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Pract Neurol. 2015 Aug;15(4):293-7. doi: 10.1136/practneurol-2014-000984. Epub 2015 May 19.

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

Author information

1
Neurology Department, Brighton and Sussex medical School, Trafford Centre for Medical Research, University of Sussex, Brighton, East Sussex, UK.

Abstract

Facial onset sensory and motor neuronopathy (FOSMN) was first described in 2006 as an apparently sporadic neurodegenerative disease. Thirty cases have been reported to date. We summarise six new cases, highlighting the key clinical aspects of FOSMN and how to differentiate it from motor neurone disease (amyotrophic lateral sclerosis). Typically, patients present with slowly evolving numbness of the face followed by bulbar and proximal (neck and arm) weakness. However, one of our patients presented with a motor syndrome and his abnormal blink reflex studies provided a useful diagnostic clue. This extends the spectrum of the syndrome and emphasises that FOSMN should be considered in the differential diagnosis of motor neurone disease. We discuss the pathophysiology, diagnosis, prognosis and management considerations of FOSMN.

KEYWORDS:

ALS; Blink reflex; FOSMN; MND - mimic; Trigeminal sensory nucleus

PMID:
25991863
DOI:
10.1136/practneurol-2014-000984
[Indexed for MEDLINE]

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