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Binswanger's Disease - Binswanger's Subcortical Arteriosclerotic Encephalopathy (BSAE)

Binswanger's Disease is a rare form of vascular dementia, akin to Alzheimer's Disease, most commonly found in people of 60+ years, but may occur at any age between 44 and 75. Not a great deal is known about it as yet and it is probable that misdiagnoses occur. Correctly diagnosed or not, it is incurable and death usually occurs within 5 years of onset.

Symptoms

The first outward sign of BSAE is memory loss, difficulty with decision-making, and cognition. Depression and mood changes are common, often the patient's personality changes dramatically.

Motor problems - problems with walking, parkinson-like tremors, incontinence, poor co-ordination, seizures.

Strokes and hemiparesis, abnormal blood pressure (usually too high), heart valve disease and diseased blood vessels in the neck are evident - it is unknown if these are causal or symptomatic, but they occur some years before the onset of the dementia. Patients show increased fibrinogin and beta-thromoglobulin levels in the blood and higher than average platelet production.

Diagnosis

Diagnosis is first based on the above symptoms - patients are usually already taking drugs for high blood pressure and aspirin to prevent clotting. An MRI (Magnetic Resonance Imaging) scan shows the full extent of the disease. The scan shows damaged blood vessels (arteriosclerosis) in the deep white matter regions of the brain, typically the pons, basal ganglia, and thalamus. The smooth muscle in these arteries stiffens and there is a build-up of arterial plaque resulting in a reduction in blood flow and ischaemic lesions within the brain. There is patchy loss of myelin surrounding nerve cells.

Treatment

The disease is incurable - drug treatment can, as yet, merely slow down its progress.

Aspirin is prescribed as a prophylactic, and the usual advice about giving up smoking and eating a healthy diet should be adhered to since they are important risk factors.

Anti-fibrinogen drugs and anti-platelet agents may prove to be helpful by reducing the chance of blood clots if there is an early diagnosis.

Anti-depressants are given to treat the symptoms, but are of no curative value.


The material in this article is provided for informational purposes only. For specific medical advice or diagnosis, consult a healthcare provider


Refs include:
http://www.butler.org/education/pdf/Binswangers.pdf
http://serendip.brynmawr.edu/bb/neuro/neuro01/web2/Ledoux.html

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