Specific Pattern of Early White-Matter Changes in Pure Hereditary Spastic Paraplegia
Identifieur interne : 000908 ( PascalFrancis/Corpus ); précédent : 000907; suivant : 000909Specific 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 DeppeSource :
- Movement disorders [ 0885-3185 ] ; 2010.
Descripteurs français
- Pascal (Inist)
English descriptors
- KwdEn :
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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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 |
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Pascal:10-0446333Le 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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<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>
<CC>002B17; 002B17F</CC>
<FD>Paraplégie spasmodique héréditaire de Strümpell-Lorrain; Pathologie du système nerveux; Substance blanche; Imagerie du tenseur de diffusion</FD>
<FG>Pathologie de l'encéphale; Maladie dégénérative; Maladie héréditaire; Pathologie de la moelle épinière; Pathologie du système nerveux central</FG>
<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>
<LO>INIST-20953.354000194841700320</LO>
<ID>10-0446333</ID>
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