Cortico-basal degeneration (DCB)

Content

Introduction

Introduction

Corticobasal degeneration (DCB) is a rare degenerative disease of the central nervous system that usually occurs after the age of 60. It is caused by the loss of nerve cells (neurons) and the reduction (atrophy) of specific areas of the brain, such as the cerebral cortex And the basal ganglia.

DCB is a rapidly progressing disease characterized by a wide variety of signs and disorders (symptoms) such as, for example, poor motor coordination, stiffness, speech, language, vision, behavior and mental abilities, reasoning and memory (cognitive skills).

Symptoms

Symptoms

The signs and clinical manifestations (symptoms) of cortico-basal degeneration (DCB) are very different and can vary from person to person. The most common are:

  • inability to control movements, in some cases sick people do not perceive or cannot control the movement of a limb (alien limb syndrome)
  • slow movement of one side or both sides of the body, which gets worse over time
  • rigidity
  • tremors
  • repeated muscle contractions
  • problems with balance and difficulty walking (ambulating)
  • disturbances in the perception of space and the ability to attribute meaning to perceived images (visuoconstructive skills)
  • speech and language disorders
  • attention and memory difficulties
  • behavior and personality disorders

Disorders caused by BCD progress rapidly; within 6-8 years, they can cause inability to walk and even serious complications.

Causes

Causes

The cause of cortico-basal degeneration (DBC) is currently unknown; Genetic and environmental factors capable of influencing the development of the disease have not yet been identified. It is estimated that fewer than one in 100,000 people get DBC each year and that the disease affects men and women equally.

It is known that a protein normally found in the brain, the protein tau, form of the abnormal aggregates in the brains of people with DBC. However, it has not yet been clarified why the protein tau is altered in DCB and whether the accumulation of this protein is responsible for the death of specific neuronal cells. In DCB, the degeneration of nerve cells mainly affects areas of the brain called cerebral cortex And basal ganglia.

The presence of protein aggregates tau it is common to other neurodegenerative diseases, defined for this reason taupathie, such as Alzheimer's disease, progressive supranuclear palsy and Pick's disease.

Diagnosis

Diagnosis

The assessment (diagnosis) of cortico-basal degeneration (DCB) is based on the neurological examination. Diagnosis of DCB, however, is not only very difficult in the early stages, it can also be very difficult in the more advanced stages of the disease. The disorders (symptoms) caused by DCB, in fact, overlap with those of other neurodegenerative diseases, such as Parkinson's disease, Alzheimer's disease, Pick's disease, progressive supranuclear palsy or Creutzfeldt-Jakob disease. It is estimated that about half of people with signs and disorders of DCB also have "another neurodegenerative disease. In some cases, the assessment of DBC can only be done by examining brain tissue after death (autopsy)."

Image-based instrumental investigations, such as computed axial tomography (CT) and magnetic resonance imaging (MRI), can be used to reveal changes in areas of the brain affected by BCD (cerebral cortex, basal ganglia) and rule out other diseases with similar disorders.

Therapy

Therapy

To date, there is no approved treatment that helps slow the rapid progression of neuro-basal degeneration (DBC).

However, individual complaints (symptoms) can be acted upon. Your doctor may recommend medications to reduce muscle stiffness, spasms, and contractures.

Physiotherapy, occupational therapy, and speech therapy can help manage the disabilities caused by the disease.

In-depth link

In-depth link

Mayo Clinic. Corticobasal degeneration (English)

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