The Worst Headache of My Life

History and Physical

A 17-year-old male with a history of obesity, Cushing syndrome, and arterial hypertension comes to the emergency room for severe headache starting 3 hours before sexual intercourse. Patient describes a history of occasional headaches, but never like this one, which qualifies as “the worst headache of his life.” Pain intensifies during intercourse and is located in the left parietal region, with a sensation of pressure and a severity of 10/10. Patient shows agitation, confusion, nausea, and vomiting. Family history: father with arterial hypertension, mother with obesity and diabetes, paternal grandfather with stroke. Vital signs: weight 110 kg, height 170 cm, head circumference 58 cm, blood pressure 190/110, pulse 120, axillary temperature 37°C. Neurological examination: nuchal rigidity, left horizontal nystagmus, alteration of right upper visual field. Fundoscopy shows papilledema and bilateral subhyaloid retinal hemorrhages.

Head CT is normal without acute hemorrhage, hydrocephalus, or mass. LP performed 7 hours after headache onset shows an opening pressure of 25 cm H ₂ O. CSF is xanthochromic with leukocytes 3 mm ³, red blood cells 4700 mm ³, protein 49 mg/dL, glucose 56 mg/dL. Gram-negative stains and cultures are negative at 72 hours.

Diagnostic Workup

MRI at 24 hours after headache onset reveals FLAIR-hyperintense signal in the occipital horn of left lateral ventricle and along the subarachnoid spaces of the interhemispheric fissure ( Fig. 5.1 ). There is no abnormal contrast enhancement. MR angiography and cerebral angiography are both normal.

An axial and coronal brain M R I with a bright signal near the occipital horn and a marked area in the interhemispheric region. Panel A shows an axial F L A I R M R I with an arrow pointing to a bright focal signal in the left occipital horn of the lateral ventricle. The ventricular system, cortical sulci, and symmetrical gyri across both hemispheres are clearly visible. Panel B displays a coronal F L A I R M R I with an arrowhead marking a linear bright area along the midline interhemispheric fissure. The lateral ventricles, corpus callosum, thalami, and overlying cortex are well defined. Brain structures are bilaterally symmetric with clear boundary between gray and white matter. — Fig. 5.1

Clinical Differential Diagnosis

Primary headache related to sexual activity: bilateral and progressive pain.

Migraine: unilateral, nausea/vomiting, photo/phonophobia.

Cluster headaches: periods of attacks, ipsilateral autonomic symptoms.

Autonomic headaches: unilateral neuralgiform headache, paroxysmal hemicrania, and reversible vasoconstriction syndrome.

Meningitis: fever, meningeal signs, stiff neck.

Hypertensive encephalopathy: persistent during hypertensive crises.

Stroke: acute neurological deficit with arterial territory infarct on neuroimaging.

Imaging Differential Diagnosis

Infectious meningitis: Contrast enhancement on MRI T1 and FLAIR sequences with gadolinium. Patterns can be regional or diffuse, with thick empyema/abscess formation in bacterial infections, thin smooth enhancement in viral or parasitic infections, and basilar nodular enhancement for fungal/tuberculous infections ( Fig. 5.2 ).

An axial brain M R I pair without and with contrast shows changes along both hemispheres. Panel A shows an axial T1 M R I without contrast. The frontal and parietal lobes appear symmetric with visible cortical folds, lateral ventricles, and interhemispheric fissure. Brain structures are evenly defined, and sulci are uniformly spaced across both hemispheres. Panel B shows the corresponding axial T1 M R I with contrast. Bright, irregular signal outlines are visible over the surface of both hemispheres, especially along the sulci. The cortex, midline structures, and convexities remain symmetric, and the enhancement follows the contours of the cortical surface. — Fig. 5.2

Leptomeningeal carcinomatosis: Irregular enhancement on MRI T1 and FLAIR sequences with gadolinium. In benign or low-grade tumors, leptomeningeal dissemination may be absent or nonenhancing. Evaluate the posterior fossa, basal cisterns, and CNs for subtle leptomeningeal disease ( Fig. 5.3 ).

An axial brain M R I set with arrows and arrowheads marking areas near the left cortex and midline. Panel A shows an axial T1 M R I without contrast. The lateral ventricles, corpus callosum, sulci, and gyri are visible with symmetric brain anatomy. Panel B displays an axial T1 M R I with contrast. Arrows highlight enhanced regions along the left parietal surface, and arrowheads mark smaller areas along the left frontal cortex and midline posteriorly. The enhancement follows the contours of the cortical surface. Panel C presents an A D C map with symmetric ventricular size and shape. White matter and cortical structures are clearly outlined. Panel D shows a D W I scan with increased signal near the left hemisphere compared to the right, and the sulcal outlines remain well defined. — Fig. 5.3

Pseudosubarachnoid hemorrhage: CT imaging appearance created by hypodense cerebral edema and/or ischemia with crowding of subarachnoid spaces. Vascular congestion due to compression and stasis may also contribute to extraaxial hyperdensity ( Fig. 5.4 ).