Conflict of Interest: None
Orthostatic Headache as the Presenting Symptom of Cervical Spine Metastasis
Article first published online: 20 DEC 2007
Headache: The Journal of Head and Face Pain
Volume 48, Issue 1, pages 161–163, January 2008
How to Cite
Kim, J. H., Choi, J.-Y., Kim, H.-J. and Oh, K. (2008), Orthostatic Headache as the Presenting Symptom of Cervical Spine Metastasis. Headache: The Journal of Head and Face Pain, 48: 161–163. doi: 10.1111/j.1526-4610.2007.00982.x
- Issue published online: 20 DEC 2007
- Article first published online: 20 DEC 2007
- Accepted for publication September 10, 2007.
- orthostatic headache;
- cervical spine metastasis;
- spinal root compression
Orthostatic headache is a key symptom of intracranial hypotension; however, not all orthostatic headaches are caused by cerebrospinal fluid leaks leading to intracranial hypotension. We report here the unusual case of a 68-year-old man presenting with orthostatic headache in which compression of the C3 spinal nerve root by metastatic tumor invasion may contribute to the development of his orthostatic headache.
An otherwise healthy, 68-year-old male ex-smoker with no history of significant headache presented with severe headache. One month before admission, he began to experience mild, nonprogressive pain and stiffness in the posterior neck. Three days before admission, he developed the abrupt onset of severe posterior headache. The headache consistently occurred within 1 minute after assuming an upright or inclined position, and was completely abolished within 5 minutes of a recumbent position. The pain was stabbing and throbbing, accompanied by lightheadedness, nausea, and moderately severe posterior neck pain. He noticed that there was no photophobia, diplopia, blurred vision, hearing disturbance, or radicular symptoms such as pain or paresthesia of the upper extremity. The headache not only was primarily confined to the left occipital and temporal regions, but also included the left frontal region and the vertex of his head after several minutes of standing, at which time the pain was excruciating. He denied history of back pain, spine surgery, or recent spinal trauma including chiropractic manipulation. On admission, vital signs were normal and neurological examination showed no deficits. No papilledema was found on funduscopic examination. A brain magnetic resonance imaging (MRI) revealed a small enhancing lesion with perifocal edema in the right cingulate gyrus (Fig. B,C), but no evidence of diffuse pachymeningeal enhancement or subdural fluid collection.The cerebrospinal fluid (CSF) examination showed an opening pressure of 16-cm H2O, no white cells, normal glucose and protein concentrations, and negative cytology. His headache continued despite intake of several analgesics, and a repeat CSF analysis 4 days later showed an opening pressure of 15-cm H2O, no pleocytosis, and negative cytology again. A diagnosis of metastatic brain tumor was suspected and a systemic evaluation confirmed nonsmall cell lung carcinoma in the left lower lobe with multiple metastases to the brain parenchyma and cervical (C3) and lumbar (L2/L4) vertebral bodies (Fig. A-H). A cervical MRI demonstrated an enhancing extradural mass involving the entire C3 vertebral body with pathologic fracture, epidural extension to the surrounding tissue, and neural foramenal invasion with encasement of the left C3 spinal nerve root (Fig. E-H). Neither epidural venous engorgement nor extradural fluid collection could be found on cervical and lumbar MRI. He was given emergent percutaneous vertebroplasty by using a bone cement mixture and placed in a hard cervical collar. Subsequent external beam radiation therapy targeting the cervical lesion (30 Gy in 10 fractions) has led to a dramatic improvement in his orthostatic headache. He died 8 weeks later owing to serious pneumonia and septicemia.
Orthostatic headache is a key feature of intracranial hypotension or CSF leaks leading to CSF volume depletion.1,2 Orthostatic headache is thought to be a consequence of descent of the brain due to loss of CSF buoyancy, causing traction on pain-sensitive structures, particularly the intracranial dura.1,2 Compensatory dilation of the cerebral veins and venous sinuses may also play a role in the production of the headache.1,2 However, not all orthostatic headaches are caused by spontaneous spinal CSF leaks, but other conditions have also been suggested as a rare but possible cause of orthostatic headaches, including type I Chiari malformation,3 cerebellar hemorrhage,4 skull base tumor,5 postural tachycardia syndrome,6 and filum terminale ependymoma.7
We assume that the orthostatic headache in our patient is not caused by intracranial hypotension secondary to a CSF leak but is related to compression of the cervical spinal nerve root. Compression of the upper cervical spinal nerve roots, attributable to either protruded disk or vascular compression, can lead to hemicrania or cervicogenic headache.8-10 In our patient, encasement of the left C3 spinal root by tumor invasion may have contributed to the development of the posterior headache and neck pain, which is accentuated by the upright posture, resulting in orthostatic headache. Several observations could support our assumption. First, the brain MRI of our patient did not reveal any characteristic features of intracranial hypotension, such as pachymeningeal enhancement, subdural fluid collection, or downward displacement of the brain. Second, although a CSF pressure that is within normal limits might not necessarily exclude the possibility of intracranial hypotension or reduced orthostatic CSF pressure,11 the great majority of the patients have a low (less than 6-cm H2O) or unmeasurable opening CSF pressure.1,2 Our findings of consistently normal CSF pressure and lack of aggravation in his orthostatic headache after the lumbar puncture could make the possibility of intracranial hypotension unlikely. Third, although we did not perform computerized tomography myelography or radionuclide cisternography to search for the site of a dural tear and a CSF leak, the cervical and lumbar MRI revealed no evidence of extradural fluid collection or engorgement of the cervical epidural veins, a characteristic spinal MRI finding of a CSF leak.12 Fourth, the headaches in intracranial hypotension may vary in their locations; however, the headache is typically holocephalic and bilateral and is rarely unilateral.1,2,13 The headache in our patient was primarily confined to the left temporal and occipital regions, which corresponds to the left C3 spinal nerve dermatome,10 suggesting involvement of left C3 spinal root in the development of his headache. Finally, our assumption could be strongly based on the finding of a dramatic improvement in our patient's orthostatic headache following percutaneous vertebroplasty and repeated radiation therapies. Both treatments are known to be effective in spinal stabilization, tumor control, and subsequent pain reduction in cases with spinal metastasis.14,15
Our case illustrates that upper cervical spine metastasis with the root compression should be suspected in patients with orthostatic headache when no evidence of intracranial hypotension is found, especially in the elderly with an increasing risk of malignancy, and particularly when the headache is strictly unilateral with posterior or nuchal distribution.
- 10Mechanical neck pain and cervicogenic headache. Neurosurgery. 2007;60(Suppl. 1):S21-S27., , , .