Atypical motor neuron disease variant: facial-onset sensory and motor neuronopathy syndrome (FOSMN)
Abstract Background Facial-onset sensory and motor neuronopathy (FOSMN) is a rare disease whose cardinal features are initial asymmetrical facial sensory deficits slowly evolving and followed by bulbar symptoms and spreading of sensory and motor deficits from face to scalp, neck, and extremities. Ca...
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| Format: | Article |
| Language: | English |
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SpringerOpen
2022-12-01
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| Series: | The Egyptian Journal of Neurology, Psychiatry and Neurosurgery |
| Subjects: | |
| Online Access: | https://doi.org/10.1186/s41983-022-00597-0 |
| _version_ | 1811199310059012096 |
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| author | Betül Özenç Zeki Odabaşı Ersin Tan |
| author_facet | Betül Özenç Zeki Odabaşı Ersin Tan |
| author_sort | Betül Özenç |
| collection | DOAJ |
| description | Abstract Background Facial-onset sensory and motor neuronopathy (FOSMN) is a rare disease whose cardinal features are initial asymmetrical facial sensory deficits slowly evolving and followed by bulbar symptoms and spreading of sensory and motor deficits from face to scalp, neck, and extremities. Case presentation We described a 70-year-old man who presented with 6-month history of facial numbness on the left side, and gradual worsening of symptoms. Over several months, facial muscle weakness, dysarthria, and fasciculation had progressed. NCS, needle EMG and blink reflex responses suggested the diagnosis of FOSMN. The ganglioside panel (anti-GM1 and Gd1b) was positive. Considering the FOSMN autoimmune pathology hypothesis, IVIG treatment was given. Conclusion In this case, we aimed to highlight the key clinical aspects of FOSMN and how to differentiate it from motor neuron disease and bring FOSMN to the attention of neurologists as a recently recognized and distinctive disorder. |
| first_indexed | 2024-04-12T01:45:47Z |
| format | Article |
| id | doaj.art-2da936daefa64d618e764621b26f2e16 |
| institution | Directory Open Access Journal |
| issn | 1687-8329 |
| language | English |
| last_indexed | 2024-04-12T01:45:47Z |
| publishDate | 2022-12-01 |
| publisher | SpringerOpen |
| record_format | Article |
| series | The Egyptian Journal of Neurology, Psychiatry and Neurosurgery |
| spelling | doaj.art-2da936daefa64d618e764621b26f2e162022-12-22T03:53:05ZengSpringerOpenThe Egyptian Journal of Neurology, Psychiatry and Neurosurgery1687-83292022-12-015811310.1186/s41983-022-00597-0Atypical motor neuron disease variant: facial-onset sensory and motor neuronopathy syndrome (FOSMN)Betül Özenç0Zeki Odabaşı1Ersin Tan2Neurology Department, Gülhane Training and Research HospitalNeurology Department, Gülhane Medicine SchoolNeurology Department, Hacettepe University Faculty of MedicineAbstract Background Facial-onset sensory and motor neuronopathy (FOSMN) is a rare disease whose cardinal features are initial asymmetrical facial sensory deficits slowly evolving and followed by bulbar symptoms and spreading of sensory and motor deficits from face to scalp, neck, and extremities. Case presentation We described a 70-year-old man who presented with 6-month history of facial numbness on the left side, and gradual worsening of symptoms. Over several months, facial muscle weakness, dysarthria, and fasciculation had progressed. NCS, needle EMG and blink reflex responses suggested the diagnosis of FOSMN. The ganglioside panel (anti-GM1 and Gd1b) was positive. Considering the FOSMN autoimmune pathology hypothesis, IVIG treatment was given. Conclusion In this case, we aimed to highlight the key clinical aspects of FOSMN and how to differentiate it from motor neuron disease and bring FOSMN to the attention of neurologists as a recently recognized and distinctive disorder.https://doi.org/10.1186/s41983-022-00597-0FOSMNNeuronopathyDysphagiaMotor neuron disease (MND) |
| spellingShingle | Betül Özenç Zeki Odabaşı Ersin Tan Atypical motor neuron disease variant: facial-onset sensory and motor neuronopathy syndrome (FOSMN) The Egyptian Journal of Neurology, Psychiatry and Neurosurgery FOSMN Neuronopathy Dysphagia Motor neuron disease (MND) |
| title | Atypical motor neuron disease variant: facial-onset sensory and motor neuronopathy syndrome (FOSMN) |
| title_full | Atypical motor neuron disease variant: facial-onset sensory and motor neuronopathy syndrome (FOSMN) |
| title_fullStr | Atypical motor neuron disease variant: facial-onset sensory and motor neuronopathy syndrome (FOSMN) |
| title_full_unstemmed | Atypical motor neuron disease variant: facial-onset sensory and motor neuronopathy syndrome (FOSMN) |
| title_short | Atypical motor neuron disease variant: facial-onset sensory and motor neuronopathy syndrome (FOSMN) |
| title_sort | atypical motor neuron disease variant facial onset sensory and motor neuronopathy syndrome fosmn |
| topic | FOSMN Neuronopathy Dysphagia Motor neuron disease (MND) |
| url | https://doi.org/10.1186/s41983-022-00597-0 |
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