Intraventricular Neurocysticercosis: Experience and Long-Term Outcome from a Tertiary Referral Center in the United States

Abstract

Ventricular involvement in neurocysticercosis (NCC), a common serious manifestation of NCC, has distinct clinical presentations, complications, and treatments primarily because of partial or complete obstruction of the cerebrospinal fluid (CSF) flow by Taenia solium cysts. We review the clinical course, treatments, and long-term outcomes in 23 of 121 (19.0%) total NCC patients with ventricular cysts referred to the National Institutes of Health from 1985 to the October 2017. Patients had a median age of 31.8 (range: 22.4-52.6 years), were 60.9% male, diagnosed a median of 6.5 years (range: 0.17-16 years) after immigration, and were followed for a median of 3.6 years (range: 0.1-30.5 years). Other forms and manifestations of NCC were present in 73.9% (17/23). The fourth ventricle was involved in a majority (15/23, 65.2%) resulting in hydrocephalus (73.9%), ventriculitis, and periventricular edema (7/23, 30.4%). Cystectomy was accomplished in 60.9%, usually by removal of a fourth ventricular cyst through a suboccipital craniotomy. Nonresectable cysts were treated medically. Ventriculoperitoneal shunts were inserted in 43.5% (10/23) and failed in four, three from infection. Other complications included surgically induced injuries (4/23, 17.4%) and entrapment of a lateral ventricle (2/23, 8.7%). Despite a common severe early course, 90.9% (20/22) stabilized without recurrence, 15% (3/20) complained of mild-to-moderate neurological complaints, and 15% (3/20) were significantly disabled. Four patients who underwent removal of ventricular cysts without significant other NCC and who received with no cysticidal treatment became CSF cestode antigen negative without recurrence indicating that after successful extraction of cysts, additional cysticidal treatment may not be needed.

Figure 1.

MRI imaging of the case. Panels (A) and (B) are imaging performed on the day of presentation, February 7, 2011, panel (C) on the day after presentation, February 8, 2011 and panel (D) on the day of admission to National Institutes of Health on June 2, 2011. Panel (A) is a sagittal T1 weighted fast field Echo (FFE) image revealing a barely visible third ventricle cyst showing [better seen in the axial view in Panel (B)] and part of the cyst in the process exiting the aqueduct into the fourth ventricle. Arrows delineate the cyst in the third and fourth ventricles. Panel (B) is an axial fast-attenuated inversion recovery image showing acute hydrocephalus with transependymal flow and a third ventricular cyst (arrow). Panel (C) is a cerebrospinal fluid-driven equilibrium radiofrequency reset pulse image demonstrating the scolex now fully situated in the fourth ventricle. Panel (D) is an axial balanced FFE image on June 2, 2011 (balanced fast field echo) image showing a cyst occupying the fourth ventricle (arrow) with a calcified scolex seen as a void in the middle of the cyst. This figure appears in color at www.ajtmh.org.

Figure 2.

Panels (A) and (B) are Axial fast-attenuated inversion recovery (FLAIR) and sagittal T1W fast field Echo (FFE) imaging, respectively, of a patient with massive enlargement of the fourth ventricle with a scolex within the cyst. Hydrocephalus is apparent in the sagittal image. Panel (C) shows two FLAIR images demonstrating extensive periventricular edema around the fourth ventricle. Panel (D) is a lateral sagittal short-T1 inversion recovery image showing the fourth ventricle cyst that has exited the fourth ventricle into the cisterna magna by way of the foramen of Luschka.