Cerebral Microangiopathy: Symptoms, Causes And Treatment

This group of diseases damages the blood vessels of the brain and the functioning of the brain.

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

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

Cerebral microangiopathy: Concept and typical symptoms

Cerebral microangiopathy is understood to be 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 are ultimately the ones that come closest to and are in contact with the target areas.

In these types of disorders, the walls of these blood vessels can become fragile and break, easily producing brain hemorrhages that can cause a wide variety of symptoms depending on the area where they occur.

The most typical symptoms of these disorders, which derive from the moment in which the cerebrovascular accidents occur, include paralysis of part of the body, speech disturbance, the presence of continuous headaches, dizziness and vomiting, loss of consciousness and slowed speech and of the movement. Seizures and convulsions, sensory losses, mood disturbances, and even hallucinations and delusions can occur.

Cerebral microangiopathy is not fatal in itself, but the cerebral infarcts that it facilitates are very dangerous and can lead to the arrival of cardiovascular dementias, incapacitation and even the death of the patient. Symptoms generally do not appear before the age of forty-five, with the majority of strokes occurring at older ages. However, there are cases where they have occurred even in childhood.

Types of brain microangiopathy

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

Thus, within brain 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 degenerate progressively, losing elasticity and breaking easily. It is an inherited autosomal dominant disorder caused by mutations in the NOTCH3 gene.

2. Cerebroretinal microangiopathy with cysts and calcifications

A 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 characteristics 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 on chromosome 17.

3. Susac syndrome

Another disorder caused by microangiopathy is Susac syndrome. It is generated by a non-inflammatory microangiopathy whose main effects are at the cerebral, retinal and auditory levels, affecting the blood vessels that connect between these areas. It usually causes hearing and visual loss. It is suspected that its origin may be 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, with 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 cerebral microangiopathy, assuming relevant risk factors and taking into account when explaining some of these disorders and in some cases being their direct cause. Certain infections can also disrupt and damage blood vessels.

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

Treatment

As with the causes and even the symptoms, the specific treatment to be applied 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 blood vessels (especially it is necessary to monitor 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 symptoms and keep blood vessels stronger. It has been shown that the continuous application of corticosteroids can improve the condition of patients. Also other substances can improve symptoms, such as. Rehabilitation after an ischemic accident and psychoeducation of the affected person and their 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, DA; Vargas, SA 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, MM; Corredera, E .; Orizaola, P, Maciñeiras, JL (2004) Susac syndrome. Differential diagnosis. Med.Clin .; 123: 558-9.
  • Madrid, C .; Arias, M .; Garcia, JC; Cortés, JJ; López, CI; Gonzalez-Spinola, J .; De San Juan, A. (2014). CADASIL disease: initial findings and evolution of lesions on CT and MRI. SERAM.

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