Reversible Frontotemporal Dementia in Spinal Cerebrospinal Fluid–Venous Fistula

Figure

Sagittal T1-weighted MRI head scans following i.v. administration of contrast medium.

A 60-year-old woman with a 1-year history of headaches prompted concern after 2 months of cognitive deterioration involving reduced drive, forgetfulness, and inability to perform household tasks. The neurological examination was normal. Magnetic resonance imaging (MRI) revealed sagging of the brain stem and cerebellum (Figure 1a), while dynamic computed tomography myelography in the lateral position showed what is referred to as a cerebrospinal fluid–venous fistula at the left T8 nerve root. The veins in the left foramen at T8/9 were retrogradely occluded via the femoral vein with a liquid artificial embolus. Postoperatively, the patient quickly recovered (Figure 1b) and had regained full functioning at 18 months following treatment. In addition to spinal dural tears, a cerebrospinal fluid–venous fistula is the cause of spontaneous intracranial hypotension in around 25% of cases. The main symptom of orthostatic headache is absent in patients with so-called frontotemporal brain sagging syndrome, among others. Every 3^rd patient in this group is found to have a cerebrospinal fluid–venous fistula, a treatable cause of dementia—one reason why patients with dementia should undergo MRI and, if there is evidence of brain sagging, special neuroradiological investigations.

Dr. med. Niklas Lützen, Prof. Dr. med. Horst Urbach, Klinik für Neuroradiologie, Universitätsklinik Freiburg, [e-mail]

Prof. Dr. med. Jürgen Beck, Klinik für Neurochirurgie, Universitätsklinik Freiburg

Conflict of interest statement: The authors state that no conflict of interest exists.

Translated from the original German by Christine Rye.

Cite this as: Lützen N, Beck J, Urbach H: Reversible frontotemporal dementia in spinal cerebrospinal fluid-venous fistula.
Dtsch Arztebl Int 2024; 121: 369. DOI: 10.3238/arztebl.m2023.0166