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On the clinico-neurophysiological side, understanding of SMS progressed with the description of abnormal excitotoxicity and paroxysmal autonomic dysfunction [13,14,15], recognition of clinical heterogeneity [4, 7,8,9], and subdividing SMS into stiff trunk (man) syndrome, stiff limb syndrome and progressive encephalomyelitis with rigidity [16]

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The reviewed studies are summarised in Table 1

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