- Sidney Kimmel Medical College, Thomas Jefferson University, Philadelphia, PA, USA
- Department of Neurological Surgery, Thomas Jefferson University, Philadelphia, PA, USA
- Division of Infectious Diseases and Environmental Medicine, Thomas Jefferson University, Philadelphia, PA, USA
Christopher J. Farrell
Department of Neurological Surgery, Thomas Jefferson University, Philadelphia, PA, USA
DOI:10.4103/2152-7806.175881Copyright: © 2016 Surgical Neurology International This is an open access article distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 3.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as the author is credited and the new creations are licensed under the identical terms.
How to cite this article: Krupa K, Krupa K, Pisculli ML, Athas DM, Farrell CJ. Racemose neurocysticercosis. Surg Neurol Int 05-Feb-2016;7:12
How to cite this URL: Krupa K, Krupa K, Pisculli ML, Athas DM, Farrell CJ. Racemose neurocysticercosis. Surg Neurol Int 05-Feb-2016;7:12. Available from: http://surgicalneurologyint.com/surgicalint_articles/racemose-neurocysticercosis/
Background:Neurocysticercosis (NCC) is an invasive parasitic infection of the central nervous system caused by the larval stage of the tapeworm Taenia solium. The clinical manifestations of NCC depend on the parasitic load and location of infection, as well as the developmental stage of the cysticerci and host immune response, with symptoms ranging from subclinical headaches to seizures, cerebrovascular events, and life-threatening hydrocephalus. Racemose NCC represents a particularly severe variant of extraparenchymal NCC characterized by the presence of multiple confluent cysts within the subarachnoid space and is associated with increased morbidity and mortality, as well as a decreased response to treatment. Albendazole is the preferred drug for the treatment of racemose NCC due to its superior cerebrospinal fluid penetration compared to praziquantel and the ability to be used concomitantly with steroids.
Case Description:In this report, we describe a 39-year-old man recently emigrated from Mexico with racemose NCC and hydrocephalus successfully treated with prolonged albendazole treatment, high-dose dexamethasone, and ventriculoperitoneal shunt placement for the relief of obstructive hydrocephalus.
Conclusions:Treatment of racemose NCC represents a significant clinical challenge requiring multimodal intervention to minimize infectious- and treatment-related morbidity. We review the clinical, diagnostic, and therapeutic features relevant to the management of this aggressive form of NCC.
Keywords: Neurocysticercosis, racemose, Taenia solium, treatment
Neurocysticercosis (NCC) is a common parasitic infection of the central nervous system (CNS) caused by the tapeworm Taenia solium. Infection is typically characterized by the presence of intraparenchymal cysts within the cerebral hemispheres at the gray-white junction and NCC is the most frequent cause of acquired epilepsy in the developing countries.[
A 39-year-old male presented to the emergency room with a complaint of worsening headaches and visual decline. The patient emigrated from Mexico 13 months prior to presentation and reported no other significant medical history. He described diffuse headaches that had progressed over a 1-month period with new-onset bilateral visual impairment and nausea for the last week. His neurologic exam was nonfocal, however, the ophthalmologic evaluation revealed bilateral papilledema and diminished visual acuity. He was afebrile on presentation and without meningeal irritation signs. Initial laboratory findings indicated a normal white blood cell count of 7400/mm3 with 73% polymorphonuclear leukocytes, 21% lymphocytes, and 1% eosinophils. A head computed tomography (CT) scan demonstrated hydrocephalus with transependymal flow and a multilobulated hypodense mass in the basal cisterns. Subsequent magnetic resonance imaging (MRI) was performed which demonstrated the presence of too numerous to count nonenhancing, T2-hyperintense cystic lesions in the lateral ventricles and subarachnoid spaces including the bilateral sylvian fissures and cerebellopontine angles, as well as the suprasellar, interpeduncular, and prepontine cisterns with the largest cyst measuring 21 mm in diameter and exerting mild mass effect upon the brainstem [
(a) Axial T2-weighted magnetic resonance imaging demonstrating multiple confluent cystic lesions in the basal cisterns and sylvian fissures. (b) Fluid-attenuated inversion recovery magnetic resonance imaging showing multiple cystic lesions with the largest cyst measuring 21 mm in maximal diameter (dashed arrow) and mild mass effect on the brainstem. (c) Sagittal T1-weighted magnetic resonance imaging postgadolinium administration showing hydrocephalus and cystic lesions in suprasellar, prepontine, and foramen magnum cisterns. (d) Fluid-attenuated inversion recovery magnetic resonance imaging demonstrating multiple cystic lesions within the lateral ventricles bilaterally (arrows) with enlarged ventricles and transependymal flow
After extensive discussion between the neurosurgical and infectious diseases teams, we elected to proceed with placement of a ventriculoperitoneal shunt to ameliorate his elevated intracranial pressure and communicating hydrocephalus. High-dose dexamethasone (4 mg QID) was initiated followed by administration of prolonged albendazole (10 mg/kg/day) treatment. The patient experienced immediate relief of his visual deficit following the CSF diversion treatment. He maintained a mild headache disorder that responded well to steroids, and he was able to be slowly weaned off of this medication over a 12 month period. MRI scan performed at that time revealed complete radiographic resolution of his subarachnoid, spinal, and intraventricular cystic lesions with very mild persistent meningeal enhancement [
Cysticercosis is the most common helminthic infection of the CNS with infection occurring primarily through the ingestion of eggs of the parasitic tapeworm T. solium.[
The diagnosis of racemose NCC is typically based on neuroimaging, serum and CSF laboratory evaluation, and clinical, epidemiologic data including immigration from or frequent travel to disease-endemic areas. Contrast-enhanced MRI is the imaging modality of choice for the detection of extraparenchymal NCC as CT has limited sensitivity for identification of intraventricular and small subarachnoid cysts.[
The intraventricular and cisternal forms of NCC typically manifest in a more clinically aggressive manner than parenchymal infection and frequently cause intracranial hypertension due to CSF outflow obstruction or inflammatory basal arachnoiditis.[
No consensus exists regarding the optimal treatment of racemose NCC including the duration of anthelmintic treatment and the role of surgical intervention. In patients presenting with hydrocephalus, the priority is relief of raised intracranial pressure.[
In our patient with hydrocephalus and relatively rapid progression of visual loss, given the bilateral sylvian fissure involvement and absence of any dominant cystic lesion or focal neurologic deficits, we elected to proceed with placement of a ventriculoperitoneal shunt prior to initiating therapy directed against the invasive infection. Delayed exacerbation of intracranial hypertension frequently occurs several days following administration of cysticidal agents as a result of cyst degeneration and subsequent host inflammatory response with the release of proinflammatory cytokines.[
In our patient, we reported the successful clinical resolution of racemose NCC following CSF diversion and prolonged anthelmintic therapy and corticosteroid administration to prevent the development of chronic subarachnoid inflammation. No shunt complications or revisions occurred, and a complete radiographic response was observed. Racemose NCC represents a, fortunately, rare but aggressive form of extraparenchymal NCC often resulting in basilar arachnoiditis, vasculitis, and cranial neuropathy. Proper recognition of this infection and appropriate management with careful consideration of the deleterious effects of the attendant host inflammatory response to cyst degeneration is necessary to reduce patient morbidity and mortality. Racemose NCC accompanied by intracranial hypertension and hydrocephalus is associated with poorer clinical outcomes, often related to ventriculoperitoneal shunt infection and malfunction. Vigilant clinical and radiographic monitoring of response to treatment is imperative to reduce neurologic sequelae and infection relapse as prolonged, or multicourse medical therapy is frequently necessary.
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