Dementia, clinical pictures

notes by dr Claudio Italiano

Dementia is a global deterioration of mental functions in both intellectual, cognitive, emotional and affective aspects, resulting in a global disintegration of the personality that manifests itself both intellectually and behaviorally with the reduction and loss of the ability to learn and therefore to adapt to changes. environment.

Alzheimer-Perusini disease (presenile dementia)

It occurs between the ages of 45 and 65, with a slight preference for female sex, most likely linked to an autosomal dominant hereditary factor. The alterations histological (senile plaques, neurofibrillary alterations, granular-vacuolar intraneuronal degeneration) usually affect the cerebral cortex extensively but sometimes the process is limited to the frontal or temporal lobes and particularly in the temporal-parieto-occipital passage zones. The essential characteristic of the disease is the deterioration of intellectual functions, however often the clinical picture can present itself as a psychogenic illness with anxiety, depression, paranoia and only later the typical symptoms of disorientation appear.

Memory disorders, impaired understanding and judgment are other prerogatives of the disease. Furthermore, with the aggravation of intellectual deterioration, aphasic, agnosic and apraxic disorders generally take on a rapid and dramatic evolution (alogical and asymbolic dementia). Neurologic signs such as the sucking reflex and "grasping reflex" are often present, and generalized seizures may occur. Death, preceded by complete mental breakdown, occurs 5 to 10 years after the onset of the disease, usually due to inanition, dehydration or respiratory infections.

Pick's disease (presenile dementia)

It is a presenile disease, characterized by the prevalence of symptoms of foco-laio for idiopathic degenerative atrophy with marked districts. The atrophic process mainly affects the frontal and temporal lobes. The brain appears atrophic with areas more affected than others (and centers of atrophy) in particular in the basic fronto-temporal cortex, with isolated prefrontal and parietal areas. In general, lesions are bilaterally more or less symmetrical. The characteristic microscopic alterations are of two types:
1) cellular swelling, which preferably affects the medium pyramidal cells;
2) Argentophile inclusions or Pick bodies, intracytoplasmic osmiophylic masses that move the nucleus, especially in the cortex of the frontal and temporal lobes and in a lesser degree diffused throughout the cortex.
For some clinical aspects (see age, familiarity, loss of intellectual abilities), EEG, neuroradiological findings, this mal. it is similar to that of Alzheimer-Perusini.
Pick's disease should be distinguished by the prevalence of outbreak symptoms (frontal syndrome).
The disease begins with a progressive mental decay similar to the senile with "reverse actions", more frequent and clamorous than in other demented forms. Sometimes the patient appears hyperactive with fatuous euphoria or irritability, sometimes apathetic with progressive disinterest of his activities, tendency to immobility.
The signs of an outbreak are evident after about a year. These occur more frequently in the prefrontal syndrome, characterized by a disturbance of the guide of thought, by difficulty and disinterest in mental work, with gross errors and inequalities in performance depending on the variations of the conative attention. The behavior can be apathetic or hyperactive. Spontaneous attention is prompt and sometimes exaggerated, to the detriment of continued conative attention. Memory disorders appear to vary in severity.
Sometimes an apathetic behavior is evident with fatuous euphoria, in some cases the apathy can be so accentuated as to lead to a tenacious mutacy. More rarely, sectoral damage can give rise to an aphasic and alogical syndrome. The disease progresses with varying speed towards constant mental breakdown.Demenza

Creutzfeldt-Jakob disease

This disease is characterized by rapid disintegration of neurons, cerebral cortex, basal ganglia, cerebellum and anterior horns of the spinal cord, accompanied by reactive gliosis and spongy degeneration. The etymology of this disease is unknown, however a viral genesis has been hypothesized. The incidence of this disease is equal to that of Pick's disease and represents approximately 15% of the cases of presenile dementia studied from an anatomo-pathological point of view. The mal. has no preference for sex, begins between 40 and 60 years. The course is rapid, the exitus occurs after months and most patients do not survive for more than a year. The initial symptoms are represented by anxiety, depression and reduction of intellectual and memory abilities with alterations of the personality. Therefore language disorders are established, progressive dulling and confusion. Concomit disturbances in the coordination of movements, tremors, coreiform or atetotic movements, myoclonic contractions, dystonia, signs of the pyramidal and extrapyramidal series. The mal. progresses to a state of mutism with postures typical of decerebration or decortication and usually the exitus occurs due to the onset of pulmonary infections.

Senile dementia

The constant aging of the population (the population of men over 65 has quadrupled in the last fifty years in Western countries), the biological characteristics of the old, its social and psychological conditions with the progressive loss of meaning and the decay of the social role have led to an increase in senile pathology in general and in particular for demented pictures in senile age. The etiopathology is unknown: it is probable that there are constitutional factors. From an anatomical point of view the brain presents the typical diffuse atrophy of gray and white matter with ventricular dilatation, deepening of the furrows, reduction of convolutions. The histopathological picture follows the one already described for the demential syndromes in general.
On the symptomatic level, some characterological features are progressively accentuated, which, by expanding, become abnormal (reduction of initiative, environmental concerns, egocentrism, hypo-local polarization, avarice, rigidity, refusal of novelties, loss of sympathetic affective participation with the environment) . Depressive manifestations of reactive type may concomit in the onset phase. Reduction of mnestic capacities with diminished ability to operate through an abstract thought, tendency to the isolani, and to hostility, irritability with defense mechanisms, senile activity sometimes exaggerated, exhibitionism, limitation of the ability to self-tracing in the cleaning and care of the body in general . Delusional projections arise with contents of the vein, damage, jealousy, repressive attitudes: generally the subject is suspicious, tends to suspect even family members, hidden money and objects for sonal values ​​of little value, forgetting the hiding place, develops persecutory themes. Memory disorders become more serious, the patient becomes disoriented, agitated, confused, especially in the evening and in the morning, reversing the sleep-wake rhythm. Hallucinatory and delusional phenomena may appear. The typical dementia syndrome with somatic and psychic symptoms is then established.

It is possible to distinguish the following clinical forms of evening dementia:
* Simple type: characterized by! prevail of intellectual impairment with memory disturbances, loss of interests, narrowing of the initiative, apathy, irritability, nocturnal excitement and insomnia.
* Confused-onyric type: the confusional component associated with hallucinatory and delusional productions of the oneiric type prevails. The picture presents characteristics of sharpness and can be triggered by the concomitation of a physical disease that disturbs the metabolic and electrolytic balance in general or from a drug-toxic state.
* Type with depression or excitement: in addition to memory disturbances to the impoverishment of the ideational patrimony, the patient presents anxiety, agitation, melancholy themes, hypochondriacs and denial of negation.
* Paranoid type: dissatisfied, suspicious, expresses hostility in the surrounding world and presents gross delirium (unstructured and sensitive to persecutory content, harm, damage, jealousy, querulomaniac, hypochondriac, fantastic type) associated with hallucinations and confabulations .
* Presbyophrenic type: more frequent in the female is characterized by hyperdisturbed memory, by fixation with confabulations. Forget, do not give up and replace the lost memories by inventing. The patient is hyperactive, apparently dynamic, talkative, bustling, unproductive, often with superficial euphoria.


Huntington's Korea

It is a rare disease, characterized by core and dementia symptoms. It is an idiopathic degenerative disease with dominant inheritance. Extensive destruction of neurons in the caudate nucleus and putamen was detected and neuronal degeneration in the cortex of the frontal lobes. The mal. it begins in the middle age. Psychic disorders appear after the beginning of the choreic syndrome. Patients become unstable, dysphoric, irritable and impulsive, sometimes they are antisocial. The pronounced mental syndrome has a distinct demential character (coreofrenia): irritability becomes quarrelsome, impulsive dysphoria brings suicide ideas, incurant and variable intellectual deficits appear. More often there is a progressive dementia syndrome with apathy, sometimes associated with fatuous euphoria. Sometimes the typical dementia picture is associated with psychotic accessory symptoms of parafrenico type with delirium on a perceptive and hallucinatory basis with persecutory or hypochondriac content.

index psichiatry