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Normotensive hydrocephalus, also known as Hakim's syndrome, does not lead to enlargement of the head circumference. Symptoms appearing in the course of normotensive hydrocephalus can be confusing and suggest other conditions, e.g. Alzheimer's disease. So how is Hakim syndrome diagnosed and how is it treated?

Normotensive hydrocephalusis also known as Hakim's syndrome. This name comes from the author of the first description of this disease - it was done in 1965 by Solomon Hakim.Hakim's syndromeis most often observed in patients aged 60 to 70 years. Due to the fact that some patients with normotensive hydrocephalus have a completely different diagnosis (which is due to the fact that the symptoms of the disease are not specific), its exact frequency is not known, however, it is estimated that Hakim's syndrome may occur in about 0, 5 percent of patients over the age of 60. Hydrocephalus is defined as a condition in which there is an excess of cerebrospinal fluid in the ventricular system of the brain. There are various forms of it, the basic division includes the distinction of non-communicating and communicating hydrocephalus (the differences include the possibility of the flow of the cerebrospinal fluid through the structures of the ventricular system). In the case of the latter category, one of the subtypes of communicating hydrocephalus is normotensive hydrocephalus (i.e. with normal intracranial pressure).

Normotensive hydrocephalus (Hakim syndrome): causes

The cause of Hakim's syndrome is disturbances in the absorption of the cerebrospinal fluid. Normotensive hydrocephalus may occur as a result of:

  • having head injuries
  • tumors of the central nervous system
  • meningitis
  • complications after surgery performed on the head
  • bleeding into the subarachnoid space

Listed above are only half of the causes of Hakim syndrome. In the rest of the patients, the causes of the disease cannot be detected - in 50% of cases normotensive hydrocephalus is idiopathic.

Normotensive hydrocephalus (Hakim's syndrome): symptoms

In the course of Hakim's syndrome, pressure is initially increasedintracranial. The effect of this phenomenon is the expansion of the ventricular system of the brain. After some time, the intracranial pressure gradually decreases, but its values ​​remain within the upper normal range. The existing expansion of the ventricular system, however, does not regress - the enlarged ventricles may put pressure on the brain tissue, leading, among others, to The following symptoms may appear in patients with normotensive hydrocephalus:

  • gait disturbance (leaning body posture, walking with widely spaced legs and small steps - it may resemble that the patient is walking on sticky ground)
  • urinary incontinence
  • dementia disorders (manifested by memory impairment, concentration disorders, difficulty making decisions and apathy, changes in personality and behavior, and slowing down of the pace of thought processes)

Not all of the above-mentioned ailments appear in all patients with normotensive hydrocephalus. If the patient has gait disturbances, urinary incontinence and dementia disorders at the same time, the Hakim triad typical of normotensive hydrocephalus can be observed.

Normotensive hydrocephalus (Hakim syndrome): recognition

Symptoms that appear in the course of Hakim's syndrome may also occur in other neurological diseases, such as Parkinson's disease, frontotemporal dementia, Wilson's disease, Alzheimer's disease or dementia with Lewy bodies. For this reason, the patient must undergo a detailed differential diagnosis. Imaging studies are of fundamental importance in the diagnosis of normotensive hydrocephalus. Patients can undergo computed tomography of the head and magnetic resonance imaging. Ventricular dilatation may be seen in these tests. In diagnostics, measurements of the pressure of the cerebrospinal fluid and the so-called Fisher's test. The Fisher test is based on a lumbar puncture and the release of a certain amount of cerebrospinal fluid (usually more than 30 ml). If the patient's condition improves 30-60 minutes after this activity, this may indicate a diagnosis of Hakim's syndrome. The Fisher test is also useful when analyzing whether the implementation of Hakim's syndrome treatment will be able to bring the expected results.

Normotensive hydrocephalus (Hakim's syndrome): treatment

In the treatment of normotensive hydrocephalus surgical treatment is used - there is no effective pharmacological treatment in patients with thisproblem. Patients undergo ventricular peritoneal valve implantation to drain excess cerebrospinal fluid from the skull structures into the abdominal cavity. After valve implantation, the greatest improvement is usually seen in motor dysfunction. In the case of other symptoms of Hakim's syndrome, such as urinary incontinence or dementia disorders, the results of surgical treatment are unfortunately not that significant.

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Category:

Neurology