Will CSF Diversion in Patients with Idiopathic Intracranial Hypertension (IIH) Lead to Long-Lasting Shunt Dependency?

Long-term shunt dependency rates in patients treated for IIH with CSF diversion have not been established. We therefore present our experience with 5 children shunted for IIH during the years 1984–2000 with very long-time follow-up. Three out of these patients have experienced late or very late episodes of severe shunt failure during the second or third decade after initial shunt treatment. They were all boys and may not be representative for IIH patients as a whole. Three of them appear, however, to be permanently shunt dependent, indicating that long-term shunt-dependency in children treated for IIH with CSF diversion may be more common than previously expected.

Dopaminergic Degeneration and Small Vessel Disease in Patients with Normal Pressure Hydrocephalus Who Underwent Shunt Surgery

The diagnosis of idiopathic normal pressure hydrocephalus (iNPH) and the outcome of lumboperitoneal shunt treatment remains to be systematically explored. Here, we aim to evaluate whether the severity of dopaminergic degeneration and white matter small vessel disease could be predictors of outcome for iNPH patients subjected to lumboperitoneal shunt treatment. This is a single center retrospective study with 39 patients with probable iNPH undergoing programmable surgical lumboperitoneal shunt from June 2016 to March 2018 at Hualien Tzu Chi Hospital. In all patients, dopaminergic degeneration was determined with 99mTc- TRODAT-1 SPECT scan, while white matter small vessel disease (Fazekas scale) was assessed with Brain MRI. The iNPH grading scale (iNPHGS) score and Karnofsky Performance Score (KPS) pre- and post-operation (6-month follow-up) were available for all patients. Linear regression was used to correlate the severities of dopaminergic degeneration and small vessel disease with lumboperitoneal shunt treatment outcomes. Their iNPHGS score improved significantly after surgery (pre-operatively, 7.8 ± 2.6; post-operatively, 5.7 ± 2.6 (26.9% improvement) (p < 0.05)). Moreover, the KPS was also improved significantly after surgery, by a mean of 24.6% from the baseline score (p < 0.05). A significant correlation was observed between the severity of dopaminergic degeneration and a poorer improvement of iNPHGS score (p = 0.03). However, improvement of the iNPHGS score was not correlated with white matter small vessel disease. Dopaminergic degeneration comorbidity neutralized the degree of improvement after surgery. Although white matter small vessel disease was correlated with iNPH incidence, it may not be a prognostic factor for shunt operation. These findings have implications for the use of dopaminergic imaging, as they might help predict the surgical outcome of patients with iNPH, while vascular mechanisms seem to be involved in iNPH pathophysiology.