Advances in the understanding of sensory neuronopathies

Evolving clinical phenotypes and causes linked to sensory neuronopathy.

Sensory neuronopathies (or ganglionopathies) represent a rare group of peripheral nerve disorders characterised by degeneration or dysfunction of dorsal root ganglia (DRG) or trigeminal sensory neurons.1 2 Large sensory fibres are typically affected, resulting in loss of proprioception and pseudoathetoid posturing of limbs, along with loss of vibration, touch and deep tendon reflects. In addition, severe gait ataxia and a positive Romberg’s sign are accompanying clinical features that result in marked disability. While motor neurons are preserved, ‘apparent’ clinical weakness secondary to loss of proprioception is often evident. Less frequently, small DRGs are only affected leading to burning, paraesthesia and stabbing pains, along with impaired pinprick and temperature sensations. In contrast to axonal neuropathies, sensory neuropathies are non-length dependent, and consequently may involve regions innervated by specific sensory neurons, such as the scalp or face, oral mucosa,…

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