Facing it in a clear way

Introduction

Dementia is a disease connected to ageing that involves the Central Nervous System with an evolutive and irreversible trend of a worsening kind (at least for non-correlated typical forms).

This is the typical definition of dementia. A pertinent though partial description, somewhat misleading, that for years has ignored prevention in the clinical approach to the disease.

We actually know very well that when we physicians diagnose dementia we are approaching the final evidence of a disease started in an insidious and silent way dozens of years before.

We should then adjust our ideas on the disease by investing much more resources on cognitive prevention intervening and making the population aware well before dementia manifests itself.

The Different Forms of Dementia

Although different forms of dementia exist with their peculiarities, they are all characterised

by a progressive loss of cognitive abilities, functional-motor competences and psycho-behavioural changes.

Alzheimer’s disease

It is a degenerative disease affecting the brain caused by the progressive brain cell death leading to an atrophy of some of its areas.
It is the most widespread form of dementia accounting for about 60% of the cases. This disease manifests in a different way from one person to another, though the symptoms are basically shared by all patients.:

Early or mild:the person finds it difficult to remember recent events, to find the right word, to pay attention, to remember the name of people, places and objects, to organise and plan such daily activities as, for instance, doing the shopping, making lunch, paying bills.

Intermediate or moderate phase:the affected people are increasingly more disorientated, they mistake the current date or the place they are in, the find it difficult to learn or memorize new information as well as to talk and understand words,
they evidence an increasing need of support when performing such basic functions as eating, getting dressed and using the toilet. Some behaviour disorders can also arise like, aggressiveness, apathy, vagrancy, rummaging in drawers and wardrobes, repetitive gestures and behaviours made with no evident end.

Final phase:the patients are increasingly less autonomous, they find it difficult to move and walk, they are incapable of properly relating and communicating, they suffer from psychic and behavioural disorders, including delusions and/or hallucinations.
They are often bound between bed and armchair, unable to eat and control urine and faeces. They show an increasingly limited capacity to recognise even the family members and the closest people.

Vascular

It is the second form of dementia. It consists in a deterioration of mental faculties associated to decompensations in the blood flow to the brain causing multiple brain injuries called ischaemiae. It can be caused by single or multiple ictuses (strokes) to the brain or by damages to the very small blood vessels within the brain called small vessel disease (“Bingswanger’s disease”).

The beginning of MID can be sudden, as many strokes can take place before the appearance of the symptoms that can damage the areas of the brain responsible for a specific function: the language or the central executive function, the ability to move, limb weakness, lethargy, difficulty when speaking, emotional bursts, epileptic fits, or general symptoms of dementia. The course can be either discontinuous or divided into phases. The cognitive faculties can deteriorate, then stabilize for a certain period to deteriorate again. Unlike the Alzheimer’s disease, memory disorders are not the main symptom.

Risk factors: age, over 65, high blood pressure (hypertension), heart diseases, diabetes, smoking, overweight, high level of cholesterol and a family history of cardio-vascular problems.

Frontotemporal

It is a progressive disease affecting specific areas: the frontal and temporal lobes. In some cases the brain cells shrink or die, in other they swell containing “Pick bodies”.

The first symptoms involve both behaviour and language, with remarkable changes in the personality of patients that can become rude, arrogant, behave in an unbecoming way and not respect social conventions. They can loose interests in their personal hygiene, get easily distracted, keep on repeating the same action. Sometimes, in the first phases of the disease, they become incontinent. The problems with the language can vary from the decrease to the total loss of the ability to speak.

Common symptoms:stammering and the repetition of other people’s words, difficulty in following a conversation, in reading and writing. In the early stages of the disease, the problems connected to behaviour and language can arise separately, while, when the disease worsens, they overlap. Those suffering from the Pick’s disease, in the early stages, are orientated in time and maintain memory. In the advanced stages, the general symptoms of dementia are evidenced, such as confusion, the loss of both memory and the motorial abilities. It affects people between 40 and 60 years of age and, on average, it lasts 6-8 years.

Some secondary forms of frontotemporal dementia exist:

Semantic dementia: difficulty in recognising people, objects and in understanding the language. Patients speak, read and write in a fluent and correct way on the grammar point of view, but they are no longer able to call things by their proper names, o not understand the meaning of words and no longer recognise sounds and objects.

Progressive primary aphasia: characterised by the difficulty to communicate fluently or to find words. The patients understand what they hear or read, but talk in a hard and ungrammatical way, use wrong words, may be as an assonance of those that they would say and express themselves through a limited vocabulary.

Dementia with Lewy’s bodies

It is the third most common cause of dementia in elderly people, accounting for 15-20% of the total.

It shows a progressive form characterised by the presence of abnormal structures in the brain cells called “Lewy’s bodies”, whose formation mechanism is unknown. It can develop alone or together with the Alzheimer’s or Parkinson’s disease. The cause is not known and risk factors have not been identified. In its initial phases, memory can be affected, but it is basically still preserved.

Symptoms: progressive difficulty of attention, problems to the motor system, development of hallucinations and derangement states, difficult in programming, organisation, adaptability and motivation.

The disease with Lewi’s bodies is different from the Alzheimer’s disease as its course is usually more rapid. The confusional state or fluctuation of attention suffered from the patients, can highly vary even in the course of the same day. Visual hallucinations (see things that are not real) are common and they can worsen when the patient is more uneasy, Some features tend to be similar to the Parkinson’s disease such as muscular rigidity, tremors, uncoordinated movements. Medicines, particularly some sedatives, can worsen such symptoms.

Rare forms of dementia 1

Mixed dementia: characterised by the presence of brain alterations caused both by the Alzheimer’s disease and by ischemic or subcortical lesions.

Posterior Cortical Atrophy (PCA): also known as “Benson’s Syndrome”, is considered an atypical variation of the Alzheimer’s disease (AD). When compared to other forms of dementia, it often develops at an early age, starting from 40 years, causing an atrophy of the posterior part of the brain cortex with a subsequent progressive interruption of the global visual elaboration. In some cases, PCA can manifest along with other forms of dementia, like the one with Lewi’s bodies and the Creutzfeldt-Jakob’s disease.

Post-traumatic or boxing dementia: due to cranial trauma repeated over time.

Dementia caused by cortical-basal degeneration: characterised by the loss of nerve cells and by the reduction of more areas in the brain, like the brain cortex and the base ganglions.

Supernuclear progressive Paralysis or “Steele-Richardson-Olszewski’s Syndrome”:characterised by a progressive and selective loss of the neurons responsible for the control of the eye movements, balance, speech and deglutition.

Gerstmann-Straussler-Scheinker’s Syndrome: Gerstmann-Straussler-Scheinker’s Syndrom: of an hereditary kind, it is a neurodegenerative disease whose first signs include the loss of balance and a poor muscular coordination. The symptoms of dementia manifest in the last phases of the disease.

Alcoholic Dementia:the prolonged and heavy consumption of alcohol can lead to a form of dementia. However, stopping to drink and following a balanced diet can result in some improvements. Excessive drinking can also be the cause for the ”Korsakoff’s Syndrome” which implies a remarkable loss of memory. This syndrome, though, is not a form of dementia as such.

Rare forms of dementia 2

Creutzfeldt-Jacob’s disease (CJD): it is a form of progressive dementia characterised by a loss of the nerve cells and the degeneration of their membrane, causing small “holes” in the brain. It develops between 45 and 75 years of age. It can be either hereditary or infective, but it is transmitted only through the ingestion of infected food. No particular risk exists in taking care of patients. It has a long incubation period varying from 1 to 30 years. Once the following first symptoms appear: memory loss, behavioural alterations, loss of interests, lack of coordination, the course is rapid. Then, various other symptoms can develop such as sight problems, involuntary movements, particularly muscular spasms and rigidity of limbs. The patient may even loose the ability to move and speak. Based on the form, the disease can last from 6 months to many years since its first development.

Hungtington’s chorea: it has a degenerative nature. It usually begins in mid-life and is characterised by an intellectual decline and irregular and involuntary movements of limbs and face muscles. Other symptoms are: personality change, memory disorders, indistinct speech, deterioration of the sense of judgement and psychiatric problems. Those suffering from such a disease, tend to develop also dementia. Even though there are no available treatments to stop the course of the disease, the disorders of movement along with the psychiatric symptoms can be controlled with medicines. A test exists to establish if an adult has the hereditary gene responsible for such a disease.

Parkinson’s disease: it is a progressive disorder of the central nervous system, caused by a deficiency in dopamine, which is an important neurotransmitter involved in the control of the muscular activity. It is often characterised by tremors, rigidity in limbs and joints, difficulty in speaking and starting movements. In the course of the disease, some patients develop a specific form of dementia whose features remind those of the Alzheimer’s disease and vascular dementia.

Normothensive hydrocephalus : it is a not common disorder caused by an obstruction of the normal cerebrospinal flux generating an increase in the volume of the cerebrospinal liquid in the brain. It can be due to various factors among which meningitis, encephalitis and cranial traumas. Besides treating the causal event, such a condition can be solved with a neurosurgical intervention through the introduction of a “shunt” carrying the exceeding liquid out of the brain. The symptoms of the normothensive hydrocephalus include dementia, incontinence and difficulty in walking.

Diagnosis, Prognosis, Clinical Management, Neuro-psychological Diagnostics

The diagnosis of dementia is a mainly clinical path and involves two figures: The general practitioner for the first phase, the neurologist for the second stage consisting in the confirmation and identification within the various forms of dementia

Anamnesis: the physician listens to both the patient and the family, investigates on the existence of a case study of possible risk factors, such as some diseases and/or dangerous life styles and performs a careful examination of the psychological status of the individual to exclude that he/she is in a temporary condition of personal demotivation o depression. He/she sets the patient in a social-behavioural domain assessing the level of school-attendance, working activity, interests, pastimes and hobbies, retirement, life habits, autonomy in the daily activities.

Lab exams: general screening to exclude that a dementia is caused by somatic, metabolic, endochriconological, infective diseases or by conditions of chronic intoxication.

Neuropsychological tests: The first MMSE (Mini Mental State Examination), assesses the cognitive abilities of the patient, in particular time and space orientation, immediate memory (fixing or registering memory), attention and calculation, the recall memory, language (denomination, repetition, oral understanding, written understanding and creation of a written sentence), constructive praxia. More specific tests are carried out to investigate such individual areas as: long term memory, short term memory, logical-abstract functions, executive and frontal functions, attention, visual-constructive functions, visual-spatial and visual perceptive, language, praxia.

Instrumental diagnostics: they use CAT (Computer Assisted Tomography), useful to measure the thickness of the brain hemispheres, FMR (Functional Magnetic Resonance of the encephalon) that help obtain a very detailed image of the brain structure including the progressive loss of grey matter in the brain, PET (Positronic Emission Tomography) able to detect metabolic anomalies even before the first clinical manifestations of dementiae are evident, SPECT (Single-photon emission computed tomography), assessing the blood flux to the brain

Clinical Management: the most efficient interventions to perform are mainly of a rehabilitative and not pharmacological kind, aimed at stimulating and preserving all the remaining potentials: the Therapy of “Reorientering” in reality, to recover the ability to orientate in time and space related to the environment where the patient lives and to himself/herself and his/her own history. The Therapies stimulating memory, exploiting the natural tendency of the elderly person to recall the past. The Validation Therapy aiming at generating in the patient the idea that the world is fully shared and understood. The Remotivation Therapy that is applied individually or in groups aiming at stimulating the social interaction. The Occupational Therapy with the main aim of making the patient recover the highest level of autonomy and independence in his/her daily life.

Making prevention is important

Dementia represents one of the most challenging problems to solve not only on a health level but also on a social and economic one of the twenty-first century.

In 2015 about 47 million people in the world suffered from dementia and this figure is expected to triplicate in 2050. Due to the exponential increase of this disease, there is a subsequent rise in the request for medical treatment and social-support interventions.

Such an increase led to a rise in the public expense that was globally estimated around 818 billion dollars in 2015. This convinced the most important countries in the world to find some strategies to implement in order to reduce, at least partially, such abnormal costs.

Among the actions undertaken by governments, the most relevant and innovative are those meant for prevention and to contrast the risk factors. For further information make reference to the section dedicated to prevention.

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