The Brain Also Faints

History and Physical

A 16-year-old right-handed girl presents with recurrent headaches intensifying over the past month. Since 13 years of age, she has experienced monthly headaches of variable duration (2–8 hours). She describes these as bifrontal with some very intense pulsatile episodes, up to 8/10 severity but mostly mild (3–4/10) and not interfering with her activities of daily living. Analgesics usually address the pain. She reports associated photophobia but never phonophobia. She also denies nausea, vomiting, abdominal pain, and changes with posture or Valsalva maneuver.

During the last month, her headaches have become frequent and almost daily, frontal and occipital in location, of moderate intensity or sometimes greater. This is accompanied by nausea but not vomiting and shows a decreased response to analgesics. She denies any visual changes, difficulty eating, or other symptoms. Her headache becomes more intense after exercising in dance class with intense cephalic rotation: “my long hair looks like the blades of a windmill.” She also reports tinnitus and cervical pain, with relief when lying down and worsening when standing up. She is currently in her second year of high school with excellent school performance and has attended dancing school since she was 12 years old. She has no history of fever, trauma, or toxic exposures. No symptoms of depression or anxiety. No relevant personal or family history.

Clinical examination shows normal vital signs and body measurements. Patient has a marfanoid appearance, with mild discomfort in the neck and trapezius muscles on palpation, as well as joint hyperlaxity. Neurological examination shows moderate neck stiffness and mild incoordination in heel-toe walking. Headache clearly improves when placed in the Trendelenburg position.

Diagnostic Workup

Brain MRI images with contrast showed bilateral subdural hygromas and smooth linear dural enhancement, engorged pituitary gland, decreased mammillary-pontine distance, low-lying posterior fossa structures with flattening of the basis pontis against the clivus and low-lying cerebellar tonsils ( Fig. 4.1 ).

An M R I set of the brain in sagittal and axial views with arrowheads and arrows marking the brain surface and posterior regions. The set of three M R I labeled A to C. Panel A shows a sagittal T 1 M R I of the brain with arrowheads near the lower cerebellum and brainstem. The cerebrum, corpus callosum, midbrain, pons, medulla, pituitary gland, and cervical spine are visible. The cerebellar tonsils extend downward toward the foramen magnum. Panel B presents an axial T 2 M R I with arrowheads pointing to thin fluid layers along the outer edges of the brain. The cerebral hemispheres, ventricles, and midline structures are visible in a horizontal slice. Panel C displays an axial T 1 M R I with gadolinium. Arrows mark smooth lines of contrast along the inner surface of the skull. — Fig. 4.1

Next, a spinal MRI was performed in search of a fistula. The lumbar spine showed a left perineural diverticulum with cerebrospinal fluid (CSF) fistula toward the paraspinal veins and paravertebral plexuses ( Fig. 4.2 ). Based on the imaging findings, no lumbar puncture was performed, since this could worsen the symptoms by further reducing the CSF volume.

An axial lumbar spine M R I series with markers near the left side of the spinal canal. The set of three M R I labeled A to C. Panel A shows an axial T 2 M R I with fat suppression at the lumbar level. An arrowhead points to a small, rounded structure on the left side near the edge of the spinal canal. The vertebral body, posterior elements, and soft tissue outlines are visible. Panel B presents another axial section with an arrow marking a bright linear area extending from the spinal canal toward the left paraspinal region. The vertebra, dural sac, and surrounding musculature are outlined. Panel C displays a lower slice with an arrow pointing to a bright elongated structure lateral to the vertebra on the left. — Fig. 4.2

Clinical Differential Diagnoses

Primary headache, including tension headache and migraine.

Postural orthostatic tachycardia syndrome is an intolerance to orthostasis with a measured increase in heart rate by at least 30 beats per minute (bpm) compared to resting rate, or a rate greater than 120 bpm with orthostasis. Headache may be associated with other symptoms: dizziness, blurred vision, presyncope-syncope chest pain or discomfort, anxiety, flushing, fatigue, dyspnea, heat intolerance, feeling cold, excessive sweating, nausea, instability, noise sensitivity, light sensitivity, and exercise intolerance.