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What is hydrocephalus?
It is an important medical condition that results from abnormalities in the flow and resorption of cerebrospinal fluid (CSF); that is why it is also called as hydrodynamic CSF disorder. It is characterized by dilation of brain ventricles.
It is actually a combination of heterogeneous complex and multifactorial disorders. Genetic factors are also involved in the causation of it. The estimated incidence of it is 1 in 1500 births1. This disorder can be classified into:
Congenital hydrocephalus- it is present at the time of birth and may be associated with developmental defects. Recent studies show that almost 40% of such cases have possible genetic etiology1.
Hydrocephalus in adults:
(acquired type) – it occurs after the development of brain and its ventricular system1.
Normal pressure hydrocephalus or idiopathic hydrocephalus in adults-it is characterized by enlargement of cerebral ventricles and gait dysfunction in the absence of another cause. It is relatively rare but a potential cause of it in elders. It is a reversible condition if diagnosed timely. Sometimes, it is also called idiopathic adult hydrocephalus syndrome as intracranial pressure is not always normal as in normal pressure hydrocephalus2,5.
The symptoms and signs of it are affected by following factors3:
Blurred vision due to papilledema and optic atrophy
Double vision due to unilateral or bilateral 6th nerve palsy
Spascity leading to difficulty in walking especially affecting the lower limbs as the periventricular pyramidal tract is stretched by increased CSF.
In addition to the above children with this condition may have following features:
Sexual maturation problems
Short Documentary: Living with Hydrocephalus: Emma’s story
In addition to above described symptoms of it, adults with this disorder may have the following features:
Nausea aggravated by head movements
The following are the clinical features of normal pressure hydrocephalus4,5:
Gait dysfunction is usually the first symptom of normal pressure hydrocephalus and it may occur months or years before the appearance of other symptoms. The term magnetic gait means the tendency of feet to remain “stuck to floor” despite the patient’s best efforts to lift them up. Dementia may be the late symptom in this type and it presents as an impairment of recent memory.
Urinary incontinence, frequency, urgency and diminished awareness of the need to urinate are some other symptoms of normal pressure hydrocephalus.
Headache and fits are rare symptoms4.
Examination of infants may result in the following findings indicating the diagnosis of it3,4:
Patients having normal pressure hydrocephalus may have the following findings4,5:
Up going planter
Difficulty in walking
Frontal release sign
Normal muscle tone
The following tests may be required in case of hydrocephalus3:
CSF examination in case of post meningitis hydrocephalus
EEG if seizures are present
CT scanning to access the size of ventricles
Skull radiography to detect erosion of sella-turcica
MRI cine to measure the CSF stroke volume in 3rd ventricle
Diffusion tensor imaging
Surgical treatment is the treatment of choice for hydrocephalus. The following types of shunts can be placed after surgery3:
Acute onset hydrocephalus is an emergency and the following surgical procedures can be performed depending upon the specific case:
Open ventricular drainage
Lumbar puncture (LP)
VP or VA shunt
Several alternative procedures can also be applied such as:
Opening of a stenosed aqueduct
Endoscopic fenestration of the floor of the third ventricle
Conservative treatment is carried out in hydrocephalus as a temporary measure to delay the surgical intervention. Drugs used as conservative therapy include furosemide and acetazolamide3.
Benjamin Warf , MD, Neurosurgeon at Boston Children’s Hospital, explains the condition hydrocephalus and our innovative approach to treating the condition.
1. Zhang J, William MA, Rigamonti D. Genetics of human hydrocephalus. J Neurol 2006; 253(10):1255-66.
2. Shprecher D, Schwalb J, Kurlan R. Normal pressure hydrocephalus: diagnosis and treatment. Curr Neurol Neurosci Rep 2008;8(5):371-6.
3. Nelson SNJr. Hydrocephalus [Internet]. [Last updated 2013 Aug 5; cited 2013 Aug 19]. Available from: http://emedicine.medscape.com/article/1135286-overview.
4. Kiefer M, Unterberg A. The differential diagnosis and treatment of normal-pressure hydrocephalus. Dtsch Arztebl Int 2012;109(1-2):15-26.
5. Williams MA, Wilson RK. Evidence that congenital hydrocephalus is a precursor to idiopathic normal pressure hydrocephalus in only a subset of patients. J Neurol Neurosurg Psychiatry 2007;78(5):508-11.
Peter Scott is a medical writer that specializes in general health and medical research surrounding Spina Bifida and other disabilities. His 15 years of experience has seen his work published in Men's Health, Disability Horizons and New Mobility Magazine. He is currently traveling around the world working as a freelance writer.
Through our content we want to empower the lives of people with SB and to promote the prevention of it through education, public awareness and research. Working together with local organizations we aim to enhance the lives of those who are affected with SB. We want to build a stronger community and create a better future for those with SB.