gliosis in the globus pallidus and substantia nigra reticulata. In the modern literature, attention has been drawn to the co‐occurrence of two other histological markers: Lewy bodies mainly composed of abnormal α‐synuclein, and neurofibrillary tangles due to hyperphosphorilated tau aggregation. Discrepancies exist regarding the importance of these molecular changes and its relevance for the nosology of HSS. Most authors have emphasized the importance of the Lewy body–like pathology, favoring the inclusion of HSS within the α‐synucleinopathies. We report on a case of late‐onset HSS, with the typical histological findings restricted to the basal ganglia and cerebellum in which tau pathology was exceedingly more abundant than α‐synuclein pathology. This case contributes to the increasing evidence about the heterogeneity of HSS. We favor the view that the molecular changes and the protein misfolding underlying the Lewy body and tangle formation in HSS/NBIA are secondary to the main pathological process and should not be taken as the basis for its nosological classification. © 2005 Movement Disorder Society</div>
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Movement Disorders (revue)

Tau‐predominant–associated pathology in a sporadic late‐onset Hallervorden–Spatz syndrome

Tau‐predominant–associated pathology in a sporadic late‐onset Hallervorden–Spatz syndrome

Source :

Descripteurs français

English descriptors

Abstract

Links toward previous steps (curation, corpus...)

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	Movement Disorders (revue)
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