Management of trigeminal neuralgia in sclerosteosis

Abstract

Background:Sclerosteosis is a rare bone disorder characterized by a progressive craniotubular hyperostosis. The diagnosis of sclerosteosis is based on characteristic clinical and radiographic features and a family history consistent with autosomal recessive inheritance. The skull overgrowth may lead to lethal elevation of intracranial pressure, distortion of the face, and entrapment of cranial nerves, resulting in recurrent facial palsy or secondary trigeminal neuralgia.Cases Description:The authors reported cases of two siblings who were diagnosed with familial sclerosteosis and presented with secondary trigeminal neuralgia. The patients were 28 and 40-year-old and presented with pain in the right V2-V3 and V3 distributions, respectively. The facial pain was resistant to medications and was treated with percutaneous techniques. The foramen ovale puncture was complicated initially and the difficulty increased over the years due to stenosis of the foramen.Conclusion:The treatment of the trigeminal neuralgia secondary to hyperostosis and resistant to medications presents a dilemma. The narrowing of the foramen oval and difficulty in the identifying and approaching of the foramen makes the percutaneous technique a challenge for the neurosurgeon in patients harboring sclerosteosis. Microvascular decompression should not be considered since the primary cause of the trigeminal neuralgia is the nerve entrapment by the narrowing of neurovascular foramina and not the neurovascular conflict related to essential trigeminal neuralgia. Stereotactic radiosurgery may be a good treatment option, but there is a lack of published data supporting the use of this method in cranial hyperostosis.

Sclerosteosis: A Rare, Sclerosing Bone Dysplasia in a Bangladeshi Male

“…Trigeminal neuralgia is relatively a rare presentation of sclerosteosis that was present in this case. It is usually refractory to medical treatment [11], but in our case, the pain responded well to corticosteroid and pregabalin. Another fatal complication is sudden death, which is caused by the impaction of the medulla oblongata in the foramen magnum due to the progressive rise of intracranial pressure (ICP) by bony overgrowth [3].…”

Inhibiting Wnt Secretion Reduces High Bone Mass Caused by Sost Deficiency or Point Mutations in Lrp5

“…Patients with Sclerosteosis, van Buchem Disease, or HBM associated with LRP5 mutations often suffer from neuropathies and other sequelae due to nerve impingements caused by increased bone mass [11][12][13][14][15]. We show here that when treated with Wnt secretion inhibitors, genetic mouse models that replicate human HBM have bone mass reduced to more normal levels, therefore establishing this approach as a potential strategy for treating HBM patients to reduce neuropathy.…”

“…Sclerosteosis patients have even more severe symptoms that can reduce lifespan. These include cranial vascular and neural foraminal narrowing and reduced intracranial volume, frequent seventh nerve palsy, progressive optic and cranial neuropathies, mixed hearing loss, brainstem compression, intracranial hypertension with increased elastance, and sudden, premature death [12][13][14][15]. Thus, it is critical to identify approaches that can reduce the pain and morbidity seen in these patients to improve their quality of life.…”

Radiosurgery for Functional Neurosurgery

“…In patients with megadolichobasilar artery, the results have been shown to be better, with a 93.1% rate of remaining pain-free without medication after 6 months to a rate of 75.7% chance after 2 years, when the results remained stable for up to 13 years after treatment 57 . In sclerosteosis, Andrade et al 6 suggested radiosurgery as a treatment option despite the lack of published data.…”