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Midbrain neurocysticercosis presenting as isolated pupil sparing third cranial nerve palsy
Journal of the Neurological Sciences 312 (2012) 36–38
Contents lists available at SciVerse ScienceDirect
Journal of the Neurological Sciences journal homepage: www.elsevier.com/locate/jns
Midbrain neurocysticercosis presenting as isolated pupil sparing third cranial nerve palsy Navdeep Khurana, Pawan Sharma, R. Shukla, Dilip Singh ⁎, Mukund Vidhate, Pravin U. Naphade Department of Neurology, CSM Medical University, Lucknow, U.P., India
a r t i c l e
i n f o
Article history: Received 17 April 2011 Received in revised form 17 August 2011 Accepted 17 August 2011 Available online 8 September 2011 Keywords: Neurocysticercosis Third nerve palsy Midbrain NCC
a b s t r a c t Brainstem alone is involved very infrequently in patients with neurocysticercosis; usually, it occurs in association with disseminated form of the disease. Isolated involvement of the third nerve is commonly due to vascular causes. We are reporting a case due to cysticercus lesion presenting as isolated third-nerve involvement with sparing of the pupil. The diagnosis of neurocysticercosis was established by the presence of characteristic granulomatous lesions in the midbrain along with positive ELISA for cysticercal antigen in the cerebrospinal fluid. The patient responded well to corticosteroids with almost complete recovery. A follow-up MRI scan showed a significant decrease in the size of the lesion. © 2011 Elsevier B.V. All rights reserved.
1. Introduction Cysticercosis is the most common parasitic disease involving the central nervous system (CNS). It is caused by the infestation of the larval form of Taenia solium, with humans being the primary host. Neurocysticercosis commonly presents with seizures, raised intracranial tension, and dementia. It is a common differential diagnosis of intracranial space-occupying lesion in India. The unusual location of the cysts may result in uncommon manifestations mimicking many neurological disorders. Third cranial nerve palsy in neurocysticercosis is rare, and very few cases have been reported in literature [1–8].
2. Case report A 22-year-old man presented with acute onset drooping of the left eyelid and double vision for 5 days, this was resolved on closing one eye. Ocular movements were not associated with pain. He had no history of trauma, diabetes, hypertension, headache, seizure, or any other significant past history. There was neither history of a similar episode nor history of tuberculosis or tubercular contact in the past. The general physical examination was normal. On neurological examination, there was marked ptosis (7 mm) on the left side. The pupils were round, symmetrical, reactive, and about 4 mm in size. On the left side, the extraocular movement examination showed normal abduction; however, adduction, supraduction, and infraduction were restricted (Fig. 1). An initial contrast-enhanced CT scan was performed, which revealed a ring-enhancing lesion in the brainstem on the left side. Subsequently, ⁎ Corresponding author. Tel.: + 91 522 2257300; fax: + 91 0522 0258138. E-mail address: [email protected] (D. Singh). 0022-510X/$ – see front matter © 2011 Elsevier B.V. All rights reserved. doi:10.1016/j.jns.2011.08.027
an MRI was performed in which SET1 and FSET2 axial images of the brain with sagittal T2W and coronal FLAIR images showed a ring lesion in the midbrain on the left side with contrast enhancement, thereby suggestive of inflammatory granuloma/neurocysticercosis (Fig. 2). Polymerase chain reaction (PCR) analysis of mycobacterium DNA was negative in both CSF and serum. Enzyme-linked immunosorbent assay (ELISA) for cysticercus was positive in CSF. The ELISA for HIV −1 and 2 was also negative. X-ray of the chest, thigh, and arm was normal. Based on the imaging results and the high prevalence of disease in our region, these findings were considered as representing neurocysticercosis [9]. The patient was treated with only edema-lowering agents (steroids). He recovered almost completely within one month. Follow-up MRI at three months revealed significant resolution of the granuloma. At one-year follow-up, he was completely asymptomatic. 3. Discussion Cerebral cysticerci are mainly located in gray matter or at the junction of gray and white matter, areas with a rich blood supply [1,9], although any site of the central nervous system (CNS) can be involved, thus producing myriad of manifestations. Rare clinical presentations previously described in patients with neurocysticercosis include papillitis, isolated bilateral ptosis, cerebral hemorrhage, dorsal midbrain syndrome, paraplegia due to an intramedullary cyst, third-ventricular cyst, dystonia, and nominal aphasia masquerading as transient ischemic attacks [6]. Neuro-ophthalmological signs are usually caused by associated meningitis or hydrocephalus, and the recognition of these findings may provide a clue to the presence of serious neurologic disease. Hydrocephalus, found in 25% of NCC, is often accompanied by sixth or third cranial nerve palsy [3]. Third cranial nerve palsy, relatively rare in NCC, usually occurs either due to
N. Khurana et al. / Journal of the Neurological Sciences 312 (2012) 36–38
37
Fig. 1. Picture of the patient showing limited movements in adduction, supraduction, infraduction and normal abduction along with severe ptosis on the left side.
Fig. 2. Picture of the patient showing slight limitation in adduction on the left side and normal supraduction abduction.
brainstem compression by a supratentorial lesion or due to meningitis with secondary hydrocephalus. Isolated third cranial nerve palsy from a midbrain parenchymal lesion has rarely been reported [1–3]. Case reports showing isolated superior divisional oculomotor nerve involvement and fascicular involvement as a part of Weber's and Claude's syndrome have been described [4–8]. Table 1 summarizes cases presented with isolated third-nerve palsy caused by neurocysticercosis, diagnosed on the
basis of neuroimaging and immunological tests. All patients were men between 15 and 59 years of age. Out of five cases, two were treated with steroids alone, and the rest were treated with both steroids and anticysticidal therapy. All patients showed symptomatic improvement with resolution of lesion on neuroimaging. All cases reported in the literature with third-nerve involvement showed pupillary dilatation; whereas in our case, the pupils were normal in size and reaction (Table 1). The presentation in our case is probably related to the
Table 1 Table showing patients clinical finding neuroimaging and follow up clinical finding neuroimaging. Patient Positive clinical findings age/sex
Neuroimaging CT/MRI lesion
1
16/M
Ring lesion R side midbrain
2
34/M
3
59/M
4
15/M
5
25/M
Present case
22/M
Ref. no.
R—right, L—left.
R ptosis, weak supraduction, adduction, infraduction pupillary dilatation Complete ptosis L eye and partial ptosis R eye, limited to abduction and intortion on attempted down gaze, L pupillary dilatation L ptosis, and reduced supraduction, infraduction, and adduction, L dilated pupil R ptosis, limitation of dextroelevation on the R side, normal pupil R ptosis, limitation of dextroelevation on the R side, normal pupil L ptosis, and reduced supraduction, infraduction, and adduction, normal pupil
Treatment
Follow up findings Clinical
Neuroimaging
Resolution in 3 weeks
Not reported
Single ring enhancing lesion (11.6 × 9.7 × 11.11 mm) with perifocal edema at the tegmentum of the L midbrain Praziquantel 15 days Nodular lesions in the and intravenous midbrain tegmentum methylprednisolone and occipital pole, repeat steroid courses Two small cystic lesions in Steroids midbrain on the R side Ring lesion R side of midbrain Albendazole and steroids
Resolution in 6 days
CT 4 months normal
Ring lesion L side of midbrain Steroids
Resolution in 4 weeks
Albendazole dexamethasone Steroids
Recurrent exacerbation MRI 4 months lesion size reduced on steroids tapering resolution after 2 years Resolution in 7 days Not reported Resolution in 7 days
MRI at 2 months showed resolution of edema and contrast enhancement MRI 3 months lesion size reduced
38
N. Khurana et al. / Journal of the Neurological Sciences 312 (2012) 36–38
present with isolated pupil sparing third cranial nerve involvement; and, therefore, it should be included in the list of etiological diagnosis of pupil sparing third-nerve palsy. Conflict of interest No conflicts of interest. References
Fig. 3. Schematic diagram showing neurocysticercosis lesion in midbrain involving third-nerve fascicules.
involvement of the third-nerve fascicle within the midbrain before they exit the midbrain (Fig. 3). The fascicular portion of the oculomotor nerve courses ventrally from the nucleus in the dorsal midbrain tegmentum, passes through the red nucleus, and emerges from the medial aspect of the cerebral peduncle. Fascicular involvement can result in individual or multiple extraocular muscles involvement. It has been proposed that pupillary fibers are situated in the superomedial portion of the midbrain fascicular fibers; so, any lesion that is situated lower and lateral to these pupillary fibers in the midbrain can involve various ocular muscles, whereas the pupil may not be involved [10–12]. The case demonstrates that midbrain cysticercosis may
[1] Ranjith MP, Divya R, Sahni A. Isolated third cranial nerve palsy: a rare presentation of neurocysticercosis. Ir J Med Sci 2009, doi:10.1007/s11845-009-0308-6. [2] Mokta JK, Mahajan S, Machhan P, Mokta KK, Patial RK, Prashar BS. Recurrent oculomotor nerve palsy: a rare presentation of neurocysticercosis. Neurol India 2004;52:402. [3] Kim JS, Jeong SM, Moon SY, Park SH. Third cranial nerve palsy from midbrain neurocysticercosis: repeated exacerbation on tapering corticosteroids. J Neuroophthalmol 2004;24(3):217–20. [4] Meena MK, Khuteta A, Vashishtha R. Isolated superior division oculomotor palsy in neurocysticercosis: a rare presentation. Br J Ophthalmol 2010;94(7):954–5. [5] Chotmongkol V, Sawanyawisuth K, Limpawattana P, Phuphatham A, Chotmongkol R, Intapan PM. Superior divisional oculomotor nerve palsy caused by midbrain neurocysticercosis. Parasitol Int 2006;55(3):223–5. [6] Sawhney IM, Singh G, Lekhra OP, et al. Uncommon presentations of neurocysticercosis. J Neurol Sci 1998;54:94–100. [7] Song Tae-Jin, Sang Hyun Suh, Hanna Cho, Kyung-Yul Lee. Claude's syndrome associated with neurocysticercosis. Yonsei Med J 2010;51(6):978–9. [8] Singh NN, Verma R, Pankaj BK, Misra S. Neurocysticercosis presenting as Weber's syndrome. Neurol India 2003;53:551–2. [9] Del Bruto OH, Rajshekhar V, White Jr AC, et al. Proposed criteria for neurocysticercosis. Neurology 2001;57:177–83. [10] Castro O, Johnson LN, Mamourian AC. Isolated interior oblique paresis from brainstem infarction: perspective on oculomotor fascicular organization in the ventral midbrain tegmentum. Arch Neurol 1990;47:235–7. [11] Schwartz TH, Lycette CA, Kargman DE. Clinicoradiographic evidence for oculomotor fascicular anatomy. J Neurol Neurosurg Psychiatry 1995;59:338. [12] Breen LA, Hopf HC, Farris RK, Gutman L. Pupil-sparing oculomotor nerve palsy due to a midbrain infarction. Arch Neurol 1991;l48:105–6.
Contents lists available at SciVerse ScienceDirect
Journal of the Neurological Sciences journal homepage: www.elsevier.com/locate/jns
Midbrain neurocysticercosis presenting as isolated pupil sparing third cranial nerve palsy Navdeep Khurana, Pawan Sharma, R. Shukla, Dilip Singh ⁎, Mukund Vidhate, Pravin U. Naphade Department of Neurology, CSM Medical University, Lucknow, U.P., India
a r t i c l e
i n f o
Article history: Received 17 April 2011 Received in revised form 17 August 2011 Accepted 17 August 2011 Available online 8 September 2011 Keywords: Neurocysticercosis Third nerve palsy Midbrain NCC
a b s t r a c t Brainstem alone is involved very infrequently in patients with neurocysticercosis; usually, it occurs in association with disseminated form of the disease. Isolated involvement of the third nerve is commonly due to vascular causes. We are reporting a case due to cysticercus lesion presenting as isolated third-nerve involvement with sparing of the pupil. The diagnosis of neurocysticercosis was established by the presence of characteristic granulomatous lesions in the midbrain along with positive ELISA for cysticercal antigen in the cerebrospinal fluid. The patient responded well to corticosteroids with almost complete recovery. A follow-up MRI scan showed a significant decrease in the size of the lesion. © 2011 Elsevier B.V. All rights reserved.
1. Introduction Cysticercosis is the most common parasitic disease involving the central nervous system (CNS). It is caused by the infestation of the larval form of Taenia solium, with humans being the primary host. Neurocysticercosis commonly presents with seizures, raised intracranial tension, and dementia. It is a common differential diagnosis of intracranial space-occupying lesion in India. The unusual location of the cysts may result in uncommon manifestations mimicking many neurological disorders. Third cranial nerve palsy in neurocysticercosis is rare, and very few cases have been reported in literature [1–8].
2. Case report A 22-year-old man presented with acute onset drooping of the left eyelid and double vision for 5 days, this was resolved on closing one eye. Ocular movements were not associated with pain. He had no history of trauma, diabetes, hypertension, headache, seizure, or any other significant past history. There was neither history of a similar episode nor history of tuberculosis or tubercular contact in the past. The general physical examination was normal. On neurological examination, there was marked ptosis (7 mm) on the left side. The pupils were round, symmetrical, reactive, and about 4 mm in size. On the left side, the extraocular movement examination showed normal abduction; however, adduction, supraduction, and infraduction were restricted (Fig. 1). An initial contrast-enhanced CT scan was performed, which revealed a ring-enhancing lesion in the brainstem on the left side. Subsequently, ⁎ Corresponding author. Tel.: + 91 522 2257300; fax: + 91 0522 0258138. E-mail address: [email protected] (D. Singh). 0022-510X/$ – see front matter © 2011 Elsevier B.V. All rights reserved. doi:10.1016/j.jns.2011.08.027
an MRI was performed in which SET1 and FSET2 axial images of the brain with sagittal T2W and coronal FLAIR images showed a ring lesion in the midbrain on the left side with contrast enhancement, thereby suggestive of inflammatory granuloma/neurocysticercosis (Fig. 2). Polymerase chain reaction (PCR) analysis of mycobacterium DNA was negative in both CSF and serum. Enzyme-linked immunosorbent assay (ELISA) for cysticercus was positive in CSF. The ELISA for HIV −1 and 2 was also negative. X-ray of the chest, thigh, and arm was normal. Based on the imaging results and the high prevalence of disease in our region, these findings were considered as representing neurocysticercosis [9]. The patient was treated with only edema-lowering agents (steroids). He recovered almost completely within one month. Follow-up MRI at three months revealed significant resolution of the granuloma. At one-year follow-up, he was completely asymptomatic. 3. Discussion Cerebral cysticerci are mainly located in gray matter or at the junction of gray and white matter, areas with a rich blood supply [1,9], although any site of the central nervous system (CNS) can be involved, thus producing myriad of manifestations. Rare clinical presentations previously described in patients with neurocysticercosis include papillitis, isolated bilateral ptosis, cerebral hemorrhage, dorsal midbrain syndrome, paraplegia due to an intramedullary cyst, third-ventricular cyst, dystonia, and nominal aphasia masquerading as transient ischemic attacks [6]. Neuro-ophthalmological signs are usually caused by associated meningitis or hydrocephalus, and the recognition of these findings may provide a clue to the presence of serious neurologic disease. Hydrocephalus, found in 25% of NCC, is often accompanied by sixth or third cranial nerve palsy [3]. Third cranial nerve palsy, relatively rare in NCC, usually occurs either due to
N. Khurana et al. / Journal of the Neurological Sciences 312 (2012) 36–38
37
Fig. 1. Picture of the patient showing limited movements in adduction, supraduction, infraduction and normal abduction along with severe ptosis on the left side.
Fig. 2. Picture of the patient showing slight limitation in adduction on the left side and normal supraduction abduction.
brainstem compression by a supratentorial lesion or due to meningitis with secondary hydrocephalus. Isolated third cranial nerve palsy from a midbrain parenchymal lesion has rarely been reported [1–3]. Case reports showing isolated superior divisional oculomotor nerve involvement and fascicular involvement as a part of Weber's and Claude's syndrome have been described [4–8]. Table 1 summarizes cases presented with isolated third-nerve palsy caused by neurocysticercosis, diagnosed on the
basis of neuroimaging and immunological tests. All patients were men between 15 and 59 years of age. Out of five cases, two were treated with steroids alone, and the rest were treated with both steroids and anticysticidal therapy. All patients showed symptomatic improvement with resolution of lesion on neuroimaging. All cases reported in the literature with third-nerve involvement showed pupillary dilatation; whereas in our case, the pupils were normal in size and reaction (Table 1). The presentation in our case is probably related to the
Table 1 Table showing patients clinical finding neuroimaging and follow up clinical finding neuroimaging. Patient Positive clinical findings age/sex
Neuroimaging CT/MRI lesion
1
16/M
Ring lesion R side midbrain
2
34/M
3
59/M
4
15/M
5
25/M
Present case
22/M
Ref. no.
R—right, L—left.
R ptosis, weak supraduction, adduction, infraduction pupillary dilatation Complete ptosis L eye and partial ptosis R eye, limited to abduction and intortion on attempted down gaze, L pupillary dilatation L ptosis, and reduced supraduction, infraduction, and adduction, L dilated pupil R ptosis, limitation of dextroelevation on the R side, normal pupil R ptosis, limitation of dextroelevation on the R side, normal pupil L ptosis, and reduced supraduction, infraduction, and adduction, normal pupil
Treatment
Follow up findings Clinical
Neuroimaging
Resolution in 3 weeks
Not reported
Single ring enhancing lesion (11.6 × 9.7 × 11.11 mm) with perifocal edema at the tegmentum of the L midbrain Praziquantel 15 days Nodular lesions in the and intravenous midbrain tegmentum methylprednisolone and occipital pole, repeat steroid courses Two small cystic lesions in Steroids midbrain on the R side Ring lesion R side of midbrain Albendazole and steroids
Resolution in 6 days
CT 4 months normal
Ring lesion L side of midbrain Steroids
Resolution in 4 weeks
Albendazole dexamethasone Steroids
Recurrent exacerbation MRI 4 months lesion size reduced on steroids tapering resolution after 2 years Resolution in 7 days Not reported Resolution in 7 days
MRI at 2 months showed resolution of edema and contrast enhancement MRI 3 months lesion size reduced
38
N. Khurana et al. / Journal of the Neurological Sciences 312 (2012) 36–38
present with isolated pupil sparing third cranial nerve involvement; and, therefore, it should be included in the list of etiological diagnosis of pupil sparing third-nerve palsy. Conflict of interest No conflicts of interest. References
Fig. 3. Schematic diagram showing neurocysticercosis lesion in midbrain involving third-nerve fascicules.
involvement of the third-nerve fascicle within the midbrain before they exit the midbrain (Fig. 3). The fascicular portion of the oculomotor nerve courses ventrally from the nucleus in the dorsal midbrain tegmentum, passes through the red nucleus, and emerges from the medial aspect of the cerebral peduncle. Fascicular involvement can result in individual or multiple extraocular muscles involvement. It has been proposed that pupillary fibers are situated in the superomedial portion of the midbrain fascicular fibers; so, any lesion that is situated lower and lateral to these pupillary fibers in the midbrain can involve various ocular muscles, whereas the pupil may not be involved [10–12]. The case demonstrates that midbrain cysticercosis may
[1] Ranjith MP, Divya R, Sahni A. Isolated third cranial nerve palsy: a rare presentation of neurocysticercosis. Ir J Med Sci 2009, doi:10.1007/s11845-009-0308-6. [2] Mokta JK, Mahajan S, Machhan P, Mokta KK, Patial RK, Prashar BS. Recurrent oculomotor nerve palsy: a rare presentation of neurocysticercosis. Neurol India 2004;52:402. [3] Kim JS, Jeong SM, Moon SY, Park SH. Third cranial nerve palsy from midbrain neurocysticercosis: repeated exacerbation on tapering corticosteroids. J Neuroophthalmol 2004;24(3):217–20. [4] Meena MK, Khuteta A, Vashishtha R. Isolated superior division oculomotor palsy in neurocysticercosis: a rare presentation. Br J Ophthalmol 2010;94(7):954–5. [5] Chotmongkol V, Sawanyawisuth K, Limpawattana P, Phuphatham A, Chotmongkol R, Intapan PM. Superior divisional oculomotor nerve palsy caused by midbrain neurocysticercosis. Parasitol Int 2006;55(3):223–5. [6] Sawhney IM, Singh G, Lekhra OP, et al. Uncommon presentations of neurocysticercosis. J Neurol Sci 1998;54:94–100. [7] Song Tae-Jin, Sang Hyun Suh, Hanna Cho, Kyung-Yul Lee. Claude's syndrome associated with neurocysticercosis. Yonsei Med J 2010;51(6):978–9. [8] Singh NN, Verma R, Pankaj BK, Misra S. Neurocysticercosis presenting as Weber's syndrome. Neurol India 2003;53:551–2. [9] Del Bruto OH, Rajshekhar V, White Jr AC, et al. Proposed criteria for neurocysticercosis. Neurology 2001;57:177–83. [10] Castro O, Johnson LN, Mamourian AC. Isolated interior oblique paresis from brainstem infarction: perspective on oculomotor fascicular organization in the ventral midbrain tegmentum. Arch Neurol 1990;47:235–7. [11] Schwartz TH, Lycette CA, Kargman DE. Clinicoradiographic evidence for oculomotor fascicular anatomy. J Neurol Neurosurg Psychiatry 1995;59:338. [12] Breen LA, Hopf HC, Farris RK, Gutman L. Pupil-sparing oculomotor nerve palsy due to a midbrain infarction. Arch Neurol 1991;l48:105–6.