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Facial Onset Sensory and Motor Neuropathy syndrome (FOSMN) is an extremely rare disease characterised by sensory and motor loss beginning in the face and spreading to involve an increasingly larger area including the upper arms. Details of the disease, and in particular its aetiology, are currently subject to debate, mainly because FOSMN syndrome is so rare. FOSMN was first described in four patients in 2006 〔S. Vucic, D. Tian, P. Siao Tick Chong, M. E. Cudkowicz, E. T. Hedley-Whyte, and D. Cros Facial onset sensory and motor neuronopathy (FOSMN syndrome): a novel syndrome in neurology Brain (2006) 129(12): 3384-3390〕 and subsequently in a further six patients 〔Isoardo, G. and Troni, W. (2008), Sporadic bulbospinal muscle atrophy with facial-onset sensory neuropathy. Muscle Nerve, 37: 659–662. 〕〔Toshihiro Hokonohara, Hiroshi Shigeto, Yuji Kawano, Yasumasa Ohyagi, Michiya Uehara, Jun-ichi Kira. Facial onset sensory and motor neuronopathy (FOSMN) syndrome responding to immunotherapies. Journal of the Neurological Sciences - 15 December 2008 (Vol. 275, Issue 1, Pages 157-158, )〕〔Fluchere, F., Verschueren, A., Cintas, P., Franques, J., Serratrice, J., Weiller, P. J., Azulay, J. P., Pouget, J. and Attarian, S. (2011), Clinical features and follow-up of four new cases of facial-onset sensory and motor neuronopathy. Muscle Nerve, 43: 136–140. 〕 but so far, these ten represent the only reported cases. ==Signs and symptoms== Sensory loss develops over the distribution of the trigeminal nerve and then spreads to involve the scalp and neck. Ultimately paraesthesiae spreads down to the arms. Sensory symptoms are followed by motor deficits which also begin in the face. Motor manifestations are characterised by weakness, atrophy, cramping and fasciculations. Difficulty swallowing and speaking may also develop. Neurophysiology studies show a generalized sensory motor neuronopathy which is most severe cranially.〔 These features lead FOSMN syndrome to be classed as one of the 'syringomyelia-like' syndromes, a group which also includes Tangier disease. 抄文引用元・出典: フリー百科事典『 ウィキペディア（Wikipedia）』 ■ウィキペディアで「Facial Onset Sensory Motor Neuropathy syndrome」の詳細全文を読む スポンサード リンク
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