Frontotemporal lobar degeneration
Alerts and Notices
Synopsis
These disorders are characterized by behavioral changes and/or language disturbance. Behavior can be impulsive or apathetic, and affected patients may exhibit inappropriate social behavior and disinhibition, lack of empathy, changes in food preference, hypersexuality, compulsive or perseverative behaviors, agitation, blunted emotions, neglect of personal hygiene, distractibility, or lack of motivation. Language disturbance can include difficulty with speech production or comprehension. Memory and visual spatial skills are typically intact. Eventually, there can be global cognitive impairment and motor decline, such as parkinsonism or motor neuron disease.
FTD can also overlap with cortical basal degeneration, progressive supranuclear palsy, frontal variant Alzheimer disease, and motor neuron disease. Age of onset is usually 50-65 years. Prognosis is poor with rapid, steady deterioration and death within 8-15 years of symptom onset, typically due to secondary infections, such as pneumonia. FTD affects men and women equally.
Patients with this disorder may have an increased risk of various general medical conditions.
Codes
ICD10CM:G31.09 – Other frontotemporal dementia
SNOMEDCT:
230270009 – Frontotemporal dementia
702426001 – GRN-related frontotemporal dementia
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Diagnostic Pearls
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Differential Diagnosis & Pitfalls
- Alzheimer disease
- Chronic alcohol abuse (see alcohol use disorder, Wernicke-Korsakoff syndrome)
- Depression
- Bipolar disorder
- Schizophrenia
- Meningioma
- Glioma
- Chronic traumatic encephalopathy / dementia pugilistica (see dementia)
- Vascular dementia
- Parkinson disease
- Corticobasal syndrome
- Progressive supranuclear palsy
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Last Reviewed:04/17/2019
Last Updated:04/03/2023
Last Updated:04/03/2023