Cerebral microangiopathy: symptoms, causes and treatment

In order for our brain to stay alive and function properly, it needs a constant supply of energy. oxygen and nutrients (especially glucose), which we get through respiration and feeding. Both elements reach the brain through irrigation carried out by the vascular system.

However, sometimes injuries and damage occur that cause blood vessels to stop working properly or rupture. One of the disorders that causes this is cerebral microangiopathy..

• Related article: "The 10 most common neurological disorders"

Cerebral microangiopathy: Concept and typical symptoms

Cerebral microangiopathy is understood as any disorder characterized by the presence of lesions or alterations in the part of the vascular system that supplies the brain. Specifically, reference is made to small blood vessels, arterioles and venules, which ultimately come closest to and are in contact with the target areas.

In this type of disorder the walls of these blood vessels can become brittle and rupture, easily producing cerebral hemorrhages that can cause a wide variety of symptoms depending on the area where they occur.

The most typical symptomatology of these disorders, which derives from the moment in which cerebrovascular accidents occur, includes paralysis of part of the body, alteration of speech, the presence of continuous headaches, dizziness and vomiting, loss of consciousness and slowing of speech and movement. Epileptic seizures and convulsions, sensory loss, mood disturbances, and even hallucinations and delusions can occur.

Cerebral microangiopathy It is not fatal in itself, but the strokes it facilitates are very dangerous and they can lead to the arrival of cardiovascular dementias, incapacitation and even the death of the patient. Generally, symptoms do not appear before the age of forty-five, with the majority of brain accidents occurring at later ages. However, there are cases in which they have occurred even in childhood.

Types of cerebral microangiopathy

The term cerebral microangiopathy does not designate a disease in itself, but rather refers to the group of disorders that cause an altered state of the small blood vessels.

Thus, within cerebral microangiopathies, various syndromes and disorders can be found, three of which we present below.

1. Autosomal dominant cerebral arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL).

Better known by its acronym, CADASIL, this disease of genetic origin especially affects the arterioles that connect with the subcortical nuclei of the brain, especially in the basal ganglia and around the ventricles.

The musculature of the walls of these blood vessels progressively degenerates, losing elasticity and breaking easily. It is an autosomal dominant hereditary disorder, caused by mutations in the NOTCH3 gene.

• Related article: "Basal ganglia: anatomy and functions"

2. Cerebroretinal microangiopathy with cysts and calcifications

Rare disease that causes various types of alterations in the connections between the visual organs and the brain, in addition to facilitating bleeding problems in the digestive tract. The main features of this disorder is the presence of cysts and calcifications in the thalamus, basal ganglia, and other subcortical regions in both hemispheres. The first symptoms usually occur during childhood, evolving rapidly. Its origin is found in mutations of the CTC1 gene of chromosome 17.

3. Susack's syndrome

Another disorder caused by microangiopathy is Susac syndrome. It is generated by a non-inflammatory microangiopathy whose main effects occur at the brain, retinal and auditory level, affecting the blood vessels that connect between these areas. It often causes hearing and visual loss. It is suspected that its origin may be found in autoimmune causes., although its exact etiology is still unknown.

Causes

The specific causes of each microangiopathy will depend on the type of disease or disorder that occurs.

In many cases, as occurs with CADASIL, the causes of this disorder are of genetic origin, presenting mutations in genes such as NOTCH3 or COL4A1. However, they can also be produced and/or favored by acquired factors. In fact, diabetes, obesity, high cholesterol and arterial hypertension play an important role both in developing it and in worsening the prognosis of a cerebral microangiopathy, assuming relevant risk factors and to be taken into account when explaining some of these disorders and in some cases being their cause direct. Certain infections can also alter and damage blood vessels.

It has also been documented the presence of a high amount of lipoprotein A in many cases of vascular accident, contributing an exaggerated amount of this substance to the facilitation of thrombi.

Treatment

As with the causes and even the symptoms, the specific treatment to apply will depend on which areas are damaged. In general, microangiopathies do not usually have a treatment that reverses the problem. However, prevention is essential both in the case of having a disorder or problem that facilitates the weakening of the vessels blood vessels (especially it is necessary to watch in those cases that suffer from hypertension, obesity and/or diabetes). That is why it is recommended to establish healthy lifestyle habits.

Apart from this, different therapeutic strategies can be applied to alleviate the symptoms and keep the blood vessels stronger. It has been shown that continuous application of corticosteroids can improve the condition of patients. Other substances also allow to improve the symptoms, such as. Rehabilitation after an ischemic stroke and psychoeducation to the affected person and her environment are other fundamental factors to take into account.

Bibliographic references:

• Aicardi J. (3rd Ed) (2009). Diseases of the Nervous System in Childhood. London: Mac Keith Press.

• Kohlschüter A, Bley A, Brockmann K, et al. (2010). Leukodystrophies and other genetic metabolic leukoencephalopathies in children and adults. Brain Dev. 32:82-9.

• Herrera, D.A.; Vargas, S.A. and Montoya, C. (2014). Neuroimaging finding of retinal cerebral microangiopathy with calcifications and cysts. Biomedical Journal. Vol.34, 2. National Institute of Health. Colombia.

• Magariños, M. M.; Corredera, E.; Orizaola, P, Maciñeiras, J.L. (2004) Susac syndrome. Differential diagnosis. Med. clin.; 123: 558-9.

• Madrid, C.; Arias, M.; Garcia, J.C.; Cortes, J. J.; Lopez, C. YO.; Gonzalez-Spinola, J.; De San Juan, A. (2014). CADASIL disease: initial findings and evolution of the lesions in CT and MRI. SERAM.