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Dysphagia due to Chiari I malformation mimicking ALS
  1. J Gamez1,
  2. E Santamarina1,
  3. A Codina1
  1. 1Department of Neurology, Hospital General Vall d’Hebron, 08035 Barcelona, Spain
  1. Correspondence to:
 Dr Josep Gamez; 

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We read with interest the article by Paulig and Prosiegel1 concerning a patient initially diagnosed with amyotrophic lateral sclerosis (ALS), who had suffered from progressive swallowing difficulties, fibrillations, and tongue atrophy for a year, in the context of a flaccid bulbar palsy. Brain and spinal MRI showed a Chiari I malformation with descent of the cerebellar tonsils. The authors accompany their article with an illustration of the patient, showing extensive bilateral paresis and atrophy of the tongue. The article notes the importance of carrying out an MRI examination on those patients who show bulbar palsy mimicking bulbar onset type ALS in order to rule out Chiari I malformation.

Chiari I malformation has also recently been reported as being associated with bulbar onset ALS.2 Nevertheless, the marked improvement in the dysphagia after neurosurgery suggests that Chiari I was the cause of the …

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