Daily headaches and intermittent vision loss relieved only by sleep

Brenda, a 21-year-old college student, had presented to our office on multiple occasions for evaluation of recurrent sinusitis and otitis media. Her treatments had consisted of nasal steroids, decongestants, antibiotics (including doxycycline), and one course of oral prednisone.

Several weeks after her latest visit for sinusitis, Brenda returned with complaints of dull headaches that had been occurring on a daily basis for more than a week. She was concerned because she rarely had headaches. She usually experienced the pain upon awakening, although it always improved with sleep or simply lying down. There were no warning signs, associated nausea or vomiting, aura, or diplopia. She did, however, experience mild photophobia and intermittent visual loss, which would last for a few seconds and usually when nodding forward. Brenda described the pain as 7/10, with no fluctuation in intensity. Although the pain was minimally relieved by OTC nonsteroidal anti-inflammatory drugs (NSAIDs), the only true alleviating factor was sleep.

1. History

Brenda's medical history was significant for Henoch-Schönlein purpura at age 5, with associated polyarthralgias, edema of the hands and feet, and glomerulonephritis. At age 16, she experienced a relapse of the polyarthralgias, with edema of the extremities and hematuria but no rash. Once again, the symptoms resolved.

When Brenda was 19, she was diagnosed with Bell's palsy. The condition improved initially during a course of steroid medication given for sinusitis but reverted to its original status following completion of the therapy.

In addition to the NSAIDs, Brenda was currently receiving medroxyprogesterone injections (Depo-Provera) every three months. She reported no adverse effects, other than weight gain. At 5 ft 5 in tall, she weighed 195 lb. She also stated that she never exercised. She denied use of alcohol, tobacco, or illicit substances. There was no personal history of depression or anxiety and no family history of migraines, vascular headaches, or tumor.

2. Physical exam

On presentation, Brenda was afebrile. Examination was significant for persistent right facial synkinesis, ptosis, and inability to wrinkle her forehead, consistent with noncentral causes for facial paralysis. These conditions had been previously documented in her records. No cranial bruits were heard. Tympanic membranes were intact, without signs of otitis media.

An eye exam demonstrated normal uncorrected vision, extraocular eye movement, and field of gaze. Pupils were 3 mm bilaterally. Results of an Ishihara's test were normal. Brenda did, however, exhibit visual obscuration with head flexion. A dilated funduscopic exam revealed bilateral edematous optic disks with increased venous-to-arterial ratio.

Brenda's neck was supple and free of any appreciable lymphadenopathy. Thyroid was normal, with no nodularity, masses, or tenderness. Cardiac, pulmonary, and abdominal exams were unremarkable. On neurologic exam, cranial nerves 3, 7, and transiently cranial 2, were not intact. Brudzinski's and Kernig's signs were negative. Deep tendon reflexes were completely intact. The remainder of Brenda's exam was free of any other acute neurologic deficits.

We immediately referred Brenda to a neurologist, who ordered a complete blood count, anti-cardiolipin antibody titer, sedimentation rate, enzyme-linked immunosorbent assay for Lyme disease, MRI, and lumbar puncture. The MRI was negative for mass effect and ventricular dilation, and the lumbar puncture (LP) showed no signs of infection, with negative results on bacterial cultures, Lyme ELISA, viral cultures, and cytopathology. Lumbar pressure was 32 mm H2O.

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