Age / Sex : 9 / M

Chief complaint: : Headache, back pain (3days ago)

What is your impression?

Two weeks later, you can see the final diagnosis with a brief discussion of this case.

(Please submit only one answer)

Courtesy: Sun Joo Lee, Inje University Busan Paik Hospital

Spinal manifestation of spontaneous intracranial hypotension (or CSF hypovolemia)

Discussion

Findings:

MRI findings revealed widening of posterior epidural space with fluid collection and engorgement of epidural veins

Diagnosis: Spinal manifestation of spontaneous intracranial hypotension (or CSF hypovolemia)

Discussion:

•       Disease characterized by orthostatic headache and low CSF pressure.

•       The criteria of the International Classification of Headache Disorders (require at least 1)

–      low CSF pressure

–      diffuse pachymeningeal enhancement of brain MR imaging

–      evidence of CSF leakage on conventional myelography, CT myelography, or radionuclide cisternography.

•       But low CSF pressure is not always present in spontaneous intracranial hypotension

•       Localization of a CSF leak 

–      Invasive diagnostic techniques

-> Radioisotope cisternography or CT cisternography

–      Noninvasive method

-> MR Imaging, MR myelography

• Spinal MR imaging   

- Extradural fluid collections

        : posterior epidural space is maximum at the midlumbar level

- Spinal meningeal enhancement

- Dilation of the epidural venous plexus

        : decreased intrathecal CSF à collapse of the spinal subarachnoid space

         à results in the enlargement of the epidural space

         à enlargement of epidural veins & accumulation of the epidural CSF

•      MR myelography

       - CSF space expansion around nerve root sleeves

         : various degrees of diffuse, streaky, and irregular high signal intensity

           along the nerve root sleeves and extraspinal area.

•       Treatment

- Generally considered to be benign, and most patients improve spontaneously or

     with conservative management.

- Treatment options

          Analgesics, sedatives, antiemetics, intravenous hydration,

          epidural blood patch (EBP), epidural saline infusion, systemic steroids

- Treatment of choice for those patients who have failed the initial conservative

treatment: Autologous EBP

References:

1.  A. Watanabe, T. Horikoshi, M. Uchida, H. Koizumi, T. Yagishita, H. Kinouchi, Diagnostic Value of Spinal MR Imaging in Spontaneous Intracranial Hypotension Syndrome, AJNR Am J Neuroradiol 30:147–51.

2. H.-M. Yoo, S.J. Kim, C.G. Choi, D.H. Lee, J.H. Lee, D.C. Suh, J.W. Choi, K.S. Jeong, S.J. Chung, J.S. Kim, S.C. Yun, Detection of CSF Leak in Spinal CSF Leak Syndrome Using MR Myelography: Correlation with Radioisotope Cisternography, AJNR Am J Neuroradiol 29:649 –54

3. Wang YF, Lirng JF, Fuh JL, Hseu SS, Wang SJ. Heavily T2-weighted MR myelography vs CT myelography in spontaneous intracranial hypotension. Neurology 2009:73:1892-8.