A 36-year-old man with a history of infantile strabismus presented with left ptosis, diplopia and bilateral distal leg paresthesias. He reported subjective fever, cough and myalgias which had developed 4 days earlier and resolved before presentation. Exam was notable for left mydriasis, mild ptosis and limited depression and adduction, consistent with a partial left oculomotor palsy. Abduction was limited bilaterally

Stenting of VSS is safe and results in reduction of intracranial pressure in patients with IIH. This is associated with improvement in papilledema, RNFL thickness, VF parameters, and symptoms associated with intracranial hypertension.

No abstract

Degeneration of neuron and axons following injury to cells with which they synapse is termed trans-synaptic degeneration. This phenomenon may be seen in postsynaptic neurons (anterograde) or in presynaptic neurons (retrograde). Retrograde trans-synaptic degeneration (RTSD) of the retinal ganglion cells and retinal nerve fiber layer following injury to the occipital lobe has been well documented histologically in animal studies, but its occurrence in the human retina was, for many years, felt to be limited to cases of neonatal injury during a critical period of neuronal development. Over the last decade, imaging techniques such as MRI and optical coherence tomography have allowed us to visualize and quantify RTSD and analyze its time course and relationship to degree of vision loss and age of cortical injury. A deeper understanding of RTSD in the human visual system may allow us to interfere with its occurrence, potentially allowing for greater recovery following visual cortex injury.

We provide evidence that there is a significant decrease in CSF-OP in patients with idiopathic intracranial hypertension 3 months after VSS, independent of acetazolamide usage or weight loss.

Idiopathic intracranial hypertension (IIH), also known as pseudotumor cerebri, is a disease primarily affecting obese women of child-bearing age, typically presenting with positional headaches, diplopia from abducens nerve palsies, pulse-synchronous tinnitus, and visual disturbances related to papilledema, including transient visual obscurations, blurry vision, and visual field defects. Controversy remains regarding the pathophysiology of IIH. Elevated intracranial pressure in IIH is thought to be the result of a dysregulation of cerebrospinal fluid (CSF) dynamics, through excessive secretion, reduced drainage, or both. Passive CSF drainage through the arachnoid granulations depends on a pressure gradient between the subarachnoid space and the cerebral venous system, such that elevations in cerebral venous pressure lead to impaired CSF drainage. Dural venous sinus stenosis (DVSS) at the junction of the transverse and sigmoid sinuses, leading to local venous hypertension and a secondary reduction in CSF drainage through the arachnoid granulations, is increasingly recognized as an important component of the pathophysiology of IIH. A growing number of studies have demonstrated a role for stenting of DVSS as an alternative to CSF shunting and optic nerve sheath fenestration in medically refractory IIH patients with vision loss or debilitating symptoms.

ObjectiveEvaluate the role of venous sinus stenting in the treatment of pulsatile tinnitus among patients with Idiopathic Intracranial Hypertension (IIH) and significant venous sinus stenosis.Subjects and MethodsA written informed consent approved by the Weill Cornell institutional review board was signed and obtained from the study participants. Thirty-seven consecutive patients with IIH and venous sinus stenosis who were treated with venous sinus stenting between Jan.2012-Jan.2016 were prospectively evaluated. Patients without pulsatile tinnitus were excluded. Tinnitus severity was categorized based on “Tinnitus Handicap Inventory” (THI) at pre-stent, day-0, 1-month, 3-month, 6-month, 12-month, 18-month and 2-year follow-up. Demographics, body-mass index (BMI), pre and post VSS trans-stenotic pressure gradient were documented. Statistical analysis performed using Pearson’s correlation, Chi-square analysis and Fischer’s exact test.Results29 patients with a mean age of 29.5±8.5 years M:F = 1:28. Median (mean) THI pre and post stenting were: 4 (3.7) and 1 (1) respectively. Median time of tinnitus resolution post VSS was 0-days. There was significant improvement of THI (Δ Mean: 2.7 THI [95% CI: 2.3–3.1 THI], p<0.001) and transverse-distal sigmoid sinus gradient (Δ Mean: -15.3 mm Hg [95% CI: 12.7–18 mm Hg], p<0.001) post-stenting. Mean follow-up duration of 26.4±9.8 months (3–44 months). VSS was feasible in 100% patients with no procedural complications. Three-patients (10%) had recurrent sinus stenosis and tinnitus at mean follow-up of 12 months (6–30 months).ConclusionVenous sinus stenting is an effective treatment for pulsatile tinnitus in patients with IIH and venous sinus stenosis.

This data provides compelling new evidence of retrograde transsynaptic degeneration causing retinal ganglion cell loss after postgeniculate visual pathway injury.