MovDisordV3, PascalFrancis, Corpus, bibRecord, 000908

Specific Pattern of Early White-Matter Changes in Pure Hereditary Spastic Paraplegia

Identifieur interne : 000908 ( PascalFrancis/Corpus ); précédent : 000907; suivant : 000909

Specific Pattern of Early White-Matter Changes in Pure Hereditary Spastic Paraplegia

Auteurs : Thomas Duning ; Tobias Warnecke ; Anja Schirmacher ; Hagen Schiffbauer ; Hubertus Lohmann ; Siawoosh Mohammadi ; Peter Young ; Michael Deppe

Source :

Movement disorders [ 0885-3185 ] ; 2010.

RBID : Pascal:10-0446333

Descripteurs français

Pascal (Inist)
- Paraplégie spasmodique héréditaire de Strümpell-Lorrain, Pathologie du système nerveux, Substance blanche, Imagerie du tenseur de diffusion.

English descriptors

KwdEn :
- Diffusion tensor imaging, Hereditary spastic paraplegia, Nervous system diseases, White matter.

Abstract

Hereditary spastic paraplegias (HSP) are genetically and clinically heterogeneous neurodegenerative disorders. Most MR studies on HSP include very heterogeneous samples of patients, and findings were inconsistent. Here, we examined six patients with pure HSP and SPG4 mutations by clinical evaluation, detailed neuropsychological testing, and neuroimaging analyses, including conventional MRI, diffusion tensor imaging (DTI), and brain volumetry. Differences of voxel-wise statistics and ROI-based analysis of DTI data between patients and 32 healthy volunteers were evaluated. Although conventional MRI and brain volumetry were normal, DTI revealed widespread disturbance of white matter (WM) integrity (P < 0.001), mainly affecting the corticospinal tract. With longer disease duration, frontal regions were also involved. The WM changes were also present in subclinical subjects harbouring the pathogenic mutation. These subtle WM abnormalities have functional relevance because they correlated with clinical symptoms. Thus, early alterations of nerve fibres, which can be detected by DTI, might serve as a biological marker in HSP, in particular with respect to future longitudinal studies.

Notice en format standard (ISO 2709)

Pour connaître la documentation sur le format Inist Standard.

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C01	`01`		`ENG`	@0 Hereditary spastic paraplegias (HSP) are genetically and clinically heterogeneous neurodegenerative disorders. Most MR studies on HSP include very heterogeneous samples of patients, and findings were inconsistent. Here, we examined six patients with pure HSP and SPG4 mutations by clinical evaluation, detailed neuropsychological testing, and neuroimaging analyses, including conventional MRI, diffusion tensor imaging (DTI), and brain volumetry. Differences of voxel-wise statistics and ROI-based analysis of DTI data between patients and 32 healthy volunteers were evaluated. Although conventional MRI and brain volumetry were normal, DTI revealed widespread disturbance of white matter (WM) integrity (P < 0.001), mainly affecting the corticospinal tract. With longer disease duration, frontal regions were also involved. The WM changes were also present in subclinical subjects harbouring the pathogenic mutation. These subtle WM abnormalities have functional relevance because they correlated with clinical symptoms. Thus, early alterations of nerve fibres, which can be detected by DTI, might serve as a biological marker in HSP, in particular with respect to future longitudinal studies.
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C07	`02`	`X`	`FRE`	`@0 Maladie dégénérative @5 38`
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C07	`03`	`X`	`FRE`	`@0 Maladie héréditaire @5 39`
C07	`03`	`X`	`ENG`	`@0 Genetic disease @5 39`
C07	`03`	`X`	`SPA`	`@0 Enfermedad hereditaria @5 39`
C07	`04`	`X`	`FRE`	`@0 Pathologie de la moelle épinière @5 40`
C07	`04`	`X`	`ENG`	`@0 Spinal cord disease @5 40`
C07	`04`	`X`	`SPA`	`@0 Médula espinal patología @5 40`
C07	`05`	`X`	`FRE`	`@0 Pathologie du système nerveux central @5 41`
C07	`05`	`X`	`ENG`	`@0 Central nervous system disease @5 41`
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Format Inist (serveur)

NO :	PASCAL 10-0446333 INIST
ET :	Specific Pattern of Early White-Matter Changes in Pure Hereditary Spastic Paraplegia
AU :	DUNING (Thomas); WARNECKE (Tobias); SCHIRMACHER (Anja); SCHIFFBAUER (Hagen); LOHMANN (Hubertus); MOHAMMADI (Siawoosh); YOUNG (Peter); DEPPE (Michael)
AF :	Department of Neurology, University Hospital of Münster/Münster/Allemagne (1 aut., 2 aut., 3 aut., 5 aut., 6 aut., 7 aut., 8 aut.); Department of Clinical Radiology, University Hospital of Münster/Münster/Allemagne (4 aut.)
DT :	Publication en série; Courte communication, note brève; Niveau analytique
SO :	Movement disorders; ISSN 0885-3185; Etats-Unis; Da. 2010; Vol. 25; No. 12; Pp. 1986-1992; Bibl. 18 ref.
LA :	Anglais
EA :	Hereditary spastic paraplegias (HSP) are genetically and clinically heterogeneous neurodegenerative disorders. Most MR studies on HSP include very heterogeneous samples of patients, and findings were inconsistent. Here, we examined six patients with pure HSP and SPG4 mutations by clinical evaluation, detailed neuropsychological testing, and neuroimaging analyses, including conventional MRI, diffusion tensor imaging (DTI), and brain volumetry. Differences of voxel-wise statistics and ROI-based analysis of DTI data between patients and 32 healthy volunteers were evaluated. Although conventional MRI and brain volumetry were normal, DTI revealed widespread disturbance of white matter (WM) integrity (P < 0.001), mainly affecting the corticospinal tract. With longer disease duration, frontal regions were also involved. The WM changes were also present in subclinical subjects harbouring the pathogenic mutation. These subtle WM abnormalities have functional relevance because they correlated with clinical symptoms. Thus, early alterations of nerve fibres, which can be detected by DTI, might serve as a biological marker in HSP, in particular with respect to future longitudinal studies.
CC :	002B17; 002B17F
FD :	Paraplégie spasmodique héréditaire de Strümpell-Lorrain; Pathologie du système nerveux; Substance blanche; Imagerie du tenseur de diffusion
FG :	Pathologie de l'encéphale; Maladie dégénérative; Maladie héréditaire; Pathologie de la moelle épinière; Pathologie du système nerveux central
ED :	Hereditary spastic paraplegia; Nervous system diseases; White matter; Diffusion tensor imaging
EG :	Cerebral disorder; Degenerative disease; Genetic disease; Spinal cord disease; Central nervous system disease
SD :	Paraplejía espasmódica hereditaria Strümpell-Lorrain; Sistema nervioso patología; Substancia blanca; Imágen de tensor de difusión
LO :	INIST-20953.354000194841700320
ID :	10-0446333

Links to Exploration step

Pascal:10-0446333

Le document en format XML

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<front><div type="abstract" xml:lang="en">Hereditary spastic paraplegias (HSP) are genetically and clinically heterogeneous neurodegenerative disorders. Most MR studies on HSP include very heterogeneous samples of patients, and findings were inconsistent. Here, we examined six patients with pure HSP and SPG4 mutations by clinical evaluation, detailed neuropsychological testing, and neuroimaging analyses, including conventional MRI, diffusion tensor imaging (DTI), and brain volumetry. Differences of voxel-wise statistics and ROI-based analysis of DTI data between patients and 32 healthy volunteers were evaluated. Although conventional MRI and brain volumetry were normal, DTI revealed widespread disturbance of white matter (WM) integrity (P < 0.001), mainly affecting the corticospinal tract. With longer disease duration, frontal regions were also involved. The WM changes were also present in subclinical subjects harbouring the pathogenic mutation. These subtle WM abnormalities have functional relevance because they correlated with clinical symptoms. Thus, early alterations of nerve fibres, which can be detected by DTI, might serve as a biological marker in HSP, in particular with respect to future longitudinal studies.</div>
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<server><NO>PASCAL 10-0446333 INIST</NO>
<ET>Specific Pattern of Early White-Matter Changes in Pure Hereditary Spastic Paraplegia</ET>
<AU>DUNING (Thomas); WARNECKE (Tobias); SCHIRMACHER (Anja); SCHIFFBAUER (Hagen); LOHMANN (Hubertus); MOHAMMADI (Siawoosh); YOUNG (Peter); DEPPE (Michael)</AU>
<AF>Department of Neurology, University Hospital of Münster/Münster/Allemagne (1 aut., 2 aut., 3 aut., 5 aut., 6 aut., 7 aut., 8 aut.); Department of Clinical Radiology, University Hospital of Münster/Münster/Allemagne (4 aut.)</AF>
<DT>Publication en série; Courte communication, note brève; Niveau analytique</DT>
<SO>Movement disorders; ISSN 0885-3185; Etats-Unis; Da. 2010; Vol. 25; No. 12; Pp. 1986-1992; Bibl. 18 ref.</SO>
<LA>Anglais</LA>
<EA>Hereditary spastic paraplegias (HSP) are genetically and clinically heterogeneous neurodegenerative disorders. Most MR studies on HSP include very heterogeneous samples of patients, and findings were inconsistent. Here, we examined six patients with pure HSP and SPG4 mutations by clinical evaluation, detailed neuropsychological testing, and neuroimaging analyses, including conventional MRI, diffusion tensor imaging (DTI), and brain volumetry. Differences of voxel-wise statistics and ROI-based analysis of DTI data between patients and 32 healthy volunteers were evaluated. Although conventional MRI and brain volumetry were normal, DTI revealed widespread disturbance of white matter (WM) integrity (P < 0.001), mainly affecting the corticospinal tract. With longer disease duration, frontal regions were also involved. The WM changes were also present in subclinical subjects harbouring the pathogenic mutation. These subtle WM abnormalities have functional relevance because they correlated with clinical symptoms. Thus, early alterations of nerve fibres, which can be detected by DTI, might serve as a biological marker in HSP, in particular with respect to future longitudinal studies.</EA>
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<ED>Hereditary spastic paraplegia; Nervous system diseases; White matter; Diffusion tensor imaging</ED>
<EG>Cerebral disorder; Degenerative disease; Genetic disease; Spinal cord disease; Central nervous system disease</EG>
<SD>Paraplejía espasmódica hereditaria Strümpell-Lorrain; Sistema nervioso patología; Substancia blanca; Imágen de tensor de difusión</SD>
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	Movement Disorders (revue)
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Movement Disorders (revue)

Specific Pattern of Early White-Matter Changes in Pure Hereditary Spastic Paraplegia

Specific Pattern of Early White-Matter Changes in Pure Hereditary Spastic Paraplegia

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