Facial onset sensory and motor neuronopathy: Difference between revisions
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'''Facial onset sensory and motor neuronopathy''', often abbreviated '''FOSMN''', is a rare disorder of the [[nervous system]] in which sensory and motor [[Spinal nerve|nerves]] of the face and limbs progressively degenerate over a period of months to years. This degenerative process, the cause of which is unknown, eventually results in sensory and motor symptoms — the former consisting mainly of paresthesia followed by numbness, and the latter in muscle weakness, atrophy, and eventual paralysis. In many ways, it is reminiscent of the much better known condition '''[[amyotrophic lateral sclerosis]]''', with which it is closely related. There is no cure; treatment is supportive. Life expectancy may be shortened by respiratory complications arising from weakness of the muscles that aid breathing and swallowing. It was first described in four patients by Vucic and colleagues<ref name="Vucic 2006">{{cite journal |last1=Vucic |first1=S |display-authors=etal |title=Facial onset sensory and motor neuronopathy (FOSMN syndrome): a novel syndrome in neurology |journal=Brain |date=Dec 2006 |volume=129 |issue=12 |pages=3384–90 |doi=10.1093/brain/awl258 |pmid=17012296 |url=https://pubmed.ncbi.nlm.nih.gov/17012296/ |access-date=1 May 2021}}</ref> working at the [[Massachusetts General Hospital]] in the [[United States of America|United States]]; subsequent reports from the United Kingdom,<ref name="Broad 2015">{{cite journal |last1=Broad |first1=R |last2=Leigh |first2=PN |title=Recognising facial onset sensory motor neuronopathy syndrome: insight from six new cases |journal=Pract Neurol |date=Aug 2015 |volume=15 |issue=4 |pages=293–7 |doi=10.1136/practneurol-2014-000984 |pmid=25991863 |url=https://pubmed.ncbi.nlm.nih.gov/25991863/ |access-date=1 May 2021}}</ref> Europe and Asia<ref name="Sonoda 2013">{{cite journal |last1=Sonoda |first1=K |display-authors=etal |title=TAR DNA-binding protein 43 pathology in a case clinically diagnosed with facial-onset sensory and motor neuronopathy syndrome: an autopsied case report and a review of the literature |journal=J Neurol Sci |date=Sep 2013 |volume=332 |issue=1-2 |pages=148–53 |doi=10.1016/j.jns.2013.06.027 |pmid=23849263 |url=https://pubmed.ncbi.nlm.nih.gov/23849263/ |access-date=1 May 2021}}</ref> point to a global incidence of the disease. It is thought to be exceptionally rare, with only approximately 100 individuals<ref name="de Boer 2021">{{cite journal |last1=de Boer |first1=EMJ |display-authors=etal|title=Facial Onset Sensory and Motor Neuronopathy: New Cases, Cognitive Changes, and Pathophysiology |journal=Neurol Clin Pract |date=Apr 2021 |volume=11 |issue=2 |pages=147–57 |doi=10.1212/CPJ.0000000000000834 |pmid=33842068 |url=https://pubmed.ncbi.nlm.nih.gov/33842068/ |access-date=1 May 2021}}</ref> described to date in the medical literature. |
'''Facial onset sensory and motor neuronopathy''', often abbreviated '''FOSMN''', is a rare disorder of the [[nervous system]] in which sensory and motor [[Spinal nerve|nerves]] of the face and limbs progressively degenerate over a period of months to years. This degenerative process, the cause of which is unknown, eventually results in sensory and motor symptoms — the former consisting mainly of paresthesia followed by numbness, and the latter in muscle weakness, atrophy, and eventual paralysis. In many ways, it is reminiscent of the much better known condition '''[[amyotrophic lateral sclerosis]]''', with which it is closely related. There is no cure; treatment is supportive. Life expectancy may be shortened by respiratory complications arising from weakness of the muscles that aid breathing and swallowing. It was first described in four patients by Vucic and colleagues<ref name="Vucic 2006">{{cite journal |last1=Vucic |first1=S |display-authors=etal |title=Facial onset sensory and motor neuronopathy (FOSMN syndrome): a novel syndrome in neurology |journal=Brain |date=Dec 2006 |volume=129 |issue=12 |pages=3384–90 |doi=10.1093/brain/awl258 |pmid=17012296 |url=https://pubmed.ncbi.nlm.nih.gov/17012296/ |access-date=1 May 2021|doi-access=free }}</ref> working at the [[Massachusetts General Hospital]] in the [[United States of America|United States]]; subsequent reports from the United Kingdom,<ref name="Broad 2015">{{cite journal |last1=Broad |first1=R |last2=Leigh |first2=PN |title=Recognising facial onset sensory motor neuronopathy syndrome: insight from six new cases |journal=Pract Neurol |date=Aug 2015 |volume=15 |issue=4 |pages=293–7 |doi=10.1136/practneurol-2014-000984 |pmid=25991863 |url=https://pubmed.ncbi.nlm.nih.gov/25991863/ |access-date=1 May 2021}}</ref> Europe and Asia<ref name="Sonoda 2013">{{cite journal |last1=Sonoda |first1=K |display-authors=etal |title=TAR DNA-binding protein 43 pathology in a case clinically diagnosed with facial-onset sensory and motor neuronopathy syndrome: an autopsied case report and a review of the literature |journal=J Neurol Sci |date=Sep 2013 |volume=332 |issue=1-2 |pages=148–53 |doi=10.1016/j.jns.2013.06.027 |pmid=23849263 |url=https://pubmed.ncbi.nlm.nih.gov/23849263/ |access-date=1 May 2021}}</ref> point to a global incidence of the disease. It is thought to be exceptionally rare, with only approximately 100 individuals<ref name="de Boer 2021">{{cite journal |last1=de Boer |first1=EMJ |display-authors=etal|title=Facial Onset Sensory and Motor Neuronopathy: New Cases, Cognitive Changes, and Pathophysiology |journal=Neurol Clin Pract |date=Apr 2021 |volume=11 |issue=2 |pages=147–57 |doi=10.1212/CPJ.0000000000000834 |pmid=33842068 |url=https://pubmed.ncbi.nlm.nih.gov/33842068/ |access-date=1 May 2021|doi-access=free }}</ref> described to date in the medical literature. |
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==References== |
==References== |
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Revision as of 20:35, 5 May 2021
Facial onset sensory and motor neuronopathy, often abbreviated FOSMN, is a rare disorder of the nervous system in which sensory and motor nerves of the face and limbs progressively degenerate over a period of months to years. This degenerative process, the cause of which is unknown, eventually results in sensory and motor symptoms — the former consisting mainly of paresthesia followed by numbness, and the latter in muscle weakness, atrophy, and eventual paralysis. In many ways, it is reminiscent of the much better known condition amyotrophic lateral sclerosis, with which it is closely related. There is no cure; treatment is supportive. Life expectancy may be shortened by respiratory complications arising from weakness of the muscles that aid breathing and swallowing. It was first described in four patients by Vucic and colleagues[1] working at the Massachusetts General Hospital in the United States; subsequent reports from the United Kingdom,[2] Europe and Asia[3] point to a global incidence of the disease. It is thought to be exceptionally rare, with only approximately 100 individuals[4] described to date in the medical literature.
References
- ^ Vucic, S; et al. (Dec 2006). "Facial onset sensory and motor neuronopathy (FOSMN syndrome): a novel syndrome in neurology". Brain. 129 (12): 3384–90. doi:10.1093/brain/awl258. PMID 17012296. Retrieved 1 May 2021.
- ^ Broad, R; Leigh, PN (Aug 2015). "Recognising facial onset sensory motor neuronopathy syndrome: insight from six new cases". Pract Neurol. 15 (4): 293–7. doi:10.1136/practneurol-2014-000984. PMID 25991863. Retrieved 1 May 2021.
- ^ Sonoda, K; et al. (Sep 2013). "TAR DNA-binding protein 43 pathology in a case clinically diagnosed with facial-onset sensory and motor neuronopathy syndrome: an autopsied case report and a review of the literature". J Neurol Sci. 332 (1–2): 148–53. doi:10.1016/j.jns.2013.06.027. PMID 23849263. Retrieved 1 May 2021.
- ^ de Boer, EMJ; et al. (Apr 2021). "Facial Onset Sensory and Motor Neuronopathy: New Cases, Cognitive Changes, and Pathophysiology". Neurol Clin Pract. 11 (2): 147–57. doi:10.1212/CPJ.0000000000000834. PMID 33842068. Retrieved 1 May 2021.
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