Hydrocephalus and Cerebral Edema
Questions
1. Which of the following is associated with communicating hydrocephalus with normal cerebrospinal fluid pressure, production, and reabsorption?
A. choroid plexus papilloma
B. cortical atrophy
C. subarachnoid hemorrhage
D. tuberculous meningitis
E. venous sinus thrombosis
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1. Answer B. (MN-110) Cortical atrophy associated with cognitive aging disorders results in hydrocephalus ex vacuo with an otherwise normal intracranial pressure, cerebrospinal fluid (CSF) production, flow, and reabsorption. Choroid plexus papilloma is the only known cause of hydrocephalus to oversecretion of CSF. Obstructive hydrocephalus describes conditions that result in near complete blockage of CSF flow within the ventricular system, such as acute intraventricular hemorrhage or a third ventricular colloid cyst, among other causes. Aside from normal pressure hydrocephalus, causes of communicating hydrocephalus often involve some degree of poor reabsorption of CSF due to blockage of the arachnoid villi (post-inflammatory or post-hemorrhagic) or occlusion of cerebral veins. Tuberculous meningitis and subarachnoid hemorrhage also impair the absorptive capacity due to inflammation in setting of infection and/or bleeding.
2. A 24-year-old otherwise healthy man presents to the ED with 3 weeks of headaches and blurred vision, and this morning he found it difficult to wake up from sleep. On examination he is somnolent, awakens only to strong physical stimulation, and is unable to count backward from 10 without falling back to sleep. He is also tachypneic and tachycardic. CT head reveals widely dilated third and lateral ventricles, a heterogenous hyperdensity within the third ventricle, and a normal-appearing 4th ventricle. Which is the next best stop in management?
A. Give high-dose IV dexamethasone
B. Obtain MRI brain with and without contrast to make a clear diagnosis
C. Place a lumbar drain (LD) to drain CSF and relieve pressure
D. Place a ventriculoperitoneal shunt (VPS) to drain CSF and relieve pressure
E. Place an external ventricular drain (EVD) to drain CSF and relieve pressure
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2. Answer E. (MN-110) The patient has acute obstructive hydrocephalus from an intraventricular mass and is rapidly decompensating. In addition to basic resuscitation of ABC’s, this is a neurosurgical emergency and this patient needs immediate intervention. Placement of an EVD is the most appropriate next step, which will immediately divert CSF flow and reduce intracranial pressure. VPS placement is not typically performed in an emergent fashion, and LD placement is dangerous in this setting as it could cause downward brain herniation. Steroids are unlikely to be helpful if vasogenic edema is not the primary cause of obstruction and will take many hours to take effect. The patient will later need an MRI brain for better characterization of the mass; however, this is lower priority and the CT provides adequate information for immediate patient care.
3. In infants with congenital hydrocephalia, tapping of the skull during clinical examination eliciting a “cracked pot” sound is known as:
A. Colier sign
B. Macewen sign
C. McConnell sign
D. Monroe sign
E. Murphy sign
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3. Answer B. (MN-110) Macewen sign, named for Sir William Macewen (1848-1924), a surgeon in Glasgow, Scotland, is a clinical sign to detect hydrocephalus and brain abscess. Percussion (tapping the middle finger) on the skull at a particular spot (near the junction of the frontal, temporal, and parietal bones) yields an unusually resonant sound in the presence of hydrocephalus or a brain abscess. Collier sign is bilateral or unilateral eyelid retraction and is an accepted medical sign of a midbrain lesion. McConnell sign is regional RV dysfunction with akinesia of the mid free wall but normal motion at the apex; it is a distinct echocardiographic finding described in patients with acute pulmonary embolism. Murphy sign, indicative of acute cholecystitis, is tested for during an abdominal examination and is performed by asking the patient to breathe out and then gently placing the hand below the costal margin on the right upper quadrant (the approximate location of the gallbladder).
4. Which of the following is typically the first symptom of normal pressure hydrocephalus (NPH)?
A. Argyll-Robertson pupil
B. Axial rigidity
C. Magnetic gait
D. Progressive cognitive impairment
E. Urinary incontinence
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4. Answer C. (MN-110) Gait change, described as magnetic gait, is usually the first symptom, as well as the most frequent symptom, of NPH. The clinical finding is more accurately an axial apraxia with the most obvious clinical changes being gait. The presumed anatomic basis is involvement of bilateral paramedian prefrontal and primary cortical and adjacent subcortical regions due to hydrocephalus. This change may be subacute, fluctuating, or more chronic but most often worsens over weeks or months. In addition, axial rigidity from extrapyramidal dysfunction, dementia, and urinary incontinence typically occur. The Argyll-Robertson pupil is usually seen in tertiary syphilis, not NPH.
5. Which of the following is true about brain edema?
A. A key component of vasogenic edema involves tightening of the blood-brain barrier
B. Interstitial edema results from bulk flow of water into the periventricular extracellular fluid compartment in patients with hydrocephalus
C. Ionic edema refers to an intermediate phase of cytotoxic edema in which sodium and other ions accumulate within the intracellular space
D. Osmotic edema results from sudden increases in serum osmolality
E. The inciting cause of cytotoxic edema is intracellular chloride accumulation due to energy failure and ATP depletion
View Answer
5. Answer B. (MN-111) Interstitial edema results from bulk flow of water into the periventricular extracellular fluid compartment in patients with hydrocephalus. Osmotic edema results from acute reductions in serum osmolality, as occurs with treatment of diabetic ketoacidosis or dialysis. The inciting cause of cytotoxic edema is intracellular sodium accumulation due to energy failure and ATP depletion. Ionic edema refers to an intermediate phase of cytotoxic edema in which sodium and other ions accumulate within the extracellular space. A key component of vasogenic edema involves blood-brain barrier disruption.
6. Which of the following is true of interventions for elevated ICP?
A. A ventricular drain should be placed in all patients
B. CPP should be optimized between 80 and 120 mm Hg
C. Hypertonic saline and mannitol are incompatible and should not be used in the same patient
D. Mannitol can be effective even if serum osmolality exceeds 320 mOsm/L
E. Sedation should be avoided to allow for serial neurological assessments
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6. Answer D. (MN-111) Mannitol and hypertonic saline boluses can continue to be effective even if osmolality exceeds 320 mOsm/L, at the expense of further increases in osmolality. Parenchymal ICP monitors can be used to monitor ICP in patients without ventriculomegaly. CPP should be optimized between 60 and 100 mm Hg in most patients. Sedation is a cornerstone intervention for ICP but should be interrupted for neurological assessments even if ICP is an issue. Hypertonic saline and mannitol can be used in the same patient.
7. A 30-year-old man presents with severe traumatic brain injury after falling off a ladder. GCS is 8 (eye 2, motor 4, and verbal 2) prior to intubation, and the pupils are 4 mm and sluggish on the right and 5 mm and fully reactive on the left. After intubation head CT shows global cerebral edema, slit ventricles, bifrontal and temporal contusions, and a 5-mm right subdural hematoma with 3 mm of right-to-left midline shift. There are no signs of extracranial trauma, and a CT and a focused assessment with sonography in trauma (FAST) ultrasound study show no sign of intra-abdominal organ injury. Neurosurgery is called to place an ICP monitor. Prior to moving the patient to the ICU, which of the following interventions is most appropriate?
A. Elevate head of bed to 30°
B. Give 1.0 g/kg of mannitol as a controlled infusion over 4 hours
C. Give 30 mL of 23% hypertonic saline as a drip over the next 6 hours
D. Hyperventilate to an end-tidal CO2 of 25 to 30 mm Hg
E. Start 0.45% saline at 100 mL/h
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7. Answer A. (MN-111) The head of bed should be elevated to 30° to improve venous drainage and reduce ICP. The other choices are all ICP reduction tools; however, they are being used incorrectly. Hypertonic saline, 23%, should be administered as a rapid infusion over 2 to 5 minutes. Mannitol should also be given as a rapid infusion (wide open) or at a pump rate of 999 mL/h. Only isotonic crystalloids should be used; hypotonic fluids such as half-normal saline should be avoided since free water will flow down its osmotic gradient into the injured brain. Excessive hyperventilation in the acute phase of TBI should be avoided; the preferred target is mild hypocapnia at 30 to 35 mm Hg.
8. A patient with severe TBI presented above has repeated elevations of ICP exceeding 30 mm Hg despite propofol sedation and paralysis, controlled hyperventilation, CPP optimization between 60 and 70 mm Hg, and multiple doses of 20% mannitol and 23.4% hypertonic saline. The family wants everything done. What is the most appropriate action at this point to give him the best chance of survival?
A. Begin high-dose methylprednisolone infusion (2-g load, then 400 mg/h for 48 hours)
B. Decompressive hemicraniectomy
C. Pentobarbital infusion titrated to ICP control and EEG burst suppression
D. Placement of a ventricular drain
E. Therapeutic temperature modulation to 33 °C
View Answer
8. Answer B. (MN-111) In the RESCUE ICP trial craniectomy resulted in a mortality reduction from 47% to 24% compared with continued medical therapy including escalation to pentobarbital coma. In the Eurotherm3235 trial hypothermia to 32 °C resulted in increased mortality, as did high-dose methylprednisolone infusion in the CRASH trial. Slit ventricles makes ventricular drain placement difficult, and at this late stage CSF drainage is not as likely as craniectomy to reduce mortality.
9. Which of the following assessments is most crucial to determine if CSF diversion with a ventriculoperitoneal shunt is indicated in a patient with idiopathic intracranial hypertension (IIH)?
A. A neuropsychometric testing battery
B. An endocrinologic evaluation including pituitary hormone levels
C. Automated perimetry visual field testing
D. Marker-based motion capture assessment of gait and balance
E. Phase-contrast MRI to assess CSF flow dynamics
View Answer

9. Answer C. (MN-111) Determining whether vision is threatened is the primary criterion for identifying candidates for CSF diversion in patients with IIH. IIH by definition does not cause mental status or gait abnormalities; if either are present, another diagnosis should be sought. Phase-contrast MRI shows reduced mean peak CSF flow rates compared with normal controls, but this test is not used to identify candidates for shunting, nor is endocrine evaluation.
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