case_images: PMC3843933_01
case_images: EMG of the upper and lower limbs of our patient. a EMG of the left biceps brachii muscle: manual muscle testing 2/5, fibrillation potentials and positive sharp waves at rest, polyphasic small-amplitude short-duration motor unit potentials with movement (myopathic potentials). b EMG of the left flexor digitorum sublimis muscle: manual muscle testing 4/5, absence of potentials or positive sharp waves at rest, polyphasic high-amplitude and high-frequency potentials with movement (neuropathic potentials). c EMG of the left flexor digitorum profundus muscle: manual muscle testing 2/5, fibrillation potentials and positive sharp waves at rest, polyphasic small-amplitude short-duration motor unit potentials with movement (myopathic potentials). d EMG of the left vastus lateralis muscle: manual muscle testing 3/5, fibrillation potentials and positive sharp waves at rest, polyphasic high-amplitude and high-frequency potentials with movement (neuropathic potentials). e EMG of the left tibialis anterior muscle: manual muscle testing 1/5, severe fibrillation potentials and positive sharp waves at rest, rare polyphasic small-amplitude short-duration motor unit potentials with movement (myopathic potentials). f EMG of the left extensor digitorum brevis muscle: manual muscle testing 4/5, absence of potentials or positive sharp waves at rest, polyphasic high-amplitude and high-frequency potentials with movement (neuropathic potentials).
case_images: crn-0005-0187-g01.jpg
case_images: PMC3843933_01_crn-0005-0187-g01.jpg
case_images: F1
case_images: An electromyography (EMG) (fig. 1) showed fibrillation potentials and positive sharp waves at rest, and myopathic or neurogenic features according to the muscles examined: polyphasic small-amplitude short-duration motor unit potentials and polyphasic long-duration with slow-frequency motor unit potentials were found together in the EMG study.
case_images: T2-FLAIR-weighted brain MRI of our proband. a Axial image throughout the hippocampal structures: bilateral temporal atrophy. b Axial image throughout the frontal lobes: bilateral frontal atrophy. c Sagittal image: frontal atrophy. d Coronal image: bilateral temporal atrophy.
case_images: crn-0005-0187-g02.jpg
case_images: PMC3843933_01_crn-0005-0187-g02.jpg
case_images: F2
case_images: Cerebral magnetic resonance imaging (MRI) was performed at 54 years of age and revealed frontal and internal temporal atrophy (fig. 2).
case_images: FDG PET scan in our proband: severe bilateral hypometabolism, involving especially the frontal and temporal lobes.
case_images: crn-0005-0187-g03.jpg
case_images: PMC3843933_01_crn-0005-0187-g03.jpg
case_images: F3
case_images: Fluorodeoxyglucose (FDG) positron emission tomography (PET) scan showed severe bilateral hypometabolism, involving especially the frontal and temporal lobes (fig. 3).
case_images: PMC3843933_02