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Froin syndrome is described in the medical literature as a combination of xanthochromia, increased protein content, and hypercoagulated liquor (CSF).
Froin syndrome is characterized by xanthochromia and CSF hypercoagulability. 1
The etiology of the disease is based either on meningeal irritation or a blockage of the spinal canal by a mass, which leads to a reduction in the flow of cerebrospinal fluid.
Papillary edema is a result of high levels of CSF protein, which disrupts the CSF resorption process that occurs at the level of arachnoid granulations.
The protein level in the CSF can rise to at least 5 g / L, compared to the normal values of 0.15 to 0.45 g / L. 2
Schully et al. suggested that the spinal subarachnoid space beneath a spinal block behaves like a dead end. 3
As a result, there is a reduced pressure in the dead end, since the connection with the spinal fluid above the compression has been interrupted.
In addition, the pial veins are widened at the compression site, which leads to transudation. The above-mentioned congestion and transudative nature of the fluid leads to hypercoagulability. 4
Working up a patient with symptoms consistent with Froin syndrome usually begins with imaging of the brain and spine
An MRI typically shows the spinal lesion causing the CSF obstruction or increased CSF protein.
A representative case presentation
A 35-year-old obese woman develops severe back pain and blurred vision. An ophthalmologist finds 20/30 eyesight in both eyes (OU) with a degree 3 Frisen papillary edema and severe visual field narrowing OU.
MRIs of the brain and orbit with MRI venography (MRV) were negative.
A lumbar puncture revealed no CSF and a “dry” MRI of the spine showed a thoracic ependymoma.
After neurosurgical resection, a spinal puncture showed an opening pressure of 30 cm of water and an increased CSF protein of 100 mg / dl, which corresponds to a Froin syndrome.
Doctors should be aware that disc edema with back pain suggests a spinal lesion.
A normal MRI of the brain with MRV creates a false sense of security for clinicians.
A dry tap (due to obstruction) or increased CSF protein are the main findings in spinal lesions that cause papilledema in Froin syndrome.
Treatment of the primary lesion in the spine may not result in complete resolution immediately due to persistent postoperative inflammatory pleocytosis of the CSF or increased CSF protein.
Junsang Cho, BA, University of Missouri Medical School, Columbia, Missouri; Anika Tanwani, BS, University of Texas Medical Department, Galveston, Texas; Sami Younes, BS, Medical School, Baylor College of Medicine, Houston, Texas; and Chao Charoenkijkajorn, MD, Department of Ophthalmology, Blanton Eye Institute, Houston Methodist Hospital, Texas, all contributed to this report.
Andrew G. Lee, MD
Lee, editor of The Neuro-Connection column, is affiliated with the Blanton Eye Institute, Houston Methodist Hospital. He has indicated no ownership interest.
- Govindarajan R, Khan T. Froin syndrome: a rare copycat of Guillain-Barre syndrome. Eur Spine J. 2012; 21 (8): 1674-1675. doi: 10.1007 / s00586-012-2277-0
- Greenfield JG. Original papers: ABOUT THE FROIN SYNDROME AND ITS RELATIONSHIP TO ALLIED CONDITIONS IN THE CEREBROSPINAL FLUID. J Neurol Psychopathol. 1921; 2 (6): 105-141. doi: 10.1136 / jnnp.s1-2.6.105
- Scully FJ. Yellow spinal fluid – its origin and meaning. Arch Neur Psych 1923 Jul; 10 (1): 83-99.
- Garispe A, Naji H, Dong F, Arabian S, Neeki M. Froin syndrome secondary to traumatic and infectious etiology. Cureus. 2019; 11 (12): e6313. doi: 10.7759 / cureus.6313
- Mirza S, Adams WM, Corkhill RA. Revisited Froin Syndrome 100 years later. Pseudo-Froin syndrome on MRI. Clin Radiol. 2008; 63 (5): 600-604. doi: 10.1016 / j.crad.2007.07.027