PMC:3654953 / 10236-11439 JSONTXT

{"target":"https://pubannotation.org/docs/sourcedb/PMC/sourceid/3654953","sourcedb":"PMC","sourceid":"3654953","source_url":"https://www.ncbi.nlm.nih.gov/pmc/3654953","text":"Table 1 Clinical and instrumental assessments\n  Pt1 Pt2\nCurrent age 68 60\nAge at onset 55 46\nDisease duration at the time of examinations, years 13 14\nInstrumental assessment *\nCognitive: MMSE score 16/30 30/30\nEMG 0 Mild motor axonal neuropathy (1)\nMEPs Bulbar/UL/LL N.A./0/0 1/1/3\nSEPs UL/LL 1/1 0/1\nBAEPs NA NA\nVEPs 0 0\nAutonomic testing 0 0\nClinical scoring **\nDysarthria/dysphagia 2 2\nGait abnormalities 1 3\nSpasticity 0 3\nAxial Ataxia 1 0\nLimb dysmetria 2 0\nLimb weakness 0 2\nMuscle wasting 0 1\nSphincter function 2 0\n* For neurophysiological assessments, values are graded as 0 (normal), 1 (abnormality not exceeding 25% of upper/lower normal ranges), 2 (between 25 and 50%), and 3 (beyond 50%).\n** For clinical dysfunctions, abnormalities are graded as 0 (normal), 1 (only objective signs), 2 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