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Postural headache in a patient with Marfan's syndrome
E Ferrante1, A Citterio, A Savino
1Departments of Neurosciences and Internal Medicine, Niguarda Cà Granda Hospital, Milan, Italy. enricoferrante@libero.it
Cephalalgia : an International Journal of Headache
|September 3, 2003
Summary
Patients with Marfan's syndrome and spinal meningeal diverticula may face increased risk of cerebrospinal fluid (CSF) leaks. This condition, causing intracranial hypotension, can resolve with conservative treatment.
Area of Science:
- Neurology
- Radiology
- Genetics
Background:
- Marfan's syndrome is a genetic disorder affecting connective tissue, potentially leading to various complications.
- Spinal meningeal diverticula are abnormal pouches in the dura mater, often associated with connective tissue disorders.
- Spontaneous intracranial hypotension (SIH) is characterized by low cerebrospinal fluid pressure, often presenting with postural headaches.
Observation:
- A 26-year-old male with Marfan's syndrome presented with postural headaches and diffuse pachymeningeal enhancement on MRI.
- MRI myelography identified multiple large meningeal diverticula at the sacral nerve root level.
- The patient's symptoms improved with bed rest and hydration, with complete resolution of pachymeningeal enhancement on follow-up MRI.
Findings:
- The patient was diagnosed with SIH, likely caused by a cerebrospinal fluid (CSF) leak from the meningeal diverticula.
- Conservative management (bed rest, hydration) led to symptom resolution and radiographic improvement.
- No neurological abnormalities were noted at the 1-year follow-up.
Implications:
- Spinal meningeal diverticula, particularly in patients with Marfan's syndrome, represent a potential source for CSF leaks.
- SIH secondary to meningeal diverticula may be triggered by Valsalva maneuvers or minor trauma.
- Early recognition and conservative management can lead to favorable outcomes in SIH associated with meningeal diverticula.
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