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Frontotemporal dementia, what does it consist of?

Frontotemporal dementia, what does it consist of?

Frontotemporal dementia usually begins with personality changes, behavioral alterations, language, affect or executive functions. It is a dementia with three variants: frontal variant, semantic dementia and progressive primary aphasia. The most frequent occurrence of this dementia is over 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 frontal variant the progression is faster. Those who enjoy more longevity are those who suffer from semantic dementia.

The complexity of this type of dementia because of its three variants, leads us to shred each type of variant throughout the article. It will be investigated at the beginning, in the diagnostic criteria, as well as in the exclusionary criteria. Each of the variants has specific characteristics that will gradually deepen. Let's get started!

Content

  • 1 Classification of frontotemporal dementia
  • 2 Front variant of frontotemporal dementia
  • 3 Diagnostic criteria of the semantic variant
  • 4 Diagnostic criteria for progressive primary aphasia

Classification of frontotemporal dementia

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

  • Front variant The associated symptoms are the change of personality and behavior. The lesion is located in the bilateral orbitofrontal cortex.
  • Primary progressive aphasia. Symptoms of non-fluent aphasia, impaired expression but preservation of understanding. The affected area is the left perisilvian area.
  • Semantic dementia It is expressed through a fluent anomic aphasia with impaired understanding and loss of meaning. The lesion is located in the left or bilateral inferolateral temporal cortex.

Front variant of frontotemporal dementia

In 90% of cases of the frontal variant behavioral alterations occur. Can be found two clinical syndromes of this variant: apathetic and uninhibited. In the first, an atrophy of the dorsolateral area of ​​the frontal lobe is seen; in the second, there is an atrophy of the orbitomedial area of ​​the frontal lobe and the temporal lobe. The Neary team (1998) establishes different diagnostic criteria for the frontal variant.

Essential criteria

  1. Insidious onset and gradual progression.
  2. Precocious disorder of the social behavior of interpersonal relationship.
  3. Early alteration of introspection.
  4. Superficial superficiality and emotional indifference.
  5. Early appearance of anosognosia.

Manifestations that support the diagnosis

  1. Start before age 65.
  2. Family grouping of dementia or a history of a similar disorder in a first-degree relative.
  3. Conduct disorder: Deterioration in care and personal hygiene. Inflexibility and mental rigidity. Impersistance and distractibility. Hyperoral behavior and changes in eating behavior. Stereotyped behavior of use.
  4. Alterations of language and speech: Alterations in the expressive facet as loss of the concretion of language, broken speech and loss of spontaneity. Presence of Ecolalia. Stereotyped language Perseveration Late mutism
  5. Physical signs: Reflexes of cortical disinhibition. Urinary incontinence Akinesia, stiffness and tremor. Low and labile blood pressure. Lumbar paralysis, weakness and muscular atrophy.
  6. Supplementary tests: Neuropsychological examination Normal electroencephalogram in stages with obvious dementia. Brain neuroimaging tests with abnormality in the anterior frontal or temporal area.

Aspects that exclude the diagnosis

  1. Clinical aspects: Rough start. Previous head trauma Intense memory disorder in the initial stages 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. Myoclonus (rapid spasms of a muscle or group of muscles). Weakness of corticospinal type. Cerebellar ataxia Choreoathetosis (uncontrolled and involuntary movements).
  2. Supplementary tests: Alterations of location, especially in the area after the Rolando fissure. Brain injuries in different areas. Affectation of an inflammatory or metabolic disorder such as multiple sclerosis, syphilis or AIDS.

Relative Exclusion Data

  1. Chronic alcoholism
  2. Hypertension of long evolution.
  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 the words. However, phonological and syntactic aspects of language can be preserved. The awareness of dementia at the beginning is low. Gradually, the ability to understand the meaning of words is lost. The same thing happens with visual stimuli, smells, flavors and nonverbal sounds. Something curious in this type of patients is the ability to use objects they say they don't know. The Neary team (1998) also elaborates the different diagnostic aspects of the semantic variant.

Essential Diagnostic Aspects

  1. Insidious onset and gradual progression.
  2. Language disorder It speaks spontaneously fluent but of poor content. Loss of meaning of words. Difficulty to name and understand. Semantic paraphases
  3. Perception disorder. Presence of prosopagnosia and associative agnosia.
  4. Normality in copying a drawing and evidence of association of elements based on perception data.
  5. Normal repetition of isolated words.
  6. Normal writing and reading to dictation.

Aspects that support the diagnosis

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

Aspects that exclude the diagnosis

  1. From the history and exploration: Abrupt start and ictal events. Cranial trauma close to eating manifestations. Intense early amnesia. Spatial disorientation Speak fast. Myoclonus Corticospinal weakness Cerebellar ataxia and choreoathetosis.
  2. Supplementary tests: Within the cerebral neuroimaging we find structural deficiencies in the retrorrollandic region and multifocal lesions. There are also signs of cerebral involvement due to inflammatory or infectious processes such as multiple sclerosis, syphilis or AIDS.

Relative Exclusion Data

  1. Chronic alcoholism
  2. Hypertension of long evolution.
  3. Personal history of vascular disease.

Diagnostic criteria of progressive primary aphasia

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

There are two variants: fluent and non-fluent. The fluent variant is characterized by preserving phonology and syntax but having altered semantic processing. The non-fluent variant presents grammatical alterations and phonological paraphrases. The conservation of understanding, nonverbal intelligence and memory is observed. Mesulam (2003) sums it up in the following chart:

  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 language tests applied in a formal neuropsychological examination.
  2. All the difficulties that are manifested for the development of daily activities will be attributable to the alteration of language, at least during the first two years of evolution.
  3. Normal premorbid language, except if there is evidence of a constitutional dyslexia.
  4. During the first two years of evolution apathy is not observed significantly, forgetfulness of recent events, visuospatial alterations, uninhibited behavior, differences in visual recognition or sensory-motor dysfunction.
  5. The Acalculia and ideomotor apraxia They may appear during the first two years.
  6. After the first two years of evolution, other functions may deteriorate, however, the language will be the most affected.
  7. Absence of specific causes, such as a 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 Journal 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 consensus on clinical diagnostic criteria.Neurology, 51, 1546-52.
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