What is progressive bulbar palsy?

Progressive bulbar paralysis is a special form of ALS in which the motor symptoms occur in the tongue and pharyngeal region (bulbar region) and remain limited to this region for long periods of time (up to several years). The transition from paralysis to the hands, arms and legs takes place with a long delay. In the first months and years of the course of the disease, the disease progresses in the bulbar region (increase in speech and swallowing disorders), while muscle weakness (paresis) or muscle stiffness (spasticity) are not detectable in other parts of the body.

ALS is not completely restricted to the bulbar region, even in progressive bulbar paralysis: After years of isolated involvement in the bulbar region, the later course of the disease leads to slowly progressive involvement of the trunk muscles (weakness of the head muscles), arms and lower extremities. Due to the severe limitation of the symptoms to the bulbar region, the diagnosis of this particular form of ALS can be difficult and delayed.

Less than 5 % of all people with ALS show the disease course of progressive bulbar paralysis.