Tracheostomy and salivary gland radiotherapy for severe sialorrhoea in motor neurone disease.

The management of excessive secretions in patients with motor neurone disease can be challenging. In this paper, we highlight the main issues from the perspectives of a patient, specialist nurse and neurologist and the importance of a multidisciplinary approach.

The post-irradiation lower motor neuron syndrome neuronopathy or radiculopathy?

It is not known whether the post-irradiation lower motor neuron syndrome results from radiation damage to motor neuron cell bodies or from damage to the nerve roots of the cauda equina. We studied six cases who had presented with testicular neoplasms, subsequently undergoing irradiation that encompassed inter alia para-aortic nodes with co-irradiation of the distal spinal cord and cauda equina. A predominantly motor disorder affecting the legs ensued after variable and often prolonged latencies (3-25 years). However, all patients also developed mild sensory features either initially or on prolonged follow-up. Sural sensory nerve action potentials (SNAPs) were normal in five. Mild sphincter symptoms occurred in three of five surviving cases after a mean of 7.9 years. MRI showed gadolinium enhancement of the cauda equina in two of three patients. The first reported neuropathological study, uncomplicated by metastatic disease, of the conus and cauda equina was performed in one patient who died. This showed a radiation-induced vasculopathy of the proximal spinal roots, with preservation of motor neuronal cell bodies and spinal cord architecture. These clinical, radiological, neurophysiological and pathological findings all point to a predominantly, but not exclusively, motor radiculopathy affecting the irradiated portion of the cauda equina proximal to the dorsal root ganglia. Radiation exposure exceeded 40 Gy both in our series and in previous reports. The natural history of this disorder is one of relentless deterioration occasionally punctuated by 1-2-year periods of stability. Post-irradiation lumbosacral radiculopathy is a more accurate name for this condition.