Sensory neuron diseases

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Abstract from Lancet Neurol. 2005 Jun;4(6):349-61.

Sensory neuron diseases.

Sghirlanzoni A, Pareyson D, Lauria G.

Neuro-Oncology Unit, National Neurological Institute " Carlo Besta " ,

Milan, Italy.

Sensory neuron diseases (SND) are a distinct subgroup of peripheral-

nervous-system diseases, first acknowledged in 1948. Acquired SND

have a subacute or chronic course and are associated with systemic

immune-mediated diseases, vitamin intoxication or deficiency,

neurotoxic drugs, and life-threatening diseases such as cancer. SND

are commonly idiopathic but can be genetic diseases; the latter tend

to involve subtypes of sensory neurons and are associated with

certain clinical pictures.

The loss of sensory neurons in dorsal root ganglia causes the

degeneration of short and long peripheral axons and central sensory

projections in the posterior columns. This pathological process leads

to a pattern of sensory nerve degeneration that is not length

dependent and explains distinct clinical and neurophysiological

abnormalities.

Here we propose a comprehensive approach to the diagnosis of acquired

and hereditary SND and discuss clinical, genetic, neurophysiological,

neuroradiological, and neuropathological assessments.