The main clinical features are signature language progressive difficulties with speech production. There can be problems in different parts of the speech production system, hence patients can present with articulatory breakdown, phonemic breakdown (difficulties with sounds) and other problems. However, it is rare for patients to have just one of these problems and most people will present with more than one problem. Features include:

- Hesitant, effortful speech

- Speech 'apraxia'

- Stutter (including return of a childhood stutter)

- Anomia

- Phonemic paraphasia (sound errors in speech e.g. 'gat' for 'cat')

- Agrammatism (using the wrong tense or word order)

As the disease develops, speech quantity decreases and many patients will become mute.

Cognitive domains other than language are rarely affected early on. However, as the disease progresses other domains can be affected. Problems with writing, reading and speech comprehension can occur as can behavioural features similar to frontotemporal dementia.

Semantic dementia (SD), also known as semantic variant primary progressive aphasia (svPPA), is a progressive neurodegenerative disorder characterized by loss of semantic memory in both the verbal and non-verbal domains. However, the most common presenting symptoms are in the verbal domain (with loss of word meaning). SD is one of the three canonical clinical syndromes associated with frontotemporal lobar degeneration (FTLD), with the other two being frontotemporal dementia and progressive nonfluent aphasia. SD is a clinically defined syndrome, but is associated with predominantly temporal lobe atrophy (left greater than right) and hence is sometimes called temporal variant FTLD (tvFTLD). SD is one of the three variants of Primary Progressive Aphasia (PPA), which results from neurodegenerative disorders such as FTLD or Alzheimer's disease. It is important to note the distinctions between Alzheimer’s Disease and Semantic dementia with regard to types of memory affected. In general, Alzheimer’s Disease is referred to as disorder affecting mainly episodic memory, defined as the memory related to specific, personal events distinct for each individual. Semantic dementia generally affects semantic memory, which refers to long-term memory that deals with common knowledge and facts.3

It was first described by Arnold Pick in 1904 and in modern times was characterized by Professor Elizabeth Warrington in 1975, but it was not given the name semantic dementia until 1989. The clinical and neuropsychological features, and their association with temporal lobe atrophy were described by Professor John Hodges and colleagues in 1992.

Three classifications of primary progressive aphasia have been described. In the classical Mesulam criteria for primary progressive aphasia, there are two variants: a non-fluent type progressive nonfluent aphasia (PNFA) and a fluent type semantic dementia (SD). A third variant of primary progressive aphasia, logopenic progressive aphasia (LPA), is an atypical form of Alzheimer's disease. Early PNFA can include such features as speech apraxia, effortful speech, and anomia, and thus can resemble Broca’s aphasia. Early LPA involves impairments in naming and sentence repetition, and thus can resemble Conduction aphasia. However, these PPA subtypes differ from these similar aphasias, as these subtypes do not occur acutely following trauma to the brain, such as following a stroke, due to differing functional and structural neuroanatomical patterns of involvement and the progressive nature of the disease.

The following diagnosis criteria were defined by Mesulam:

- As opposed to having followed trauma to the brain, a patient must show an insidious onset and a gradual progression of aphasia, defined as a disorder of sentence and/or word usage, affecting the production and comprehension of speech.

- The disorder in question must be the only determinant on functional impairment in the activities of the patient’s daily living.

- On the basis of diagnostic procedures, the disorder in question must be unequivocally attributed to a neurodegenerative process.

Whether or not PPA and other aphasias are the only source of cognitive impairment in a patient is often difficult to assess because: 1) as with other neurologically degenerative diseases, such as Alzheimer's disease, there are currently no reliable non-invasive diagnostic tests for aphasias, and thus neuropsychological assessments are the only tool physicians have for diagnosing patients; and 2) aphasias often affect other, non-language portions of these neuropsychological tests, such as those specific for memory.

The defining characteristic of SD is decreased performance on tasks that require semantic memory. This includes difficulty with naming pictures and objects, single word comprehension, categorizing, and knowing uses and features of objects. SD patients also have difficulty with spontaneous speech creation, using words such as "this" or "things" where more specific and meaningful words can be used. Syntax is spared, and SD patients have the ability to discern syntactic violations and comprehend sentences with minimal lexical demands. SD patients have selectively worse concrete word knowledge and association, but retain knowledge and understanding of abstract words. SD patients are able to retain knowledge of numbers and music, but have more difficulty with concrete concepts with visual associations. Impairments of processing of phonemic structure and prosodic predictability have also been observed.

There is some confusion in the terminology used by different neurologists. Mesulam's original description in 1982 of progressive language problems caused by neurodegenerative disease (which he called primary progressive aphasia (PPA) included patients with progressive non-fluent (PNFA), semantic dementia (SD), and logopenic progressive aphasia (LPA).

Agnosia is the inability to recognize certain objects, persons or sounds. Agnosia is typically caused by damage to the brain (most commonly in the occipital or parietal lobes) or from a neurological disorder. Treatments vary depending on the location and cause of the damage. Recovery is possible depending on the severity of the disorder and the severity of the damage to the brain. Many more specific types of agnosia diagnoses exist, including: associative visual agnosia, astereognosis, auditory agnosia, auditory verbal agnosia, prosopagnosia, simultanagnosia, topographical disorientation, visual agnosia etc.

Patients with hypergraphia exhibit a wide variety of writing styles and content. While some write in a coherent, logical manner, others write in a more jumbled style (sometimes in a specific pattern). In some cases hypergraphia can manifest with compulsive drawing. Drawings by patients with hypergraphia exhibit repetition and a high level of detail, sometimes mixing both compulsive writing and drawing together.

In addition to writing in different forms (poetry, books, repetition of one word), hypergraphia patients differ in the complexity of their writings. While some writers (see Alice Flaherty and Dyane Harwood, author of "Birth of a New Brain—Healing from Postpartum Bipolar Disorder" who had postpartum hypergraphia and bipolar, peripartum onset, also known as postpartum bipolar disorder.) use their hypergraphia to help them write extensive papers and books, most patients do not write things of substance. Flaherty defines hypergraphia, as a result of temporal lobe epilepsy, as a condition that "increase[s] idea generation, sometimes at the expense of quality." Patients hospitalized with temporal lobe epilepsy and other disorders causing hypergraphia have written memos and lists (like their favorite songs) and recorded their dreams in extreme length and detail. Some patients who also suffer from temporal lobe epilepsy record the times and locations of each seizure compiled as a list.

There are many accounts of patients writing in nonsensical patterns including writing in a center-seeking spiral starting around the edges of a piece of paper. In one case study, a patient even wrote backwards, so that the writing could only be interpreted with the aid of a mirror. Sometimes the writing can consist of scribbles and frantic, random thoughts that are quickly jotted down on paper very frequently. Grammar can be present, but the meaning of these thoughts is generally hard to grasp and the sentences are loose. In some cases, patients write extremely detailed accounts of events that are occurring or descriptions of where they are.

The signs and symptoms of frontal lobe disorder can be indicated by Dysexecutive syndrome which consists of a number of symptoms which tend to occur together. Broadly speaking, these symptoms fall into three main categories; cognitive (movement and speech), emotional or behavioural. Although many of these symptoms regularly co-occur, it is common to encounter patients who have several, but not all of these symptoms. This is one reason why some researchers are beginning to argue that dysexecutive syndrome is not the best term to describe these various symptoms. The fact that many of the dysexecutive syndrome symptoms can occur alone has led some researchers to suggest that the symptoms should not be labelled as a "syndrome" as such. Some of the latest imaging research on frontal cortex areas suggests that executive functions may be more discrete than was previously thought.

Signs/symptoms can be divided as follows:

Frontal lobe disorder is an impairment of the frontal lobe that occurs due to disease or head trauma. The frontal lobe of the brain plays a key role in higher mental functions such as motivation, planning, social behaviour, and speech production. A frontal lobe syndrome can be caused by a range of conditions including head trauma, tumours, degenerative diseases, neurosurgery and cerebrovascular disease. Frontal lobe impairment can be detected by recognition of typical clinical signs, use of simple screening tests, and specialist neurological testing.

Mild cognitive impairment (MCI), also known as incipient dementia and isolated memory impairment, is a neurological disorder that occurs in older adults which involves cognitive impairments with minimal impairment in instrumental activities of daily living. MCI involves the onset and evolution of cognitive impairments beyond those expected based on the age and education of the individual, but which are not significant enough to interfere with their daily activities. It may occur as a transitional stage between normal aging and dementia. Causation of the syndrome in and of itself remains unknown, as, therefore, do prevention and treatment.

Alzheimer's disease (AD) is a progressive, degenerative and fatal brain disease, in which cell to cell connections in the brain are lost. Alzheimer's disease is the most common form of dementia. Globally approximately 1–5% of the population is affected by Alzheimer's disease. Women are disproportionately the victims of Alzheimer's disease, with evidence suggesting that women with AD display more severe cognitive impairment relative to age-matched males with AD, as well as a more rapid rate of cognitive decline.

Phonagnosia (from Ancient Greek φωνή "phone", "voice" and γνῶσις "gnosis", "knowledge") is a type of agnosia, or loss of knowledge, that involves a disturbance in the recognition of familiar voices and the impairment of voice discrimination abilities in which the affected individual does not suffer from comprehension deficits. Phonagnosia is an auditory agnosia, an acquired auditory processing disorder resulting from brain damage, other auditory agnosias include cortical deafness and auditory verbal agnosia also known as pure word deafness.

Since people suffering from phonagnosia do not suffer from aphasia, it is suggested that the structures of linguistic comprehension are functionally separate from those of the perception of the identity of the speaker who produced it.

Phonagnosia is the auditory equivalent of prosopagnosia. Unlike Prosopagnosia, investigations of phonagnosia have not been extensively pursued. Phonagnosia was first described by a study by Van Lancker and Cantor in 1982. The subjects in this study were asked to identify which of four names or faces matched a specific famous voice. The subjects could not complete the task. Since then, there have been a couple studies done on patients with phonagnosia. The clinical and radiologic findings with computerized tomographic scans cat scan in these cases suggest that recognition of familiar voices is impaired by damage to the inferior and parietal regions of the right hemisphere while voice discrimination is impaired by temporal lobe damage of either hemisphere. These studies have also shown evidence for a double dissociation between voice recognition and voice discrimination. Some patients will perform normally on the discrimination tasks but poorly on the recognition tasks; whereas the other patients will perform normally on the recognition tasks but poorly on the discrimination tasks. Patients did not perform poorly on both tasks.

Associative phonagnosia is a form of phonagnosia that develops with dementia or other focal neurodegenerative disorders. Some research has led to questions of other impairments in phonagnosics. Recently, studies have shown that phonagnosics also have trouble in recognizing the sounds of familiar instruments. As it is with voices, they also show deficiency in distinguishing between sounds from different instruments. Although the disability is shown, phonagnosics are much less affected in this area of sound discrimination. In distinguishing voices, it is a complete agnosia, but this is not the case for musical instrument sounds, as they can correctly identify some of them. Controversy arises in that not all phonagnosics exhibit these symptoms, and so not all researchers agree that it should be attributed to the damage suffered that causes phonagnosia. Much debate has arisen over the fact that it seems that separate areas of the brain are utilized to handle information from language and music. This has led some researchers to skeptically consider this impairment as a clear symptom of the disorder. Again, more research is needed to create a clearer conclusion.

An interesting attribute that phonagnosics possess is that they can correctly detect emotions in voices when someone talks to them. They can also correctly match an emotion with a facial expression. Although surprising, this finding is sensible because it is known and well agreed upon that the limbic system, involved in expressing emotions and detecting emotions of others, is a separate system within the brain. The limbic system is made up of several brain structures including the hippocampus, amygdala, anterior thalamic nuclei, septum, limbic cortex and fornix.

Presently, there is no therapy or treatment for phonagnosia. Clearly, more research is needed to accomplish the feat of developing treatment for the disorder. The lack of treatment stems from the lack of knowledge about the disorder. Increased research will reveal vital information needed to formulate effective treatments and therapies.

Although MCI can present with a variety of symptoms, when memory loss is the predominant symptom it is termed "amnestic MCI" and is frequently seen as a prodromal stage of Alzheimer's disease. Studies suggest that these individuals tend to progress to probable Alzheimer's disease at a rate of approximately 10% to 15% per year.

When individuals have impairments in domains other than memory it is classified as nonamnestic single- or multiple-domain MCI and these individuals are believed to be more likely to convert to other dementias (e.g., dementia with Lewy bodies).

Anosodiaphoria is a condition in which a person who suffers disability due to brain injury seems indifferent to the existence of their handicap. Anosodiaphoria is specifically used in association with indifference to paralysis. It is a somatosensory agnosia, or a sign of neglect syndrome. It might be specifically associated with defective functioning of the frontal lobe of the right hemisphere.

Joseph Babinski first used the term anosodiaphoria in 1914 to describe a disorder of the body schema in which patients verbally acknowledge a clinical problem (such as hemiparesis) but fail to be concerned about it. Anosodiaphoria follows a stage of anosognosia, in which there may be verbal, explicit denial of the illness, and after several days to weeks, develop the lack of emotional response. Indifference is different from denial because it implies a lack of caring on the part of the patient whom otherwise acknowledges his or her deficit.

A few possible explanations for anosodiaphoria exist:

1. The patient is aware of the deficit but does not fully comprehend it or its significance for functioning

2. May be related to an affective communication disorder and defective arousal. These emotional disorders cannot account for the verbal explicit denial of illness of anosognosia.

Other explanations include reduced emotional experience, impaired emotional communication, alexithymia, behavioral abnormalities, dysexecutive syndrome, and the frontal lobes.

FTD is traditionally difficult to diagnose due to the heterogeneity of the associated symptoms. Signs and symptoms are classified into three groups based on the functions of the frontal and temporal lobes:

- Behavioural variant frontotemporal dementia (BvFTD) is characterized by changes in social behavior and conduct, with loss of social awareness and poor impulse control.

- Semantic dementia (SD) is characterized by the loss of semantic understanding, resulting in impaired word comprehension, although speech remains fluent and grammatically faultless.

- Progressive nonfluent aphasia (PNFA) is characterized by progressive difficulties in speech production.

However, the following abilities in the person with FTD are preserved:

- Perception

- Spatial Skills

- Memory

- Praxis

In later stages of FTD, the clinical phenotypes may overlap. FTD patients tend to struggle with binge eating and compulsive behaviors. These binge eating habits are often associated with abnormal eating behavior including overeating, stuffing oneself with food, changes in food preferences (cravings for more sweets, carbohydrates), eating inedible objects and snatching food from others. Recent findings from structural MRI research have indicated that eating changes in FTD are associated with atrophy (wasting) in the right ventral insula, striatum, and orbitofrontal cortex.

Patients with FTD show marked deficiencies in executive functioning and working memory. Most FTD patients become unable to perform skills that require complex planning or sequencing. In addition to the characteristic cognitive dysfunction, a number of primitive reflexes known as frontal release signs are often able to be elicited. Usually the first of these frontal release signs to appear is the palmomental reflex which appears relatively early in the disease course whereas the palmar grasp reflex and rooting reflex appear late in the disease course.

In rare cases, FTD can occur in patients with motor neuron disease (MND) (typically amyotrophic lateral sclerosis). The prognosis for people with MND is worse when combined with FTD, shortening survival by about a year.

Echolalia can be categorized as immediate (occurring immediately after the stimulus) vs. delayed (some time after the occurrence of a stimulus). Immediate echolalia results from quick recall of information from the short-term memory and "superficial linguistic processing". A typical pediatric presentation of immediate echolalia might be as follows: a child is asked "Do you want dinner?"; the child echoes back "Do you want dinner?", followed by a pause, and then a response, "Yes. What's for dinner?"

In delayed echolalia the patient repeats words, phrases, or multiple sentences after a delay that can be anywhere from hours to years later. Immediate echolalia can be indicative that a developmental disorder exists, but this is not necessarily the case. Sometimes echolalia can be observed when an individual echoes back a statement to indicate they are contemplating a response and fully heard the original statement.

Researchers observed the daily repetitions of an autistic six-year-old in order to examine the differences between triggers for delayed versus immediate echolalia. Researchers further distinguished immediate echos by the sequential context in which they occur: after corrections, after directives, or in indiscernible sequential positions. Delayed echos are distinguished on the basis of ownership: self-echos, other-echos, and impersonal echos. The results showed that nearly all immediate echos produced by the six-year-old were found in sequential contexts, while the delayed echoes also occurred in the basis of ownership.

Although echolalia can be an impairment, the symptoms can involve a large selection of underlying meanings and behaviors across and within subjects. "Mitigated echolalia" refers to a repetition in which the original stimulus is somewhat altered, and "ambient echolalia" refers to the repetition (typically occurring in individuals with dementia) of environmental stimuli such as a television program running in the background.

Examples of mitigated echolalia are pronoun changes or syntax corrections. The first can be seen in the example of asking the patient “Where are you going?” and with patient responding “Where am I going?” The latter would be seen in the clinician asking “Where are I going?” and the patient repeating “Where am I going?” In mitigated echolalia some language processing is occurring. Mitigated echolalia can be seen in dyspraxia and aphasia of speech.

A Japanese case report describes a 20-year-old college student who was admitted to the hospital complaining about headaches and meningitis; however, he also exhibited signs of ambient echolalia. The researchers stated that the young patient's repetition was occurring at approximately the same tempo as his normal speech rate. The patient did not simply repeat words he had heard one after another. The patient reported that his ambient echolalia appeared to be random but appeared when he was distracted. He was also aware of his echolalia, but said he is unable to stop the repetitions.

Echolalia can be an indicator of communication disorders in autism, but is neither unique to, nor synonymous with syndromes.

Echophenomena (particularly echolalia and echopraxia) were defining characteristics in the early descriptions of Tourette syndrome (TS). Echolalia also occurs in aphasia, schizophrenia, dementia, catatonia, epilepsy, after cerebral infarction (stroke), closed head injury, in blind children, children with language impairments, as well as certain developing neurotypical children. Other disorders associated with echolalia are Pick's disease, frontotemporal dementia, corticobasal degeneration, progressive supranuclear palsy, as well as pervasive developmental disorder.

In transcortical sensory aphasia, echolalia is common, with the patient incorporating another person's words or sentences into his or her own response. While these patients lack language comprehension, they are still able to read.

Frontotemporal dementia (FTD) is the clinical presentation of frontotemporal lobar degeneration, which is characterized by progressive neuronal loss predominantly involving the frontal or temporal lobes, and typical loss of over 70% of spindle neurons, while other neuron types remain intact.

It was first described by Arnold Pick in 1892 and was originally called "Pick's disease", a term now reserved for Pick disease, one specific type of frontotemporal dementia. Second only to Alzheimer's disease (AD) in prevalence, FTD accounts for 20% of young-onset dementia cases. Signs and symptoms typically manifest in late adulthood, more commonly between the ages of 55 and 65, approximately equally affecting men and women.

Common signs and symptoms include significant changes in social and personal behavior, apathy, blunting of emotions, and deficits in both expressive and receptive language. Currently, there is no cure for FTD, but there are treatments that help alleviate symptoms.

Aphasia in CBD is revealed through the inability to speak or a difficulty in initiating spoken dialogue and falls under the non-fluent (as opposed to fluent or flowing) subtype of the disorder. This may be related to speech impairment such as dysarthria, and thus is not a true aphasia, as aphasia is related to a change in language function, such as difficulty retrieving words or putting words together to form meaningful sentences. The speech and/or language impairments in CBD result in disconnected speech patterns and the omission of words. Individuals with this symptom of CBD often lose the ability to speak as the disease progresses.

In both case studies, patients showed an altered sense of humor, mostly in regard to producing and appreciating humor. The right hemisphere is involved with processing speed and problem solving, which plays a role in humor processing. These patients have difficulty fully interpreting a joke's content, but can recognize the importance of the form of a joke. Patients with witzelsucht often find non sequiturs, slapstick humor, and puns funniest since these forms of humor do not require integration of content across sentences. In other words, the end of the joke is not dependent on the first part; one does not need to make a logical connection to understand humor. Patients show no change in understanding simple logic, and understand the importance of surprise in humor (hence why they choose slapstick humor instead of the “correct” punch line); however, once they have registered this surprise, they cannot connect the punch line to the body of the joke to fully appreciate the true humor behind the joke. Successful jokes require a juxtaposition of the sound and the meaning of words used to understand the punch line. However, patients with witzelsucht have difficulty connecting the two, resulting in an inability to appreciate humor.

Additionally, patients show no emotional reaction to humor, whether produced by themselves or others. This lack of responsiveness is due to dissociation between their cognitive and affective responses to humorous stimuli. That is, even when a patient understands that a joke is funny (based on quantitative brain activity), they do not respond with laughter, or even a smile. While they have grasped the cognitive basis of humor, they do not affectively respond. This also considered a cognitive component of empathy, affecting ability to take the perspective of others; hence why patients often do not respond to humor produced by other people.

Psychiatric problems associated with CBD often present as a result of the debilitating symptoms of the disease. Prominent psychiatric and cognitive conditions cited in individuals with CBD include dementia, depression, and irritability, with dementia forming a key feature that sometimes leads to the misdiagnosis of CBD as another cognitive disorder such as Alzheimer's disease (AD). Frontotemporal dementia can be an early feature.

Corticobasal degeneration is a rare form of dementia that is characterized by many different types of neurological problems that get progressively worse over time. This is because the disorder affects the brain in many different places, but at different rates. One common sign is difficulty with using only one limb. One symptom that is extremely rare in any condition other than corticobasal degeneration is the "alien limb." The alien limb is a limb of the person that seems to have a mind of its own, it moves without control of the person's brain. Other common symptoms include jerky movements of one or more limbs (myoclonus), symptoms that are different in different limbs (asymmetric), difficulty with speech that is due to not being able to move the mouth muscles in a coordinated way, numbness and tingling of the limbs and neglecting one side of the person's vision or senses. In neglect, a person ignores the opposite side of the body from the one that has the problem. For example, a person may not feel pain on one side, or may only draw half of a picture when asked. In addition, the person's affected limbs may be rigid or have muscle contractions causing strange repetitive movements (dystonia).

The area of the brain most often affected in corticobasal degeneration is the posterior frontal lobe and parietal lobe. Still, many other part of the brain can be affected.