Propriospinal myoclonus as a sequela of Guillain-Barré syndrome Teaching NeuroImages Neurology Resident and Fellow Section.

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Propriospinal myoclonus as a sequela of Guillain-Barré syndrome Teaching NeuroImages Neurology Resident and Fellow Section

Vignette A previously healthy 27-year-old female developed Guillain-Barré syndrome (GBS) with severe tetraplegia, requiring immunoglobulins. During remission, non-rhythmic, stimulus-sensitive abdominal jerks, propagating to the hips, appeared, worsening in supine position. Bedarf et al.

Imaging Bedarf et al. Figure 1 Surface multichannel electromyography Propagation pattern of propriospinal myoclonus, recorded by surface multichannel EMG of left-sided muscles. Myoclonic jerks have a duration of 200-400 ms. Note the first jerk of the rectus abdominis muscle, driven by the Th8 myelomere (myoclonic generator), followed by a typical rostral and caudal spread involving the iliopsoas and pectoralis muscles. Bedarf et al.

Imaging Bedarf et al. Figure 2 Spinal Imaging T2-weighted sagittal (A) and axial (D) spinal cord MRI without focal pathology at Th8, and spinal diffusion tensor imaging with fiber tracking (B, C) without microstructural abnormalities. FA, fractional anisotropy values were lowest within segment Th8, compatible with an altered axonal integrity (B); ADC, apparent diffusion coefficient were normal. Bedarf et al.

Video Bedarf et al. Video Clinical and electrophysiological presentation of PSM Clinical presentation of non-rhythmic myoclonic jerks, originating from the abdominal muscles with involvement of the iliopsoas and pectoralis muscle. Surface electromyography reveals typical slow propagation pattern from the myoclonic generator at Th8 in rostral and caudal direction. Bedarf et al.

Propriospinal myoclonus as a sequela of Guillain-Barré syndrome Surface electromyography confirmed propriospinal myoclonus (PSM)1 originating from Th8 (Figure 1, Video). Myoclonus ceased with levetiracetam. Normal backward averaging, somatosensory evoked potentials, and absent Bereitschaftspotential argued against cortical or functional origins. PSM is mainly based on myelon lesions1. Occurrence as a sequela of GBS is rare, though myoclonus through radiculitis was described before2. Although spinal imaging was within normal range (Figure 2), we believe ephaptic transmission following segmental inflammatory demyelination to be the likely cause here. Literature 1. Roze E, Bounolleau P, Ducreux D, Cochen V, Leu-Semenescu S, Beaugendre Y, Lavallard-Rousseau MC, Blancher A, Bourdain F, Dupont P, Carluer L, Verdure L, Vidailhet M, Apartis E. Propriospinal myoclonus revisited: Clinical, neurophysiologic, and neuroradiologic findings. Neurology. 2009 Apr 14;72(15):1301-9. doi: 10.1212/WNL.0b013e3181a0fd50. 2. Miki Y, Tomiyama M, Ueno T, Haga R, Nishijima H, Arai A, Suzuki C, Nunomura J, Baba M. Radicular myoclonus in a patient with Guillain-Barré Syndrome. Intern Med. 2012;51(15):2021-3. Epub 2012 Aug 1 Bedarf et al.