Luciana Pelosi1, Ruth Leadbetter2, Eoin Mulroy3, Andrew M Chancellor1, Stuart Mossman2, Richard Roxburgh3. 1. Department of Neurology and Clinical Neurophysiology, Bay of Plenty District Health Board, Tauranga, 3143, New Zealand. 2. Capital and Coast District Health Board, Wellington, New Zealand. 3. Auckland District Health Board, Auckland, New Zealand.
Abstract
INTRODUCTION: We report preliminary findings of nerve ultrasound in patients with cerebellar ataxia neuropathy vestibular areflexia syndrome (CANVAS) who have sensory impairment due to dorsal root ganglionopathy. METHODS: The ultrasound cross-sectional area (CSA) of median and ulnar nerves of 7 CANVAS patients was compared with 7 age- and gender-matched controls and with the mean CSA of our reference population. RESULTS: The nerve CSA of CANVAS patients was significantly smaller than that of controls at all sites (P < 0.01). All but 1 individual measurement of CSA at the mid-forearm level in the CANVAS patients fell outside the normal control range and was >2 standard deviations below the reference mean. CONCLUSIONS: The small nerves in CANVAS probably reflect nerve thinning from axonal loss secondary to ganglion cell loss. Our data show a role for ultrasound in the diagnosis of CANVAS ganglionopathy. This may also be applicable to ganglionopathy from other causes. Muscle Nerve 56: 160-162, 2017.
INTRODUCTION: We report preliminary findings of nerve ultrasound in patients with cerebellar ataxia neuropathy vestibular areflexia syndrome (CANVAS) who have sensory impairment due to dorsal root ganglionopathy. METHODS: The ultrasound cross-sectional area (CSA) of median and ulnar nerves of 7 CANVAS patients was compared with 7 age- and gender-matched controls and with the mean CSA of our reference population. RESULTS: The nerve CSA of CANVAS patients was significantly smaller than that of controls at all sites (P < 0.01). All but 1 individual measurement of CSA at the mid-forearm level in the CANVAS patients fell outside the normal control range and was >2 standard deviations below the reference mean. CONCLUSIONS: The small nerves in CANVAS probably reflect nerve thinning from axonal loss secondary to ganglion cell loss. Our data show a role for ultrasound in the diagnosis of CANVAS ganglionopathy. This may also be applicable to ganglionopathy from other causes. Muscle Nerve 56: 160-162, 2017.
Authors: Sarah J Beecroft; Andrea Cortese; Roisin Sullivan; Wai Yan Yau; Zoe Dyer; Teddy Y Wu; Eoin Mulroy; Luciana Pelosi; Miriam Rodrigues; Rachael Taylor; Stuart Mossman; Ruth Leadbetter; James Cleland; Tim Anderson; Gianina Ravenscroft; Nigel G Laing; Henry Houlden; Mary M Reilly; Richard H Roxburgh Journal: Brain Date: 2020-09-01 Impact factor: 13.501