Nephrogenic encephalopathy is a disorder of central nervous system function. The disease does not have a uniform set of symptoms, as the symptoms vary greatly in individual cases. Nephrogenic encephalopathy results as a sequelae disorder from decompensated renal failure or uremia.
What is nephrogenic encephalopathy?
Nephrogenic encephalopathy is also sometimes referred to by the synonymous terms uremic encephalopathy or renal encephalopathy. The main symptom of nephrogenic encephalopathy at the onset of the disease is intermittent cerebral edema. Due to this edema, typical brain pressure signs appear, for example, pain in the head, vomiting and so-called papilledema. In some cases, nephrogenic encephalopathy leads to further complaints, such as so-called high-pressure angiopathies. As a consequence, perivascular necrosis and so-called mass hemorrhages occur in some patients. Demyelinating foci and necrosis of ganglion cells are also possible. These symptoms are identified during histologic analysis.
Causes
To date, the exact factors and mechanisms of the development of nephrogenic encephalopathy have not been conclusively investigated. Basically, most conjectures assume that urinary substances are retained in the patient’s blood. This results in brain dysfunction as a consequence of various processes. After some time, the urinary substances develop a neurotoxic effect, primarily affecting the central nervous system. The main cause of nephrogenic encephalopathy is therefore the accumulation of certain substances in the blood. These include creatinine, uric acid and other substances that affect the acid-base balance. An increased concentration of urea is particularly relevant here. In addition, electrolyte metabolism is negatively affected, which usually leads to the development of hypercalcemia. As the corresponding urine-associated substances accumulate in the person’s blood, the tissue of the nerves is damaged. As a result, the typical symptoms of nephrogenic encephalopathy develop. In addition, associations with diseases such as diabetes mellitus and hypertension have been observed.
Symptoms, complaints, and signs
The leading symptoms of nephrogenic encephalopathy focus on neurologic deficits. The symptoms develop over a shorter or longer period of time, depending on the individual case. In numerous cases of the disease, the symptoms are first noticed by other people and only later by the patients themselves. Neurological symptoms often occur in nephrogenic encephalopathy. Possible symptoms include dysarthria, hyperreflexia, tremor, and myoclonia. In addition, some patients present with symptoms such as cerebral spasms. Very rarely, exogenous psychoses develop in those suffering from nephrogenic encephalopathy. These result primarily from the uremia that accompanies the disease. The characteristic complaints of nephrogenic encephalopathy are irritable symptoms of a psychomotor nature, difficulty falling asleep and staying asleep, and a general feeling of restlessness. In addition, many affected patients suffer from affect lability. The general neurological symptoms of nephrogenic encephalopathy are primarily manifested by an increased need for sleep and disturbances in the sense of orientation and concentration. Individuals exhibit a general slowing down that relates to both thinking and movement processes. Sometimes a so-called stupor develops. The characteristic complaints of nephrogenic encephalopathy arise primarily from the effects of kidney weakness. The effects on the digestive tract as well as the cardiovascular system and blood result in the typical symptoms of nephrogenic encephalopathy.
Diagnosis and course of the disease
A diagnosis of nephrogenic encephalopathy often takes a long time because the symptoms of the disease are relatively nonspecific and suggest numerous other conditions. Consequently, it is often only possible to narrow down the diagnosis on the basis of the symptoms to a limited extent. In addition, the patients themselves often experience no or hardly any symptoms for a long time.In most cases, it is relatives or other close persons who draw attention to the abnormalities. Thus, the factor of lack of insight into the disease is added, which often delays a diagnosis. People with suspected nephrogenic encephalopathy initially confide in their general practitioner, who makes a referral to an appropriate specialist. When evaluating the patient’s medical history in the first part of the diagnostic process, any renal impairment that may be present is of great importance. This may provide the decisive clue to the possibility of nephrogenic encephalopathy. During the clinical examination, the treating specialist usually uses several diagnostic techniques. Significant are blood analyses by laboratory tests. For example, an increased concentration of parathormone and hypercalcemia are detected. An EEG examination is also used in the majority of cases to diagnose nephrogenic encephalopathy. Imaging methods may find evidence of cerebral atrophy in some cases.
Complications
Nephrogenic encephalopathy is already a complication of renal failure. It occurs when urinary substances accumulate more and more in the blood. Initially, the disorder is manifested by nonspecific neurological symptoms that can also be attributed to other diseases. The neurological deficits range from concentration disorders to coma. As mentioned earlier, the main disease feature of nephrogenic encephalopathy is cerebral edema. With the help of dialysis (blood washing), the symptoms can initially be pushed back. However, the high intracranial pressure sometimes causes mass hemorrhage and vascular necrosis. In extreme cases, these complications can lead to death or permanent brain damage. However, if treatment is given in time, at least partial regression of symptoms is possible. However, inadequate treatment of decompensated renal failure, i.e. incomplete removal of urinary substances from the blood, results in chronic damage to the central nervous system. These substances have a neurotoxic effect and destroy the nerve cells, which are then unable to regenerate. As a result, permanent dementia may develop, associated with affect lability, disorientation and forgetfulness. Cerebral seizures are also observed as complications of nephrogenic encephalopathy. These manifest similarly to epileptic seizures in sudden loss of consciousness and muscle spasms throughout the body. Occasionally, involuntary biting of the tongue is also possible. The urinary substances in the blood can also cause psychosis in rare cases.
When should you go to the doctor?
If people show disturbances in general functioning, there is cause for concern. Interruptions in speech, changes in speech comprehension, and declines in mental performance must be observed. If they persist unabated for several days or continue to increase, a physician is needed. Any abnormality in motor function, disturbances in general movements, as well as an increased risk of accidents and falls must be clarified by a physician. If a tremor occurs, a doctor should be contacted immediately or an ambulance should be alerted. In the case of muscle weakness, interruptions of the usual movements of the musculoskeletal system as well as pain, clarification of the cause is necessary. Sleep disturbances or the inability to rest are characteristic of the disease. In most cases, the affected person suffers from difficulty falling asleep, a general restlessness and restlessness. If headaches occur, behavioral changes are noticed, or the affected person suffers from an inner weakness, he or she needs help. Mood swings and aggressive behavior should be discussed with a doctor. If attention and orientation deficits are noticed, if concentration is impaired, or if everyday obligations can no longer be performed, a visit to the doctor is necessary. Disturbances in kidney function, changes in weight or skin appearance are further indications of a health impairment. Action is required, since in severe cases and if left untreated, organ failure is imminent.
Treatment and therapy
The most important measure of therapy for nephrogenic encephalopathy is dialysis.In this way, the superfluous urea-bearing substances of the blood are removed. The top priority here is to cleanse the blood of urea. This prevents further damage to the nervous tissue caused by the neurotoxic substances. Successful treatment of nephrogenic encephalopathy sometimes results in the regression of some symptoms.
Outlook and prognosis
The earlier treatment for nephrogenic encephalopathy begins, the better the prognosis for patients. It is also important to reduce renal exposure to nephrotoxic drugs for a good prognosis. The typical neurological deficits of patients can range from mild concentration disorders to life-threatening coma, as the main disease feature is cerebral edema. With the help of blood washing, also known as dialysis, the symptoms can initially be pushed back, but the high intracranial pressure that occurs often causes vascular necrosis and mass hemorrhages in those affected. In individual cases, such complications lead to death or even permanent brain damage in the affected patients. However, if treatment is given in time, at least a partial regression of the symptoms that occur is possible. However, insufficient therapy can lead to chronic damage to the central nervous system. As a result, dementia may develop. Cerebral seizures are also often observed as complications in the course of the disease. These manifest themselves with sudden muscle spasms and unconsciousness. In addition, a chronic course of the disease is to be expected. There is a continuous deterioration in the general condition of those affected. In addition, there is also a risk of patients falling into a coma. If the patient awakens from this coma again, very severe health impairments should be expected.
Prevention
Prevention of nephrogenic encephalopathy is not possible in every individual case. In principle, appropriate therapy of existing renal impairment supports prevention.
Follow-up
After successful treatment, it is important to train independence and mobility. Various therapy options are available for this purpose. These can either be monitored in a rehabilitation program or performed independently at home. Doctors and physiotherapists prescribe the exercises and adapt them individually depending on progress. Depending on the severity of the condition, an occupational therapist may also be involved. An occupational therapist helps to cope with everyday activities. Often, some things have to be learned anew. In most cases, a combination of previous supervised rehabilitation and later independence is useful. Yoga and special massage and meditation techniques help to cope with pain. An intact circle of family and friends is also of great importance here. The social environment supports further stabilization, at least psychologically. However, regular monitoring of blood and urine levels is also very important for aftercare. Initially, this is done at very short intervals, but later longer intervals are likely. With the control the urine production of the kidney must be checked, in order to determine whether the minimum quantity of urine is produced in the second, in order to guarantee the function ability. This is referred to as the glomerular filtration rate. If it falls below a set value, dialysis (blood washing) is required.
What you can do yourself
Since nephrogenic encephalopathy usually affects the entire musculoskeletal system, the goal of therapy is to preserve mobility and independence. In addition to medical measures, other alternative therapy methods can be used, which should be performed independently at home. Doctors, physiotherapists or occupational therapists provide instructions for exercises that challenge and promote the patient’s mobility. These should be individually adapted to the state of the disease and continuously updated. Regularity in the performance of the exercises is also important, as this is the only way to achieve the desired success of maintaining performance. Massage techniques can also be learned to treat skin hardening, which patients can then incorporate into their everyday lives and apply independently.In addition to a positive influence on the affected connective tissue, such measures also have positive effects on the psychological condition of the affected person. Through a self-determined occupation with one’s own body, the personal quality of life can be significantly increased. Alternative therapy methods such as yoga and meditation can be ways of learning to cope with symptomatically caused pain. Involving the social environment of the ill person is always an important measure. Maintaining an intact social network can give new strength to deal with the disease. Friends and relatives can form a support in case of setbacks and show new perspectives.
