Normotensive hydrocephalus( also called Hakim-Adams syndrome) is a disease that is clinically manifested by disordered gait, impaired cognitive function and retention.
These symptoms, especially in older patients, are quite common and are not always easily associated with suspected hydrocephalus.
- etiopathogenesis and pathophysiology
- Varieties of flow disturbances
- clinical picture
- Methods setting
- diagnosis CT brain
- lumbar puncture
- lumbar drainage
- Opportunities therapy
- Complications and prognosis
etiopathogenesis and pathophysiology
a history of some patients suspected of Hakim-Adams syndromeyou can find a bruise, brain injury, subarachnoid hemorrhage or inflammation of the central nervous system, whichOver the years, led to the expansion of the ventricular system, and subsequently - to decompensation and clinical manifestations.
Most patients, however, have an idiopathic disease where a clear causal relationship is not obser
From the pathophysiological point of view, the hydrodynamic concept of increased pressure of liquor pulsations in the ventricles is recognized without increasing intraventricular pressure, which leads to the formation of hydrocephalus.
Causes of cerebrospinal fluid drainage disorders can be many. It can be associated with the manifestation of congenital defects, meningitis, tumors, hemorrhages in the brain, a consequence of a stroke or head trauma, and also develop in view of age.
Variation of the course of the
disorder There are two types of Hakim-Adams syndrome flow in acute and chronic forms.
Functional classification of normotensive hydrocephalus:
- is obstructive;
- is hypersecretory;
- is hypersorptive.
In terms of dynamics( important for treatment)
Clinical picture of
Symptoms that indicate the development of normotensive hydrocephalus:
- walking disorder ( frontal apraxia): slowing of walking, shortening of pitch, decreasing step height;
- cognitive impairment of , reminiscent of signs of dementia - apathy, decreased concentration, loss of interest, memory problems, psychomotor slowdown as a whole;
- discontinuation of the continuum - first intermittent urinary incontinence up to incontinence, including stool incontinence.
Symptoms can be differently expressed. Most often, a walking disorder that first precedes cognitive disorders first manifests itself, and, in the last instance, discontinuation of the continent develops.
Additional symptoms that may be present( headaches, tinnitus, psychoaffective disorders) are not typical, but do not exclude the diagnosis of NG.As a rule, they are characteristic for patients older than 60 years, so these clinical manifestations can be attributed to aging and their occurrence can be underestimated by the attending physician.
Methods for diagnosing
The following research methods are used to diagnose the disease.
CT of the brain
If a disease is suspected, a CT scan of the brain should be performed, which attests to the expansion of the ventricular system.
Other pathological changes leading to hydrocephalus( tumors, congenital anomalies, vascular malformations) and possible hydrocephalus "ex vacuo" should be excluded, in which expansion of the ventricular system develops from brain atrophy and clinical manifestations may be similar.
Based on the results of CT and clinical suspicions of normotensive hydrocephalus, the patient is examined by a neurologist who, using structurally more appropriate MRI and clinical evaluation, conducts a differential diagnosis( other types of dementia and gait disorders - degenerative, intoxication, metabolic, infectious)causes the patient to be sent to the appropriate department for further clinical investigation, checking walking and conducting cognitive tests.
In case of suspicion of the disease, a liquorodynamic examination is performed.
The simplest test is spinal puncture, during which the needle is inserted into the lumbar region of the spinal canal in the lumbar region and intracranial pressure( potential exclusion of intracranial hypertension) is measured, after which 30-50 ml of cerebrospinal fluid( CSF) are collected. The sensitivity of the test is about 50-60%.
With negative tests given above, but with a high clinical suspicion, lumbar drainage can be performed with a sensitivity of up to 90%.
Continuous drainage of cerebrospinal fluid 10 ml / h for 3 days simulates the introduction of VP shunt and leads to clinical improvement.
The possibilities of therapy
The main method of treatment for Hakim-Adams syndrome is the introduction of a ventriculoperitoneal( VP) shunt. The operation takes about an hour, and in its course the lateral ventricle is treated, which is connected through a tube with a valve, driven by a subcutaneous tissue of the abdominal cavity.
There are also alternatives - ventriculoatrial and lumboritoneal shunt. In recent years, also performed ventokulostroemia, in which endoscopically fenestrirotsya 3 ventricles.
Brain CT scan is performed 2-3 days after surgery in order to avoid possible complications. These include bleeding, improper position of the shunt, etc. In the long term, it can reach the infection, which can lead to a disruption in the closure of the shunt and the need for repeated treatment.
To reduce the number of infections leads to impregnation with antibiotics of the injected shunts. The risk of infection during the first year was only 5%.
With proper indication, a relatively rapid and significant improvement in gait disorders occurs. Improvement of continent disorders is gradual, and patients with a marked significant deterioration in cognitive abilities react less positively to it.
After effective treatment, nevertheless, regular clinical monitoring with CT( 1 time per year) is necessary.
Complications and prognosis
The incidence of complications after operations in patients with NG is different in the literature. Mortality does not exceed 2% and, according to some authors, is more associated with concomitant diseases than with shunt implantation.
The formation of subdural disturbances is in the range of 2-17%.The percentage of these complications is significantly reduced with the use of modern programmable valves.
The risk of infectious complications is relatively low, and does not exceed 5-6%.Other complications may include the failure of the shunt itself, the formation of hematomas after a puncture of the ventricles and even the development of epilepsy.
An important role is played by the duration of worsening walking before surgery, and patients whose gait disorder lasted less than 1 year have the best prognosis.
The positive effect of the above methods of treating the disease is 70-80% of patients. Over the course of several years, the shunt effect decreases, and it is also necessary to regularly reduce the bypass pressure.
In order not to become a victim of the disease, a person should be aware of precautions. You should always cover your head in a risky job, use helmets when riding a motorcycle, detect and treat infectious and inflammatory diseases on time, and avoid hypothermia of the head.