Case Report Author's Personal Copy

Chiari Malformation and Masking Neurocysticercosis Sharad Rajpal1, Colson Tomberlin2, Andrew Bauer1, Robert C. Forsythe3, Sigita Burneikiene1,4

Key words - BACKGROUND: Various diagnostic characteristics associated with neuro- - Chiari malformation cysticercosis have been well studied; however, their potential to be implicated - Hydrocephalus - Neurocysticercosis in other differential diagnoses has not been well demonstrated. - Subarachnoid - CASE DESCRIPTION: We report the case of a 55-year-old Hispanic man who Abbreviations and Acronyms underwent a Chiari decompression surgery, which was complicated with hy- CP: Cerebellopontine drocephalus. Despite a ventriculoperitoneal placement, he continued to MRI: Magnetic resonance imaging have headaches and was soon found to have several base subarachnoid VP: Ventriculoperitoneal lesions, which were later diagnosed as the sequelae of an active neuro- From the 1Boulder Neurosurgical Associates, 2University of cysticercosis infection. Colorado Boulder, 3Bouder Valley Pathology, and 4Justin Parker Neurological Institute, Boulder, Colorado, USA - CONCLUSION: This case report highlights the importance of overlapping To whom correspondence should be addressed: symptoms between diseases in a short temporal context. Sharad Rajpal, M.D. [E-mail: [email protected]] Citation: World Neurosurg. (2018) 114:68-71. https://doi.org/10.1016/j.wneu.2018.03.010 duraplasty. He had an uneventful hospital After approximately 8 months, the pa- Journal homepage: www.WORLDNEUROSURGERY.org course and was discharged home after 3 tient was seen in the emergency depart- days. The patient did well for several ment again for a fever, headache, balance Available online: www.sciencedirect.com months but then presented with recurrent problems, and myalgias. MRI of the brain 1878-8750/$ - see front matter ª 2018 Elsevier Inc. All rights reserved. headaches about 5 months after his showed a large arachnoid in the left surgery. Brain imaging demonstrated a cerebellopontine (CP) angle measuring   BACKGROUND large 7.4 9.6 cm pseudomeningocele 3.1 1.7 cm and another in within the surgical site and extending into the right ambient cistern measuring 2.8  Neurocysticercosis is the leading neuro- the posterior tissues of the (Figure 2A 1.7 cm (Figure 5A and B). The larger cyst parasitic infection in the United States, and B). in the CP angle was causing compression affecting approximately 2000 individuals 1 The patient was taken to the operating on the . The VP shunt valve every year. The disease is caused by the was readjusted in the clinic over 2 ingestion of eggs from the tapeworm Taenia room for exploration and revision of the suboccipital pseudomeningocele and place- separate visits in an attempt to increase solium that are excreted in the stool of fl ment of a lumbar drain. At the time of sur- the ow rate with close monitoring of people with taeniasis. Neurocysticercosis the skull-based cysts for any size changes. symptoms range from headaches to gery, a small pinhole was visualized at the edge of the duraplasty graft and revised with a Five months later, the patient continued seizures but can also present with to complain of headaches, dizziness, loss symptoms of hydrocephalus, which can 6-0 nonabsorbable polypropylene suture, fat be caused by chronic arachnoiditis, graft, and dural sealant. He was hospitalized meningeal fibrosis, or intraventricular with continuous lumbar drainage and dis- cysts.2 Here we report a unique presentation charged home on the eighth day reporting an of neurocysticercosis following surgical improvement in his headaches. Approxi- intervention for a Chiari I malformation. mately 4 weeks later, the patient returned to the emergency department complaining of worsening bitemporal headaches, nausea, CASE DESCRIPTION and vomiting. A magnetic resonance imaging A 55-year-old Hispanic male presented to the (MRI) scan indicated that he had developed clinic complaining of balance issues, hydrocephalus with reaccumulation of fluid diplopia, visual field deficits, and ongoing in the subarachnoid space consistent with a headaches that had persisted for about a pseudomeningocele measuring 5.6  4.8 cm year. Radiographic imaging demonstrated (Figure 3A and B). Due to the recurring nature thepresenceofanArnoldChiariImalfor- of hydrocephalus, the patient was taken to mation (Figure 1). He was taken to the the operating room and underwent a right operating room and underwent posterior frontal ventriculoperitoneal (VP) shunt Figure 1. Radiographic imaging fossa decompression via a suboccipital placement (Figure 4). He did well and was demonstrated the presence of an Arnold Chiari I malformation. craniectomy with C1 laminectomy and discharged home the next day.

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Figure 4. Due to the recurring nature of Figure 2. (A and B) Brain imaging demonstrated a large 7.4 Â 9.6 cm pseudomeningocele within the hydrocephalus, the patient was taken to the surgical site and extending into the posterior tissues of the neck. operating room and underwent a right frontal ventriculoperitoneal shunt placement. of lateral vision, and left sided face and the cyst fluid and sent to Pathology. tongue numbness. The cyst in the left CP Figure 5C His postoperative MRI, angle had increased to 3.8 x 2.6 cm demonstrating an improvement in his but no scolex were observed. Pathologic fi although the cyst in the right ambient compressive cyst. and histologic evidence was suf cient to fi cistern decreased in size to 1.8 x 1.4 cm. Histologic analysis (Figure 6AeC)ofthe con rm the diagnosis of neurocysticercosis. The decision was made to proceed with firm lesion, measuring 1.4 Â 1.1 cm, The patient was seen by an infectious surgical exploration and cyst fenestration showed the classic 3-layer distinctive disease expert and placed on close obser- because they were becoming more symp- wall of pleomorphic neurocysticercosis vation for further possible infection and tomatic and not responding to his VP with microtriches/microvilli, an outer symptoms related to neurocysticercosis. In shunt valve adjustments. The patient eosinophilic cuticle layer, a cellular layer this case, anthelminthic drugs were not subsequently underwent a retrosigmoid with uniform cellular dark nuclei, and an prescribed because it was felt the patient fi craniotomy for fenestration of the left CP inner reticular layer containing loose fibrils. had undergone de nitive surgical treat- angle cyst. During his surgery, a small In addition, intense lymphoplasmacytic ment of his neurocysticercosis. The patient firm lesion was unexpectedly noted within inflammation and dystrophic calcifications recovered without any postoperative com- plications or remaining symptoms other than slight balance impairment. His follow-up imaging at 4 months (Figure 7A and B) has demonstrated complete resolution of the arachnoidal cyst in the left CP angle and near-complete resolu- tion of the right ambient cistern cyst measuring 1.2 Â 0.4 cm.

DISCUSSION A host of symptomatic or asymptomatic signs can occur depending on the size, location (parenchymal or extrapar- enchymal), and number of cysts in patients infected with neurocysticercosis. An extraparenchymal racemose form of neu- rocysticercosis is observed to compose up to 54% of cases with cysts located in the cisternal/subarachnoid spaces, , Figure 3. (A and B) Magnetic resonance imaging scan indicated that he had developed hydrocephalus or ocular bulb.3 Seizures are the most with reaccumulation of fluid in the subarachnoid space consistent with a pseudomeningocele common clinical presentation of this measuring 5.6 Â 4.8 cm. form, but hydrocephalus may occur due to

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Figure 5. (AeC) Magnetic resonance imaging of the brain measuring 3.1 Â 1.7 cm and another arachnoid cyst in the right showed a large arachnoid cyst in the left cerebellopontine angle ambient cistern measuring 2.8 Â 1.7 cm. mechanical obstruction or inflammatory hydrocephalus, which is also a common to elevated from an reaction and has been associated with comorbidity of a Chiari malformation and obstructive process secondary to a higher mortality.4 later develop arachnoid cysts. neurocysticercosis infection. However, given Although signs of hydrocephalus can be Our case is unique in that the patient the close temporal proximity of the easily recognized on imaging studies, initially presented with a Chiari malformation Chiari symptoms and the observed cysts, uninflamed extraparenchymal cysticerci without any clinical evidence of a neuro- it is also possible that intracranial may be difficult to identify on computed cysticercosis infection. It was only after hy- hypertension from the cysts caused tomography or MRI scans due to the thin drocephalus was treated with a VP shunt that an acquired Chiari malformation or membrane and the cyst being isodense to he developed enlarging subarachnoidal cysts, worsened the congenital form and it cerebrospinal fluid.5 This diagnostic aspect which were later diagnosed to be infectious in became symptomatic. Future considerations can often be overlooked when diagnosing etiology; this was also associated with regarding recurring hydrocephalus should neurocysticercosis because hydrocephalus symptoms of fever and myalgias. There be met with a high level of scrutiny for their without accompanying radiologic was another similar case reported in the possible association with both morphologic evidence of cysts is typically attributed to literature, but in that case a patient developed and pathophysiologic problems. and treated as something else. To make an acquired Chiari malformation after VP things even more complicated, arachnoid shunt placement for hydrocephalus associ- cysts may develop as a of ated with neurocysticercosis.7 It is more likely CONCLUSIONS overdrainage.6 This had happened in in our case that Chiari malformation Management of neurocysticercosis can be our case when the patient developed was congenital and did not develop due contentious due to the wide variety of

Figure 6. Histologic analysis (AeC) of the firm lesion, measuring outer eosinophilic cuticle layer, a cellular layer with uniform 1.4 Â 1.1 cm, showed the classic 3-layer distinctive wall of cellular dark nuclei, and an inner reticular layer containing loose pleomorphic neurocysticercosis with microtriches/microvilli, an fibrils.

70 www.SCIENCEDIRECT.com WORLD NEUROSURGERY, https://doi.org/10.1016/j.wneu.2018.03.010 Author's Personal Copy CASE REPORT SHARAD RAJPAL ET AL. CHIARI MALFORMATION AND NEUROCYSTICERCOSIS

2. Zymberg ST. Neurocysticercosis. World Neurosurg. 2013;79(suppl 2):S24.e5-S24.e8.

3. Mahale RR, Mehta A, Rangasetty S. Extrapar- enchymal (racemose) neurocysticercosis and its multitude manifestations: a comprehensive review. J Clin Neurol. 2015;11:203-211.

4. Sotelo J, Marin C. Hydrocephalus secondary to cysticercotic arachnoiditis. A long-term follow-up review of 92 cases. J Neurosurg. 1987;66:686-689.

5. Garcia HH, Evans CA, Nash TE, Takayanagui OM, White AC Jr, Botero D, et al. Current consensus guidelines for treatment of neurocysticercosis. Clin Microbiol Rev. 2002;15:747-756.

6. Martinez-Lage JF, Poza M, Lopez F. Arachnoid cyst as a complication of ventricular shunting. Childs Nerv Syst. 1991;7:356-357.

7. Lee JK, Park JY, Chung HS, Suh JK, Lee KC, A B Figure 7. Follow-up imaging at 4 months ( and ) has demonstrated complete resolution of the Lee HK. Acquired Chiari-malformation after ven- arachnoidal cyst in the left cerebellopontine angle and near-complete resolution of the right ambient triculoperitoneal shunt for hydrocephalus associ- Â cistern cyst measuring 1.2 0.4 cm. ated with neurocysticercosis. J Korean Neurosurg Soc. 1996;25:1313-1317. symptoms depending on the location, among subarachnoid cysts, Chiari mal- Conflict of interest statement: Sharad Rajpal, M.D., received developmental stage, and number of cysts formations, and recurring hydrocephalus a consultant fee from Medtronic and an honorarium from the when presenting. With cases of neuro- are important considerations to study Cleveland Clinic Foundation. cysticercosis increasing in the United when making differential diagnoses on Received 28 November 2017; accepted 1 March 2018 States, it is essential that the various neurocysticercosis. Citation: World Neurosurg. (2018) 114:68-71. symptoms and accompanying radio- https://doi.org/10.1016/j.wneu.2018.03.010 graphic results are recognized. Our case REFERENCES Journal homepage: www.WORLDNEUROSURGERY.org report is the first in the literature to Available online: www.sciencedirect.com 1. O’Neal SE, Flecker RH. Hospitalization frequency illustrate a unique manifestation of neu- and charges for neurocysticercosis, United States, 1878-8750/$ - see front matter ª 2018 Elsevier Inc. All rocysticercosis. The potential interactions 2003-2012. Emerg Infect Dis. 2015;21:969-976. rights reserved.

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