Common Presentations of ALS with FTD

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Partners in FTD Care
Spring 2018

ALS can present in myriad ways, with weakness in any segment of the body. Roughly 75% of patients present with limb weakness, 25% present with bulbar weakness (trouble speaking or swallowing), and a small number present with respiratory insufficiency. Similarly, FTD can manifest early on as a predominantly behavioral syndrome or as a disorder of language; these patterns of disease can look very different. Both ALS and FTD can have variable disease courses as well.

Unsurprisingly, ALS with FTD presents and develops in many different ways – no two cases are the same. Here are some examples of persons diagnosed:

  • A 58-year-old man develops walking difficulty due to stiffness in his leg. His wife reports that subtle personality changes, which she first noticed five years ago, have now begun to cause problems at his job.
  • A 61-year-old man, already diagnosed with severe bvFTD, is referred by a cognitive clinic for further neuromuscular evaluation because of new onset right-hand weakness.
  • A 53-year-old woman with dysarthria and hand weakness, whose sister has ALS, shows a lack of insight into her disability along with inappropriate public affection to her husband in the doctor’s office during an examination.
  • A 69-year-old man presented with dysarthria and over the course of two years began to exhibit anarthria (no audible speech) and developed a need for a feeding tube and power wheelchair. He then developed a language disorder that manifested in his writing: He confused “yes” and “no,” showed a lack of grammar ability and made frequent spelling errors.

ALS is unpredictable – it can first manifest in any part of the body, then spread in a variety of patterns. One common presentation is when weakness starts in one arm: It will then spread to the opposite arm, then to the leg on the side of the body where it initially started. But it is harder to predict when respiratory or bulbar muscles may become involved. In both ALS and FTD, the speed of progression can be variable and is hard to predict. The best predictor of rate of progression is the patient’s individual history, as rate of progression tends to remain constant within an individual.

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