Frontotemporal dementia
00:00 / 00:00
Videos
Notes
Frontotemporal dementia
Nervous system
Central and peripheral nervous system disorders
Amyotrophic lateral sclerosis
Neurofibromatosis
Sturge-Weber syndrome
Tuberous sclerosis
von Hippel-Lindau disease
Central nervous system disorders
Brain herniation
Broca aphasia
Kluver-Bucy syndrome
Wernicke aphasia
Wernicke-Korsakoff syndrome
Acoustic neuroma (schwannoma)
Adult brain tumors
Pediatric brain tumors
Pituitary adenoma
Ischemic stroke
Transient ischemic attack
Brain abscess
Cavernous sinus thrombosis
Creutzfeldt-Jakob disease
Encephalitis
Epidural abscess
Meningitis
Neonatal meningitis
Aqueductal stenosis
Cerebral palsy
Chiari malformation
Dandy-Walker malformation
Septo-optic dysplasia
Spina bifida
Spinocerebellar ataxia (NORD)
Syringomyelia
Tethered spinal cord syndrome
Alzheimer disease
Creutzfeldt-Jakob disease
Delirium
Frontotemporal dementia
Lewy body dementia
Normal pressure hydrocephalus
Vascular dementia
Acute disseminated encephalomyelitis
Central pontine myelinolysis
JC virus (Progressive multifocal leukoencephalopathy)
Multiple sclerosis
Transverse myelitis
Cavernous sinus thrombosis
Cluster headache
Idiopathic intracranial hypertension
Migraine
Tension headache
Trigeminal neuralgia
Arteriovenous malformation
Epidural hematoma
Intracerebral hemorrhage
Saccular aneurysm
Subarachnoid hemorrhage
Subdural hematoma
Essential tremor
Huntington disease
Opsoclonus myoclonus syndrome (NORD)
Parkinson disease
Restless legs syndrome
Torticollis
Early infantile epileptic encephalopathy (NORD)
Epilepsy
Febrile seizure
Brown-Sequard Syndrome
Cauda equina syndrome
Friedreich ataxia
Neurogenic bladder
Syringomyelia
Treponema pallidum (Syphilis)
Vitamin B12 deficiency
Concussion and traumatic brain injury
Peripheral nervous system disorders
Poliovirus
Spinal muscular atrophy
Charcot-Marie-Tooth disease
Guillain-Barre syndrome
Bell palsy
Trigeminal neuralgia
Carpal tunnel syndrome
Erb-Duchenne palsy
Klumpke paralysis
Sciatica
Thoracic outlet syndrome
Ulnar claw
Winged scapula
Lambert-Eaton myasthenic syndrome
Myasthenia gravis
Nervous system pathology review
Adult brain tumors: Pathology review
Central nervous system infections: Pathology review
Cerebral vascular disease: Pathology review
Congenital neurological disorders: Pathology review
Dementia: Pathology review
Demyelinating disorders: Pathology review
Headaches: Pathology review
Movement disorders: Pathology review
Neurocutaneous disorders: Pathology review
Neuromuscular junction disorders: Pathology review
Pediatric brain tumors: Pathology review
Seizures: Pathology review
Spinal cord disorders: Pathology review
Traumatic brain injury: Pathology review
Assessments
Frontotemporal dementia
Flashcards
0 / 5 complete
USMLE® Step 1 questions
0 / 2 complete
High Yield Notes
9 pages
Flashcards
Frontotemporal dementia
of complete
Questions
USMLE® Step 1 style questions USMLE
of complete
Image reproduced from Wikimedia Commons
Further testing reveals round cytoplasmic inclusions containing aggregates of tau proteins that stain with silver. Which of the following symptoms did this patient most likely exhibit before his death?
External References
First Aid
2022
2021
2020
2019
2018
2017
2016
Dementia
Pick disease p. 722
Pick bodies p. 538, 722
Transcript
Content Reviewers
Frontotemporal dementia, or FTD for short, refers to a degeneration of the frontal and temporal lobes of the brain. FTD is often mistakenly called Pick disease, or Pick’s disease. However, this is only a specific subtype of frontotemporal dementia, characterized by the presence of Pick bodies, which are tangles of abnormal nerve cell proteins called tau proteins.
Now, if we take a step back and take a look at the brain - it can be divided into four lobes: the frontal, temporal, parietal, and occipital lobes. Within each lobe is a dense network of neurons, which allows neurons to communicate with one another. Like most cells, neurons have a cytoskeleton made up of filaments and microtubules that give the cell its structure. Microtubules help neurons move molecules along the length of the cell, kind of like a railway track. And just like in a railway track, the individual units of a microtubule, called tubulins are tied together with a protein called tau. Tau comes in six different shapes and sizes, or isoforms, and one of the key features of these isoforms is how many times a particular sequence of 29 amino acids gets repeated. For three of those isoforms, it's repeated three times -- they're called the 3R isoforms -- and for the other three, it's repeated four times -- they're called the 4R isoforms.
Although it's not completely understood why, in frontotemporal dementia, abnormal protein inclusions form in the cytoplasm or nuclei of neurons. These inclusions are often made up of tau proteins. Specifically, in the case of Pick disease, 3R isoforms of the tau protein get hyperphosphorylated, meaning that phosphate groups keep binding onto the proteins until no more will fit. These hyperphosphorylated tau proteins change shape and stop being able to tie together the tubulins in the neuron's cytoskeleton. What's more, the hyperphosphorylated tau proteins start clumping together, forming tangles of tau protein. This makes Pick disease what's called a tauopathy, just like Alzheimer’s disease. A key difference is that the tangles in Pick disease, are called Pick bodies, and they’re only made up of the 3R tau isoforms, whereas the tangles in Alzheimer’s disease, are called neurofibrillary tangles, and are made up of both 3R and 4R tau isoforms. In other cases, intracellular inclusions are made up of a protein called TDP-43, which is involved in transcription regulation in the nucleus of normal cells.
Summary
Frontotemporal dementia (FTD) is a type of dementia that affects the frontal and/or temporal lobes of the brain. These are the areas of the brain responsible for personality, behavior, judgment, language, and movement.
The main symptoms of FTD include changes in personality and behavior, problems with judgment and planning, difficulty with language, and problems with movement. FTD may also cause changes in eating habits and sleep patterns.
Sources
- "Robbins Basic Pathology" Elsevier (2017)
- "Harrison's Principles of Internal Medicine, Twentieth Edition (Vol.1 & Vol.2)" McGraw-Hill Education / Medical (2018)
- "Pathophysiology of Disease: An Introduction to Clinical Medicine 8E" McGraw-Hill Education / Medical (2018)
- "Frontotemporal lobar degeneration—building on breakthroughs" Nature Reviews Neurology (2014)
- "Towards a nosology for frontotemporal lobar degenerations—A meta-analysis involving 267 subjects" NeuroImage (2007)
For Institutions
Copyright © 2023 Elsevier, its licensors, and contributors. All rights are reserved, including those for text and data mining, AI training, and similar technologies.
Cookies are used by this site.
USMLE® is a joint program of the Federation of State Medical Boards (FSMB) and the National Board of Medical Examiners (NBME). COMLEX-USA® is a registered trademark of The National Board of Osteopathic Medical Examiners, Inc. NCLEX-RN® is a registered trademark of the National Council of State Boards of Nursing, Inc. Test names and other trademarks are the property of the respective trademark holders. None of the trademark holders are endorsed by nor affiliated with Osmosis or this website.
