Frontotemporal dementia, what is it?

Frontotemporal dementia usually begins with personality changes, behavioral, language, affection or executive functions. It is a dementia with three variants: frontal variant, semantic dementia and progressive primary aphasia. The most frequent appearance of this dementia is about sixty years. The frontal variant and semantic dementia occur more in men; On the other hand, progressive primary aphasia is more frequent in women. In the front variant the progression is faster. Those who enjoy longevity are those who suffer from semantic dementia.

The complexity of this type of dementia because of its three variants, leads us to crumble each type of variant throughout the article. It will be investigated at the beginning, in the diagnostic criteria, as well as in the exclusive criteria. Each of the variants has specific characteristics in which it will be deepened little by little. Let's start!

Content

• Frontotemporal dementia classification
• Front variant of frontotemporal dementia
  • Essential criteria
  • Manifestations that support the diagnosis
  • Aspects that exclude diagnosis
  • Relative exclusion data
• Diagnostic criteria of the semantic variant
  • Essential diagnostic aspects
  • Aspects that support diagnosis
  • Aspects that exclude diagnosis
  • Relative exclusion data
• Diagnostic criteria of progressive primary aphasia
  • Bibliography

Frontotemporal dementia classification

Hodges and Miller (2001) classify the frontotemporal dementia in three groups:

• Front variant. Associated symptoms are personality and behavior change. The lesion is located in the bilateral orbitofrontal cortex.
• Progressive primary aphasia. Symptoms of non -fluent aphasia, alteration in expression but conservation of understanding. The affected area is the left Perisilvian area.
• Semantic dementia. It is expressed through an anomic aphasia fluent with alteration in the understanding and loss of meaning. The lesion is located in the left or bilateral infercolateral temporal cortex.

Front variant of frontotemporal dementia

In 90% of the cases of the front variant there are alterations in behavior. Can be found Two clinical syndromes of this variant: apathetic and uninhibited. In the first, there is an atrophy of the dorsolateral area of ​​the frontal lobe; In the second, there is atrophy of the orbitomedial area of ​​the frontal lobe and the temporal lobe. Neary's team (1998) establishes different diagnostic criteria of the front variant.

Essential criteria

1. Insidious Home and Gradual Progression.
2. Early interpersonal relationship social behavior disorder.
3. Early alteration of introspection.
4. Emotional superficiality and indifference.
5. Early appearance of anosognosia.

Manifestations that support the diagnosis

1. Start before 65 years.
2. Family group of dementia or antecedent of a similar family disorder.
3. Behavioral disorder: Deterioration in care and personal toilet. Inflexibility and mental stiffness. Impersistence and distractibility. Hyperoral behavior and changes in eating behavior. Stereotyped behavior of use.
4. Language and speech alterations: Alterations in the expressive facet as loss of language concretion, speaks broken and loss of spontaneity. Presence of Ecolalia. Stereotyped language. Perseveration. Late mutism.
5. Physical signs: Cortical disinhibition reflexes. Urinary incontinence. Acinesia, rigidity and tremor. Low and labile blood pressure. Lumbar paralysis, weakness and muscle atrophy.
6. Supplementary tests: Neuropsychological exploration. Normal electroencephalogram in stages with obvious dementia. Cerebral neuroimaging tests with abnormality in the front or temporary area.

Aspects that exclude diagnosis

1. Clinical aspects: Sudden home. Previous cranial trauma. Intense memory disorder in the initial phases of dementia. Spatial disorientation. Logoclonies (spasmodic repetitions of a syllable in the middle or at the end of a word) and rapid loss of the chain of thought. Myoclonies (fast spasms of a muscle or muscle group). Corticospinal weakness. Cerebellar ataxia. Choreoatetosis (uncontrolled and involuntary movements).
2. Supplementary tests: Location alterations, especially in the area after the fissure of Rolando. Brain injuries in different areas. Affectation of an inflammatory or metabolic alteration such as multiple sclerosis, syphilis or AIDS.

Relative exclusion data

1. Chronic alcoholism.
2. Long evolution arterial hypertension.
3. Personal history of vascular disease.

Diagnostic criteria of the semantic variant

The main characteristic of the semantic variant is the loss of the meaning of words. However, phonological and syntactic aspects of language can be preserved. The consciousness of dementia at the beginning is low. Little by little, the ability to understand the meaning of words is lost. The same happens with visual stimuli, smells, flavors and nonverbal sounds. Something curious in this type of patients is the ability to use objects that say they do not know. Neary's team (1998), also elaborates the different diagnostic aspects of the semantic variant.

Essential diagnostic aspects

1. Insidious Home and Gradual Progression.
2. Language disorder. Spontaneous spontaneous but poor content. Loss of the meaning of words. Difficulty calling and understanding. Semantic parafasias.
3. Perception disorder. Presence of Prosopagnosia and Associative Agnosia.
4. Normality in the copy of a drawing and association tests based on perception data.
5. Normal repetition of isolated words.
6. Normal writing and reading.

Aspects that support diagnosis

1. Aspects that support the diagnosis of frontotemporal degeneration: Start before 65 years. Background of similar family -like disorder. Motor neuron disease with bulbar paralysis, weakness and muscle thinning and fasciculations.
2. Speech and language: Logorrhea. Idiosyncratic use of words. Absence of phonesic parafasias. Dyslexia and surface dysgraphia. The arithmetic calculation is not altered.
3. Conduct: Loss of interest and lack of empathy. Decrease of interest in the environment. Avarice.
4. Physical signs: Absence of late appearance of cortical disinhibition reflexes. Acinesia, hypertonia and tremor.
5. Supplementary tests: Within these tests we find those of neuropsychology, electroencephalogram and cerebral neuroimaging. With respect to neuropsychological tests, a notable semantic deterioration is appreciated but special skills and episodic memory are preserved. The electroencephalogram appears normal. Cerebral neuroimagen shows predominant abnormality in the anterior temporal region.

Aspects that exclude diagnosis

1. Of anamnesis and exploration: Brushed Home And ICTAL EVENTS. Cranial trauma close to the eating of the manifestations. Intense Press Amnesia. Spatial disorientation. Speak accelerated. Myoclonia. Corticospinal weakness. Cerebellar ataxia and choreatetosis.
2. Supplementary tests: Within the cerebral neuroimagen we find structural deficiencies in the retrontroroly region and multifocal lesions. There are also signs of brain involvement by inflammatory or infectious processes such as multiple sclerosis, syphilis or AIDS.

Relative exclusion data

1. Chronic alcoholism.
2. Long evolution arterial hypertension.
3. Personal history of vascular disease.

Diagnostic criteria of progressive primary aphasia

One of the main characteristics of this variant is the difficulty for nomination (anomia), as well as abnormalities in speech. There is a decrease in the size of the phrases, phonological parafasias, prosody and grammatical errors.

There are two variants: fluente and not fluent. The fluent variant is characterized by conserving phonology and syntax but having altered semantic processing. The non -fluente variant has grammatical alterations and phonological paraphraphy. The conservation of understanding, nonverbal and memory intelligence is observed. Mesulam (2003) summarizes it in the following table:

1. Insidious beginning and gradual progression of alterations in the memory of words, denomination of objects or understanding of words, manifested during a spontaneous conversation or through applied language tests in a formal neuropsychological exploration.
2. All difficulties that manifest for the development of daily activities will be attributable to language alteration, at least during the first two years of evolution.
3. Normal Premóbido Language, except if there is evidence of a constitutional dyslexia.
4. During the first two years of evolution Apathy is not significantly observed, Forgetting recent facts, visuospatial alterations, disinhibited behavior, differences in visual recognition or sensitive-motor dysfunction.
5. The Acalculia and Ideomotora Apraxia may appear during the first two years.
6. After the first two years of evolution, other functions can be deteriorated, however, Language will be the most affected.
7. Absence of specific causes, such as a cerebral stroke or a tumor.

Bibliography

• Hodges, j. and Miller, B. (2001). The Classification, Genetics and Neuropathology of Frontotemporal Dementia. Neurocase, 7, 31-35.
• Igorri, a. (2007). Frontotemporal dementia. Colombian Magazine of Psychiatry, 35, 139-156.
• Mesulam, m. (2003). Primary Progressive Aphasia: A Language-Based Dementia. The New England Journal of Medicine, 349, 1535-1542.
• Neary, d., Snowden, j., Gustafson, l., Passant, u., Stuss, d., Black, S, et al. (1998). Frontotemporal Lobar Degeneration: A Connsus on Clinical Diagnostic Criteria. Neurology, 51, 1546-52.