OVERVIEW
Binswanger’s disease, also known as subcortical atherosclerotic encephalopathy, is a relatively common form of small-vessel dementia with a significant history of hypertension. Middle-aged and elderly patients may present with cognitive dysfunction, mild limb dyskinesia, ataxia, and urinary incontinence. Neuroimaging reveals cerebral white matter atrophy and paraventricular white matter sparing with multiple lacunar infarcts, and early detection of this disease may prevent the risk of stroke.
Causes
The etiology of Binswanger’s disease is unclear. Fisher (1989) found in 72 patients with pathologically confirmed Binswanger’s disease that 94% had a history of hypertension, suggesting that the disease may be related to hypertension and vitreous degeneration of deep small arteries in the white matter.
Symptoms
1. Most cases develop at the age of 55-65 years, and most cases have a history of hypertension for many years, with an insidious onset and a subacute or chronic course.
2. Chronic progressive dementia, focal neurological signs and psychiatric symptoms; the condition may be stable for a long time or rapidly aggravated after stroke. Cognitive impairment is the first symptom, memory loss, depression, disorientation, and the development of life can not be completely self-care. Limb movement disorders are mild and may include ataxia and urinary incontinence; signs of complete hemiparesis are rare, and pseudo medullary palsy may occur.
Examination
Routine examination of cerebrospinal fluid and determination of APOE polymorphism and quantification of Tau protein and β-amyloid fragment in cerebrospinal fluid and serum have diagnostic and differential significance.
1. Electroencephalogram
The rhythm slows down to below 8-9 Hz, and diffuse theta waves appear bilaterally in frontal, temporal and central regions, which may be accompanied by focal paroxysmal high-amplitude δ rhythms. The latencies of visual evoked potentials (VEP), brainstem auditory evoked potentials (BAEP) and event-related potentials (ERP) P300 were significantly prolonged compared with those of the same-age control group, and 40% of the patients could not evoke obvious P300 waveforms, suggesting severe cognitive impairment.
2.Imaging examination
CT showed mild cortical atrophy, varying degrees of ventricular dilatation, and patchy low-density shadows with blurred borders in the anterior and posterior corners of bilateral ventricles and on both sides of the body, which could be accompanied by multiple lacunar infarcts in the basal nuclei, thalamus, and pontine plexuses and other areas rich in small arteries that penetrate the medulla oblongata. MRI showed brain atrophy mainly in the white matter, with a lighter cortical appearance, and scattered multiple T1WI low signals around bilateral ventricles and the center of the hemi-oven, with high signals, accompanied by multiple lacunar infarcts. high signal with multiple foci of lacunar infarction.PET examination showed diffuse reduction of cerebral blood flow in the white matter around bilateral ventricles, with significant reduction of glucose and oxygen metabolism.
Diagnosis
The diagnosis was based on the presence of cognitive dysfunction, mild limb movement disorders, ataxia, and urinary incontinence in chronically hypertensive middle-aged and elderly patients, neuroimaging showing cerebral white matter atrophy, paraventricular white matter sparing with multiple lacunar infarct foci, and adjunctive examinations.
Differential diagnosis
1. Normal cranial pressure hydrocephalus
Progressive gait abnormalities, urinary incontinence, and dementia triad are also manifested in this disease. Enlarged ventricles. With insidious onset, history of traumatic brain injury, subarachnoid hemorrhage or meningitis before the disease, no history of stroke, younger age of onset, normal intracranial pressure on lumbar puncture, symmetrical enlargement of bilateral ventricles on CT, obvious dilatation of the third and fourth ventricles and midbrain aqueducts, and no evidence of cerebral infarction on imaging.
2. Multiple sclerosis (MS)
MRI shows scattered multiple T1WI low signal and T2WI high signal in the white matter adjacent to the body of lateral ventricles, and the lesions are not related to the distribution of blood vessels. MS has a younger age of onset, and presents with spinal cord, brainstem, cerebellum and optic nerve signs and symptoms, a remission-relapse course of the disease, and elevated CSF lymphocytes, elevated IgG index and oligoclonal bands.
3. Alzheimer’s disease (AD)
Gradually appearing memory impairment, cognitive dysfunction, daily life requires the help of others, serious cases are bedridden, CT can see obvious cerebral cortical atrophy and ventricular dilatation, confirmed diagnosis requires brain tissue biopsy. Sometimes AD can coexist with vascular dementia, when AD is often accompanied by amyloid cerebrovascular disease and combined with lobar hemorrhage.
Complications
There may be a combination of autonomic dysfunction, hypertension manifestations. In addition, secondary lung infections, urinary tract infections and pressure sores should be noted.
Treatment
1. Traditional Chinese medicine treatment
Triphala total saponin (Thromboxane), Puerarin (Pulein) and Chuanxiongxizine (Methylpyrazine) are used, which have the effects of activating blood circulation and removing stagnation, improving blood viscosity and anti-platelet aggregation.
2. Western medical treatment
(1) Improve cerebral circulation, increase cerebral blood flow, and improve oxygen utilization ① Dihydroergot alkaloids eliminate vascular spasm and increase blood flow to improve neuron function, commonly used diergot alkaloids, orally, as well as ergot alkaloids (ergot bromide nicotinic ester). ② Calcium antagonists increase cerebral blood flow, prevent calcium overload and free radical damage, dihydropyridines such as nimodipine; treatment of leukodystrophy patients with cognitive deficits, 1 year after the disease is stable or improved, dibenzylamines such as flunarizine. (iii) Niacin can increase cerebral blood flow and improve memory.
(2) Brain metabolizers Promote the utilization of amino acids, phospholipids and glucose by brain cells, enhance the patient’s responsiveness and excitability, and enhance memory. (1) Pyrrolidone commonly used piracetam (cerebrofacial) and fennel racetam can increase the formation and operation of adenosine triphosphate (ATP) in the brain, increase the utilization of glucose and protein synthesis, and promote the transmission of information in the cerebral hemisphere. ② Meclofenoxate can act as a central hormone, increase glucose utilization, excite the central nervous system and improve learning and memory functions. ③Dihydroergotoxine mesylate (dihydroergotoxine) enhances the release of transmitters from presynaptic nerve endings, stimulates postsynaptic receptors, and improves nerve function and energy balance of brain cells. ④ Amitriptyline such as amitriptyline/lobacillin (Docusate) can increase arterial oxygen partial pressure and oxygen saturation, increase oxygen supply, improve microcirculation and cerebral metabolism. ⑤ Others, such as brain protein hydrolyzate (cerebrolysin), cytidine (cytidine diphosphate), adenosine triphosphate (ATP), coenzyme A and so on.
(3) Brain protective drugs ① Calcium antagonists such as nimodipine and flunarizine. ② Excitatory amino acid receptor antagonists such as magnesium sulfate and MK801. ③ Free radical scavengers such as vitamin E, vitamin C and ginkgo biloba preparations.
Prevention
1. Early detection and avoidance of risk factors for stroke, such as hypertension, diabetes mellitus and hyperlipidemia, and active treatment. Those with high carotid artery stenosis can be treated with surgery, which can help reduce the occurrence of vascular dementia.
2. Quit smoking, control alcohol consumption and have a reasonable diet.
3. Those with clear genetic background should undergo genetic diagnosis and treatment.