Generality
Encephalopathies are diseases characterized by an "anatomo-physiological alteration" of the brain. In other words, the brain of an individual affected by encephalopathy is modified in its structure and / or its functions.
Encephalopathies can be due to various causes; the triggering reasons also depend on the symptoms, the duration of the disease, the treatment and the prognosis.
Thanks to an accurate diagnosis, it is possible to precisely establish the characteristics of the encephalopathy in progress and the causes of its onset.Only once these points have been clarified, it is possible to plan an adequate therapeutic path.
The prognosis is variable: if the encephalopathy is temporary, there is a concrete possibility of recovery; if it is permanent, the patient may even be doomed to death.
What is encephalopathy?
Encephalopathy is the term used to refer to a group of diseases that alter the structure and / or functions of the brain.
The various forms of encephalopathy can be permanent or temporary. If they are permanent, they last for a lifetime; if they are temporary, there is a possibility of recovery.
Some encephalopathies are congenital (ie present from birth) and remain unchanged for the entire existence, without worsening; others are acquired (ie they arise in the course of life) and can undergo worsening.
The various types of encephalopathy differ from each other in their triggering causes, symptoms, complications and prognosis.
Causes
The causes that can lead to the development of an "encephalopathy are numerous and very varied.
The following table summarizes the triggers of the main known encephalopathies.
Type of encephalopathy
Causes
It occurs as a result of multiple trauma or blows to the brain. It is quite common in those who practice high-level contact sports, such as professional boxers (not surprisingly, it is also known as boxing dementia).
It is the result of a genetic condition, as a result of which there are excessive concentrations of glycine in the brain. Glycine is an ordinary amino acid.
It is a rare consequence of an autoimmune disease affecting the thyroid gland. The disease in question is Hashimoto's thyroiditis.
It is the result of a severely diseased liver, which no longer functions properly. It is a classic complication of liver failure secondary to cirrhosis.
Diabetic encephalopathy
It is due to the high levels of glucose in the blood (hyperglycemia), which is typical of diabetes.
It arises as a result of a state of persistent arterial hypertension.
It occurs when the brain does not get the right amount of oxygen. The reasons for a lack of oxygen are related to respiratory or cardiovascular problems, temporary or permanent.
It is one of the consequences of Lyme disease. Lyme disease is a "tick-borne bacterial infection. The bacterium responsible is." Borrelia burgdorferi.
The exact causes are unknown. Some researchers have advanced the hypothesis that it may be due to a hemorrhage based in the brain or an infectious meningitis.
It can be the consequence of some viral, bacterial and parasitic infections, or of coming into contact with some toxic substances, such as cleaning products, pesticides, building materials, etc.
It is a consequence of prion diseases. Prions are proteins normally found in many animal species, including humans.
If, for some reason, prions mutate, they can cause the following diseases: Kuru, Creutzfeldt-Jakob disease, fatal familial insomnia and Gerstmann-Str syndromeäussler-Scheinker.
It is a possible consequence of kidney failure.
It arises as a result of a lack of vitamin B1. Vitamin B1 deficiencies occur in cases of severe alcoholism or malnutrition.
It is linked to metabolic diseases that cause various types of dysfunctions in the mitochondrial DNA.
This congenital encephalopathy occurs during labor or delivery, and is due to a temporary lack / deficiency of oxygen in the blood to the brain of the unborn child.
It is caused by food poisoning contaminated with salmonella bacteria.
It is a combination of an "encephalopathy and a myopathy. Among the causes are mitochondrial diseases and chronic hypophosphatemia (N.B: hypophosphatemia is the medical term that indicates low levels of phosphorus in the blood).
Symptoms and Complications
Figure: Head injuries due to contact sports are a cause of chronic traumatic encephalopathy. From the site: sodahead.com
The characteristic sign of all encephalopathies is the alteration of the mental state. Typical expressions of this alteration include: memory loss, personality changes, depression and inability to solve even simple problems of everyday life.
Depending, then, on what are the triggering causes and what is the affected brain area, encephalopathies can cause a long series of neurological disorders, such as:
- Lethargy
- Tremors
- Myoclonus. These are involuntary tics of one or more muscles.
- Asterypses. It consists of a sudden, but temporary, loss of muscle tone.
- Difficulty speaking and swallowing
- Epilepsy attacks
- Inability to concentrate
- Nystagmus. It is a rapid and involuntary eye movement.
- Muscle weakness
- Loss of cognitive functions
- Dementia
- Breath of Cheyne-Stokes. It is a very particular disorder, due to which, cyclically, the patient's breathing slows down until it stops (apnea), and then recovers after a few seconds reaching a high depth (hyperpnea) and subsequently becoming more and more superficial until it culminates in a new one. apnea phase.
Such situations are all the more serious the more severe are the changes that take place inside the brain.
For example, in the case of hepatic encephalopathy, the symptoms can evolve quite quickly and with more than evident consequences; in the case of chronic traumatic encephalopathy, on the other hand, the clinical course tends to be very slow, so much so that it is difficult to establish an early diagnosis.
WHEN TO SEE THE DOCTOR?
If you experience one or more of the above symptoms, you should contact your doctor immediately, without hesitation.
COMPLICATIONS
In general, the signs that indicate a worsening of an "encephalopathy" are:
- Marked worsening of symptoms already present
- Severe state of confusion
- Severe state of disorientation
- Coma
Diagnosis
To diagnose an "encephalopathy, both the physical examination, during which the doctor evaluates the symptoms and signs in progress, and the examination of the clinical history, which involves a" careful investigation of the pathologies that have afflicted or afflict the patient are very important. patient, lifestyle, etc.
Therefore, at the end of these first two analyzes, it is usually necessary to subject the patient to more in-depth checks: the intent is to precisely identify the causes and the precise brain areas involved.
BLOOD TESTS
Thanks to a blood sample and its analysis in the laboratory, the doctor can establish whether the symptoms of encephalopathy are due to:
- A "viral, bacterial or parasitic infection
- Inhalation or contact with toxic substances
- Some prion disease.
Therefore, blood tests help to clarify the triggering causes.
LUMBAR PUNCTURE
Lumbar puncture consists of taking a sample of cerebrospinal fluid (or CSF) and analyzing it in the laboratory. To withdraw the CSF, a needle is used that the doctor inserts between the lumbar vertebrae L3-L4 or L4-L5.
Like blood tests, lumbar puncture provides an aid in identifying the triggering causes: in fact, if at the origin of the encephalopathy there are infections, intoxications or prion diseases, these are readily identified.
CT and MRI
Computed axial tomography (CT) and magnetic resonance imaging (MRI) are two diagnostic imaging tests that, in the case of encephalopathy, help to recognize the affected brain areas.
Figure: nuclear magnetic resonance imaging of three individuals with Wernicke's encephalopathy. The white arrows indicate the signs of the brain alteration. From the site: qjmed.oxfordjournals.org
ELECTROENCEPHALOGRAM
The electroencephalogram is an instrumental examination that allows to record the electrical activity of the brain and to detect any anomalies. It is a completely safe and painless diagnostic procedure.
Treatment
The treatment of an "encephalopathy depends on the causes that triggered it".
This is why it is very important to study the disease in progress and its symptoms thoroughly and with the appropriate diagnostic tests.
If the causes are treatable, the encephalopathy is curable. In these cases, the therapeutic remedies can consist of: drugs of various kinds (antibiotics, anticonvulsants, hypoglycaemics, sugar substitutes, etc.), targeted rehabilitation therapies, surgery for the transplant of a diseased organ and proper diet.
If, on the other hand, the causes are not treatable, the encephalopathy results: incurable, possible cause of permanent brain damage and, in some situations (transmissible spongiform encephalopathy), cause of death.
SOME EXAMPLES
The following table summarizes some examples of possible treatments for some forms of encephalopathy.
In case of
Treatments
Hypoxic encephalopathy, due to temporary and short-lived breathing problems.
- Oxygen therapy, to increase the amount of circulating oxygen.
Hypoxic encephalopathy, due to a severe and prolonged respiratory inability.
- Oxygen therapy
- Respiratory rehabilitation
- Respiratory physiotherapy
Physiotherapy and rehabilitation serve, respectively, to restore (at least in part) the ability to breathe and to improve tolerance to efforts.
Hepatic encephalopathy
- Oral lactulose
- Diet low in protein and rich in vegetables
- Antibiotics
Lactulose, a low-protein diet and antibiotics are used to reduce the amount of ammonia in the blood (ammonaemia).
Lactulose hinders the growth of ammonia-producing bacteria and lowers the colonic pH (which is why the conversion of ammonia into a less dangerous substance, namely the ammonium ion, is favored).
The low protein diet obviously reduces the introduction of nitrogen compounds, therefore also the production of ammonia.
Finally, the antibiotics kill the ammonia-producing bacteria residing in the colon.
Uremic encephalopathy
- Correction of the causes of kidney disease
- Dialysis
- Kidney transplant
Kidney transplant is the ultimate solution to cases of severe kidney failure.
Diabetic encephalopathy
- Administration of glucose
- Insulin administration
Glucose is used to resolve any excessive drops in blood sugar (severe hypoglycemia), which are very dangerous.
Insulin, on the other hand, is used to control the opposite state, that is, the conditions of hyperglycemia.
Hypertensive encephalopathy
- Hypotensive, to reduce high blood pressure.
Encephalopathies that permanently alter the brain structure and / or that can lead to death are treatable only from a symptomatic point of view. In fact, in such situations, relieving and managing symptoms in the best possible way is the only form of treatment that can be implemented.
Prognosis
The prognosis depends on the type of encephalopathy in progress and its level of severity.
For some encephalopathies, such as transmissible spongiforms, the prognosis is always negative, as the brain damage is permanent and the patient is doomed to death sooner or later.
For treatable encephalopathies, on the other hand, the prognosis can also be positive (or, if you prefer, not negative), since there is a possibility of recovery or, at least, of a partial restoration of normal brain functions.
Attention: according to National Institute of Neurological Disorders and Stroke (a US agency that deals with diseases of the brain and central nervous system), transmissible spongiform encephalopathies can lead to the death of the affected person even within 3-12 months.
Prevention
Unlike congenital encephalopathies, some acquired encephalopathies can be prevented.
In fact, if you adopt a healthy lifestyle without excesses (therefore the right doses of alcohol and correct diet), and if you avoid exposure to certain toxic substances, you can reduce the risk of getting sick from various forms of encephalopathy.
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