Thalamic atrophy in patients with pure hereditary spastic paraplegia type 4
| Autor: | Yasser Alemán-Gómez, Andrés Ordóñez-Ugalde, Manuel Desco, Beatriz Quintáns, Francisco Grandas, Susanna Carmona, María-Jesús Sobrido, Julia Romero, Francisco J. Navas-Sánchez, Pilar Fernández-García, Luis Marcos-Vidal, Julio Pardo, Laura Lillo, Alberto Fernández-Pena, Irene Catalina, Daniel Martín de Blas, Juan A Guzmán-De-Villoria, José Luis Muñoz-Blanco |
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| Rok vydání: | 2021 |
| Předmět: |
Pathology
medicine.medical_specialty Hereditary spastic paraplegia business.industry Upper motor neuron Putamen Thalamus Caudate nucleus medicine.disease 03 medical and health sciences 0302 clinical medicine medicine.anatomical_structure Globus pallidus nervous system Neurology Basal ganglia Corticospinal tract medicine 030212 general & internal medicine Neurology (clinical) business 030217 neurology & neurosurgery |
| Zdroj: | Journal of Neurology |
| ISSN: | 0340-5354 |
| DOI: | 10.1007/s00415-020-10387-4 |
| Popis: | SPG4 is an autosomal dominant pure form of hereditary spastic paraplegia (HSP) caused by mutations in the SPAST gene. HSP is considered an upper motor neuron disorder characterized by progressive spasticity and weakness of the lower limbs caused by degeneration of the corticospinal tract. In other neurodegenerative motor disorders, the thalamus and basal ganglia are affected, with a considerable impact on disease progression. However, only a few works have studied these brain structures in HSP, mainly in complex forms of this disease. Our research aims to detect potential alterations in the volume and shape of the thalamus and various basal ganglia structures by comparing 12 patients with pure HSP and 18 healthy controls. We used two neuroimaging procedures: automated segmentation of the subcortical structures (thalamus, hippocampus, caudate nucleus, globus pallidus, and putamen) in native space and shape analysis of the structures. We found a significant reduction in thalamic volume bilaterally, as well as an inward deformation, mainly in the sensory-motor thalamic regions in patients with pure HSP and a mutation in SPG4. We also observed a significant negative correlation between the shape of the thalamus and clinical scores (the Spastic Paraplegia Rating Scale score and disease duration). Moreover, we found a ‘Group × Age’ interaction that was closely related to the severity of the disease. No differences in volume or in shape were found in the remaining subcortical structures studied. Our results suggest that changes in structure of the thalamus could be an imaging biomarker of disease progression in pHSP. |
| Databáze: | OpenAIRE |
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