0 CSF is the physiological 3rd compartment fluid produced at 20 ml per hour (500 cc/day).
0 It is secreted mainly from the choroid plexuses of the lateral, third, and fourth ventricles.
0 Transition from the ventricular system to the subarachnoid space is via the foramen Magendie and Luschka.
0 Absorption; it is towards the venous system with arachnoid granulations located in the superior sagittal sinus.
0 It is usually caused by an increase in CSF volume and impaired absorption, and rarely by hypersecretion.
0 Hydrocephalus: It is characterized by increased CSF volume, dilatation of cerebral ventricles.
0 Hydrocephalus Ex Vacuo: It is a condition that develops due to cerebral atrophy despite an increase in the CSF volume. CSF pressure is normal.
Signs and symptoms (adult)
1. Headache, lethargy
2. Incoordination, weakness
3. Cranial nerve palsies (Abducens paralysis makes this nerve more sensitive because the intracranial course of the nerve is long).
4. Corticospinal findings
MRI is the best diagnostic method.
In treatment, shunt is applied.
Shunts; It consists of a proximal ventricular catheter, a one-way valve, and a distal catheter.
The most common cause of shunt obstruction is choroid plexus intrusion into the shunt.
Causes of hydrocephalus
1. Congenital: Danny Walker, Arnold Chiari, primary aqueductal stenosis
2. Acquired: Infectious (most common cause of communicating hydrocephalus), post-hemorrhagic (second most common cause of communicating hydrocephalus), secondary to mass
Hydrocephalus classification
1. Obstructive (Non-communicating): There is a block in the proximal arachnoid granulation.
2. Communicative: CSF circulation is blocked at the level of arachnoid granulation.
Dandy-Walker Syndrome
Foramen Magendie and Luschka atresia.
Enlarged posterior fossa with cystic dilatation of the 4th ventricle
Hypoplastic cerebellar hemispheres, hypoplastic or agenetic vermis
Dilatation of the lateral and third ventricles
Hydrocephalus develops in 90% of cases.
There is agenesis of the corpus callosum in 17% of cases and occipital encephalocele in 7% of cases.
It may be associated with syringomyelia.
Ataxia, spasticity and weakness in motor control are seen.
Seizure may accompany.
Arnold-Chiari Malformation
There are four different types.
Type 1:
The cerebellar tonsils are located at the lower level of the foramen magnum. The medulla is not displaced caudally.
Syringomyelia and hydromyelia accompany in 50% of the patients.
May be asymptomatic.
Primary cerebellar ectopia is also called adult Chiari malformation (as it is diagnosed in the 2nd or 3rd decade of life).
The most common symptom is pain. Headache with head tilt or valsalva may occur.
Lhermitte's sign can be detected. Lower extremity involvement is usually in the form of bilateral spasticity.
Type 2:
Part of the cerebellar vermis and the brainstem extends down through the foramen magnum.
Syringomyelia, hydromyelia, spina bifida, meningomyelocele, Klippel-Feil (cervical fusion), CVS anomalies, GIS anomalies, GUS anomalies may accompany.
Type 3:
It is the most serious type.
Suboccipital encephalomeningocele is the displacement of all posterior fossa structures down the foramen magnum.
Type 4:
Cerebellar hypoplasia
Klippel-Feil Syndrome
• It is formed by cervical vertebra fusion.
• The main clinical features are short neck, lower nape-hairline, and limited neck movements.
Childhood hydrocephalus
1. Intraventricular hemorrhages
2. Ventriculitis
3. Colloid cysts of the 3rd ventricle
4. Suprasellar masses (blocks the foramen of Monro)
5. Pineal region tumors (compresses Aquaductus)
6. Aquaductus sylvius stenosis
7. Foramen Magendi and Luschka atresia (Dandy-Walker syndrome)
8. Tumors, meningitis, subarachnoid hemorrhage
9. Arnold-Chiari syndrome
• The most important finding is the enlargement of the head circumference.
• The fontanelles become prominent, cambered and taut. The veins on the head and face become prominent.
• Irregular breathing with apneic attacks
• Separation in cranial sutures
• Due to the increase in intracranial pressure, the eyes look down. This view is called the setting sign.
With rigid skull in older children/adults:
• Symptoms of increased intracranial pressure: papilledema, headache, nausea and vomiting, gait disturbances are common.
• Results:
Spastic walking,
Increased deep tendon reflexes,
motor neuron findings,
Endocrine and mental disorders,
Diabetes insipidus,
Precocious puberty and hypothalamic findings,
Cranial nerve palsies (most common 3rd and 6th cranial pairs),
Vision loss due to optic nerve compression,
Nystagmus,
drowsiness,
Epileptic seizures,
Irritability develops.
In patients who develop hydrocephalus after the fontanelles are closed, the clinical picture is only increased intracranial pressure syndrome.
Mc-Even symptom: Hearing the sound of a broken jug when hit on the head.
Normal pressure hydrocephalus
Also known as Adams-Judge Syndrome. It is seen around the age of 60.
0 3 characteristic findings in order
1. Gait disturbance-ataxia
2. Mental changes (Dementia)
3. Urinary incontinence
0 Gait disturbance is the first finding.
0 Gait disturbance progresses over weeks or months.
0 It causes more subcortical dementia.
0 CT and MRI are used for diagnosis.
0 The ventricles appear large on imaging without cortical atrophy. (must be distinguished from Alzheimer's)
0 It is the recovery of the patient's walking typically within hours after 25-40 cc CSF is taken at diagnosis.
0 Although dementia and incotinance improve somewhat after CSF removal, this is not a clear finding.
0 In treatment, shunt is applied.