Progressive jerky movements in a 58 year old man

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Presentation transcript:

Progressive jerky movements in a 58 year old man Teaching NeuroImages Neurology Resident and Fellow Section © 2015 American Academy of Neurology

Vignette A 58 year old man presented with a 7 year history of progressive involuntary jerks of his arms and legs At neurological examination myoclonus was present in the trunk, arms and legs Eye movements examination revealed a remarkable ‘looping’ trajectory of the vertical saccades Eggink et al. © 2015 American Academy of Neurology

Video The video shows normal pursuit in all directions and saccadic horizontal eye movements are conducted. Vertical saccades show a ‘looping’ trajectory with an inability to produce pure vertical saccades, also referred to as the ‘round the houses’ sign. Spontaneous myoclonic jerks are present in rest, most prominent in the hands, exacerbating with posturing. Eggink et al. © 2015 American Academy of Neurology

The ‘round the houses’ sign as a clinical clue for Niemann-Pick Disease type C The ‘round the houses’ sign is an early indicator of vertical supranuclear gaze palsy (VSGP) Degeneration of the rostral interstitial nuclei of the medial longitudinal fasciculus leads to VSGP. It affects up to 81% of patients with Niemann-Pick Disease type C (NPC) This case illustrates that the ´round the houses’ sign can point towards the treatable diagnosis of NPC Quinn N. The “Round the houses” sign in progressive supranuclear palsy. Ann Neurol 1996;40:951. Salanso E, Umeh C, Rufa A, et al. Vertical supranuclear gaze palsy in Niemann-Pick type C disease. Neurol Sci 2012;33:1225-1232. Eggink et al. © 2015 American Academy of Neurology