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Digoxin as a Treatment for Patients With Idiopathic Intracranial Hypertension
Jen Haagensen, MS; Carl Hoegerl, DO, MSc
The Journal of the American Osteopathic Association, March 2014
Abstract
Idiopathic intracranial hypertension (IIH)—sometimes called pseudotumor cerebri—is a neurologic condition distinguished by any of the following symptoms:
headache,
increased cerebrospinal fluid pressure,
papilledema,
vision loss,
diplopia,
tinnitus,
deafness,
nausea and vomiting,
or sixth nerve palsy.
Medical and surgical management options are available for patients with IIH. The authors report a case of IIH that was successfully resolved with digoxin after standard avenues of therapy were exhausted.
Risk factors include
female sex,
weight gain,
obesity,
certain medications (eg, growth hormone, minocycline, doxycycline1),
problems with metabolizing vitamin A supplements,
and rheumatologic disorders (eg, lupus).
The condition is difficult to diagnose because it may involve specialist consultations (eg, neurology, ophthalmology) or procedures (eg, lumbar puncture).
Neuroimaging studies do not show evidence of hydrocephalus, masses, or vascular or structural abnormalities. Cerebrospinal fluid composition is usually normal.2 At a rate of 7.9 per 100,000, obese females of childbearing age are disproportionately affected by IIH.3
The pathophysiologic process of IIH is uncertain.
One theory posits that there is resistance of CSF outflow at the level of the arachnoid granulations.
Other theories attribute the condition to abnormalities of the cerebral venous outflow
or a change in sodium and water retention mechanisms.4
Another cause may be problems with vitamin A metabolism5
or obesity-related increased abdominal pressure and intracranial venous pressure.5,6
Management of IIH involves both medical and surgical modalities.
If a patient is obese, weight loss is encouraged, and acetazolamide (Diamox) and topiramate4 are frequently prescribed, as is furosemide.7 A physician may also perform serial lumbar punctures to decrease CSF pressure.
Patients whose conditions cannot be managed with these methods, or whose vision is failing, have surgical options, including CSF shunting and optic nerve sheath fenestration (ONSF).8 However, these treatment options have some risks. Shunting can be highly effective, but the shunts often need to be revised or replaced, especially for obese patients.9 Whereas many patients experience relief from headaches with shunts, Rosenberg et al10 found that only 14 of 37 patients remained “cured” after a single surgical procedure. Physicians may use ONSF to preserve or stabilize a patient's vision, but the procedure often does not alleviate headache symptoms.11
We report a case of IIH that demonstrates the effects of digoxin, a medication that may benefit patients for whom other treatments have failed or who are unable or unwilling to undergo surgical procedures.
Few cases of IIH managed with digoxin have been reported, to our knowledge.
Digoxin acts on the ouabain-sensitive Na+/K+ ATPase receptors in the choroid plexus, thereby decreasing CSF production.
Neblett et al12 discussed 3 cases in which the use of digoxin correlated with decreasing CSF production by up to 78%. They concluded that digoxin may be beneficial to conditions of increased CSF pressure. Schott and Holt,13 however, used digoxin in a patient with IIH without success.
This instance may illustrate that there is more than 1 mechanism for the increased intracranial pressure and perhaps the mechanism in our patient is different from the mechanism in the patient noted by Schott and Holt.13 Borsody et al14 noted that levels of brain ouabain-like compounds, which inhibit the Na+/K+ ATPase receptors, were lower in patients with unsuccessfully managed IIH. Digoxin is similar to ouabain-like compounds, which could explain our patient's improvement.
Full article in the attached file
Jen Haagensen, MS; Carl Hoegerl, DO, MSc
The Journal of the American Osteopathic Association, March 2014
Abstract
Idiopathic intracranial hypertension (IIH)—sometimes called pseudotumor cerebri—is a neurologic condition distinguished by any of the following symptoms:
headache,
increased cerebrospinal fluid pressure,
papilledema,
vision loss,
diplopia,
tinnitus,
deafness,
nausea and vomiting,
or sixth nerve palsy.
Medical and surgical management options are available for patients with IIH. The authors report a case of IIH that was successfully resolved with digoxin after standard avenues of therapy were exhausted.
Risk factors include
female sex,
weight gain,
obesity,
certain medications (eg, growth hormone, minocycline, doxycycline1),
problems with metabolizing vitamin A supplements,
and rheumatologic disorders (eg, lupus).
The condition is difficult to diagnose because it may involve specialist consultations (eg, neurology, ophthalmology) or procedures (eg, lumbar puncture).
Neuroimaging studies do not show evidence of hydrocephalus, masses, or vascular or structural abnormalities. Cerebrospinal fluid composition is usually normal.2 At a rate of 7.9 per 100,000, obese females of childbearing age are disproportionately affected by IIH.3
The pathophysiologic process of IIH is uncertain.
One theory posits that there is resistance of CSF outflow at the level of the arachnoid granulations.
Other theories attribute the condition to abnormalities of the cerebral venous outflow
or a change in sodium and water retention mechanisms.4
Another cause may be problems with vitamin A metabolism5
or obesity-related increased abdominal pressure and intracranial venous pressure.5,6
Management of IIH involves both medical and surgical modalities.
If a patient is obese, weight loss is encouraged, and acetazolamide (Diamox) and topiramate4 are frequently prescribed, as is furosemide.7 A physician may also perform serial lumbar punctures to decrease CSF pressure.
Patients whose conditions cannot be managed with these methods, or whose vision is failing, have surgical options, including CSF shunting and optic nerve sheath fenestration (ONSF).8 However, these treatment options have some risks. Shunting can be highly effective, but the shunts often need to be revised or replaced, especially for obese patients.9 Whereas many patients experience relief from headaches with shunts, Rosenberg et al10 found that only 14 of 37 patients remained “cured” after a single surgical procedure. Physicians may use ONSF to preserve or stabilize a patient's vision, but the procedure often does not alleviate headache symptoms.11
We report a case of IIH that demonstrates the effects of digoxin, a medication that may benefit patients for whom other treatments have failed or who are unable or unwilling to undergo surgical procedures.
Few cases of IIH managed with digoxin have been reported, to our knowledge.
Digoxin acts on the ouabain-sensitive Na+/K+ ATPase receptors in the choroid plexus, thereby decreasing CSF production.
Neblett et al12 discussed 3 cases in which the use of digoxin correlated with decreasing CSF production by up to 78%. They concluded that digoxin may be beneficial to conditions of increased CSF pressure. Schott and Holt,13 however, used digoxin in a patient with IIH without success.
This instance may illustrate that there is more than 1 mechanism for the increased intracranial pressure and perhaps the mechanism in our patient is different from the mechanism in the patient noted by Schott and Holt.13 Borsody et al14 noted that levels of brain ouabain-like compounds, which inhibit the Na+/K+ ATPase receptors, were lower in patients with unsuccessfully managed IIH. Digoxin is similar to ouabain-like compounds, which could explain our patient's improvement.
Full article in the attached file