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Case Reports / Journal of Clinical Neuroscience 21 (2014) 1066–1068
Paradoxical migrating cyst: An unusual presentation of intraventricular neurocysticercosis with a coincidental pituitary adenoma Shanchita Ghosh a, Rend Al-Khalili a, James K. Liu b, Shira E. Slasky a,⇑ a b
Department of Radiology, Rutgers University, New Jersey Medical School, 150 Bergen Street, University Hospital Suite C-318, Newark, NJ 07103, USA Department of Neurological Surgery, Rutgers University, New Jersey Medical School, Newark, NJ, USA
a r t i c l e
i n f o
Article history: Received 9 October 2013 Accepted 18 October 2013
Keywords: Computer tomography Magnetic resonance imaging Migrating cyst Neurocysticercosis
a b s t r a c t Intraventricular neurocysticercosis is an uncommon entity which may become symptomatic due to cerebrospinal fluid flow obstruction. Migration of intraventricular cysts through the ventricular spaces is a rare occurrence. This phenomenon is poorly understood but may be due to pressure changes within the ventricular cavities. We present a patient with intraventricular neurocysticercosis with paradoxical transaqueductal migration of the cyst from the cerebral aqueduct to the fourth ventricle shortly after ventricular drain placement for acute hydrocephalus. The patient also presented with a coincidental sellar and suprasellar mass, later pathologically proven to be a pituitary adenoma. The migration of this cyst resulted in spontaneous relief of obstruction at the cerebral aqueduct, thus restoring normal cerebrospinal fluid pathways and avoiding permanent shunting. We discuss the possible mechanisms and implications of cyst migration, and the diagnostic challenges of concomitant findings of a pituitary mass and neurocysticercosis. Although the presence of a sellar and suprasellar mass in a patient with known neurocysticercosis should raise clinical suspicion for the possibility of sellar neurocysticercosis, pituitary macroadenoma is a more common entity and a more likely etiology for a sellar lesion. Ó 2013 Elsevier Ltd. All rights reserved.
1. Case report A 26-year-old Ecuadorian immigrant presented with headaches and altered mental status for 2 days. In the emergency department, the patient became obtunded within 2 hours. CT scan of the head showed obstructive hydrocephalus at the level of the cerebral aqueduct (Fig. 1). A heterogeneous soft tissue mass extending from the sella to the suprasellar cistern, as well as a small cystic lesion within the right parietal lobe, was also seen. A focus of calcification was noted in the dilated cerebral aqueduct. An external ventricular drain placed emergently to relieve the hydrocephalus which resulted in improvement in neurological status. MRI of the brain showed obstructive hydrocephalus due to an ovoid lesion in the aqueduct of Sylvius, demonstrating T1-weighted shortening, measuring 6.5 mm 6.7 mm 13 mm (craniocaudal) (Fig. 2). Ring-enhancing lesions in the right postcentral sulcus and in the left anteromedial frontal lobe were also identified. A mixed solid and cystic sellar and suprasellar lesion exerted mass effect on the optic apparatus (Fig. 2). The ring-enhancing right frontal and parietal lesions were typical for neurocysticercosis; however, the lesion in the cerebral aqueduct had characteristics consistent with a proteinaceous or hemorrhagic cyst, rather than the typical appearance of neurocysticercosis as a simple cyst with or without a scolex. Neurocysticercosis, however, was considered given the other lesions. The sellar and suprasellar lesion was suggestive of a pituitary macroadenoma but neurocysticercosis was also considered. The patient was started on albendazole and dexamethasone for neurocysticercosis. The serum prolactin level was found to be 1108 ng/mL, consistent with a prolactin-secreting pituitary adenoma. The patient was started on bromocriptine without adequate response and therefore operative management was chosen. Serum cysticercosis immunoglobulin G was positive. A repeat pituitary MRI, performed about 48 hours after the initial MRI, showed a ventriculostomy tube with a decrease in size of the ventricles. The cystic lesion in the cerebral aqueduct had ⇑ Corresponding author. Tel.: +1 646 337 8170; fax: +1 973 972 7429. E-mail address: [email protected] (S.E. Slasky).
migrated to the fourth ventricle, where it now measured 9.1 mm 8.6 mm 8.6 mm and had a more rounded configuration (Fig. 3). The mixed solid and cystic sellar and suprasellar lesion was unchanged in size and configuration. Because the cerebral aqueduct was no longer obstructed, the external ventricular drain was removed without recurrent hydrocephalus. The patient underwent endoscopic endonasal transsphenoidal resection of the pituitary mass and surgical pathology confirmed a pituitary adenoma, prolactin-secreting type. Followup MRI performed after 2.5 weeks of treatment with albendazole showed collapse and involution of the fourth ventricular cyst which now measured 5.6 mm 4.2 mm 5.2 mm, consistent with treatment response (Fig. 3). Postoperatively, the patient remained neurologically intact.
2. Discussion Clinical manifestations of neurocysticercosis result from host inflammatory responses to cyst degeneration; however, intraventricular cysticerci can cause ventricular obstruction depending on cyst location [1]. We demonstrate that neurocysticercosis may obstruct cerebrospinal fluid flow at a hazardous location, such as the narrow cerebral aqueduct, and later migrate into a space better suited to accommodate the mass effect of the cyst, in this case the fourth ventricle. This resulted in spontaneous resolution of obstruction without further intervention. Additionally, migrating cysts present a unique challenge to operative planning for cyst removal, potentially resulting in surgical exploration of the incorrect ventricular cavity. Our case showed cyst migration through the cerebral aqueduct within 2 days. To our knowledge the only other case of transaqueductal migration in the literature suggests that a cyst may complete this path within 45 minutes [2]. Rapid cyst migration must be considered to avoid surgical exploration of the incorrect ventricular cavity. Intraventricular cysts are known to be freely mobile within the ventricles. A buildup of pressure above the cyst may induce pressure gradients producing mass effect on the cyst, forcing migration [1]. Additionally, pressure gradients caused by manipulation of the
Case Reports / Journal of Clinical Neuroscience 21 (2014) 1066–1068
1067
Fig. 1. (a) Axial non-contrast CT scan demonstrating enlargement of the lateral and third ventricles consistent with obstructive hydrocephalus. (b) Sagittal reformatted image from the same CT scan demonstrates a calcified focus (arrow) within the cerebral aqueduct.
Fig. 2. Sagittal T1-weighted MRI (a) without contrast and (b) after intravenous gadolinium showing a non-enhancing hyperintense lesion (long arrow) within the aqueduct of Sylvius causing obstructive hydrocephalus. There is an enhancing heterogeneous solid and cystic lesion (short arrow) within the sella and suprasellar cistern.
Fig. 3. (a) Sagittal T1-weighted non-contrast MRI 48 hours after the initial MRI showing the hyperintense lesion previously seen in the aqueduct is now seen within the fourth ventricle (arrow). The lateral and third ventricles are now of normal size. (b) Sagittal T1-weighted MRI with intravenous gadolinium after 2.5 weeks of treatment with albendazole showing involution of the fourth ventricular cyst (arrow). The pituitary lesion has been resected.
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Case Reports / Journal of Clinical Neuroscience 21 (2014) 1068–1070
ventricles, usually in an attempt to decrease elevated intracranial pressures through ventriculostomy, have been associated with cyst migration [3,4]. In this fashion it would be expected that the cyst would have migrated from its original location in the cerebral aqueduct into the third ventricle after ventricular drain placement. This case is unusual in that migration occurred in the opposite of the predicted direction, a phenomenon not illustrated in the current literature. The cyst must have been partially compressible in nature because it was able to migrate through the cerebral aqueduct, which is in accordance with the known histopathology of neurocysticercosis cysts as thin walled flexible structures. The calcifications of an involuted cyst would be substantially less compressible [2]. The simultaneous presentation of a pituitary adenoma and neurocysticercosis in this patient was coincidental but confounded the diagnosis. While there is only one reported patient with simultaneous presentation of pituitary macroadenoma and neurocysticercosis to our knowledge, there are multiple accounts of neurocysticercosis mimicking pituitary masses [5,6]. Given that pituitary macroadenoma is a common entity in the general population [7], a coincidental pituitary mass in a patient with neurocysticercosis will most likely represent a macroadenoma; however, sellar neurocysticercosis can mimic a pituitary
macroadenoma and therefore a high degree of clinical suspicion is necessary for accurate diagnosis. 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. References [1] Cuetter A, Andrews R. Intraventricular neurocysticercosis: 18 consecutive patients and review of the literature. Neurosurg Focus 2002;12: e5. [2] Kramer J, Carrazana E, Cosgrove G, et al. Transaqueductal migration of a neurocysticercus cyst. Case Report. J Neurosurg 1992;77:956–8. [3] Przybojewski S, Griffith-Richards SB, Strachan M, et al. ‘Migrating’ intraventricular neurocysticercus cyst. S Afr J Radiol 2007;11:25–6. [4] Chowdhary A, Abel TJ, Gabikian P, et al. Case report: resolution of acute hydrocephalus and migration of neurocysticercosis cyst with external ventricular drainage. Care Rep Med 2010;2010:245259. [5] Cohn-Zurita F, Guinto-Balanzar G, Pérez-Cerdán H. Neurocysticercosis associated with pituitary adenoma: case report and literature review. Cir Cir 2006;74:47–9. [6] Arriada-Mendicoa N, Celis-Lopez MA, Higuera-Calleja J, et al. Imaging features of sellar cysticercosis. AJNR Am J Neuroradiol 2003;24:1386–9. [7] Feldkamp J, Santen R, Harms E, et al. Incidentally discovered pituitary lesions: high frequency of macroadenomas and hormone-secreting adenomas – results of a prospective study. Clin Endocrinol 1999;51:109–13.
http://dx.doi.org/10.1016/j.jocn.2013.10.010
Central nervous system lymphoma associated with natalizumab Angelika Na a, Nick Hall a, Bhadrakant Kavar a, John King b,⇑ a b
Department of Neurosurgery, Royal Melbourne Hospital, Parkville, Melbourne, VIC, Australia Department of Neurology, Royal Melbourne Hospital, Grattan Street, Parkville, Melbourne, VIC 3050, Australia
a r t i c l e
i n f o
Article history: Received 13 October 2013 Accepted 18 October 2013
a b s t r a c t Patients with primary central nervous system lymphoma (PCNSL) after treatment with natalizumab have been considered co-incidental. We report another case of PCNSL in a patient where the explosive onset suggests a causal link. Ó 2013 Elsevier Ltd. All rights reserved.
Keywords: Lymphoma Multiple sclerosis Natalizumab
1. Case report A 28-year-old man with multiple sclerosis (MS) was seen in January 2013 with 1 week of confusion and increasing left arm weakness. In 2009 he presented with 3 years of leg weakness, bilateral optic disc pallor and spasticity of both legs. MRI of the brain and spinal cord and visual evoked responses confirmed the diagnosis of MS. He completed four doses of mitoxantrone 20 mg (one dose every 3 months) and was then lost to follow-up until December 2010, when he was stable. In May 2012 he returned with a left footdrop and brain MRI showed a gadolinium enhancing lesion in the right precentral gyrus but no other right frontal lesion. Because of compliance issues, he was treated with natalizumab 300 mg every 4 weeks and was well until January 2013 when he had seven doses of ⇑ Corresponding author. Tel.: +61 3 9348 2500; fax: +61 3 9348 2522. E-mail address: [email protected] (J. King).
natalizumab. Brain MRI in late January 2013 showed a large enhancing right frontal lesion with gross mass effect (Fig. 1). Biopsy revealed a diffuse large B-cell lymphoma. Immunostaining and in situ hybridization for Epstein Barr virus (EBV) were negative. EBV – viral capsid antigen immunoglobulin G (IgG) and EBV – nuclear antigen IgG were present in serum. His John Cunningham virus (JCV) and human immunodeficiency virus (HIV) serology were negative. There was no evidence of extracerebral lymphoma. Despite chemotherapy and radiotherapy he died in June 2013.
2. Discussion The first reported case of PCNSL after the use of natalizumab occurred in a 40-year-old MS patient treated with 21 infusions of natalizumab, who developed a PCNSL intermediate between high grade diffuse large B-cell lymphoma and Burkitt lymphoma, which was EBV negative [1]. An association between lymphoma and natalizumab was doubted by others because the absence of
Case Reports / Journal of Clinical Neuroscience 21 (2014) 1066–1068
Paradoxical migrating cyst: An unusual presentation of intraventricular neurocysticercosis with a coincidental pituitary adenoma Shanchita Ghosh a, Rend Al-Khalili a, James K. Liu b, Shira E. Slasky a,⇑ a b
Department of Radiology, Rutgers University, New Jersey Medical School, 150 Bergen Street, University Hospital Suite C-318, Newark, NJ 07103, USA Department of Neurological Surgery, Rutgers University, New Jersey Medical School, Newark, NJ, USA
a r t i c l e
i n f o
Article history: Received 9 October 2013 Accepted 18 October 2013
Keywords: Computer tomography Magnetic resonance imaging Migrating cyst Neurocysticercosis
a b s t r a c t Intraventricular neurocysticercosis is an uncommon entity which may become symptomatic due to cerebrospinal fluid flow obstruction. Migration of intraventricular cysts through the ventricular spaces is a rare occurrence. This phenomenon is poorly understood but may be due to pressure changes within the ventricular cavities. We present a patient with intraventricular neurocysticercosis with paradoxical transaqueductal migration of the cyst from the cerebral aqueduct to the fourth ventricle shortly after ventricular drain placement for acute hydrocephalus. The patient also presented with a coincidental sellar and suprasellar mass, later pathologically proven to be a pituitary adenoma. The migration of this cyst resulted in spontaneous relief of obstruction at the cerebral aqueduct, thus restoring normal cerebrospinal fluid pathways and avoiding permanent shunting. We discuss the possible mechanisms and implications of cyst migration, and the diagnostic challenges of concomitant findings of a pituitary mass and neurocysticercosis. Although the presence of a sellar and suprasellar mass in a patient with known neurocysticercosis should raise clinical suspicion for the possibility of sellar neurocysticercosis, pituitary macroadenoma is a more common entity and a more likely etiology for a sellar lesion. Ó 2013 Elsevier Ltd. All rights reserved.
1. Case report A 26-year-old Ecuadorian immigrant presented with headaches and altered mental status for 2 days. In the emergency department, the patient became obtunded within 2 hours. CT scan of the head showed obstructive hydrocephalus at the level of the cerebral aqueduct (Fig. 1). A heterogeneous soft tissue mass extending from the sella to the suprasellar cistern, as well as a small cystic lesion within the right parietal lobe, was also seen. A focus of calcification was noted in the dilated cerebral aqueduct. An external ventricular drain placed emergently to relieve the hydrocephalus which resulted in improvement in neurological status. MRI of the brain showed obstructive hydrocephalus due to an ovoid lesion in the aqueduct of Sylvius, demonstrating T1-weighted shortening, measuring 6.5 mm 6.7 mm 13 mm (craniocaudal) (Fig. 2). Ring-enhancing lesions in the right postcentral sulcus and in the left anteromedial frontal lobe were also identified. A mixed solid and cystic sellar and suprasellar lesion exerted mass effect on the optic apparatus (Fig. 2). The ring-enhancing right frontal and parietal lesions were typical for neurocysticercosis; however, the lesion in the cerebral aqueduct had characteristics consistent with a proteinaceous or hemorrhagic cyst, rather than the typical appearance of neurocysticercosis as a simple cyst with or without a scolex. Neurocysticercosis, however, was considered given the other lesions. The sellar and suprasellar lesion was suggestive of a pituitary macroadenoma but neurocysticercosis was also considered. The patient was started on albendazole and dexamethasone for neurocysticercosis. The serum prolactin level was found to be 1108 ng/mL, consistent with a prolactin-secreting pituitary adenoma. The patient was started on bromocriptine without adequate response and therefore operative management was chosen. Serum cysticercosis immunoglobulin G was positive. A repeat pituitary MRI, performed about 48 hours after the initial MRI, showed a ventriculostomy tube with a decrease in size of the ventricles. The cystic lesion in the cerebral aqueduct had ⇑ Corresponding author. Tel.: +1 646 337 8170; fax: +1 973 972 7429. E-mail address: [email protected] (S.E. Slasky).
migrated to the fourth ventricle, where it now measured 9.1 mm 8.6 mm 8.6 mm and had a more rounded configuration (Fig. 3). The mixed solid and cystic sellar and suprasellar lesion was unchanged in size and configuration. Because the cerebral aqueduct was no longer obstructed, the external ventricular drain was removed without recurrent hydrocephalus. The patient underwent endoscopic endonasal transsphenoidal resection of the pituitary mass and surgical pathology confirmed a pituitary adenoma, prolactin-secreting type. Followup MRI performed after 2.5 weeks of treatment with albendazole showed collapse and involution of the fourth ventricular cyst which now measured 5.6 mm 4.2 mm 5.2 mm, consistent with treatment response (Fig. 3). Postoperatively, the patient remained neurologically intact.
2. Discussion Clinical manifestations of neurocysticercosis result from host inflammatory responses to cyst degeneration; however, intraventricular cysticerci can cause ventricular obstruction depending on cyst location [1]. We demonstrate that neurocysticercosis may obstruct cerebrospinal fluid flow at a hazardous location, such as the narrow cerebral aqueduct, and later migrate into a space better suited to accommodate the mass effect of the cyst, in this case the fourth ventricle. This resulted in spontaneous resolution of obstruction without further intervention. Additionally, migrating cysts present a unique challenge to operative planning for cyst removal, potentially resulting in surgical exploration of the incorrect ventricular cavity. Our case showed cyst migration through the cerebral aqueduct within 2 days. To our knowledge the only other case of transaqueductal migration in the literature suggests that a cyst may complete this path within 45 minutes [2]. Rapid cyst migration must be considered to avoid surgical exploration of the incorrect ventricular cavity. Intraventricular cysts are known to be freely mobile within the ventricles. A buildup of pressure above the cyst may induce pressure gradients producing mass effect on the cyst, forcing migration [1]. Additionally, pressure gradients caused by manipulation of the
Case Reports / Journal of Clinical Neuroscience 21 (2014) 1066–1068
1067
Fig. 1. (a) Axial non-contrast CT scan demonstrating enlargement of the lateral and third ventricles consistent with obstructive hydrocephalus. (b) Sagittal reformatted image from the same CT scan demonstrates a calcified focus (arrow) within the cerebral aqueduct.
Fig. 2. Sagittal T1-weighted MRI (a) without contrast and (b) after intravenous gadolinium showing a non-enhancing hyperintense lesion (long arrow) within the aqueduct of Sylvius causing obstructive hydrocephalus. There is an enhancing heterogeneous solid and cystic lesion (short arrow) within the sella and suprasellar cistern.
Fig. 3. (a) Sagittal T1-weighted non-contrast MRI 48 hours after the initial MRI showing the hyperintense lesion previously seen in the aqueduct is now seen within the fourth ventricle (arrow). The lateral and third ventricles are now of normal size. (b) Sagittal T1-weighted MRI with intravenous gadolinium after 2.5 weeks of treatment with albendazole showing involution of the fourth ventricular cyst (arrow). The pituitary lesion has been resected.
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Case Reports / Journal of Clinical Neuroscience 21 (2014) 1068–1070
ventricles, usually in an attempt to decrease elevated intracranial pressures through ventriculostomy, have been associated with cyst migration [3,4]. In this fashion it would be expected that the cyst would have migrated from its original location in the cerebral aqueduct into the third ventricle after ventricular drain placement. This case is unusual in that migration occurred in the opposite of the predicted direction, a phenomenon not illustrated in the current literature. The cyst must have been partially compressible in nature because it was able to migrate through the cerebral aqueduct, which is in accordance with the known histopathology of neurocysticercosis cysts as thin walled flexible structures. The calcifications of an involuted cyst would be substantially less compressible [2]. The simultaneous presentation of a pituitary adenoma and neurocysticercosis in this patient was coincidental but confounded the diagnosis. While there is only one reported patient with simultaneous presentation of pituitary macroadenoma and neurocysticercosis to our knowledge, there are multiple accounts of neurocysticercosis mimicking pituitary masses [5,6]. Given that pituitary macroadenoma is a common entity in the general population [7], a coincidental pituitary mass in a patient with neurocysticercosis will most likely represent a macroadenoma; however, sellar neurocysticercosis can mimic a pituitary
macroadenoma and therefore a high degree of clinical suspicion is necessary for accurate diagnosis. 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. References [1] Cuetter A, Andrews R. Intraventricular neurocysticercosis: 18 consecutive patients and review of the literature. Neurosurg Focus 2002;12: e5. [2] Kramer J, Carrazana E, Cosgrove G, et al. Transaqueductal migration of a neurocysticercus cyst. Case Report. J Neurosurg 1992;77:956–8. [3] Przybojewski S, Griffith-Richards SB, Strachan M, et al. ‘Migrating’ intraventricular neurocysticercus cyst. S Afr J Radiol 2007;11:25–6. [4] Chowdhary A, Abel TJ, Gabikian P, et al. Case report: resolution of acute hydrocephalus and migration of neurocysticercosis cyst with external ventricular drainage. Care Rep Med 2010;2010:245259. [5] Cohn-Zurita F, Guinto-Balanzar G, Pérez-Cerdán H. Neurocysticercosis associated with pituitary adenoma: case report and literature review. Cir Cir 2006;74:47–9. [6] Arriada-Mendicoa N, Celis-Lopez MA, Higuera-Calleja J, et al. Imaging features of sellar cysticercosis. AJNR Am J Neuroradiol 2003;24:1386–9. [7] Feldkamp J, Santen R, Harms E, et al. Incidentally discovered pituitary lesions: high frequency of macroadenomas and hormone-secreting adenomas – results of a prospective study. Clin Endocrinol 1999;51:109–13.
http://dx.doi.org/10.1016/j.jocn.2013.10.010
Central nervous system lymphoma associated with natalizumab Angelika Na a, Nick Hall a, Bhadrakant Kavar a, John King b,⇑ a b
Department of Neurosurgery, Royal Melbourne Hospital, Parkville, Melbourne, VIC, Australia Department of Neurology, Royal Melbourne Hospital, Grattan Street, Parkville, Melbourne, VIC 3050, Australia
a r t i c l e
i n f o
Article history: Received 13 October 2013 Accepted 18 October 2013
a b s t r a c t Patients with primary central nervous system lymphoma (PCNSL) after treatment with natalizumab have been considered co-incidental. We report another case of PCNSL in a patient where the explosive onset suggests a causal link. Ó 2013 Elsevier Ltd. All rights reserved.
Keywords: Lymphoma Multiple sclerosis Natalizumab
1. Case report A 28-year-old man with multiple sclerosis (MS) was seen in January 2013 with 1 week of confusion and increasing left arm weakness. In 2009 he presented with 3 years of leg weakness, bilateral optic disc pallor and spasticity of both legs. MRI of the brain and spinal cord and visual evoked responses confirmed the diagnosis of MS. He completed four doses of mitoxantrone 20 mg (one dose every 3 months) and was then lost to follow-up until December 2010, when he was stable. In May 2012 he returned with a left footdrop and brain MRI showed a gadolinium enhancing lesion in the right precentral gyrus but no other right frontal lesion. Because of compliance issues, he was treated with natalizumab 300 mg every 4 weeks and was well until January 2013 when he had seven doses of ⇑ Corresponding author. Tel.: +61 3 9348 2500; fax: +61 3 9348 2522. E-mail address: [email protected] (J. King).
natalizumab. Brain MRI in late January 2013 showed a large enhancing right frontal lesion with gross mass effect (Fig. 1). Biopsy revealed a diffuse large B-cell lymphoma. Immunostaining and in situ hybridization for Epstein Barr virus (EBV) were negative. EBV – viral capsid antigen immunoglobulin G (IgG) and EBV – nuclear antigen IgG were present in serum. His John Cunningham virus (JCV) and human immunodeficiency virus (HIV) serology were negative. There was no evidence of extracerebral lymphoma. Despite chemotherapy and radiotherapy he died in June 2013.
2. Discussion The first reported case of PCNSL after the use of natalizumab occurred in a 40-year-old MS patient treated with 21 infusions of natalizumab, who developed a PCNSL intermediate between high grade diffuse large B-cell lymphoma and Burkitt lymphoma, which was EBV negative [1]. An association between lymphoma and natalizumab was doubted by others because the absence of
