- Email: [email protected]
Cervicomedullary neurocysticercosis causing obstructive hydrocephalus
Journal of Clinical Neuroscience xxx (2015) xxx–xxx
Contents lists available at ScienceDirect
Journal of Clinical Neuroscience journal homepage: www.elsevier.com/locate/jocn
Case Report
Cervicomedullary neurocysticercosis causing obstructive hydrocephalus Doris D. Wang a,⇑, Michael C. Huang a,b a b
Department of Neurological Surgery, University of California San Francisco, 505 Parnassus Avenue, Room 779M, San Francisco, CA 94143-0112, USA San Francisco General Hospital, Brain and Spinal Injury Center, San Francisco, CA, USA
a r t i c l e
i n f o
Article history: Received 18 February 2015 Accepted 3 March 2015 Available online xxxx Keywords: Cervical medullary junction Craniocervical junction Foramen magnum Hydrocephalus Posterior fossa Neurocysticercosis Neurosurgery
a b s t r a c t We present a 45-year-old man with tussive headache and blurred vision found to have obstructive hydrocephalus from a neurocysticercal cyst at the cervicomedullary junction who underwent surgical removal of the cyst. We performed a suboccipital craniectomy to remove the cervicomedullary cyst en bloc. Cyst removal successfully treated the patient’s headaches without necessitating permanent cerebrospinal fluid diversion. Neurocysticercosis is the most common parasite infection of the central nervous system causing seizures and, less commonly, hydrocephalus. Intraventricular cysts or arachnoiditis usually cause hydrocephalus in neurocysticercosis but craniocervical junction cysts causing obstructive hydrocephalus are rare. Neurocysticercosis at the craniocervical junction may cause Chiari-like symptoms. In the absence of arachnoiditis and leptomeningeal enhancement, surgical removal of the intact cyst can lead to favorable outcomes. Published by Elsevier Ltd.
1. Introduction Neurocysticercosis is the infection of the central nervous system (CNS) by the larval stage of the pork tapeworm Taenia solium. It is the most common cause of acquired seizure disorders in Latin American countries, sub-Saharan Africa, and Southeast Asia, as well as the most common parasitic infection of the CNS worldwide [1,2]. The prevalence of neurocysticercosis in the USA is low, estimated to be 0.2–0.6 per 100,000 inhabitants [1]. However, with the increasing immigrant population, its incidence is on the rise. Clinical manifestations of neurocysticercosis vary significantly depending on the severity and location of infection. Neurocysticercosis is regarded as the great imitator because it can mimic almost any neurological disorder [3] from Brown–Séquard syndrome [4] to parkinsonism [5] and psychosis [5]. Generally, parenchymal brain cysticercosis presents with seizures as the most common symptom [6]. However, viable T. solium cysts, fluid-filled vesicles with a tapeworm head (scolex) inside, may survive for years with intermittent neurological symptoms. Extraparenchymal neurocysticercosis carries a much worse prognosis with high mortality rates of around 20%, presumably due to intracranial hypertension from cerebrospinal fluid (CSF) flow obstruction [6]. Here, we present 45-year-old man with rare chronic obstructive hydrocephalus from a neurocysticercal cyst at the cervicomedullary
⇑ Corresponding author. Tel.: +1 415 353 3904; fax: +1 415 353 2889. E-mail address: [email protected] (D.D. Wang).
junction causing Chiari-like symptoms, and describe the en bloc resection of the cyst for treatment. 2. Case report A 45-year-old man from Guatemala presented with 4 years of progressive vision loss, headache and thoracic back pain. His vision loss consisted of blurred vision, more noticeable in the bilateral temporal region. An ophthalmology exam revealed bilateral optic disc pallor and optic nerve atrophy. His headache was occipital in nature, non-positional, but worse with valsalva maneuvers. A previous MRI of the thoracic spine revealed perineural cysts in the T11 foramen, thought to be genetic in origin. Besides bitemporal hemianopsia and optic nerve atrophy, he had intact cranial nerves and full motor and sensory functions. He underwent a brain CT scan which revealed hydrocephalus throughout the ventricular system without significant transependymal flow. His brain MRI revealed obstructive hydrocephalus with mass effect on the optic chiasm, sella, and midbrain with a 1.5 cm cystic lesion abutting the cervicomedullary junction at the foramen magnum (Fig. 1A, B). There was no evidence of leptomeningeal enhancement. Additionally, he had a suprasellar cyst elevating the optic nerve (Fig. 1C). The man was admitted for workup. CSF studies revealed 44 white cells per lL, protein of 72 mg/dL, and glucose of 20 mg/dL. Cryptococcal antigen, venereal disease research laboratory, and coccidioidomycosis antibody were all negative. Serum cysticercosis antibody was positive. Given the diagnosis of racemose
http://dx.doi.org/10.1016/j.jocn.2015.03.031 0967-5868/Published by Elsevier Ltd.
Please cite this article in press as: Wang DD, Huang MC. Cervicomedullary neurocysticercosis causing obstructive hydrocephalus. J Clin Neurosci (2015), http://dx.doi.org/10.1016/j.jocn.2015.03.031
2
Case Report / Journal of Clinical Neuroscience xxx (2015) xxx–xxx
Fig. 1. Pre (A–C) and postoperative (D–F) MRI of a patient with neurocysticercosis. (A) Preoperative sagittal T1-weighted MRI with gadolinium showing a 1.5 cm cyst at the cervicomedullary junction causing obstructive hydrocephalus. (B) T2-weighted axial MRI through the level of the medulla showing a round neurocysticercal cyst. (C) Sagittal T1-weighted MRI with gadolinium showing a suprasellar cyst with elevation of the optic tract. Note that there is no leptomeningeal enhancement on the preoperative scans. (D) Postoperative sagittal T1-weighted MRI showing complete removal of the cyst at the craniocervical junction. (E) T2-weighted axial MRI through the level of the medulla showing no residual cyst with expansion of the foramen magnum. (F) The suprasellar cyst remains stable. (G) Intraoperative photo of the suboccipital craniectomy showing the T. solium cyst. Cerebellar tonsils are located to the left of the image. This figure is available in colour at www.sciencedirect.com.
Please cite this article in press as: Wang DD, Huang MC. Cervicomedullary neurocysticercosis causing obstructive hydrocephalus. J Clin Neurosci (2015), http://dx.doi.org/10.1016/j.jocn.2015.03.031
Case Report / Journal of Clinical Neuroscience xxx (2015) xxx–xxx
neurocysticercosis causing obstructive hydrocephalus and increasing headaches and nausea, the man was taken to the operating room for a suboccipital craniectomy for cyst removal with duraplasty and external ventricular drain placement. Intraoperatively, a thin-walled T. solium larva was seen at the cervicomedullary junction just below the cerebellar tonsils (Fig. 1G). We carefully removed the entire cyst without disrupting the contents using forceps (Supp. Fig. 1). In addition, we dissected and fenestrated the arachnoid adhesions at the obex. We were able to visualize CSF egression from the fourth ventricle. The man tolerated the procedure well without complications. He was rapidly weaned off his external ventricular drain and recovered well, though without improvement of his vision. His postoperative MRI showed complete removal of the cervicomedullary cyst without immediate resolution of hydrocephalus (Fig. 1C, D, F). He was started on albendazole treatment with dexamethasone and discharged home in good condition. The postoperative follow up at 3 months showed resolution of his headache with head CT scans showing improvement in hydrocephalus. 3. Discussion Neurocysticercosis is the most prevalent parasitic CNS infection and the most common cause of acquired seizures worldwide, though its presence in the USA is less common [7]. Neurocysticercosis is acquired when T. solium eggs are ingested by humans via the fecal-oral route from close contact with a tapeworm carrier. After ingestion, the embryos are released from the egg in the intestines and cross the intestinal mucosa to the bloodstream which carries them to the peripheral tissues including the CNS, where they develop into cysticerci [2]. Pigs or humans that ingest infective eggs develop cysticercosis and become intermediate hosts [8]. Neurocysticercosis causing hydrocephalus has been well described in the literature and occurs in nearly 30% of patients with neurocysticercosis infection [9]. It usually arises from one of two causes: cysts in the ventricular system causing CSF flow obstruction, or an inflammatory reaction causing arachnoiditis [10–12]. The clinical course of hydrocephalus from arachnoiditis may be subacute or chronic, whereas ventricular cysts may cause acute decompensation such as Bruns syndrome when there is sudden loss of consciousness related to head movement [13,14]. Hydrocephalus related to neurocysticercosis is associated with high mortality rates, except when neurosurgical intervention is possible [2,13,15]. When there is ventricular cysticercosis, consensus guidelines (level 3 evidence) favor surgical removal of the cyst when possible [16–19]. Even though some reports suggest that albendazole treatment is effective against ventricular cysts, these may cause systemic inflammatory arachnoiditis and meningitis [17]. Intraventricular cysts in the lateral and third ventricles can be removed with an endoscopic approach which allows for simultaneous resection of the lesion and complements with CSF diversion via ventriculostomy [17,18]. A simple hydraulic maneuver by placing the patient in the Trendelenburg position intraoperatively can facilitate the endoscopic en bloc removal of third ventricular neurocysticercosis [20]. Cysts in the fourth ventricle can by removed from a posterior approach, either with neuroendoscopy or microdissection [17,21–24]. This has been described in several case series with successful treatment of hydrocephalus without the need for permanent CSF diversion. Our patient presented with evidence of intracranial hypertension with tussive headaches and visual loss from optic nerve atrophy. The optic nerve atrophy is likely secondary to chronic elevated intracranial pressure from CSF obstruction. Given the constellation of symptoms and acute worsening of his headaches, we chose to
3
surgically remove the cervicomedullary cyst to treat the hydrocephalus. A suboccipital craniectomy with duraplasty was successful to remove the cyst en bloc without spilling the cyst contents. We were also able to microdissect and fenestrate the arachnoid adhesions. Even though the patient’s hydrocephalus did not completely resolve radiographically by 3 months, his headache was completely treated. We believe the patient may have had prior arachnoiditis given the chronicity of his headaches and the lack of transependymal fluid-attenuate inversion recovery abnormality on MRI. His intracranial pressure reading from the intraoperative external ventricular drain also showed low pressure. Even though his hydrocephalus was not completely resolved on the CT scan, the improvement in ventricle size and symptomatic relief demonstrated the utility of surgical removal without the need for permanent CSF diversion. To our knowledge, there is only one other published case report of a neurocysticercosis cyst in the cervicomedullary junction [10]. Because of leptomeningeal enhancement and septation of the subarachnoid spaces in that patient, the patient underwent a ventriculoperitoneal shunt placement and albendazole therapy for treatment. Here, we show that surgical removal of a cyst at this location can lead to a successful outcome without necessitating CSF diversion. While management strategies must be case-dependent, we believe that previous guidelines for surgical management of fourth ventricle intraventricular neurocysticercosis can apply to cervicomedullary cysts that are causing obstructive hydrocephalus as well. In series published by Loyo et al., Citow et al., and Apuzzo et al., the presence of gadolinium enhancement of the leptomeninges is highly suggestive of active ependymal inflammation, in which case a permanent ventriculoperitoneal shunt should be used instead of cyst excision, given the high likelihood of worsening hydrocephalus [22–24]. In patients without MRI enhancement, surgical removal of the cyst is favored. Care should be taken to avoid intraoperative cyst rupture whenever possible to avoid arachnoiditis, though there is no clear evidence that there is a difference in outcome between patients with intraoperative cyst rupture and those with intact cyst extraction [22,23]. In summary, neurocysticercosis can occur at the cervicomedullary junction causing obstructive hydrocephalus and Chiari-like symptoms. When there is no MRI evidence of leptomeningeal enhancement, en bloc surgical resection of the cyst can successfully treat the symptoms without the need for permanent CSF diversion. Conflicts of Interest/Disclosures The authors declare that they have no financial or other conflicts of interest in relation to this research and its publication. Appendix A. Supplementary material Supplementary data associated with this article can be found, in the online version, at http://dx.doi.org/10.1016/j.jocn.2015.03.031. References [1] Singh G, Burneo JG, Sander JW. From seizures to epilepsy and its substrates: neurocysticercosis. Epilepsia 2013;54:783–92. [2] Garcia HH, Nash TE, Del Brutto OH. Clinical symptoms, diagnosis, and treatment of neurocysticercosis. Lancet Neurol 2014;13:1202–15. [3] Garcia HH, Del Brutto OH, Cysticercosis Working Group in Peru. Neurocysticercosis: updated concepts about an old disease. Lancet Neurol 2005;4:653–61. [4] Salazar Noguera EM, Pineda Sic R, Escoto Solis F. Intramedullary spinal cord neurocysticercosis presenting as brown-sequard syndrome. BMC Neurol 2015;15:1. [5] Verma R, Anand KS, Sharma BB, et al. Neurocysticercosis presenting as parkinsonism. Neurol India 2014;62:110–1.
Please cite this article in press as: Wang DD, Huang MC. Cervicomedullary neurocysticercosis causing obstructive hydrocephalus. J Clin Neurosci (2015), http://dx.doi.org/10.1016/j.jocn.2015.03.031
4
Case Report / Journal of Clinical Neuroscience xxx (2015) xxx–xxx
[6] Nash TE, Garcia HH. Diagnosis and treatment of neurocysticercosis. Nat Rev Neurol 2011;7:584–94. [7] Serpa JA, White Jr AC. Neurocysticercosis in the United States. Pathog Glob Health 2012;106:256–60. [8] Flisser A. Taeniasis and cysticercosis due to Taenia solium. Prog Clin Parasitol 1994;4:77–116. [9] Lobato RD, Lamas E, Portillo JM, et al. Hydrocephalus in cerebral cysticercosis. Pathogenic and therapeutic considerations. J Neurosurg 1981;55:786–93. [10] Amelot A, Faillot T. Hydrocephalus and neurocysticercosis: cases illustrative of three distinct mechanisms. J Clin Neurol 2014;10:363–6. [11] Monteiro L, Almeida-Pinto J, Stocker A, et al. Active neurocysticercosis, parenchymal and extraparenchymal: a study of 38 patients. J Neurol 1993;241:15–21. [12] Salazar A, Sotelo J, Martinez H, et al. Differential diagnosis between ventriculitis and fourth ventricle cyst in neurocysticercosis. J Neurosurg 1983;59:660–3. [13] Torres-Corzo J, Rodriguez-della Vecchia R, Rangel-Castilla L. Bruns syndrome caused by intraventricular neurocysticercosis treated using flexible endoscopy. J Neurosurg 2006;104:746–8. [14] Teegala R, Rajesh KG, Raviprasad VY, et al. Emergency neuroendoscopic management of third ventricular neurocysticercosis cyst presented with Bruns syndrome: report of two cases and review of literature. J Korean Neurosurg Soc 2014;55:173–7. [15] Santo AH. Cysticercosis-related mortality in the State of Sao Paulo, Brazil, 1985–2004: a study using multiple causes of death. Cad Saude Publica 2007;23:2917–27.
[16] Garcia HH, Evans CA, Nash TE, et al. Current consensus guidelines for treatment of neurocysticercosis. Clin Microbiol Rev 2002;15:747–56. [17] Proano JV, Torres-Corzo J, Rodriguez-Della Vecchia R, et al. Intraventricular and subarachnoid basal cisterns neurocysticercosis: a comparative study between traditional treatment versus neuroendoscopic surgery. Childs Nerv Syst 2009;25:1467–75. [18] Rajshekhar V. Surgical management of neurocysticercosis. Int J Surg 2010;8:100–4. [19] Rangel-Castilla L, Serpa JA, Gopinath SP, et al. Contemporary neurosurgical approaches to neurocysticercosis. Am J Trop Med Hyg 2009;80:373–8. [20] Rapoport BI, Baird LC, Cohen AR. Third-ventricular neurocysticercosis: hydraulic maneuvers facilitating endoscopic resection. Childs Nerv Syst 2014;30:541–6. [21] Hanak BW, Walcott BP, Codd PJ, et al. Fourth ventricular neurocystercercosis presenting with acute hydrocephalus. J Clin Neurosci 2011;18:867–9. [22] Loyo M, Kleriga E, Estanol B. Fourth ventricular cysticercosis. Neurosurgery 1980;7:456–8. [23] Apuzzo ML, Dobkin WR, Zee CS, et al. Surgical considerations in treatment of intraventricular cysticercosis. An analysis of 45 cases. J Neurosurg 1984;60:400–7. [24] Citow JS, Johnson JP, McBride DQ, et al. Imaging features and surgery-related outcomes in intraventricular neurocysticercosis. Neurosurg Focus 2002;12:e6.
Please cite this article in press as: Wang DD, Huang MC. Cervicomedullary neurocysticercosis causing obstructive hydrocephalus. J Clin Neurosci (2015), http://dx.doi.org/10.1016/j.jocn.2015.03.031
Contents lists available at ScienceDirect
Journal of Clinical Neuroscience journal homepage: www.elsevier.com/locate/jocn
Case Report
Cervicomedullary neurocysticercosis causing obstructive hydrocephalus Doris D. Wang a,⇑, Michael C. Huang a,b a b
Department of Neurological Surgery, University of California San Francisco, 505 Parnassus Avenue, Room 779M, San Francisco, CA 94143-0112, USA San Francisco General Hospital, Brain and Spinal Injury Center, San Francisco, CA, USA
a r t i c l e
i n f o
Article history: Received 18 February 2015 Accepted 3 March 2015 Available online xxxx Keywords: Cervical medullary junction Craniocervical junction Foramen magnum Hydrocephalus Posterior fossa Neurocysticercosis Neurosurgery
a b s t r a c t We present a 45-year-old man with tussive headache and blurred vision found to have obstructive hydrocephalus from a neurocysticercal cyst at the cervicomedullary junction who underwent surgical removal of the cyst. We performed a suboccipital craniectomy to remove the cervicomedullary cyst en bloc. Cyst removal successfully treated the patient’s headaches without necessitating permanent cerebrospinal fluid diversion. Neurocysticercosis is the most common parasite infection of the central nervous system causing seizures and, less commonly, hydrocephalus. Intraventricular cysts or arachnoiditis usually cause hydrocephalus in neurocysticercosis but craniocervical junction cysts causing obstructive hydrocephalus are rare. Neurocysticercosis at the craniocervical junction may cause Chiari-like symptoms. In the absence of arachnoiditis and leptomeningeal enhancement, surgical removal of the intact cyst can lead to favorable outcomes. Published by Elsevier Ltd.
1. Introduction Neurocysticercosis is the infection of the central nervous system (CNS) by the larval stage of the pork tapeworm Taenia solium. It is the most common cause of acquired seizure disorders in Latin American countries, sub-Saharan Africa, and Southeast Asia, as well as the most common parasitic infection of the CNS worldwide [1,2]. The prevalence of neurocysticercosis in the USA is low, estimated to be 0.2–0.6 per 100,000 inhabitants [1]. However, with the increasing immigrant population, its incidence is on the rise. Clinical manifestations of neurocysticercosis vary significantly depending on the severity and location of infection. Neurocysticercosis is regarded as the great imitator because it can mimic almost any neurological disorder [3] from Brown–Séquard syndrome [4] to parkinsonism [5] and psychosis [5]. Generally, parenchymal brain cysticercosis presents with seizures as the most common symptom [6]. However, viable T. solium cysts, fluid-filled vesicles with a tapeworm head (scolex) inside, may survive for years with intermittent neurological symptoms. Extraparenchymal neurocysticercosis carries a much worse prognosis with high mortality rates of around 20%, presumably due to intracranial hypertension from cerebrospinal fluid (CSF) flow obstruction [6]. Here, we present 45-year-old man with rare chronic obstructive hydrocephalus from a neurocysticercal cyst at the cervicomedullary
⇑ Corresponding author. Tel.: +1 415 353 3904; fax: +1 415 353 2889. E-mail address: [email protected] (D.D. Wang).
junction causing Chiari-like symptoms, and describe the en bloc resection of the cyst for treatment. 2. Case report A 45-year-old man from Guatemala presented with 4 years of progressive vision loss, headache and thoracic back pain. His vision loss consisted of blurred vision, more noticeable in the bilateral temporal region. An ophthalmology exam revealed bilateral optic disc pallor and optic nerve atrophy. His headache was occipital in nature, non-positional, but worse with valsalva maneuvers. A previous MRI of the thoracic spine revealed perineural cysts in the T11 foramen, thought to be genetic in origin. Besides bitemporal hemianopsia and optic nerve atrophy, he had intact cranial nerves and full motor and sensory functions. He underwent a brain CT scan which revealed hydrocephalus throughout the ventricular system without significant transependymal flow. His brain MRI revealed obstructive hydrocephalus with mass effect on the optic chiasm, sella, and midbrain with a 1.5 cm cystic lesion abutting the cervicomedullary junction at the foramen magnum (Fig. 1A, B). There was no evidence of leptomeningeal enhancement. Additionally, he had a suprasellar cyst elevating the optic nerve (Fig. 1C). The man was admitted for workup. CSF studies revealed 44 white cells per lL, protein of 72 mg/dL, and glucose of 20 mg/dL. Cryptococcal antigen, venereal disease research laboratory, and coccidioidomycosis antibody were all negative. Serum cysticercosis antibody was positive. Given the diagnosis of racemose
http://dx.doi.org/10.1016/j.jocn.2015.03.031 0967-5868/Published by Elsevier Ltd.
Please cite this article in press as: Wang DD, Huang MC. Cervicomedullary neurocysticercosis causing obstructive hydrocephalus. J Clin Neurosci (2015), http://dx.doi.org/10.1016/j.jocn.2015.03.031
2
Case Report / Journal of Clinical Neuroscience xxx (2015) xxx–xxx
Fig. 1. Pre (A–C) and postoperative (D–F) MRI of a patient with neurocysticercosis. (A) Preoperative sagittal T1-weighted MRI with gadolinium showing a 1.5 cm cyst at the cervicomedullary junction causing obstructive hydrocephalus. (B) T2-weighted axial MRI through the level of the medulla showing a round neurocysticercal cyst. (C) Sagittal T1-weighted MRI with gadolinium showing a suprasellar cyst with elevation of the optic tract. Note that there is no leptomeningeal enhancement on the preoperative scans. (D) Postoperative sagittal T1-weighted MRI showing complete removal of the cyst at the craniocervical junction. (E) T2-weighted axial MRI through the level of the medulla showing no residual cyst with expansion of the foramen magnum. (F) The suprasellar cyst remains stable. (G) Intraoperative photo of the suboccipital craniectomy showing the T. solium cyst. Cerebellar tonsils are located to the left of the image. This figure is available in colour at www.sciencedirect.com.
Please cite this article in press as: Wang DD, Huang MC. Cervicomedullary neurocysticercosis causing obstructive hydrocephalus. J Clin Neurosci (2015), http://dx.doi.org/10.1016/j.jocn.2015.03.031
Case Report / Journal of Clinical Neuroscience xxx (2015) xxx–xxx
neurocysticercosis causing obstructive hydrocephalus and increasing headaches and nausea, the man was taken to the operating room for a suboccipital craniectomy for cyst removal with duraplasty and external ventricular drain placement. Intraoperatively, a thin-walled T. solium larva was seen at the cervicomedullary junction just below the cerebellar tonsils (Fig. 1G). We carefully removed the entire cyst without disrupting the contents using forceps (Supp. Fig. 1). In addition, we dissected and fenestrated the arachnoid adhesions at the obex. We were able to visualize CSF egression from the fourth ventricle. The man tolerated the procedure well without complications. He was rapidly weaned off his external ventricular drain and recovered well, though without improvement of his vision. His postoperative MRI showed complete removal of the cervicomedullary cyst without immediate resolution of hydrocephalus (Fig. 1C, D, F). He was started on albendazole treatment with dexamethasone and discharged home in good condition. The postoperative follow up at 3 months showed resolution of his headache with head CT scans showing improvement in hydrocephalus. 3. Discussion Neurocysticercosis is the most prevalent parasitic CNS infection and the most common cause of acquired seizures worldwide, though its presence in the USA is less common [7]. Neurocysticercosis is acquired when T. solium eggs are ingested by humans via the fecal-oral route from close contact with a tapeworm carrier. After ingestion, the embryos are released from the egg in the intestines and cross the intestinal mucosa to the bloodstream which carries them to the peripheral tissues including the CNS, where they develop into cysticerci [2]. Pigs or humans that ingest infective eggs develop cysticercosis and become intermediate hosts [8]. Neurocysticercosis causing hydrocephalus has been well described in the literature and occurs in nearly 30% of patients with neurocysticercosis infection [9]. It usually arises from one of two causes: cysts in the ventricular system causing CSF flow obstruction, or an inflammatory reaction causing arachnoiditis [10–12]. The clinical course of hydrocephalus from arachnoiditis may be subacute or chronic, whereas ventricular cysts may cause acute decompensation such as Bruns syndrome when there is sudden loss of consciousness related to head movement [13,14]. Hydrocephalus related to neurocysticercosis is associated with high mortality rates, except when neurosurgical intervention is possible [2,13,15]. When there is ventricular cysticercosis, consensus guidelines (level 3 evidence) favor surgical removal of the cyst when possible [16–19]. Even though some reports suggest that albendazole treatment is effective against ventricular cysts, these may cause systemic inflammatory arachnoiditis and meningitis [17]. Intraventricular cysts in the lateral and third ventricles can be removed with an endoscopic approach which allows for simultaneous resection of the lesion and complements with CSF diversion via ventriculostomy [17,18]. A simple hydraulic maneuver by placing the patient in the Trendelenburg position intraoperatively can facilitate the endoscopic en bloc removal of third ventricular neurocysticercosis [20]. Cysts in the fourth ventricle can by removed from a posterior approach, either with neuroendoscopy or microdissection [17,21–24]. This has been described in several case series with successful treatment of hydrocephalus without the need for permanent CSF diversion. Our patient presented with evidence of intracranial hypertension with tussive headaches and visual loss from optic nerve atrophy. The optic nerve atrophy is likely secondary to chronic elevated intracranial pressure from CSF obstruction. Given the constellation of symptoms and acute worsening of his headaches, we chose to
3
surgically remove the cervicomedullary cyst to treat the hydrocephalus. A suboccipital craniectomy with duraplasty was successful to remove the cyst en bloc without spilling the cyst contents. We were also able to microdissect and fenestrate the arachnoid adhesions. Even though the patient’s hydrocephalus did not completely resolve radiographically by 3 months, his headache was completely treated. We believe the patient may have had prior arachnoiditis given the chronicity of his headaches and the lack of transependymal fluid-attenuate inversion recovery abnormality on MRI. His intracranial pressure reading from the intraoperative external ventricular drain also showed low pressure. Even though his hydrocephalus was not completely resolved on the CT scan, the improvement in ventricle size and symptomatic relief demonstrated the utility of surgical removal without the need for permanent CSF diversion. To our knowledge, there is only one other published case report of a neurocysticercosis cyst in the cervicomedullary junction [10]. Because of leptomeningeal enhancement and septation of the subarachnoid spaces in that patient, the patient underwent a ventriculoperitoneal shunt placement and albendazole therapy for treatment. Here, we show that surgical removal of a cyst at this location can lead to a successful outcome without necessitating CSF diversion. While management strategies must be case-dependent, we believe that previous guidelines for surgical management of fourth ventricle intraventricular neurocysticercosis can apply to cervicomedullary cysts that are causing obstructive hydrocephalus as well. In series published by Loyo et al., Citow et al., and Apuzzo et al., the presence of gadolinium enhancement of the leptomeninges is highly suggestive of active ependymal inflammation, in which case a permanent ventriculoperitoneal shunt should be used instead of cyst excision, given the high likelihood of worsening hydrocephalus [22–24]. In patients without MRI enhancement, surgical removal of the cyst is favored. Care should be taken to avoid intraoperative cyst rupture whenever possible to avoid arachnoiditis, though there is no clear evidence that there is a difference in outcome between patients with intraoperative cyst rupture and those with intact cyst extraction [22,23]. In summary, neurocysticercosis can occur at the cervicomedullary junction causing obstructive hydrocephalus and Chiari-like symptoms. When there is no MRI evidence of leptomeningeal enhancement, en bloc surgical resection of the cyst can successfully treat the symptoms without the need for permanent CSF diversion. Conflicts of Interest/Disclosures The authors declare that they have no financial or other conflicts of interest in relation to this research and its publication. Appendix A. Supplementary material Supplementary data associated with this article can be found, in the online version, at http://dx.doi.org/10.1016/j.jocn.2015.03.031. References [1] Singh G, Burneo JG, Sander JW. From seizures to epilepsy and its substrates: neurocysticercosis. Epilepsia 2013;54:783–92. [2] Garcia HH, Nash TE, Del Brutto OH. Clinical symptoms, diagnosis, and treatment of neurocysticercosis. Lancet Neurol 2014;13:1202–15. [3] Garcia HH, Del Brutto OH, Cysticercosis Working Group in Peru. Neurocysticercosis: updated concepts about an old disease. Lancet Neurol 2005;4:653–61. [4] Salazar Noguera EM, Pineda Sic R, Escoto Solis F. Intramedullary spinal cord neurocysticercosis presenting as brown-sequard syndrome. BMC Neurol 2015;15:1. [5] Verma R, Anand KS, Sharma BB, et al. Neurocysticercosis presenting as parkinsonism. Neurol India 2014;62:110–1.
Please cite this article in press as: Wang DD, Huang MC. Cervicomedullary neurocysticercosis causing obstructive hydrocephalus. J Clin Neurosci (2015), http://dx.doi.org/10.1016/j.jocn.2015.03.031
4
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[6] Nash TE, Garcia HH. Diagnosis and treatment of neurocysticercosis. Nat Rev Neurol 2011;7:584–94. [7] Serpa JA, White Jr AC. Neurocysticercosis in the United States. Pathog Glob Health 2012;106:256–60. [8] Flisser A. Taeniasis and cysticercosis due to Taenia solium. Prog Clin Parasitol 1994;4:77–116. [9] Lobato RD, Lamas E, Portillo JM, et al. Hydrocephalus in cerebral cysticercosis. Pathogenic and therapeutic considerations. J Neurosurg 1981;55:786–93. [10] Amelot A, Faillot T. Hydrocephalus and neurocysticercosis: cases illustrative of three distinct mechanisms. J Clin Neurol 2014;10:363–6. [11] Monteiro L, Almeida-Pinto J, Stocker A, et al. Active neurocysticercosis, parenchymal and extraparenchymal: a study of 38 patients. J Neurol 1993;241:15–21. [12] Salazar A, Sotelo J, Martinez H, et al. Differential diagnosis between ventriculitis and fourth ventricle cyst in neurocysticercosis. J Neurosurg 1983;59:660–3. [13] Torres-Corzo J, Rodriguez-della Vecchia R, Rangel-Castilla L. Bruns syndrome caused by intraventricular neurocysticercosis treated using flexible endoscopy. J Neurosurg 2006;104:746–8. [14] Teegala R, Rajesh KG, Raviprasad VY, et al. Emergency neuroendoscopic management of third ventricular neurocysticercosis cyst presented with Bruns syndrome: report of two cases and review of literature. J Korean Neurosurg Soc 2014;55:173–7. [15] Santo AH. Cysticercosis-related mortality in the State of Sao Paulo, Brazil, 1985–2004: a study using multiple causes of death. Cad Saude Publica 2007;23:2917–27.
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Please cite this article in press as: Wang DD, Huang MC. Cervicomedullary neurocysticercosis causing obstructive hydrocephalus. J Clin Neurosci (2015), http://dx.doi.org/10.1016/j.jocn.2015.03.031