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.
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.
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