Subcortical Arteriosclerotic Encephalopathy: Causes, Symptoms & Treatment

Subcortical arteriosclerotic encephalopathy (SAE) is the name given to a brain disease. It is also known as Binswanger’s disease.

What is subcortical arteriosclerotic encephalopathy?

Subcortical arteriosclerotic encephalopathy (SAE) is a disease of the brain that results from vascular changes such as hardening of the arteries (arteriosclerosis). Damage occurs in the subcortical area under the cerebral cortex. The disease is also known as multi-infarct dementia, vascular encephalopathy and Binswanger’s disease. Subcortical arteriosclerotic encephalopathy was first described in the late 19th century by the psychiatrist and neurologist Otto Ludwig Binswanger (1852-1929) of Switzerland. Subcortical arteriosclerotic encephalopathy represents the most common form of vascular dementia. It is one of the encephalopathies and is associated with arterial hypertension. It also results in microangiopathy.

Causes

Subcortical arteriosclerotic encephalopathy results from years of progressive arterial hypertension in which the arterioles of the central nervous system (CNS) are persistently damaged by fibrinoid necrosis. This results in tissue death. Because the small blood vessels are affected, the affected structures can no longer be supplied properly. This leads to extensive demyelination of the medullary canal. Furthermore, thromboembolic microinfarcts occur in the medullary canal, the ventral brainstem, and the basal ganglia. In earlier years, demyelination of the medullary canal was considered the sole reason for the development of dementia symptoms. However, according to recent research, dementia does not develop concomitantly with medullary camp damage. Instead, neuropathological changes similar to Alzheimer’s disease occur. So far, however, it has not been possible to determine the exact origin of the disease. Often, affected patients with subcortical arteriosclerotic encephalopathy already suffer from diabetes mellitus, arterial hypertension, or infarcts in multiple brain sections.

Symptoms, complaints, and signs

In its early stages, subcortical arteriosclerotic encephalopathy takes an insidious course and progresses in episodes. Parkinsonian-like symptoms are considered the earliest symptom of SAE. This involves tremors, immobility and rigidity. In addition, cognitive characteristics such as concentration, attention and retentiveness are reduced. However, the old memory is hardly affected. On the other hand, new information can only be processed inadequately. Those affected are therefore hardly able to cope with new situations. However, they still succeed in routine tasks and perform them meticulously. In some patients, affective and intellectual flattening occurs after a few years, accompanied by neuropsychological disorders. As the subcortical arteriosclerotic encephalopathy progresses, vascular dementia often develops. Other typical symptoms of SAE include bladder dysfunction, in which patients suffer from urinary leakage and urinary incontinence, and gait disturbances. The latter are characterized by a clumsy, wide-legged and unsteady gait. Furthermore, there is a spastic increase in muscle tone. In addition, patients suffer from subcortical dementia, which leads to a Parkinsonian-like loss of drive as well as deceleration. Not infrequently, paranoia and hallucinations also become apparent.

Diagnosis and course of the disease

The diagnosis of subcortical arteriosclerotic encephalopathy can be made using imaging techniques such as computed tomography (CT) or magnetic resonance imaging (MRI). With these methods, extensive demyelination of the medullary layer as well as lacunar infarcts can be easily detected. These are indicated as whitish foci around the ventricles. Differential diagnosis is also important. For example, similar symptoms can occur in Alzheimer’s disease, multi-infarct dementia, multiple sclerosis, HIV encephalopathy, cerebral edema, or radiation damage, among others. If subcortical arteriosclerotic encephalopathy progresses to vascular dementia, it shortens the patient’s life expectancy. Thus, mortality is higher than in Alzheimer’s dementia.It also often results in severe falls or bedriddenness.

Complications

Subcortical arterisclerotic encephalopathy is always associated with severe mobility limitations. The affected person may become less able to walk as the disease progresses and eventually becomes immobile. Falls and accidents also frequently occur, rendering the patient bedridden. Delayed wound healing and constant lying can cause secondary complaints such as edema, circulatory problems and inflammation. Prolonged bedriddenness also deteriorates cognitive perceptual ability and, in the course, causes psychological complaints and changes in personality. Apart from this, subcortical arterisclerotic encephalopathy can cause bladder dysfunction. Urinary leakage and even incontinence frequently occur. Subsequently, dementia progresses and causes paranoid-hallucinatory symptoms. The patient’s life expectancy is usually reduced. Treatment of the brain disease usually proceeds without major complications. However, the prescribed sedatives may cause severe side effects. In connection with existing mental illnesses, addictive behavior may also develop. Occupational therapy can cause frustration and anxiety in the affected person, as progress is usually very slow. Physical therapy carries the risk of temporary tension or bruising, but otherwise proceeds without symptoms.

When should you see a doctor?

If memory disturbances persist or increase gradually, there is cause for concern. A physician should be consulted for control so that clarification of the cause can be made. Restrictions in attention, general retentiveness and a decrease in mental performance should be investigated. If the person’s ability to cope with stress decreases, his or her personality changes, or if abnormalities in behavior become apparent, a physician is needed. Trembling of the limbs, gait unsteadiness or disturbances of the movement sequences are further signs of a health impairment. A visit to the doctor is necessary so that an investigation into the cause can be initiated. If the affected person experiences an uncontrolled loss of urine, increasing feelings of shame or shows a withdrawal from social life, there is a need for action. In the event of body rigidity or immobility, a physician should be consulted immediately. In severe cases, an ambulance must be alerted. Hallucinations, listlessness, as well as irregularities of the muscular system are other complaints of subcortical arteriosclerotic encephalopathy. They need to be presented to a physician as soon as possible so that medical care can take place. Faintness, bedriddenness, and persistent fatigue should be presented to a physician for examination. Mental and emotional problems, a decrease in well-being, and a general feeling of illness should be discussed with a physician. If daily responsibilities can no longer be performed independently, the affected person needs help.

Treatment and therapy

Because the causes of subcortical arteriosclerotic encephalopathy are still largely unknown, no specific therapy exists for its treatment. Surgical interventions also fail to bring about improvement. Even treatment with drugs rarely leads to success. For this reason, the avoidance of long- or short-term hypertension is at the forefront of therapy. Thus, these represent a significant risk factor of subcortical arteriosclerotic encephalopathy. Furthermore, extrapyramidal movement disorders are treated, which are a typical feature of SAE. The focus of therapy is to compensate for gait disturbances, balance disorders and coordination weaknesses. It is important to start treatment as early as possible. Another pillar of SAE therapy is occupational therapy. It is considered particularly useful for the treatment of coordination disorders. Furthermore, incontinence counseling and the administration of appropriate remedies take place. The supply of incontinence material helps to make the patient’s life easier, which also applies to their relatives. If the patient suffers from restlessness, sedative medications such as haloperidol, melperone or clomethiazole can be administered to calm them down at night. Another important component of treatment is cognitive training.This can also be done jointly by occupational therapists and psychologists. The aim is to give the patient better orientation and more independence and personal responsibility. If behavioral disorders are present, therapists now prefer non-pharmacological interventions. If these treatment measures are not sufficient, the patient is given appropriate medication.

Prevention

Because the causes of subcortical arteriosclerotic encephalopathy are largely unknown, targeted prevention is almost impossible. Also, there are no medications that can prevent or at least delay SAE or vascular dementia. Although certain preparations are available, their harms mostly exceed their benefits.

Follow-up

SAE cannot be completely cured. Medication can only slightly affect the progression of the disease. Because of its chronic nature, concomitant aftercare is useful. A largely normal life is the goal of aftercare therapy approaches. The patient’s quality of life should be stabilized and his independence maintained for as long as possible. In the case of subcortical arteriosclerotic encephalopathy, follow-up care is physical and psychotherapeutic. Concurrent care by a neurologist is also advisable. Physiotherapeutic exercises should improve the patient’s mobility. Existing vascular diseases require medical treatment. This will reduce the risk of SAE. When medications are used, a specialist must check their tolerability. Side effects must be recognized and treated at an early stage. Aftercare also involves family members. They receive advice from the therapist on how to deal with the patient on a day-to-day basis. The patient himself has the opportunity to take precautions: A healthy lifestyle can reduce the likelihood of SAE. A varied diet and abstaining from nicotine or alcohol have a favorable effect. A change in diet, on the other hand, is part of follow-up care. Patients should refrain from cigarettes or drinking too much alcohol after receiving the diagnosis.

What you can do yourself

Once this disease is diagnosed, therapies can only relieve the symptoms and slow down any progression of the disease. To do this, patients must carefully adhere to the treatment plans of their treating physicians, take their prescribed medications regularly, and keep their physical therapy appointments. It may be that keeping appointments in particular is difficult due to decreased short-term memory, so it is not uncommon for patients to need help and supervision early on. Visiting a psychologist or psychiatrist can also help. On the one hand, to cope with the stressful disease situation, and on the other hand, to participate in cognitive training to prevent or slow down further memory loss. Family members may also benefit from adjunctive psychotherapy, as caring for a person with subcortical arteriosclerotic encephalopathy can be very stressful. In any case, the hypertension that may underlie the disease must be permanently and persistently lowered to prevent further damage. This means that, in addition to taking the appropriate medication, patients can do a number of things themselves to improve their situation. This includes, for example, abstaining from alcohol and nicotine. Nicotine in particular closes the vessels and thus aggravates the subcortical arteriosclerotic encephalopathy. Taking omega-3 fatty acids, on the other hand, is advisable. Fish oil capsules containing these fatty acids are commercially available, but flax oil is also a good source of omega-3 fatty acids.