FTD-ALS
In 2011, researchers identified a variant in the C9orf72 gene as the most common cause of both genetic FTD and genetic amyotrophic lateral sclerosis (ALS). Previously, ALS was thought to be purely a movement disorder, while FTD was considered purely a cognitive or behavioral form of dementia.
FTD and ALS can occur in the same person, a clinical subtype known as FTD-ALS. Describing this unique clinical syndrome has been an area of active research, and our knowledge of its underlying genetics, pathology, and clinical features is increasing.
The current understanding of ALS with language deficits is still incomplete. Both the nonfluent agrammatic variant and the semantic variant of primary progressive aphasia, an FTD disorder that affects language, have been reported in association with ALS.
FTD-ALS Symptoms
In persons with FTD-ALS, symptoms of one of the FTD disorders (usually behavioral variant FTD) are often first to appear, and can include changes in behavior, personality, and language. Motor symptoms that are more typically associated with ALS – including difficulty walking, swallowing, and/or using one’s hands – develop later. The former symptoms are caused by the degeneration of the brain’s frontal and temporal lobes, while the latter are a result of the deterioration of the nerves in the spinal cord that communicate with muscles in the rest of the body.
FTD-ALS Diagnostic Checklist
Not all physicians know about FTD, so it is often misdiagnosed or not diagnosed at all. AFTD developed a checklist to help identify red flags for FTD-ALS that you can bring to your neurologist. You can download the checklist and indicate which symptoms you or your loved one is experiencing on the front side of the document. The back of the document is designed to help your physician better understand FTD diagnostic criteria.
Webinars
Additional webinars:
- Bringing the ALS-FTD Clinical Experience into Focus (December 2019 webinar)
- Managing ALS/FTD & Movement Disorders (AFTD Education Conference 2019 session)