A 19-year-old woman presents to her primary care physician with headaches, blurry vision, and tinnitus over the past month. She started treatment for a skin condition with a new oral medication 2 months prior. She has not noticed any changes in her complexion as of yet. Her temperature is 98.2°F (36.8°C), blood pressure 100/65 mmHg, pulse 87/min, and respiratory rate 14/min. She is oriented to person, place, and time. Neurological exam reveals papilledema upon examination of both eyes. Skin examination is notable for the findings in Figure A. Which of the following is the most likely diagnosis?
Idiopathic intracranial hypertension
Meniere disease
Giant cell arteritis
Cerebrovascular accident
Idiopathic intracranial hypertension
This young patient with severe nodulocytic acne who was started on a new oral medication (likely isotretinoin) is likely suffering from idiopathic intracranial hypertension, which can present with papilledema, headache, blurry vision, and diplopia.

Idiopathic intracranial hypertension is characterized by an increase in intracranial pressure without an identifiable cause. Patients often present with symptoms that include headache, blurry vision, and tinnitus. Fundoscopic examination typically reveals bilateral papilledema. Vision loss is a complication of this condition with high morbidity. While there is no identifiable cause, common associations include obesity or a recent increase in weight as well as medications such as isotretinoin - a vitamin A derivative that is often used in the management of severe nodulocystic acne vulgaris. Management should first involve imaging of the brain (CT or MRI) to exclude elevated cerebrospinal fluid pressure due to other causes such as brain tumor, dural sinus thrombosis, or hydrocephalus. The diagnosis is made via lumbar puncture revealing increased opening pressure. Lumbar puncture can also result in therapeutic relief. The specimen should be sent for gram stain, culture, and viral PCR to rule out other etiologies. Long term treatment is centered on weight loss and avoiding causative agents. Acetazolamide and serial lumbar punctures are other treatments.

Boyter reviews the clinical presentation and management of patients with idiopathic intracranial hypertension. They found the condition is most common among obese women in their 20's, but it also may occur in children, men, and older adults. They recommend referral for patients to see a neurologist for ongoing management of headaches and other symptoms, as well as an ophthalmologist or neuro-ophthalmologist for monitoring papilledema, given the risk of vision loss.

Figure/Illustration A: The figure depicts a patient with severe nodules and cysts (red arrows) consistent with severe inflammatory nodulocystic acne that would be suitable for treatment with isotretinoin.

Incorrect Answers:
Answer A: Cerebrovascular accidents can be embolic or hemorrhagic in nature and often result in persistent neurologic deficits or resolution of an acute neurologic deficit in a patient with cerebrovascular risk factors (diabetes, hypertension, hyperlipidemia). This young, healthy patient with intermittent headache and neurologic symptoms without a family history of hypercoagulable state is unlikely to have suffered from a cerebrovascular accident.

Answer B: Giant cell arteritis (GCA) is a vasculitis that is typically seen in elderly patients (>60 years of age) and presents with tenderness of the ipsilateral temple and elevated inflammatory markers. Many patients with GCA have one or more constitutional symptoms (weight loss, fever, fatigue, anorexia, malaise) in addition to symptoms such as new onset headache or jaw claudication.

Answer D: Meniere disease affects the inner ear and is characterized by severe episodes of vertigo, tinnitus, hearing loss, and fullness in the ear. Patients are often women between the ages of 40 and 60. Headaches and blurry vision are not a classic part of the clinical presentation.

Answer E: Migraines present with severe headaches that can be incapacitating with associated photophobia and phonophobia. In some cases, patients may present with an aura that precedes or occurs during the migraine. The presence of papilledema suggests elevated intracranial pressure and is not typically seen in migraines.

Bullet Summary:
Idiopathic intracranial hypertension may be a side effect of isotretinoin.