Is Generalized and Segmental Dystonia Accompanied by Impairments in the Dopaminergic System?
Background: The pathogenesis of dystonia is remarkably diverse. Some types of dystonia, such as DYT5 (DYT-GCH1) and tardive dystonia, are related to dysfunction of the dopaminergic system. Furthermore, on pathological examination, cell loss in the substantia nigra (SN) of patients with dystonia has...
Guardado en:
| Autores principales: | , , , , , , , , , , |
|---|---|
| Formato: | article |
| Lenguaje: | EN |
| Publicado: |
Frontiers Media S.A.
2021
|
| Materias: | |
| Acceso en línea: | https://doaj.org/article/a5fac26e44144125924b2e8d031a0abe |
| Etiquetas: |
Agregar Etiqueta
Sin Etiquetas, Sea el primero en etiquetar este registro!
|
| id |
oai:doaj.org-article:a5fac26e44144125924b2e8d031a0abe |
|---|---|
| record_format |
dspace |
| spelling |
oai:doaj.org-article:a5fac26e44144125924b2e8d031a0abe2021-11-18T14:07:12ZIs Generalized and Segmental Dystonia Accompanied by Impairments in the Dopaminergic System?1664-229510.3389/fneur.2021.751434https://doaj.org/article/a5fac26e44144125924b2e8d031a0abe2021-11-01T00:00:00Zhttps://www.frontiersin.org/articles/10.3389/fneur.2021.751434/fullhttps://doaj.org/toc/1664-2295Background: The pathogenesis of dystonia is remarkably diverse. Some types of dystonia, such as DYT5 (DYT-GCH1) and tardive dystonia, are related to dysfunction of the dopaminergic system. Furthermore, on pathological examination, cell loss in the substantia nigra (SN) of patients with dystonia has been reported, suggesting that impaired dopamine production may be involved in DYT5 and in other types of dystonia.Objectives: To investigate functional dopaminergic impairments, we compared patients with dystonia and those with Parkinson's disease (PD) with normal controls using neuromelanin-sensitive magnetic resonance imaging (NM-MRI) and dopamine transporter single photon emission computed tomography (DAT SPECT).Methods: A total of 18, 18, and 27 patients with generalized or segmental dystonia, patients with PD, and healthy controls, respectively, were examined using NM-MRI. The mean area corresponding to NM in the SN (NM-SN) was blindly quantified. DAT SPECT was performed on 17 and eight patients with dystonia and PD, respectively. The imaging data of DAT SPECT were harmonized with the Japanese database using striatum phantom calibration. These imaging data were compared between patients with dystonia or PD and controls from the Japanese database in 256 healthy volunteers using the calibrated specific binding ratio (cSBR). The symptoms of dystonia were evaluated using the Fahn–Marsden Dystonia Rating Scale (FMDRS), and the correlation between the results of imaging data and FMDRS was examined.Results: The mean areas corresponding to NM in the SN (NM-SN) were 31 ± 4.2, 28 ± 3.8, and 43 ± 3.8 pixels in patients with dystonia, PD, and in healthy controls, respectively. The mean cSBRs were 5 ± 0.2, 2.8 ± 0.2, 9.2 (predictive) in patients with dystonia, PD, and in healthy controls, respectively. The NM-SN area (r = −0.49, p < 0.05) and the cSBR (r = −0.54, p < 0.05) were inversely correlated with the FMDRS. There was no significant difference between the dystonia and PD groups regarding NM-SN (p = 0.28). In contrast, the cSBR was lower in patients with PD than in those with dystonia (p < 0.5 × 10−6).Conclusions: Impairments of the dopaminergic system may be involved in developing generalized and segmental dystonia. SN abnormalities in patients with dystonia were supposed to be different from degeneration in PD.Jun IkezawaJun IkezawaFusako YokochiRyoichi OkiyamaSatoko KumadaMaya TojimaMaya TojimaTsutomu KamiyamaTakashi HanakawaTakashi HanakawaHiroshi MatsudaFumiaki TanakaYasuhiro NakataEiji IsozakiFrontiers Media S.A.articledystoniasubstantia nigradopaminergic systemdopamine transporter single photon emission computed tomographyneuromelanin-sensitive MRIParkinson's diseaseNeurology. Diseases of the nervous systemRC346-429ENFrontiers in Neurology, Vol 12 (2021) |
| institution |
DOAJ |
| collection |
DOAJ |
| language |
EN |
| topic |
dystonia substantia nigra dopaminergic system dopamine transporter single photon emission computed tomography neuromelanin-sensitive MRI Parkinson's disease Neurology. Diseases of the nervous system RC346-429 |
| spellingShingle |
dystonia substantia nigra dopaminergic system dopamine transporter single photon emission computed tomography neuromelanin-sensitive MRI Parkinson's disease Neurology. Diseases of the nervous system RC346-429 Jun Ikezawa Jun Ikezawa Fusako Yokochi Ryoichi Okiyama Satoko Kumada Maya Tojima Maya Tojima Tsutomu Kamiyama Takashi Hanakawa Takashi Hanakawa Hiroshi Matsuda Fumiaki Tanaka Yasuhiro Nakata Eiji Isozaki Is Generalized and Segmental Dystonia Accompanied by Impairments in the Dopaminergic System? |
| description |
Background: The pathogenesis of dystonia is remarkably diverse. Some types of dystonia, such as DYT5 (DYT-GCH1) and tardive dystonia, are related to dysfunction of the dopaminergic system. Furthermore, on pathological examination, cell loss in the substantia nigra (SN) of patients with dystonia has been reported, suggesting that impaired dopamine production may be involved in DYT5 and in other types of dystonia.Objectives: To investigate functional dopaminergic impairments, we compared patients with dystonia and those with Parkinson's disease (PD) with normal controls using neuromelanin-sensitive magnetic resonance imaging (NM-MRI) and dopamine transporter single photon emission computed tomography (DAT SPECT).Methods: A total of 18, 18, and 27 patients with generalized or segmental dystonia, patients with PD, and healthy controls, respectively, were examined using NM-MRI. The mean area corresponding to NM in the SN (NM-SN) was blindly quantified. DAT SPECT was performed on 17 and eight patients with dystonia and PD, respectively. The imaging data of DAT SPECT were harmonized with the Japanese database using striatum phantom calibration. These imaging data were compared between patients with dystonia or PD and controls from the Japanese database in 256 healthy volunteers using the calibrated specific binding ratio (cSBR). The symptoms of dystonia were evaluated using the Fahn–Marsden Dystonia Rating Scale (FMDRS), and the correlation between the results of imaging data and FMDRS was examined.Results: The mean areas corresponding to NM in the SN (NM-SN) were 31 ± 4.2, 28 ± 3.8, and 43 ± 3.8 pixels in patients with dystonia, PD, and in healthy controls, respectively. The mean cSBRs were 5 ± 0.2, 2.8 ± 0.2, 9.2 (predictive) in patients with dystonia, PD, and in healthy controls, respectively. The NM-SN area (r = −0.49, p < 0.05) and the cSBR (r = −0.54, p < 0.05) were inversely correlated with the FMDRS. There was no significant difference between the dystonia and PD groups regarding NM-SN (p = 0.28). In contrast, the cSBR was lower in patients with PD than in those with dystonia (p < 0.5 × 10−6).Conclusions: Impairments of the dopaminergic system may be involved in developing generalized and segmental dystonia. SN abnormalities in patients with dystonia were supposed to be different from degeneration in PD. |
| format |
article |
| author |
Jun Ikezawa Jun Ikezawa Fusako Yokochi Ryoichi Okiyama Satoko Kumada Maya Tojima Maya Tojima Tsutomu Kamiyama Takashi Hanakawa Takashi Hanakawa Hiroshi Matsuda Fumiaki Tanaka Yasuhiro Nakata Eiji Isozaki |
| author_facet |
Jun Ikezawa Jun Ikezawa Fusako Yokochi Ryoichi Okiyama Satoko Kumada Maya Tojima Maya Tojima Tsutomu Kamiyama Takashi Hanakawa Takashi Hanakawa Hiroshi Matsuda Fumiaki Tanaka Yasuhiro Nakata Eiji Isozaki |
| author_sort |
Jun Ikezawa |
| title |
Is Generalized and Segmental Dystonia Accompanied by Impairments in the Dopaminergic System? |
| title_short |
Is Generalized and Segmental Dystonia Accompanied by Impairments in the Dopaminergic System? |
| title_full |
Is Generalized and Segmental Dystonia Accompanied by Impairments in the Dopaminergic System? |
| title_fullStr |
Is Generalized and Segmental Dystonia Accompanied by Impairments in the Dopaminergic System? |
| title_full_unstemmed |
Is Generalized and Segmental Dystonia Accompanied by Impairments in the Dopaminergic System? |
| title_sort |
is generalized and segmental dystonia accompanied by impairments in the dopaminergic system? |
| publisher |
Frontiers Media S.A. |
| publishDate |
2021 |
| url |
https://doaj.org/article/a5fac26e44144125924b2e8d031a0abe |
| work_keys_str_mv |
AT junikezawa isgeneralizedandsegmentaldystoniaaccompaniedbyimpairmentsinthedopaminergicsystem AT junikezawa isgeneralizedandsegmentaldystoniaaccompaniedbyimpairmentsinthedopaminergicsystem AT fusakoyokochi isgeneralizedandsegmentaldystoniaaccompaniedbyimpairmentsinthedopaminergicsystem AT ryoichiokiyama isgeneralizedandsegmentaldystoniaaccompaniedbyimpairmentsinthedopaminergicsystem AT satokokumada isgeneralizedandsegmentaldystoniaaccompaniedbyimpairmentsinthedopaminergicsystem AT mayatojima isgeneralizedandsegmentaldystoniaaccompaniedbyimpairmentsinthedopaminergicsystem AT mayatojima isgeneralizedandsegmentaldystoniaaccompaniedbyimpairmentsinthedopaminergicsystem AT tsutomukamiyama isgeneralizedandsegmentaldystoniaaccompaniedbyimpairmentsinthedopaminergicsystem AT takashihanakawa isgeneralizedandsegmentaldystoniaaccompaniedbyimpairmentsinthedopaminergicsystem AT takashihanakawa isgeneralizedandsegmentaldystoniaaccompaniedbyimpairmentsinthedopaminergicsystem AT hiroshimatsuda isgeneralizedandsegmentaldystoniaaccompaniedbyimpairmentsinthedopaminergicsystem AT fumiakitanaka isgeneralizedandsegmentaldystoniaaccompaniedbyimpairmentsinthedopaminergicsystem AT yasuhironakata isgeneralizedandsegmentaldystoniaaccompaniedbyimpairmentsinthedopaminergicsystem AT eijiisozaki isgeneralizedandsegmentaldystoniaaccompaniedbyimpairmentsinthedopaminergicsystem |
| _version_ |
1718420802513141760 |