Normal pressure hydrocephalus

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Classification according to ICD-10
G91.2 Normal pressure hydrocephalus
ICD-10 online (WHO version 2019)

At normal pressure (also age intracranial pressure ; abbreviation NPH of English normal pressure hydrocephalus ) is a hydrocephalus in which the intracranial pressure (pressure within the ventricles, where the cerebrospinal fluid is circulated) only for a short time, especially at night, is increased.

A distinction is made between primary or idiopathic normal pressure hydrocephalus with an unknown cause, and secondary forms, which are mainly caused by resorption disorders of the cerebrospinal fluid. In normal pressure hydrocephalus, there is a typical triad ( Hakim triad ) consisting of three symptoms : gait disorder , dementia and urinary incontinence .

Historical

It was first described in 1964 by the Colombian doctor Salomón Hakim (1922-2011). The first case was a 16-year-old Colombian boy who was in a car accident in 1957. However, after irreversible brain damage was diagnosed, Salómon Hakim recognized an enlarged ventricle in the X-ray. The boy was now incontinent and passed out. However, after a CSF puncture by Hakim, the patient woke up again, and after Hakim succeeded in continuously draining his brain water, the boy was able to go back to school.

This was the first time a form of dementia that could be treated was described.

etiology

The exact causes of the disease are not known; possibly various changes can lead to normal pressure hydrocephalus ( aqueduct stenosis , hydrocephalus malresorptivus - see also the article hydrocephalus ). In normal pressure hydrocephalus, the intracerebral pressure is normal for most of the time, there are only brief increases in intracranial pressure.

The expression "normal pressure hydrocephalus" has historical roots. It was shaped in the sixties by Salomon Hakim , who first described this clinical picture. At this point in time it was not yet possible to record the intracranial pressure continuously over several days. In the meantime we know that the so-called normal pressure hydrocephalus is not a hydrocephalus with always normal intracranial pressure.

Rather, there is a strongly fluctuating course of intracranial pressure, especially at night, and intracranial pressure peaks often occur at abnormal levels. Correctly, this form of hydrocephalus should therefore be called intermittent normal pressure hydrocephalus or intermittent high pressure hydrocephalus. However, it is the sum of all short-term nocturnal increases in intracranial pressure that damage the brain in the long term and cause failure symptoms.

If you carry out a pressure measurement (e.g. as part of a liquor puncture), the measurement is only meaningful if you accidentally puncture during the time during which there is an increase in pressure. To prove a pathologically increased pressure increase in the skull in the context of normal pressure hydrocephalus, a nerve water pressure measurement over several days is necessary.

Epidemiology

Most adults around the age of 60 develop idiopathic normal pressure hydrocephalus. The secondary normal pressure hydrocephalus, on the other hand, occurs in all age groups. The idiopathic and the secondary form are roughly equally common. There is no gender preference. There are no reliable data for the prevalence and incidence . The information on the incidence of NPH varies widely and is between 2 and 20 per million inhabitants per year in different studies. Estimates assume a much greater frequency. A door-to-door study in Starnberg in the early 1990s found a prevalence of 0.4% among people over 65 years of age.

Symptoms

The disease manifests itself in a classic triad ( Hakim triad ), consisting of

  • Gait disorder with slow, small steps, with feet typically sticking to the ground, hence also called " magnetic gait ", difficulties in getting up and insecurity with falling. Walking seems apractical , which is why it is also called "G angapraxie ". The gait pattern is partly similar to a Parkinson's gait, but other symptoms of Parkinson's syndrome are missing, from which the misleading term " Lower Body Parkinsonism " is derived. Pyramid orbit signs can be added.
  • Dementia with apathy
  • Bladder dysfunction, usually in the form of bladder autonomy with an urge to incontinence

The gait disorder is required by some authors.

diagnosis

If clinical symptoms are present, a CT scan or, alternatively, an MRI scan of the brain should be performed; If further suspicious factors are found here, a larger amount of cerebrospinal fluid is taken as a sample, after which the symptoms of the disease should improve (but not necessarily - even if NPH is present). Alternatively, hemodynamic tests or an intracranial pressure measurement can be performed.

In the differential diagnosis, secondary normal pressure hydrocephalus (e.g. meningitis or subarachnoid hemorrhage), chronic occlusive hydrocephalus, Alzheimer's disease, Parkinson's disease, but also cervical myelopathy and lumbar canal stenosis must be excluded.

therapy

Normal pressure hydrocephalus is often easy to treat. The earlier therapy is started, the better the result. The problem is that the diagnosis is made too seldom and is often confused with other diseases. The therapeutic gold standard is the creation of a ventriculoperitoneal shunt . After early therapy, the gait disorder in particular, and to a lesser extent the continence, can improve.

literature

Web links

Commons : Normal Pressure Hydrocephalus  - Collection of images, videos, and audio files

Individual evidence

  1. ^ Sabine Schuchardt: Salomón Hakim, the engineer doctor. Deutsches Ärzteblatt 2019, volume 116, issue 31–32 of August 5, 2019, page [96]; Link accessed on August 15, 2019
  2. ↑ Clinical Neurophysiology, University Medical Center Göttingen: Normal pressure hydrocephalus ( Memento of the original from January 15, 2016 in the Internet Archive ) Info: The archive link was inserted automatically and has not yet been checked. Please check the original and archive link according to the instructions and then remove this notice. . Retrieved February 27, 2013. @1@ 2Template: Webachiv / IABot / www.neurologie.uni-goettingen.de
  3. ^ S. Hakim, RD Adams: The special clinical problem of symptomatic hydrocephalus with normal cerebrospinal fluid pressure. Observations on cerebrospinal fluid hydrodynamics. In: Journal of the neurological sciences. Volume 2, Number 4, 1965 Jul-Aug, pp. 307-327, ISSN  0022-510X . PMID 5889177 .
  4. ↑ Clinic for Neurosurgery, Saarland University Hospital: Hydrocephalus - Questions and Answers ( Memento of the original from October 11, 2015 in the Internet Archive ) Info: The @1@ 2Template: Webachiv / IABot / www.uniklinikum-saarland.de archive link has been inserted automatically and has not yet been checked. Please check the original and archive link according to the instructions and then remove this notice. . Retrieved February 27, 2013.
  5. ↑ RC Petersen, B. Mokri, ER Laws: Surgical treatment of idiopathic hydrocephalus in elderly patients. In: Neurology . Volume 35, Number 3, March 1985, pp. 307-311, ISSN  0028-3878 . PMID 3974888 .
  6. ↑ A. Marmarou et al: Diagnosis and management of idiopathic normal-pressure hydrocephalus: a prospective study in 151 patients. In: Journal of Neurosurgery . Volume 102, Number 6, June 2005, pp. 987-997, ISSN  0022-3085 . doi: 10.3171 / jns.2005.102.6.0987 . PMID 16028756 .
  7. ^ JK Krauss, B. Halve: Normal pressure hydrocephalus: survey on contemporary diagnostic algorithms and therapeutic decision-making in clinical practice. In: Acta Neurochirurgica . Volume 146, Number 4, April 2004, pp. 379-388, ISSN  0001-6268 . doi: 10.1007 / s00701-004-0234-3 . PMID 15057532 .
  8. ↑ C. Trenkwalder et al: Starnberg trial on epidemiology of Parkinsonism and hypertension in the elderly. Prevalence of Parkinson's disease and related disorders assessed by a door-to-door survey of inhabitants older than 65 years. In: Archives of Neurology . Volume 52, Number 10, October 1995, pp. 1017-1022, ISSN  0003-9942 . PMID 7575219 .
  9. ^ S1- guideline normal pressure hydrocephalus of the German Society for Neurology. In: AWMF online (as of 2008)
  10. ^ U. Kehler: Normal Pressure Hydrocephalus - Clinical Characteristics and Differential Diagnosis . Ed .: M. Fritsch, U. Kehler, U. Meier. Georg Thieme Verlag, Stuttgart / New York / Delhi / Rio 2014, ISBN 978-3-13-164601-9 , pp. 16-22 .
  11. ↑ M. Kiefer, A. Unterberg: The differential diagnosis and treatment of normal-pressure hydrocephalus. In: Deutsches Ärzteblatt international. Volume 109, Number 1-2, January 2012, pp. 15-25, ISSN  1866-0452 . doi: 10.3238 / arztebl.2012.0015 . PMID 22282714 . PMC 3265984 (free full text). (Review).