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Facial onset sensory and motor neuronopathy

MedGen UID:: 1374397
•Concept ID:: C4509818
•: Disease or Syndrome

Synonyms:	facial onset sensorimotor neuronopathy syndrome; facial onset sensory and motor neuronopathy; Facial onset sensory and motor neuronopathy syndrome; facial onset sensory and motor neuronopathy syndrome; FOSMN (facial onset sensory and motor neuronopathy) syndrome; FOSMN syndrome
SNOMED CT:	Facial onset sensory and motor neuronopathy syndrome (723306004); FOSMN syndrome (723306004); FOSMN (facial onset sensory and motor neuronopathy) syndrome (723306004)
Modes of inheritance:	Unknown inheritance MedGen UID: 989040 •Concept ID: CN307042 • Finding Source: Orphanet Hereditary clinical entity whose mode of inheritance is unknown. See: This record Unknown inheritance (Orphanet)

Monarch Initiative:	MONDO:0019405
Orphanet:	ORPHA85162

Definition

Facial onset sensory and motor neuronopathy is characterized initially by paresthesia and numbness in the region of the trigeminal nerve distribution, which later progresses to involve the scalp, neck, upper trunk and upper limbs. Onset of motor manifestations occurs later with cramps, fasciculations, dysphagia, dysarthria, muscle weakness and atrophy. This syndrome has been described in four males and appears to be a slowly progressive neurodegenerative disease. [from SNOMEDCT_US]

Term Hierarchy

GTR
MeSH

CClinical test, RResearch test, OOMIM, GGeneReviews, VClinVar

CROGVFacial onset sensory and motor neuronopathy

Inherited neurodegenerative disorder
- Facial onset sensory and motor neuronopathy

Recent clinical studies

Etiology

Facial Onset Sensory and Motor Neuronopathy-Like Syndrome: A Case Report.

Bruss DM, Venkataraman P, Mozaffar T
J Clin Neuromuscul Dis 2022 Sep 1;24(1):55-58. doi: 10.1097/CND.0000000000000402. PMID: 36005474

Atypical Motor Neuron Disease variants: Still a diagnostic challenge in Neurology.

Pinto WBVR, Debona R, Nunes PP, Assis ACD, Lopes CG, Bortholin T, Dias RB, Naylor FGM, Chieia MAT, Souza PVS, Oliveira ASB
Rev Neurol (Paris) 2019 Apr;175(4):221-232. Epub 2019 Mar 4 doi: 10.1016/j.neurol.2018.04.016. PMID: 30846210

Oral phase dysphagia in facial onset sensory and motor neuronopathy.

Watanabe M, Shiraishi W, Yamasaki R, Isobe N, Sawatsubashi M, Yasumatsu R, Nakagawa T, Kira JI
Brain Behav 2018 Jun;8(6):e00999. Epub 2018 May 21 doi: 10.1002/brb3.999. PMID: 29781209 Free PMC Article

Amyotrophic lateral sclerosis causes small fiber pathology.

Dalla Bella E, Lombardi R, Porretta-Serapiglia C, Ciano C, Gellera C, Pensato V, Cazzato D, Lauria G
Eur J Neurol 2016 Feb;23(2):416-20. doi: 10.1111/ene.12936. PMID: 26806218

Clinical features and follow-up of four new cases of facial-onset sensory and motor neuronopathy.

Fluchere F, Verschueren A, Cintas P, Franques J, Serratrice J, Weiller PJ, Azulay JP, Pouget J, Attarian S
Muscle Nerve 2011 Jan;43(1):136-40. doi: 10.1002/mus.21884. PMID: 21108321

See all (6)

Diagnosis

Facial onset sensory and motor neuronopathy (FOSMN syndrome): Cases series and systematic review.

Hu N, Zhang L, Yang X, Fu H, Cui L, Liu M
Neurol Sci 2023 Jun;44(6):1969-1978. Epub 2023 Mar 3 doi: 10.1007/s10072-023-06703-1. PMID: 36864244

Facial Onset Sensory and Motor Neuronopathy-Like Syndrome: A Case Report.

Bruss DM, Venkataraman P, Mozaffar T
J Clin Neuromuscul Dis 2022 Sep 1;24(1):55-58. doi: 10.1097/CND.0000000000000402. PMID: 36005474

Electrodiagnostic findings in facial onset sensory motor neuronopathy (FOSMN).

De Oliveira HM, Silsby M, Jaiser SR, Lai HM, Pavey N, Kiernan MC, Williams TL, Vucic S, Baker MR
Clin Neurophysiol 2022 Aug;140:228-238. Epub 2022 May 10 doi: 10.1016/j.clinph.2022.04.020. PMID: 35599162

Atypical Motor Neuron Disease variants: Still a diagnostic challenge in Neurology.

Facial onset sensory and motor neuronopathy.

Zheng Q, Chu L, Tan L, Zhang H
Neurol Sci 2016 Dec;37(12):1905-1909. Epub 2016 Jul 29 doi: 10.1007/s10072-016-2686-7. PMID: 27473302

See all (15)

Therapy

Recognising facial onset sensory motor neuronopathy syndrome: insight from six new cases.

Broad R, Leigh PN
Pract Neurol 2015 Aug;15(4):293-7. Epub 2015 May 19 doi: 10.1136/practneurol-2014-000984. PMID: 25991863

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.

Sonoda K, Sasaki K, Tateishi T, Yamasaki R, Hayashi S, Sakae N, Ohyagi Y, Iwaki T, Kira J
J Neurol Sci 2013 Sep 15;332(1-2):148-53. Epub 2013 Jul 9 doi: 10.1016/j.jns.2013.06.027. PMID: 23849263

IVIG- responsive multiple cranial neuropathy: a pharyngo-facial variant of Guillain-Barré syndrome.

Unal-Cevik I, Onal MZ, Odabasi Z, Tan E
Acta Neurol Belg 2009 Dec;109(4):317-21. PMID: 20120214

See all (3)

Prognosis

Facial onset sensory and motor neuronopathy (FOSMN syndrome): Cases series and systematic review.

Hu N, Zhang L, Yang X, Fu H, Cui L, Liu M
Neurol Sci 2023 Jun;44(6):1969-1978. Epub 2023 Mar 3 doi: 10.1007/s10072-023-06703-1. PMID: 36864244

New findings in facial-onset sensory and motor neuronopathy (FOSMN) syndrome.

Pinto WBVR, Naylor FGM, Chieia MAT, de Souza PVS, Oliveira ASB
Rev Neurol (Paris) 2019 Apr;175(4):238-246. Epub 2018 Oct 5 doi: 10.1016/j.neurol.2018.04.010. PMID: 30293881

Oral phase dysphagia in facial onset sensory and motor neuronopathy.

Facial onset sensory and motor neuronopathy.

Zheng Q, Chu L, Tan L, Zhang H
Neurol Sci 2016 Dec;37(12):1905-1909. Epub 2016 Jul 29 doi: 10.1007/s10072-016-2686-7. PMID: 27473302

Recognising facial onset sensory motor neuronopathy syndrome: insight from six new cases.

Broad R, Leigh PN
Pract Neurol 2015 Aug;15(4):293-7. Epub 2015 May 19 doi: 10.1136/practneurol-2014-000984. PMID: 25991863

See all (10)

Clinical prediction guides

Facial onset sensory and motor neuronopathy (FOSMN syndrome): Cases series and systematic review.

Hu N, Zhang L, Yang X, Fu H, Cui L, Liu M
Neurol Sci 2023 Jun;44(6):1969-1978. Epub 2023 Mar 3 doi: 10.1007/s10072-023-06703-1. PMID: 36864244

Taste disorder in facial onset sensory and motor neuronopathy: a case report.

Ohashi N, Nonami J, Kodaira M, Yoshida K, Sekijima Y
BMC Neurol 2020 Feb 29;20(1):71. doi: 10.1186/s12883-020-01639-x. PMID: 32113480 Free PMC Article

Atypical Motor Neuron Disease variants: Still a diagnostic challenge in Neurology.

New findings in facial-onset sensory and motor neuronopathy (FOSMN) syndrome.

Pinto WBVR, Naylor FGM, Chieia MAT, de Souza PVS, Oliveira ASB
Rev Neurol (Paris) 2019 Apr;175(4):238-246. Epub 2018 Oct 5 doi: 10.1016/j.neurol.2018.04.010. PMID: 30293881

Oral phase dysphagia in facial onset sensory and motor neuronopathy.

See all (7)