Identified as the second most common type of early-onset dementia and responsible for approximately 10-20% of all dementias, frontotemporal dementia (FTD) is characterized by a progressive neuronal loss in the frontal and temporal lobes [1]. FTD appears at a rate of 3-15 per 100,000 individuals between 55-65 years of age and affects both sexes almost equally [1]. Several distinct clinical syndromes have been described in the literature [1] [2] [3] [4] [5] [7]:

• Behavioral-variant frontotemporal dementia (bvFTD) - Considered as the most common form of FTD (presumably caused by genetic mutations), this subtype is responsible for about 50% of all cases and is distinguished by various changes in personality and behavior due to loss of neuronal circuits in the frontal lobe [3] [4]. Disinhibition is the most prominent feature manifested as inappropriate social behavior (insensitive remarks to others, inclination toward violence, assault, excessive eating, hypersexual and sociopathic behavior), absence of empathy, lack of insight and awareness, abnormal behavioral patterns regarding their thoughts or habits, as well as a lack of personal hygiene and sphincter control are present in the majority of patients [3] [5] [6]. About 50% of bvFTD patients show either probable or possible signs of amyotrophic lateral sclerosis (ALS, which may develop together with all other forms of FTD, but much rarely as comapred to bvFTD). In that case, symptoms of motor neuron disease are present [2] [3] [4].
• Semantic dementia (SD) - Progressive neuronal loss in the anterior temporal lobes is the main pathological entity described in SD, consequently leading to aphasia and loss in semantic memory (presenting as the inability to attach words with objects and an overall loss of vocabulary) [2] [4] [7]. As a result, patients interact by using nonspecific words and imprecise terms, while agnosia and prosopagnosia (inability to recognize visual objects or familiar faces, respectively), as well as behavioral disturbances seen in bvFTD, develop in later stages of the disease [2] [3] [5] [6].
• Progressive non-fluent aphasia (PNFA) - Agrammatism, apraxia (difficulties in speech initiation), dysarthria, and a slow speech full of errors are main characteristics of this type of FTD.
• Overlap syndromes - Some authors have confirmed overlapping of FTD and two clinical syndromes - corticobasal syndrome (CBD) and supranuclear palsy (in addition to ALS). Deficits in vertical gaze, postural instability, ophthalmoplegia and disinhibitory symptoms suggest an association of FTD with supranuclear palsy, whereas apraxia, rigidity, and myoclonus indicate CBD as the overlapping syndrome [2] [3] [4].

• Swelling

  His meninges were swollen but when we biopsied the tissue we found no signs of a tumor or bacterial infection to explain the swelling.” [doi.org]

  Upon autopsy, the patient's brain showed asymmetric atrophy. 1 Pathologically, FTD is associated with atrophy of the frontal and/or temporal lobes, gliosis, neuronal swelling, and eventual microvacuolation. 2, 3 Pick bodies are hyperphosphorylated tau [ajnr.org]

  My foot swelled up, and I was unable to walk, or put any weight on it for several weeks. I took Ibuprofen and other stuff for inflammation and pain. That pretty much sums up the month of June. [bvftd.blogspot.com]

• Fatigue

  Ensure personal comforts by checking for pain, hunger, thirst, infection, constipation, diarrhea, full bladder, fatigue. [memory.ucsf.edu]

• Aspiration

  They may inhale (aspirate) food, sometimes resulting in aspiration pneumonia. Different types of symptoms develop, depending on which part of the frontal or the temporal lobe is affected. [msdmanuals.com]

• Cough

  GETTY Frontotemporal dementia symptoms: Lance was diagnosed at 45 years old TYPES OF DEMENTIA Lance, who worked as a steel erector before his diagnosis, now communicates to Adele with his eyes, through coughing, his body language and an Etch A Sketch [express.co.uk]

  On examination: There may be impairment of orofacial movements such as swallowing, coughing or yawning on command (although still present as a reflex). There may be stuttering, impairment of ability to write or read, or impaired repetition ability. [patient.info]

  Apraxia of other orofacial movements or swallowing often accompanies speech apraxia and is tested by asking the patient to yawn or cough, which they are unable to do to command, although they can as a reflex. [bmj.com]

• Abnormal Behavior

  […] and awareness, abnormal behavioral patterns regarding their thoughts or habits, as well as a lack of personal hygiene and sphincter control are present in the majority of patients. [symptoma.com]

  Researchers looked at when the dementia symptoms began based on the earliest report from family members telling of persistently abnormal behavior. Survival was defined as from the time symptoms began until death. [psychcentral.com]

  Some people with frontotemporal dementia become uninhibited, resulting in increasingly inappropriate behavior. They may speak rudely. Their interest in sex may increase abnormally. Behavior may become impulsive and compulsive. [msdmanuals.com]

• Inappropriate Sexual Behavior

  Examples include shoplifting, encroaching on someone’s personal space, and inappropriate sexual behavior. Social skills like tact and empathy may be lost. They may lose interest in everything, or they may suddenly need to be active all the time. [dementiacarecentral.com]

• Motivation Decreased

  […] in motivation, decrease in energy, and generally compulsive behavior. [disabilitybenefitscenter.org]

• Mood Swings

  Your loved one might have: Memory and concentration problems Poor judgment Confusion Mood swings Depression Sleep problems Twitching or jerky muscles Trouble walking Normal Pressure Hydrocephalus This type of dementia is caused by a buildup of fluid in [webmd.com]

• Personality Change

  The second patient presented with progressive memory impairment and marked personality changes after a transient ischaemic attack. [ncbi.nlm.nih.gov]

  In fact, personality changes and behavior problems are hallmarks of the disorder. [alzheimers.net]

  Principal symptoms include behavioral disinhibition and personality changes, as well as deficits in language, both in speech production and word comprehension. The diagnosis can be made only after a thorough clinical and imaging workup. [symptoma.com]

  Because behavior and personality change may be a presenting symptom, FTDs are often initially diagnosed as psychiatric disorders. [neuropathology-web.org]

• Agitation

  Non-Drug Strategies Treating potential underlying causes of hallucinations or agitation is preferred. [memory.ucsf.edu]

  Aggressive, agitated, or dangerous behaviors could require medication. Anti-depressants and tranquilizers have been shown to improve some symptoms. PROGRESSION The outcome for individuals with FTD is poor. [secure.ssa.gov]

  […] symptoms of bvFTD are behavioral or emotional challenges, including compulsively overeating, having an emotionally-detached demeanor, making socially inappropriate responses, and displaying hypersexual conversation and actions, along with irritability, agitation [alzheimers.about.com]

  - in some instances there is an increase in purposeless activity (e.g., pacing, constant cleaning) or agitation. [brain.northwestern.edu]

• Memory Impairment

  The second patient presented with progressive memory impairment and marked personality changes after a transient ischaemic attack. [ncbi.nlm.nih.gov]

  Also, deficits in verbal processing abilities and word retrieval may contribute to the decreased memory performance and to the impression of the caregiver that memory is impaired. [doi.org]

  Memory impairment is not an initial symptom, and yet those with PPA/FTD who are in the earlier stages may be perceived by others as having Alzheimer’s disease. [crisisprevention.com]

• Irritability

  […] most common symptoms of bvFTD are behavioral or emotional challenges, including compulsively overeating, having an emotionally-detached demeanor, making socially inappropriate responses, and displaying hypersexual conversation and actions, along with irritability [alzheimers.about.com]

  These possibilities include: Behavioral variant FTD – these patients have prominent, early changes in their behavior (such as being more impulsive or irritable), social interaction with others (kissing strangers, crude or explicit remarks), initiative [alzheimers.emory.edu]

  Personality changes can also involve increased irritability, anger and even verbal or physical outbursts toward others (usually the caregiver). [brain.northwestern.edu]

  There are medications that can reduce agitation, irritability and/or depression. These treatments should be used to help improve quality of life. [alz.org]

• Behavior Problem

  Common treatments include: Medications Some antidepressants can help reduce the behavioral problems caused by changes to the brain. Antipsychotic medications are also used to treat behavioral problems. [healthline.com]

  In fact, personality changes and behavior problems are hallmarks of the disorder. [alzheimers.net]

  Diseases in this group include the following: Pick’s Disease Frontotemporal Lobar Degeneration (FTLD) Behavior Variant Frontotemporal Dementia (bvFTD) – problems with behavior, personality, judgment, and emotions Primary Progressive Aphasia (PPA) – problems [dementiacarecentral.com]

Evaluation of patients in whom dementia of any type is suspected must start with a comprehensive history and a thorough physical examination, both being equally important. Physicians should suspect FTD if they detect language deficits and disinhibitory symptoms (which are often noticed by friends or family members, but not by the patients themselves), and if the patients are somewhat younger than the known age for the onset of dementia ( i.e.< 65 years of age). A complete neurological exam (to assess whether motor neuron disease is concomitantly present) and cognitive testing, an integral component in the assessment of dementia, provides vital clues to differentiate between various forms of FTD [2] [3]. Imaging studies, however, are necessary to rule out other causes of such symptoms (eg. tumors), and magnetic resonance imaging (MRI) is the recommended method [2] [3] [4] [5]. Atrophy of the frontal and temporal lobes are present, with the dorsolateral prefrontal cortex being involved in the majority of cases [2] [3]. In addition, the anterior cingulate, orbitofrontal and frontoinsular cortices are commonly affected, often correlating with the extent of manifested symptoms [2]. If findings on MRI are inconclusive, then single photon emission computed tomography (SPECT) or fluorodeoxyglucose positron emission tomography (FDG-PET) are recommended due to their ability to detect dysfunction of specific areas of the brain [2].

One subject stopped sertraline treatment because of diarrhea, while another stopped paroxetine treatment due to increased anxiety. [ncbi.nlm.nih.gov]

Care plans Before treatment starts, your current and future health and social care needs will be assessed, and a care plan drawn up. This is a way of ensuring you receive the right treatment for your needs. [nhs.uk]

Best prognosis tends to be in those with the semantic type, and survival may be as long as ten years or more. Worst prognosis is associated in the overlap syndrome with motor neurone disease, and there may only be 3-5 years between onset and death. [patient.info]

KEYWORDS: Amyotrophic lateral sclerosis; disease progression; frontotemporal dementia; incidence; primary progressive aphasia; prognosis [ncbi.nlm.nih.gov]

What are The Prognosis and Options for Treatment? FTD worsens over time and impairs the individual’s ability to live and function independently. [uofmhealth.org]

The case highlights the limitations of our diagnostic tools and the complex relationship between clinical symptoms, neuroimaging, and etiology. © The Author(s) 2014. [ncbi.nlm.nih.gov]

Neuroimaging (magnetic resonance imaging) usually demonstrates focal atrophy in addition to excluding other etiologies. [aafp.org]

At a minimum, these SNPs are known to be related, and others may also be Known genetic loci according to "The epidemiology of frontotemporal dementia" [ PMID 23611343 ]: Chromosome 9 open reading frame 72 ( C9orf72 ), chromosomal location 9p21.2, found [snpedia.com]

In the meeting the participants refreshed their knowledge in epidemiology, clinical diagnosis, neuropsychology, neuroimaging, neuropathology, genetics, cell biology, animal models and management for their daily practice in patient care. [icftd2016.de]

The clinic is conducting many research projects, including clinical trials and studies of day-to-day care, as well as epidemiologic, neuropsychiatric, neuropathological, and genetic investigations. [hopkinsmedicine.org]

We emphasized the key molecular actors in these processes, proposing them as novel FTD biomarkers that could be crucial for further epidemiological and molecular studies. [ncbi.nlm.nih.gov]

Discussion While the clinical presentation of frontotemporal dementia was described as early as the nineteenth century, recent advances in genetics have resulted in greater understanding of the pathophysiology of this disease. [racgp.org.au]

Recent advances in the understanding of FTD pathophysiology and genetics have led to the development of potentially disease-modifying therapies. [ncbi.nlm.nih.gov]

Currently, there is no specific treatment available to prevent disease progression. FTD treatment is based on symptomatic management, and most therapies lack quality evidence from randomized, placebo-controlled clinical trials. [ncbi.nlm.nih.gov]

Preventing or delaying dementia Recent research suggests that healthy lifestyle habits and mental stimulation may prevent dementia altogether or at least delay its onset. [helpguide.org]

[…] for up to one month Engineers have developed a Jell-O-like pill that quickly expands on reaching the stomach and uses an embedded sensor to monitor health status over an extended period of time. ‹ › New understanding could help improve prediction and prevention [news-medical.net]

Instead, risk factors for developing FTD include: Mutations in the MAPT and/or GRN genes of chromosome 17 A family history of FTD Treatments Like other varieties of degenerative dementia, there is no cure or treatment that can eradicate, prevent or stop [dementia.org]

1. Ferrari R, Hernandez DG, Nalls MA, et al. Frontotemporal dementia and its subtypes: a genome-wide association study. Lancet neurology. 2014;13(7):686-699.
2. Warren JD, Rohrer JD, Rossor MN. Frontotemporal dementia. BMJ. 2013;347:f4827.
3. Rabinovici GD, Miller BL. Frontotemporal Lobar Degeneration: Epidemiology, Pathophysiology, Diagnosis and Management. CNS drugs. 2010;24(5):375-398.
4. Tsai RM, Boxer AL. Treatment of Frontotemporal Dementia. Curr Treat Options Neurol. 2014;16(11):319.
5. Goedert M, Ghetti B, Spillantini MG. Frontotemporal Dementia: Implications for Understanding Alzheimer Disease. Cold Spring Harb Perspect Med. 2012;2(2):a006254.
6. Arvanitakis Z. Update on Frontotemporal Dementia. The neurologist. 2010;16(1):16-22
7. Forman MS, Farmer J, Johnson JK, et al. Frontotemporal Dementia: Clinicopathological Correlations. Annals of neurology. 2006;59(6):952-962.