Laurence D. Watkins

Idiopathic intracranial hypertension: 120-day clinical, radiological, and manometric outcomes after stent insertion into the dural venous sinus

OBJECTIVE Idiopathic intracranial hypertension (IIH) is commonly associated with venous sinus stenosis. In recent years, transvenous dural venous sinus stent (DVSS) insertion has emerged as a potential therapy for resistant cases. However, there remains considerable uncertainty over the safety and efficacy of this procedure, in particular the incidence of intraprocedural and delayed complications and in the longevity of sinus patency, pressure gradient obliteration, and therapeutic clinical outcome. The aim of this study was to determine clinical, radiological, and manometric outcomes at 3-4 months after DVSS in this treated IIH cohort. METHODS Clinical, radiographic, and manometric data before and 3-4 months after DVSS were reviewed in this single-center case series. All venographic and manometric procedures were performed under local anesthesia with the patient supine. RESULTS Forty-one patients underwent DVSS venography/manometry within 120 days. Sinus pressure reduction of between 11 and 15 mm Hg was achieved 3-4 months after DVSS compared with pre-stent baseline, regardless of whether the procedure was primary or secondary (after shunt surgery). Radiographic obliteration of anatomical stenosis correlating with reduction in pressure gradients was observed. The complication rate after DVSS was 4.9% and stent survival was 87.8% at 120 days. At least 20% of patients developed restenosis following DVSS and only 63.3% demonstrated an improvement or resolution of papilledema. CONCLUSIONS Reduced venous sinus pressures were observed at 120 days after the procedure. DVSS showed lower complication rates than shunts, but the clinical outcome data were less convincing. To definitively compare the outcomes between DVSS and shunts in IIH, a randomized prospective study is needed.

The predictive value of DESH for shunt responsiveness in idiopathic normal pressure hydrocephalus

OBJECTIVE Selecting probable idiopathic normal pressure hydrocephalus (INPH) patients for shunt insertion presents a challenge because of coexisting comorbidities and other conditions that could mimic NPH. The characteristic appearance of DESH (Disproportionately Enlarged Subarachnoid Space Hydrocephalus) on brain imaging has been shown to have a high positive predictive value in identifying shunt responsive INPH patients (SINPHONI trial). However, the negative predictive value of this radiological sign was not clearly demonstrated. The aim of the present study was to calculate the negative predictive value of the DESH sign. METHODS A single centre study of probable INPH patients, who underwent ventriculoperitoneal (VP) shunt insertion. Shunt responsive INPH patients were identified as those having improvement in their walking speed, neuropsychological assessment and continence one year post operatively. Preoperative images were reviewed for DESH sign. Negative and Positive Predictive Values (NPV and PPV) of DESH sign were determined post analysis. RESULTS A total of 103 probable INPH patients were included (31 were DESH positive (30%) and 72 were DESH negative (70%)). A total of 78 patients showed measurable improvement one year post shunt insertion (76%); 24 (31%) of these patients were DESH positive and 54 (69%) were DESH negative (p=<0.001). Therefore, the DESH sign had an estimated PPV of 77% and NPV of 25%. CONCLUSION DESH sign demonstrates a low negative predictive value. We conclude that DESH negative patients should still undergo prognostic tests for iNPH, such as an extended lumbar drainage protocol, and should not be excluded from shunt insertion.

Occipital Nerve Stimulation for Medically Refractory Chronic Paroxysmal Hemicrania

To describe the outcome of a patient with refractory chronic paroxysmal hemicrania (CPH) to occipital nerve stimulation (ONS).

The predictive value of T-tau and AB1-42 levels in idiopathic normal pressure hydrocephalus

BackgroundIdiopathic normal pressure hydrocephalus (INPH) has no reliable biomarker to assist in the selection of patients who could benefit from ventriculo-peritoneal (VP) shunt insertion. The neurodegenerative markers T-tau and Aβ1-42 have been found to successfully differentiate between Alzheimer’s disease (AD) and INPH and therefore are candidate biomarkers for prognosis and shunt response in INPH. The aim of this study was to test the predictive value of cerebrospinal fluid (CSF) T-tau and Aβ1-42 for shunt responsiveness. In particular, we pay attention to the subset of INPH patients with raised T-tau, who are often expected to be poor surgical candidates.MethodsSingle-centre retrospective analysis of probable INPH patients with CSF samples collected from 2006 to 2016. Index test: CSF levels of T-tau and Aβ1-42. Reference standard: postoperative outcome. ROC analysis assessed the predictive value.ResultsA total of 144 CSF samples from INPH patients were analysed. Lumbar T-tau was a good predictor of post-operative mobility (AUROC 0.80). The majority of patients with a co-existing neurodegenerative disease responded well, including those with high T-tau levels.ConclusionINPH patients tended to exhibit low levels of CSF T-tau, and this can be a good predictor outcome. However levels are highly variable between individuals. Raised T-tau and being shunt-responsive are not mutually exclusive, and such patients ought not necessarily be excluded from having a VP shunt. A combined panel of markers may be a more specific method for aiding selection of patients for VP shunt insertion. This is the most comprehensive presentation of CSF samples from INPH patients to date, thus providing further reference values to the current literature.

Intracranial pressure in patients with papilloedema

Papilloedema is a clinical manifestation of chronically raised intracranial pressure (ICP), often seen in idiopathic intracranial hypertension (IIH). However, the extent of intracranial hypertension required to produce papilloedema is not known. We compare ICP values in IIH patients who developed papilloedema and those who did not. We aim to identify a pathological ICP threshold predictive of the development of papilloedema in IIH patients.

Transtentorial Distortion Syndrome: Consistent Complication Following Lateral and Fourth Ventricular Shunting in Adults

BACKGROUND Complex hydrocephalus affecting lateral and fourth ventricles separately is occasionally managed with cerebrospinal fluid diversion via supratentorial and infratentorial ventricular catheters. The optimal configuration to reduce complications is currently unknown in adults. We describe a consistently similar clinical presentation of patients with complex hydrocephalus and a fourth ventricle separately drained by infratentorial shunt insertion. METHODS This was a retrospective single-center case series. Medical notes were reviewed for clinical presentation, brain imaging, and neurophysiologic tests results. All patients underwent intracranial pressure monitoring (ICPM). Outcomes were determined by ventricular appearance on brain imaging computed tomography and symptomatic improvements postoperatively. RESULTS Five adult patients referred to the hydrocephalus service had separate infratentorial and supratentorial shunt systems. A common clinical presentation was observed, including lower motor neuron facial palsy (confirmed with electrophysiology), ophthalmoplegia, dysarthria, impaired gait headache, and nausea. We refer to this as transtentorial distortion syndrome. Twenty-four-hour ICPM demonstrated clear low pressures. All patients underwent shunt revision connecting the transtentorial shunts via a Y-connector and the addition of a distal valve. All subjects had improved ventricular appearance on computed tomography scans post revision, and normalization of ICPM was observed. In the follow-up period of 6 months, no patient required further shunt revision. CONCLUSION To prevent transtentorial distortion syndrome, supratentorial and infratentorial shunt constructs in adults with encysted fourth ventricles should be similar to the shunt systems widely known in the pediatric population with Dandy-Walker syndrome (i.e., joint output to a single valve distal to the connection of the 2 proximal drainage catheters).

Persistent hydrocephalus due to postural activation of a ventricular shunt anti-gravity device

The ever present need to balance over drainage with under drainage in hydrocephalus has required innovations including adjustable valves with antigravity devices. These are activated in the vertical position to prevent siphoning. We describe a group of bedridden patients who presented with unexplained under drainage caused by activation of antigravity shunt components produced by peculiar head/body position. Retrospective single centre case series of hydrocephalus patients, treated with ventriculo-peritoneal (VP) shunt insertion between April 2014 - February 2016. These patients presented with clinical and radiological under drainage syndrome. Medical notes were reviewed for clinical picture and outcome. Radiological studies were reviewed assessing shunt placement and ventricular size. Seven patients presented with clinical and radiological under drainage syndrome. A consistent posturing of long term hyper-flexion of the neck whilst lying supine was observed. All patients had similar shunt construct (adjustable Miethke ProGAV valve and shunt assistant anti-gravity component). In each of those patients a hypothesis was formulated that neck flexion was activating the shunt assistance anti-gravity component in supine position. Five patients underwent shunt revision surgery removing the shunt assistant device from the cranium and adding an anti-gravity component to the shunt system at the chest. One had the shunt assistant completely removed and one patient was managed conservatively with mobilisation. All patients had clinical and radiological improvement. Antigravity shunt components implanted cranially in bedridden hydrocephalus patients will produce underdrainage due to head flexion induced anti-gravity device activation. In these patients, anti-gravity devices should be placed at the chest. Alternatively, special nursing attention should be paid to head-trunk angle.

Cerebral venous sinus stent insertion as a primary versus secondary procedure in the treatment of intracranial hypertension.

Venous sinus stent insertion is being increasingly used as a primary treatment for intracranial hypertension patients (BIH). However, the value of this treatment modality is still controversial. This study looks into the difference in effectiveness of stents inserted as a primary procedure and those inserted in patients who already had cerebrospinal fluid diverting shunt in place i.e. as a secondary procedure.