Motor neurone disease: diagnostic pitfalls

Imaging of patients with dysphagia greater than dysarthria. (a) Midline T2 weighted sagittal MR head. This patient presented with 12 months of increasing dysphagia without dysarthria. There were no abnormalities on examination. Extensive assessment by gastroenterology, ENT, upper gastro-intestinal surgery and investigation (endoscopic examination, barium swallow and blood work) had failed to yield a diagnosis. MR imaging was requested to exclude a structural lesion as a cause for dysphagia. The imaging demonstrated a large midline clival meningioma, which was successfully removed by surgery. The patient has a residual left facial palsy, but near normal swallow. (b) Representative non-enhanced CT brain head from a patient who presented with the abrupt onset of mild dysphasia and right-sided weakness, followed over the next 48 hours by complete aphagia. Although initial CT imaging demonstrated infarction of the left operculum, repeat CT imaging 24 hours later (shown here) demonstrated bilateral infarcts representing the opercular or Foix–Chavany–Marie-syndrome (dysphagia, dysarthria, drooling and facial weakness, often with limb sparing), which is seen as a result of stroke, infection and occasional neurodegenerative disease. Supportive treatment and temporary naso-gastric tube feeding resulted in a good functional recovery.