M. Neil Woodall

Cerebral vascular malformations in hereditary hemorrhagic telangiectasia: Clinical article

OBJECT Hereditary hemorrhagic telangiectasia (HHT) is a hereditary disorder characterized by mucocutaneous telangiectasias, frequent nosebleeds, and visceral arteriovenous malformations (AVMs). Few reports have outlined the prevalence of the various cerebral vascular malformations found in patients with HHT. The authors set out to define the prevalence of cerebral vascular malformations in a population of HHT patients who underwent imaging with 3-T imaging (MRI/MR angiography [MRA]) of the brain. METHODS A retrospective review of prospectively collected data was carried out using a database of 372 HHT patients who were seen and examined at the Georgia Regents University HHT Center and screened with 3-T MRI/MRA. Data were tabulated for numbers and types of vascular malformations in this population. RESULTS Arteriovenous malformations were identified in 7.7%, developmental venous anomalies in 4.3%, and cerebral aneurysms in 2.4% of HHT patients. The HHT AVMs tended to be supratentorial, small, and cortical in this series, findings consistent with other recent studies in the literature. An arteriovenous fistula, cavernous malformation, and capillary telangiectasia were identified in 0.5%, 1%, and 1.9% of HHT patients, respectively. CONCLUSIONS Few studies have investigated the prevalence of the various vascular malformations found in HHT patients screened with 3-T MRI/MRA of the brain. Hereditary hemorrhagic telangiectasia AVMs are more likely to be multiple and have a tendency toward small size and cortical location. As such, they are often treated using a single-modality therapy.

Teaching neuroimages: microvascular decompression of the optic nerve.

Vascular compression is a known cause of cranial nerve dysfunction in a variety of disease states including trigeminal neuralgia, hemifacial spasm, and hypoglossal neuralgia.1,2

Bilateral transverse sinus stenosis causing intracranial hypertension

Idiopathic intracranial hypertension (IIH) has been associated with transverse sinus stenosis in a number of cases. Some authors advocate for stent placement when transverse sinus stenosis is responsible for pseudotumor symptoms. A 19-year-old man with IIH, bilateral transverse sinus stenosis was apparent on a CT venography, with reconstitution of the lateral sinus. MRI was then performed, and transverse sinus stenosis was confirmed. An impressive network of dilated collateral veins became apparent on VENBOLD sequences. Treatment options in this case include cerebrospinal fluid (CSF) shunting, optic nerve fenestrations and transverse sinus stenting. A ventriculoperitoneal shunt was placed in this patient, with prompt resolution of pseudotumor symptoms.

Arteriovenous fistula mimicking vestibular schwannoma

A 59-year-old woman presented with progressive left-sided sensorineural hearing loss. Workup revealed an enhancing lesion of the internal auditory canal (IAC) consistent with vestibular schwannoma (figure 1).

Teaching NeuroImages: Microvascular decompression of the optic nerveAuthor Response

We were interested to read the Neuro Image by Woodall et al.1 In addition to ectatic internal cerebral artery abutting the optic nerve, we also noted the tortuous course of intraorbital optic nerves with dilated perineural subarachnoid space, features consistent with idiopathic intracranial hypertension (IIH). IIH also results in vision diminution and even though the cause-and-effect relationship of vascular loop compression is unclear, it is important to rule out other causes and provide conservative management before resorting to surgery. Compression over …