Marfan syndrome [Neurosurgery Wiki]

This page is read only. You can view the source, but not change it. Ask your administrator if you think this is wrong.
====== Marfan syndrome ======
Marfan [[syndrome]] can demonstrate tortuous and elongated [[intracranial]] [[artery]]. However, these arteries rarely cause neurovascular compression resulting in [[hemifacial spasm]] or [[trigeminal neuralgia]]
((Sakakura K, Akutsu H, Yamamoto T, Masuda Y, Ishikawa E, Matsumura A.
Trigeminal Neuralgia in a Patient with Marfan Syndrome: Case Report. Neurol Med
Chir (Tokyo). 2013 Dec 27. [Epub ahead of print] PubMed PMID: 24390183.)).

===== Complications =====

The underlying cause of [[spontaneous intracranial hypotension]] is usually a spontaneous [[cerebrospinal fluid fistula]], however, some cases may be due to low [[cerebrospinal fluid volume]]. Evidence supports an underlying weakness of the meninges as a contributing factor; for instance, connective tissue disorders like [[Marfan syndrome]], and [[Ehlers-Danlos syndrome]].

===== Case reports =====
Marques et al. report a five-generation kindred with two brothers with pituitary [[gigantism]] due to [[AIP]] [[mutation]]-positive [[GH secreting pituitary neuroendocrine tumor]]s and their first-cousin coincidently also having gigantism due to [[Marfan syndrome]] 
((Marques P, Collier D, Barkan A, Korbonits M. Coexisting pituitary and
non-pituitary gigantism in the same family. Clin Endocrinol (Oxf). 2018 Sep 17.
doi: 10.1111/cen.13852. [Epub ahead of print] PubMed PMID: 30223298.
)).
----
A 33-year-old woman who was diagnosed as Marfan syndrome, suffered from trigeminal neuralgia. Magnetic resonance (MR) angiography showed tortuous and elongated left vertebral artery (VA). The coronal section of three dimensional (3D) MR cisternography with contrast enhancement showed that the left trigeminal nerve was compressed from underneath by the tortuous and elongated left VA. After successful surgery of microvascular decompression, the patient's symptom resolved and no recurrence was encountered. 

Neurosurgeons should not only be aware of hemifacial spasm but also of trigeminal neuralgia caused by elongated vessels in a patient with Marfan syndrome, although it is an extremely rare condition. In addition, offending vessel is not atherosclerotic in younger patients unlike usual cases of trigeminal neuralgia. Thus, microvascular decompression can be easier than usual cases 
((Sakakura K, Akutsu H, Yamamoto T, Masuda Y, Ishikawa E, Matsumura A.
Trigeminal Neuralgia in a Patient with Marfan Syndrome: Case Report. Neurol Med
Chir (Tokyo). 2013 Dec 27. [Epub ahead of print] PubMed PMID: 24390183.)).