Hydrocephalus (Water on the Brain) – Top USMLE Diseases

Neurosurgery is a specialized field focused on the surgical management of pathologies of the brain, spine, spinal cord, and peripheral nerves. General neurosurgery includes cases of trauma and emergencies. There are a number of specialized neurosurgical practices, including oncologic neurosurgery, spinal neurosurgery, and pediatric neurosurgery. Common neurosurgery cases treat tumors, masses, herniations, various types of hemorrhages, and radicular pain. Although neurosurgery is a surgical specialty, neurosurgeons must be very competent in neurology, critical care, trauma care, and radiology.

Idiopathic intracranial hypertension (IIH), also known as pseudotumor cerebri, is a clinical disorder that presents with symptoms due to increased intracranial pressure (ICP; ≥ 20 mm Hg) or CSF pressure (> 250 mm H2O), with no structural changes or other attributable causes. The condition is most commonly observed in obese women and after intake of certain drugs, such as growth hormones, tetracycline antibiotics, and high dosages of vitamin A. Classic manifestations include headache, vision loss or visual-field defects, and papilledema. Diagnosis is made by clinical exam, imaging, and lumbar puncture. Management is aimed at decreasing ICP and includes medication, therapeutic CSF removal, and shunting.

Normal intracranial pressure (ICP) is defined as < 15 mm Hg, whereas pathologically increased ICP is any pressure ≥ 20 mm Hg. Increased ICP may result from several etiologies, including trauma, intracranial hemorrhage, mass lesions, cerebral edema, increased CSF production, and decreased CSF absorption. Increased ICP can lead to brain herniation and death if not treated promptly. Clinical presentation includes headache, drowsiness or altered level of consciousness, and papilledema. Diagnosis is suspected based on the clinical presentation and confirmed with urgent brain imaging. Immediate management includes measures to decrease ICP, medications including diuretics, and surgery.

Osmotic diuretics increase tubular fluid osmolarity, pulling water into the collecting tubules and preventing water reabsorption, which results in osmotic diuresis. The primary osmotic diuretic used clinically is mannitol. The primary indication for mannitol is to treat cases of increased intracranial or intraocular pressure, which can have significant effects on fluid volume and sodium concentration, so caution must be exercised when using these agents.

The ventricular system is an extension of the subarachnoid space into the brain consisting of a series of interconnecting spaces and channels. Four chambers are filled with cerebrospinal fluid (CSF): the paired lateral ventricles, the unpaired 3rd ventricle, and the unpaired 4th ventricle. Connections between the structures occur via the interventricular foramen of Monro and the cerebral aqueduct (sylvian aqueduct). The foramen of Magendie and the foramen of Luschka, are additional channels present in the 4th ventricle.

Normal pressure hydrocephalus (NPH) is a neurodegenerative disorder characterized by the triad of gait abnormalities, dementia, and urinary urgency or incontinence. Normal pressure hydrocephalus can be either idiopathic or secondary to intraventricular or subarachnoid hemorrhage. Symptoms may be similar to those of Alzheimer and Parkinson's diseases. Diagnosis of NPH is clinical, in addition to lumbar puncture testing and neuroimaging. Management is with surgical shunt placement to drain excess CSF from the cerebral ventricles.

Hydrocephalus is a potentially life-threatening condition caused by the excess accumulation of cerebrospinal fluid (CSF) within the ventricular system. Hydrocephalus can be classified as communicating, which is caused by either impaired CSF absorption or excess CSF production; or non-communicating, which is caused by a structural blockage in CSF flow. The causes of hydrocephalus can be congenital or acquired. The clinical presentation is often nonspecific with symptoms including headache, behavioral changes, developmental delays, or nausea and vomiting. If the hydrocephalus is congenital or acquired at a young age, infants often present with macrocephaly. Diagnosis is confirmed with neuroimaging (ultrasound, computed tomography of the head, or magnetic resonance imaging) showing ventriculomegaly. Treatment is most commonly surgical with the placement of a CSF shunt.