Academic Journal
Neuroimaging signatures of facial onset sensory and motor neuronopathy (FOSMN).
| Τίτλος: | Neuroimaging signatures of facial onset sensory and motor neuronopathy (FOSMN). |
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| Συγγραφείς: | Rosenbohm A; Department of Neurology, University Hospital Ulm, Ulm, Germany. angela.rosenbohm@uni-ulm.de., Herrera-Angeles F; Department of Neurology, University Hospital Ulm, Ulm, Germany., Huppertz HJ; Swiss Epilepsy Clinic, Klinik Lengg, Zurich, Switzerland., Haeusler KG; Department of Neurology, University Hospital Ulm, Ulm, Germany., Weishaupt JH; Department of Neurology, University Hospital Ulm, Ulm, Germany., Müller HP; Department of Neurology, University Hospital Ulm, Ulm, Germany.; Department of Diagnostic and Interventional Radiology, University Hospital Ulm, Oberer Eselsberg 45, 89081, Ulm, Germany.; Department of Nuclear Medicine, University Hospital Ulm, Ulm, Germany., Kassubek J; Department of Neurology, University Hospital Ulm, Ulm, Germany. |
| Πηγή: | Journal of neurology [J Neurol] 2026 Oct 05; Vol. 273 (10). Date of Electronic Publication: 2026 Oct 05. |
| Τύπος έκδοσης: | Journal Article |
| Γλώσσα: | English |
| Στοιχεία περιοδικού: | Publisher: Springer-Verlag Country of Publication: Germany NLM ID: 0423161 Publication Model: Electronic Cited Medium: Internet ISSN: 1432-1459 (Electronic) Linking ISSN: 03405354 NLM ISO Abbreviation: J Neurol Subsets: MEDLINE |
| Imprint Name(s): | Original Publication: Berlin ; New York, Springer-Verlag |
| Ιατρικοί όροι (MeSH): | Brain*/diagnostic imaging , Brain*/pathology , Facial Nerve Diseases*/diagnostic imaging , Facial Nerve Diseases*/pathology , Neuroimaging* , Motor Neuron Disease*, Humans ; Female ; Male ; Diffusion Tensor Imaging ; Magnetic Resonance Imaging ; Middle Aged ; Atrophy ; Adult ; Image Processing, Computer-Assisted ; Aged |
| Περίληψη: | Facial onset sensory and motor neuronopathy (FOSMN) is an exceptionally rare condition characterized by progressive facial paresthesia and the subsequent appearance of bulbar symptoms with rostro-caudal motor and sensory deficits sometimes extending to the hand muscles. The pathophysiological understanding of this disorder is very limited. In order to identify patterns of neuroanatomical involvement within the brain, we applied advanced magnetic resonance imaging-based techniques to a group of six patients with the diagnosis of FOSMN, using unbiased analysis techniques with atlas-based volumetry of volume-rendering T1-weighted data and whole brain-based spatial statistics of diffusion tensor imaging (DTI). The volumetric data showed significant global atrophy with prominent involvement of the medulla oblongata, while DTI demonstrated significant decreases in the right-hemispheric frontal lobe and bilaterally along the superior and inferior longitudinal fasciculi. In summary, this application of volumetry and DTI to a group of FOSMN patients demonstrated, beyond global brain atrophy as a correlate of a systemic disease process, regional atrophy in the brainstem/medulla oblongata, together with tract alterations of polymodal frontotemporoparietal interconnections. Future neuroimaging studies in larger patient groups of FOSMN are needed for detailed phenotype-neuroimaging correlations. (© 2026. The Author(s).) |
| Competing Interests: | Declarations. Conflicts of interest and financial disclosures (for the preceding 12 months): None of the authors has any potential conflicts of interest for this manuscript. All authors exclude financial support for this study. |
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| Contributed Indexing: | Keywords: Amyotrophic lateral sclerosis; Atlas-based volumetry; Diffusion tensor imaging; Facial onset sensory and motor neuronopathy; Magnetic resonance imaging; Motor neuron disease; Neuroimaging |
| Entry Date(s): | Date Created: 20261005 Date Completed: 20261005 Latest Revision: 20261008 |
| Update Code: | 20261008 |
| PubMed Central ID: | PMC13638678 |
| DOI: | 10.1007/s00415-026-14174-5 |
| PMID: | 42831972 |
| Βάση Δεδομένων: | MEDLINE |
| ISSN: | 1432-1459 |
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| DOI: | 10.1007/s00415-026-14174-5 |