Brain damage in RVCL-S patients can cause a wide range of symptoms. This brain damage can lead to forgetfulness or dementia. Sudden neurological deficits can also occur. The age at which people first develop neurological complaints varies greatly.
In RVCL-S, damage of the small vessels of the brain causes injury to the brain tissue. This can lead to several neurological complaints, such as forgetfulness and dementia, or changes in personality and behavior. For instance, patients can become apathic and loose the ability to take initiative, or patients can become more easily irritated or display a lack of social awareness. Sometimes, family and friends notice this before patient themselves, causing conflicts at work or in relationships. Furthermore, depression and anxiety are common complaints of RVCL-S. With age, complaints can become worse.
The injury in the brain tissue can also cause acute focal neurological deficits, such as sudden loss of strength and/or sensation in a part of the body. Swallowing or speech can also be suddenly disrupted. These neurological deficits can resolve completely, but they can also be permanent.
Lastly, RVCL-S patients suffer more frequently than average from migraine attacks.
What happens in the brain in RVCL-S?
Injury to the brain tissue is often visible on a brain MRI scan. In RVCL-S, this damage seems to occur mainly in the so-called white matter, this is the deeper part of the brain where the nerve connections run. We call these lesions white matter abnormalities.
Sometimes MRI scans show that the neurological complaints are caused by an inflammatory reaction in the brain. Often these inflammatory reactions are minor and disappear spontaneously after some time.
However, sometimes an inflammatory reaction becomes so severe that it displaces healthy brain tissue, then we call it a pseudotumor or tumefactive lesion. In that case, acute treatment with anti-inflammatory drugs is often required to reduce the inflammatory response.
More information about the treatment of and support for neurological complaints in RVCL-S patients can be found here.