- Email: [email protected]
Bilateral trigeminal neuralgia associated with limited systemic sclerosis
708
Case Reports / Journal of Clinical Neuroscience 16 (2009) 708–710
Table 1 (continued) No. Age, Gender
Symptoms
CT image
MR image
Hydrocephalus Treatment
Outcome
Author
6
33, M
CN V, VII paresis cerebellar signs
Enhanced
( )
Subtotal resection, Chemo Tx
CR at 7 months No neurological deficits
Yoshida et al.8
7
16, F
IICP, cerebellar signs
n.d.
(+)
Subtotal resection, FB+WS RTx
CR at 7 years No neurological deficits
Yen et al.7
8
30, F
CN VI, IX, X, XII paresis, nystagmus
Slight high density homogeneously enhanced
( )
Subtotal resection, Chemo Tx, FB RTx
CR at 15 months Present CN VI, VII paresis case 1
9
24, M
IICP
Slight high density Homogeneously enhanced
TIWI-iso, T2WI-high signal Homogeneously enhanced TIWI-iso, T2WI-high signal Heterogeneously enhanced, cystic TIWI-low, T2WI-high signal Homogeneously enhanced TIWI-iso, T2WI-high signal Homogeneously enhanced
(+)
Partial resection, ChemoTx, FB RTx
CR at 10 months Present No neurological case 2 deficits
Footnotes to table: CN = cranial nerve, CR = complete remission, FB = focal brain, F = female, GK = gamma knife, IICP = increased intracranial pressure, M = male, n.d. = not described, RTx = radiotherapy, Tx = treatments, T1WI = T1-weighted image, T2WI = T2-weighted image WB = whole brain, WS = whole spine.
Appendix A. Supplementary data Supplementary data associated with this article can be found, in the online version, at doi:10.1016/j.jocn.2008.06.009. References 1. Matsutani M, Sano K, Takakura K, et al. Primary intracranial germ cell tumors: a clinical analysis of 153 histologically verified cases. J Neurosurg 1993;86:446–55. 2. Hashimoto M, Hatasa M, Shinoda S, et al. Medulla oblongata germinoma in association with Klinefelter syndrome. Surg Neurol 1992;37:384–7.
3. Israel Z, Lossos A, Ashkenazi E, et al. Germinoma and choroid plexus papilloma coexisting in the fourth ventricle. Acta Neurochir (Wien) 1996;138:123–5. 4. Nakajima H, Iwai Y, Yamanaka K, et al. Primary intracranial germinoma in the medulla oblongata. Surg Neurol 2000;53:448–51. 5. Sugiyama K, Uozumi T, Goishi J, et al. Germinoma of the medulla oblongata: case report. Neurol Med Chir (Tokyo) 1994;34:291–4. 6. Tashiro T, Yoshida J, Wakabayashi T, et al. Primary intracranial germinoma involving the medulla oblongata: case report. Neurol Med Chir (Tokyo) 1993;33:251–4. 7. Yen PS, Chou ASB, Chen CJ, et al. Primary medulla oblongata germinoma: a case report and review of the literature. J Neuro-Oncol 2003;62:339–42. 8. Yoshida K, Nakao Y, Yamamoto T, et al. Germinoma in the fourth ventricle. Acta Neurochir (Wien) 2003;145:789–92.
doi:10.1016/j.jocn.2008.06.009
Bilateral trigeminal neuralgia associated with limited systemic sclerosis Atta Mohyuddin a,*, Donald Myers b, Eric Guazzo c a b c
Birmingham Heartland Hospital, 35 Bordesley Green East, Bordesley Green, Birmingham B9 5SS, UK School of Medicine, University of California, San Francisco, Fresno, California, USA Department of Neurosurgery, Townsville Hospital, Townsville, Queensland, Australia
a r t i c l e
i n f o
Article history: Received 10 January 2008 Accepted 15 June 2008
a b s t r a c t A young patient with limited systemic sclerosis and medically resistant bilateral trigeminal neuralgia was successfully treated with microvascular decompression. To our knowledge, this is the first published report of this type of case. Ó 2008 Elsevier Ltd. All rights reserved.
Keywords: Bilateral trigeminal neuralgia Systemic sclerosis Microvascular decompression
1. Introduction Trigeminal neuralgia (TNG) is a painful unilateral neuralgia of the trigeminal nerve characterised by paroxysms of excruciating, lancinating spasms affecting one or more divisions of the nerve. * Corresponding author. Tel.: +44 791 3607282. E-mail address: [email protected] (A. Mohyuddin).
It occurs in about 5 people per 100,000 per year. TNG is more frequent in older age groups and women are more likely to suffer. TNG develops bilaterally in about 2% to 3% of patients with systemic disease.1 The pathophysiology of TNG is thought to be focal demyelination of the trigeminal nerve at a point close to the brain stem, where central myelination by oligodendroglial cells is replaced by myelination from Schwann cells. This demyelination can occasionally be caused by lesions such as meningiomas, sch-
Case Reports / Journal of Clinical Neuroscience 16 (2009) 708–710
wannomas, multiple sclerosis, or by vascular compression, usually by a loop of the superior cerebellar artery.2 Demyelination can also occur in patients with collagen vascular diseases, including systemic lupus erythematosus, rheumatoid arthritis, scleroderma, dermatomyositis and systemic sclerosis (SSc).3 We present a patient with bilateral trigeminal neuralgia (BTNG) that has been diagnosed with limited systemic sclerosis (lSSc). To our knowledge, this is the first published report of this type of case. 2. Case report A 34-year-old woman presented with a 1-year history of mild occasional left facial pain that was triggered by a cold breeze across her face. The pain was in both the maxillary (V2) and mandibular (V3) branches, was electric shock-like and fluctuant. Neurological examination was normal. The patient suffered from spondyloarthritis, multiple teleangiectasias of the mucosal lining of the stomach (watermelon stomach) and iron-deficiency anaemia. Her fingers were puffy with shiny hardened nails. There were multiple telengiectasias present on her face and arms. Nail-fold microscopy revealed multiple dilated capillary loops. MRI of the head was normal. Echocardiogram and pulmonary function, including diffusion lung CT scan, were normal. The patient’s liver function test (LFT), erythrocyte sedimentation rate (ESR) and rheumatoid factor (RF) antibody levels were normal. Anti-nuclear antibody (ANA) and antibodies to SCl-70 (associated with scleroderma) and double strand-DNA were normal, but the anti-centromere antibody reaction was strongly positive. The patient was diagnosed with lSSc. She did not respond to medical treatment for the TNG. We performed a left-sided retrosigmoid craniectomy and microvascular decompression (MVD). During this procedure we noted arterial compression of the nerve by a loop of the superior cerebellar artery (SCA) and microsurgically freed it. A Teflon sponge was interposed as described by Lovely and Janetta.4 Postoperatively, the patient had excellent symptom relief. Eight months later, the patient noted right-sided facial pain. The diagnosis was similar to her prior symptoms. Similarly, after failed conservative treatment, a right-sided MVD was performed about 1 year after her first operation. On this occasion 2 loops of the SCA were found to indent the trigeminal nerve near the root entry zone, which were removed in a similar fashion. Again an uncomplicated post-operative course ensued with symptom relief. However, 9 months later her TNG recurred on the right side, for which further re-exploration was performed. Post-operatively, the patient experienced relief without operative complications and remained free from any residual symptoms for almost 3 years. 3. Discussion The average age of onset of SSc occurs between 40 and 50 years. In women, however, the average age is in the later childbearing years, between 30 and 39 years. Fewer than 10% of patients develop SSc before 20 years of age.1–3 SSc is a condition characterized by widespread fibrosis of the skin and internal organs with damage to small blood vessels. The incidence is 2.6 to 28 per million people, with a male to female ratio of 1 to 3.3,5 SSc is usually separated into two fairly distinct classifications: limited systemic and diffuse cutaneous.5 The lSSc subset has been referred to as the CREST syndrome (calcinosis circumscripta, Raynaud’s phenomenon, esophageal sclerosis, sclerodactyly and telangiectasia). It is associated with a more benign course, a much better prognosis and a lower incidence of renal and restrictive pulmonary disease as compared to the diffuse type,6 although some
709
patients have gastric antral vascular ectasia (GAVE, or watermelon stomach). The link between SSc and trigeminal neuropathy has been investigated in previous studies. Farrell and Medsger looked at 442 cases of SSc and found an association with trigeminal neuropathy in 4% of cases.2 That study, together with 25 other documented cases, resulted in interest and speculation about a link between trigeminal neuropathy and SSc. Serum antibodies to ribonucleoprotein were found in 9 out of 20 (45%) SSc patients with trigeminal neuropathy, as compared to 25 out of 329 (8%) patients with SSc. Hagen et al. documented scleroderma in 15 out of 81 (19%) cases in patients with trigeminal sensory neuropathy, diagnosed on clinical grounds.7 These studies, combined with our case report, highlight an important debate about whether there is a causative link between SSc and trigeminal neuropathy or whether the presence of both conditions is coincidental. Further larger studies to investigate the link between SSc and trigeminal neuropathy need to be conducted. BTNG is an idiopathic condition with an incidence of 1% to 5%. BTNG is more common in females with an occasional occurrence of additional cranial nerve dysfunction.4 Tacconi and Miles found similar incidences and, in addition, that BTGN was more common in patients with SSc and multiple sclerosis (MS).6 In one study, TNG was present in 35 (1.9%) out of 1882 patients with MS and 14% of these TNG patients had bilateral symptoms.6 MRI with and without gadolinium, or a magnetic resonance angiogram (MRA), should be performed to exclude any structural or anatomical pathology.8 The initial treatment of TNG is almost always medical, once structural lesions such as a tumour or aneurysm have been excluded. Medications such as carbamazepine, phenytoin or gabapentin and misoprostol may be helpful.5 There are various other treatment options. Percutaneous radiofrequency rhizotomy (PRFC) can suppress pain but it can be associated with sensory loss and a 4% risk of post-operative corneal anaesthetic complications.10 Gamma knife radiosurgery (GKR) has been used for the treatment of TNG with encouraging results in patients who are not good candidates for surgery.9 Radionecrosis at the pontine root entry zone is one of the significant risks of this procedure.10 MVD is generally considered to be the procedure of choice for medically refractory TNG patients who have vascular compression, and who are generally young and fit for the anaesthesia. MVD has a high reported success rate of 80% to 90% in unilateral cases at 1-2 years follow-up and a very low rate of permanent neurological complications with a mortality rate of less than 0.5%.4,10 However, the role of BTGN has not yet been investigated in a larger study. We have described that MVD is an effective and safe option for younger patients diagnosed with scleroderma that is refractory to medical treatment, if the facilities and expertise are available.4,10
4. Conclusion If there is any suggestion of bilateral trigeminal symptoms, particularly in a younger person, further workup for the presence of systemic, collagen vascular, or demyelinating disease is important. In such a case, MVD is a good treatment option if the symptoms recur.7 References 1. Mazoni GC, Torelli P. Epidemiology of typical and atypical craniofacial neuralgias. Neurol Sci 2005;26:65–7. 2. Farrell DA, Medsger TA. Trigeminal neuropathy in progressive systemic sclerosis. Am J Med 1982;73:57–62.
710
Case Reports / Journal of Clinical Neuroscience 16 (2009) 710–711
3. Steen VD, Medsger Jr TA. Epidemiology and natural history of systemic sclerosis. Rheum Dis Clin N Am 1990;16:1–10. 4. Lovely TJ, Janetta PJ. Microvascular decompression for trigeminal neuralgia. Surgical technique and long term results. Neurosurg Clin N Am 1997;8:11–29. 5. Brisman R. Trigeminal neuralgia and multiple sclerosis. Arch Neurol 1987;44:379–81. 6. Tacconi L, Miles JB. Bilateral trigeminal neuralgia: a therapeutic dilemma. Br J Neurosurg 2000;14:33–9. 7. Hagen NA, Stevens JC, Michet Jr CJ. Trigeminal sensory neuropathy associated with connective tissue diseases. Neurology 1990;40:891–6.
8. Taha JM, Tew Jr JM, Buncher CR. A prospective 15 year follow up of 154 consecutive patients with trigeminal neuralgia treated by percutaneous stereotactic radiofrequency thermal rhizotomy. J Neurosurg 1995;83:989–93. 9. Kondziolka D, Lunsford LD, Habeck M, et al. Gamma knife radiosurgery for trigeminal neuraligia. Neurosurg Clin N Am 1997;8:79–85. 10. Pollack IF, Janetta PJ, Bissonette DJ. Bilateral trigeminal neuralgia: a 14 year experience with microvascular decompression. J Neurosurg 1988;68: 559–65.
doi:10.1016/j.jocn.2008.06.024
Gamma knife radiosurgery in jugular foramen endolymphatic sac adenocarcinoma Srikant Balasubramaniam a, Ramesh B. Deshpande b, Basant K. Misra c,* a
Department of Neurosurgery, PD Hinduja National Hospital and Medical Research Centre, Mahim, Mumbai, India Department of Pathology, PD Hinduja National Hospital and Medical Research Centre, Mahim, Mumbai, India c Department of Neurosurgery & Gamma Knife Radiosurgery, PD Hinduja National Hospital and Medical Research Centre, Veer Savarkar Marg, Mahim, Mumbai 400016, India b
a r t i c l e
i n f o
Article history: Received 26 June 2008 Accepted 24 July 2008
Keywords: Endolymphatic sac tumor Gamma knife radiosurgery Jugular foramen adenocarcinoma
a b s t r a c t A 41-year-old male presented to us with a history of right-sided temporal headache and ear discharge. MRI revealed an extra-axial space-occupying lesion in the region of the right jugular foramen. The patient was operated upon and a radical excision of the tumor was performed. Histopathology revealed an adenocarcinoma, probably of endolymphatic sac origin. The patient had a recurrence of tumor at 2-year follow-up and was subjected to gamma knife radiosurgery. The patient was subsequently asymptomatic at a 2.5-year follow-up and imaging revealed regression of the tumor size. Stereotactic radiosurgery in recurrent endolymphatic sac tumors involving the jugular foramen has not been reported previously. We review the literature on this novel treatment protocol for this rare skull base tumor. Ó 2008 Elsevier Ltd. All rights reserved.
1. Introduction Endolymphatic sac tumors are rare intracranial tumors.1,2 They extend uncommonly to the jugular foramen. Radical excision has been described as the treatment of choice for these tumors.2 Recurrent tumors pose a difficult problem for determining management protocols. Gamma knife radiosurgery (GKR) is a newer option to treat such recurrent tumors. Very few reports are available on the long-term effect of GKR on these tumors.1 We report on one patient with a 3-year follow-up. 2. Case report A 41-year-old healthy male, presented to us in 2003 with a history of right-sided temporal headache (4–5 months) and rightsided ear discharge (2 months). Neurological examination revealed no deficits. MRI showed an extra-axial space-occupying lesion in the region of jugular foramen that was hyperintense on T1weighted MRI, hypointense on T2-weighted MRI and enhancing on contrast administration (Fig. 1). The pre-operative diagnosis was a glomus jugulare tumor. The patient was operated upon by the retrosigmoid approach and a radical excision was performed. The tumor was soft and quite vascular. Post operatively, the patient had no deficits and imaging revealed no residual tumor. Histopathology revealed a tubulopapillary low-grade epithelial tumor. The tubules and papillae were lined by a single layer of cuboidal epithelial cells. Nuclear atypia or pleomorphism was
* Corresponding author. Tel.: +91 9821092142; fax: +91 22 24447220. E-mail address: [email protected] (B.K. Misra).
notably absent. This was suggestive of a low grade cystic adenocarcinoma probably of endolymphatic sac origin (Fig. 2). On regular follow-up he was asymptomatic. After 2 years he had a recurrence of symptoms and MRI revealed a recurrent tumor. He was treated by GKR. The tumor volume was 7.4 cm. A prescription dose of 15 Gy was given to 50.00% with a confirmative index of 1.4. Twenty shots were administered using 3 collimators (8, 14 and 18 mm). The cochlea received less than 10 Gy of radiation. The patient’s symptoms progressively improved and he was asymptomatic after 1 year. MRI performed 2.5 years later showed a reduction in tumor size (Fig. 3). He continues to be clinically asymptomatic with no neurologic deficits at his 3-year follow-up. 3. Discussion Paragangliomas, schwannomas and meningiomas are the common tumors seen in the jugular foramen.3,4 Adenocarcinomas in the jugular foramen are rare tumors.1–3 These tumors are usually metastatic, arising from the prostate, breast, kidney or lung.4 Primary adenocarcinomas of the jugular foramen are usually of endolymphatic sac origin. A differential diagnosis to these lesions is ceruminous gland adenocarcinoma.5 Adenocarcinomas of the endolymphatic sac (also known as Heffner’s tumors) are low grade cystic adenocarcinomas arising from the pars rugosa of the endolymphatic sac.1 They grow in one of two directions: either laterally towards the middle/external ear and jugular foramen or medially in the direction of the cerebellopontine angle.6 They are commonly associated with Von HippelLindau disease. Bambakidis et al., in their study of endolymphatic sac tumors, have concluded that patients without Von HippelLindau disease are generally older and the sexes are equally represented.7 Tumors in these patients are of a higher grade and are usually unilateral.7
Case Reports / Journal of Clinical Neuroscience 16 (2009) 708–710
Table 1 (continued) No. Age, Gender
Symptoms
CT image
MR image
Hydrocephalus Treatment
Outcome
Author
6
33, M
CN V, VII paresis cerebellar signs
Enhanced
( )
Subtotal resection, Chemo Tx
CR at 7 months No neurological deficits
Yoshida et al.8
7
16, F
IICP, cerebellar signs
n.d.
(+)
Subtotal resection, FB+WS RTx
CR at 7 years No neurological deficits
Yen et al.7
8
30, F
CN VI, IX, X, XII paresis, nystagmus
Slight high density homogeneously enhanced
( )
Subtotal resection, Chemo Tx, FB RTx
CR at 15 months Present CN VI, VII paresis case 1
9
24, M
IICP
Slight high density Homogeneously enhanced
TIWI-iso, T2WI-high signal Homogeneously enhanced TIWI-iso, T2WI-high signal Heterogeneously enhanced, cystic TIWI-low, T2WI-high signal Homogeneously enhanced TIWI-iso, T2WI-high signal Homogeneously enhanced
(+)
Partial resection, ChemoTx, FB RTx
CR at 10 months Present No neurological case 2 deficits
Footnotes to table: CN = cranial nerve, CR = complete remission, FB = focal brain, F = female, GK = gamma knife, IICP = increased intracranial pressure, M = male, n.d. = not described, RTx = radiotherapy, Tx = treatments, T1WI = T1-weighted image, T2WI = T2-weighted image WB = whole brain, WS = whole spine.
Appendix A. Supplementary data Supplementary data associated with this article can be found, in the online version, at doi:10.1016/j.jocn.2008.06.009. References 1. Matsutani M, Sano K, Takakura K, et al. Primary intracranial germ cell tumors: a clinical analysis of 153 histologically verified cases. J Neurosurg 1993;86:446–55. 2. Hashimoto M, Hatasa M, Shinoda S, et al. Medulla oblongata germinoma in association with Klinefelter syndrome. Surg Neurol 1992;37:384–7.
3. Israel Z, Lossos A, Ashkenazi E, et al. Germinoma and choroid plexus papilloma coexisting in the fourth ventricle. Acta Neurochir (Wien) 1996;138:123–5. 4. Nakajima H, Iwai Y, Yamanaka K, et al. Primary intracranial germinoma in the medulla oblongata. Surg Neurol 2000;53:448–51. 5. Sugiyama K, Uozumi T, Goishi J, et al. Germinoma of the medulla oblongata: case report. Neurol Med Chir (Tokyo) 1994;34:291–4. 6. Tashiro T, Yoshida J, Wakabayashi T, et al. Primary intracranial germinoma involving the medulla oblongata: case report. Neurol Med Chir (Tokyo) 1993;33:251–4. 7. Yen PS, Chou ASB, Chen CJ, et al. Primary medulla oblongata germinoma: a case report and review of the literature. J Neuro-Oncol 2003;62:339–42. 8. Yoshida K, Nakao Y, Yamamoto T, et al. Germinoma in the fourth ventricle. Acta Neurochir (Wien) 2003;145:789–92.
doi:10.1016/j.jocn.2008.06.009
Bilateral trigeminal neuralgia associated with limited systemic sclerosis Atta Mohyuddin a,*, Donald Myers b, Eric Guazzo c a b c
Birmingham Heartland Hospital, 35 Bordesley Green East, Bordesley Green, Birmingham B9 5SS, UK School of Medicine, University of California, San Francisco, Fresno, California, USA Department of Neurosurgery, Townsville Hospital, Townsville, Queensland, Australia
a r t i c l e
i n f o
Article history: Received 10 January 2008 Accepted 15 June 2008
a b s t r a c t A young patient with limited systemic sclerosis and medically resistant bilateral trigeminal neuralgia was successfully treated with microvascular decompression. To our knowledge, this is the first published report of this type of case. Ó 2008 Elsevier Ltd. All rights reserved.
Keywords: Bilateral trigeminal neuralgia Systemic sclerosis Microvascular decompression
1. Introduction Trigeminal neuralgia (TNG) is a painful unilateral neuralgia of the trigeminal nerve characterised by paroxysms of excruciating, lancinating spasms affecting one or more divisions of the nerve. * Corresponding author. Tel.: +44 791 3607282. E-mail address: [email protected] (A. Mohyuddin).
It occurs in about 5 people per 100,000 per year. TNG is more frequent in older age groups and women are more likely to suffer. TNG develops bilaterally in about 2% to 3% of patients with systemic disease.1 The pathophysiology of TNG is thought to be focal demyelination of the trigeminal nerve at a point close to the brain stem, where central myelination by oligodendroglial cells is replaced by myelination from Schwann cells. This demyelination can occasionally be caused by lesions such as meningiomas, sch-
Case Reports / Journal of Clinical Neuroscience 16 (2009) 708–710
wannomas, multiple sclerosis, or by vascular compression, usually by a loop of the superior cerebellar artery.2 Demyelination can also occur in patients with collagen vascular diseases, including systemic lupus erythematosus, rheumatoid arthritis, scleroderma, dermatomyositis and systemic sclerosis (SSc).3 We present a patient with bilateral trigeminal neuralgia (BTNG) that has been diagnosed with limited systemic sclerosis (lSSc). To our knowledge, this is the first published report of this type of case. 2. Case report A 34-year-old woman presented with a 1-year history of mild occasional left facial pain that was triggered by a cold breeze across her face. The pain was in both the maxillary (V2) and mandibular (V3) branches, was electric shock-like and fluctuant. Neurological examination was normal. The patient suffered from spondyloarthritis, multiple teleangiectasias of the mucosal lining of the stomach (watermelon stomach) and iron-deficiency anaemia. Her fingers were puffy with shiny hardened nails. There were multiple telengiectasias present on her face and arms. Nail-fold microscopy revealed multiple dilated capillary loops. MRI of the head was normal. Echocardiogram and pulmonary function, including diffusion lung CT scan, were normal. The patient’s liver function test (LFT), erythrocyte sedimentation rate (ESR) and rheumatoid factor (RF) antibody levels were normal. Anti-nuclear antibody (ANA) and antibodies to SCl-70 (associated with scleroderma) and double strand-DNA were normal, but the anti-centromere antibody reaction was strongly positive. The patient was diagnosed with lSSc. She did not respond to medical treatment for the TNG. We performed a left-sided retrosigmoid craniectomy and microvascular decompression (MVD). During this procedure we noted arterial compression of the nerve by a loop of the superior cerebellar artery (SCA) and microsurgically freed it. A Teflon sponge was interposed as described by Lovely and Janetta.4 Postoperatively, the patient had excellent symptom relief. Eight months later, the patient noted right-sided facial pain. The diagnosis was similar to her prior symptoms. Similarly, after failed conservative treatment, a right-sided MVD was performed about 1 year after her first operation. On this occasion 2 loops of the SCA were found to indent the trigeminal nerve near the root entry zone, which were removed in a similar fashion. Again an uncomplicated post-operative course ensued with symptom relief. However, 9 months later her TNG recurred on the right side, for which further re-exploration was performed. Post-operatively, the patient experienced relief without operative complications and remained free from any residual symptoms for almost 3 years. 3. Discussion The average age of onset of SSc occurs between 40 and 50 years. In women, however, the average age is in the later childbearing years, between 30 and 39 years. Fewer than 10% of patients develop SSc before 20 years of age.1–3 SSc is a condition characterized by widespread fibrosis of the skin and internal organs with damage to small blood vessels. The incidence is 2.6 to 28 per million people, with a male to female ratio of 1 to 3.3,5 SSc is usually separated into two fairly distinct classifications: limited systemic and diffuse cutaneous.5 The lSSc subset has been referred to as the CREST syndrome (calcinosis circumscripta, Raynaud’s phenomenon, esophageal sclerosis, sclerodactyly and telangiectasia). It is associated with a more benign course, a much better prognosis and a lower incidence of renal and restrictive pulmonary disease as compared to the diffuse type,6 although some
709
patients have gastric antral vascular ectasia (GAVE, or watermelon stomach). The link between SSc and trigeminal neuropathy has been investigated in previous studies. Farrell and Medsger looked at 442 cases of SSc and found an association with trigeminal neuropathy in 4% of cases.2 That study, together with 25 other documented cases, resulted in interest and speculation about a link between trigeminal neuropathy and SSc. Serum antibodies to ribonucleoprotein were found in 9 out of 20 (45%) SSc patients with trigeminal neuropathy, as compared to 25 out of 329 (8%) patients with SSc. Hagen et al. documented scleroderma in 15 out of 81 (19%) cases in patients with trigeminal sensory neuropathy, diagnosed on clinical grounds.7 These studies, combined with our case report, highlight an important debate about whether there is a causative link between SSc and trigeminal neuropathy or whether the presence of both conditions is coincidental. Further larger studies to investigate the link between SSc and trigeminal neuropathy need to be conducted. BTNG is an idiopathic condition with an incidence of 1% to 5%. BTNG is more common in females with an occasional occurrence of additional cranial nerve dysfunction.4 Tacconi and Miles found similar incidences and, in addition, that BTGN was more common in patients with SSc and multiple sclerosis (MS).6 In one study, TNG was present in 35 (1.9%) out of 1882 patients with MS and 14% of these TNG patients had bilateral symptoms.6 MRI with and without gadolinium, or a magnetic resonance angiogram (MRA), should be performed to exclude any structural or anatomical pathology.8 The initial treatment of TNG is almost always medical, once structural lesions such as a tumour or aneurysm have been excluded. Medications such as carbamazepine, phenytoin or gabapentin and misoprostol may be helpful.5 There are various other treatment options. Percutaneous radiofrequency rhizotomy (PRFC) can suppress pain but it can be associated with sensory loss and a 4% risk of post-operative corneal anaesthetic complications.10 Gamma knife radiosurgery (GKR) has been used for the treatment of TNG with encouraging results in patients who are not good candidates for surgery.9 Radionecrosis at the pontine root entry zone is one of the significant risks of this procedure.10 MVD is generally considered to be the procedure of choice for medically refractory TNG patients who have vascular compression, and who are generally young and fit for the anaesthesia. MVD has a high reported success rate of 80% to 90% in unilateral cases at 1-2 years follow-up and a very low rate of permanent neurological complications with a mortality rate of less than 0.5%.4,10 However, the role of BTGN has not yet been investigated in a larger study. We have described that MVD is an effective and safe option for younger patients diagnosed with scleroderma that is refractory to medical treatment, if the facilities and expertise are available.4,10
4. Conclusion If there is any suggestion of bilateral trigeminal symptoms, particularly in a younger person, further workup for the presence of systemic, collagen vascular, or demyelinating disease is important. In such a case, MVD is a good treatment option if the symptoms recur.7 References 1. Mazoni GC, Torelli P. Epidemiology of typical and atypical craniofacial neuralgias. Neurol Sci 2005;26:65–7. 2. Farrell DA, Medsger TA. Trigeminal neuropathy in progressive systemic sclerosis. Am J Med 1982;73:57–62.
710
Case Reports / Journal of Clinical Neuroscience 16 (2009) 710–711
3. Steen VD, Medsger Jr TA. Epidemiology and natural history of systemic sclerosis. Rheum Dis Clin N Am 1990;16:1–10. 4. Lovely TJ, Janetta PJ. Microvascular decompression for trigeminal neuralgia. Surgical technique and long term results. Neurosurg Clin N Am 1997;8:11–29. 5. Brisman R. Trigeminal neuralgia and multiple sclerosis. Arch Neurol 1987;44:379–81. 6. Tacconi L, Miles JB. Bilateral trigeminal neuralgia: a therapeutic dilemma. Br J Neurosurg 2000;14:33–9. 7. Hagen NA, Stevens JC, Michet Jr CJ. Trigeminal sensory neuropathy associated with connective tissue diseases. Neurology 1990;40:891–6.
8. Taha JM, Tew Jr JM, Buncher CR. A prospective 15 year follow up of 154 consecutive patients with trigeminal neuralgia treated by percutaneous stereotactic radiofrequency thermal rhizotomy. J Neurosurg 1995;83:989–93. 9. Kondziolka D, Lunsford LD, Habeck M, et al. Gamma knife radiosurgery for trigeminal neuraligia. Neurosurg Clin N Am 1997;8:79–85. 10. Pollack IF, Janetta PJ, Bissonette DJ. Bilateral trigeminal neuralgia: a 14 year experience with microvascular decompression. J Neurosurg 1988;68: 559–65.
doi:10.1016/j.jocn.2008.06.024
Gamma knife radiosurgery in jugular foramen endolymphatic sac adenocarcinoma Srikant Balasubramaniam a, Ramesh B. Deshpande b, Basant K. Misra c,* a
Department of Neurosurgery, PD Hinduja National Hospital and Medical Research Centre, Mahim, Mumbai, India Department of Pathology, PD Hinduja National Hospital and Medical Research Centre, Mahim, Mumbai, India c Department of Neurosurgery & Gamma Knife Radiosurgery, PD Hinduja National Hospital and Medical Research Centre, Veer Savarkar Marg, Mahim, Mumbai 400016, India b
a r t i c l e
i n f o
Article history: Received 26 June 2008 Accepted 24 July 2008
Keywords: Endolymphatic sac tumor Gamma knife radiosurgery Jugular foramen adenocarcinoma
a b s t r a c t A 41-year-old male presented to us with a history of right-sided temporal headache and ear discharge. MRI revealed an extra-axial space-occupying lesion in the region of the right jugular foramen. The patient was operated upon and a radical excision of the tumor was performed. Histopathology revealed an adenocarcinoma, probably of endolymphatic sac origin. The patient had a recurrence of tumor at 2-year follow-up and was subjected to gamma knife radiosurgery. The patient was subsequently asymptomatic at a 2.5-year follow-up and imaging revealed regression of the tumor size. Stereotactic radiosurgery in recurrent endolymphatic sac tumors involving the jugular foramen has not been reported previously. We review the literature on this novel treatment protocol for this rare skull base tumor. Ó 2008 Elsevier Ltd. All rights reserved.
1. Introduction Endolymphatic sac tumors are rare intracranial tumors.1,2 They extend uncommonly to the jugular foramen. Radical excision has been described as the treatment of choice for these tumors.2 Recurrent tumors pose a difficult problem for determining management protocols. Gamma knife radiosurgery (GKR) is a newer option to treat such recurrent tumors. Very few reports are available on the long-term effect of GKR on these tumors.1 We report on one patient with a 3-year follow-up. 2. Case report A 41-year-old healthy male, presented to us in 2003 with a history of right-sided temporal headache (4–5 months) and rightsided ear discharge (2 months). Neurological examination revealed no deficits. MRI showed an extra-axial space-occupying lesion in the region of jugular foramen that was hyperintense on T1weighted MRI, hypointense on T2-weighted MRI and enhancing on contrast administration (Fig. 1). The pre-operative diagnosis was a glomus jugulare tumor. The patient was operated upon by the retrosigmoid approach and a radical excision was performed. The tumor was soft and quite vascular. Post operatively, the patient had no deficits and imaging revealed no residual tumor. Histopathology revealed a tubulopapillary low-grade epithelial tumor. The tubules and papillae were lined by a single layer of cuboidal epithelial cells. Nuclear atypia or pleomorphism was
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notably absent. This was suggestive of a low grade cystic adenocarcinoma probably of endolymphatic sac origin (Fig. 2). On regular follow-up he was asymptomatic. After 2 years he had a recurrence of symptoms and MRI revealed a recurrent tumor. He was treated by GKR. The tumor volume was 7.4 cm. A prescription dose of 15 Gy was given to 50.00% with a confirmative index of 1.4. Twenty shots were administered using 3 collimators (8, 14 and 18 mm). The cochlea received less than 10 Gy of radiation. The patient’s symptoms progressively improved and he was asymptomatic after 1 year. MRI performed 2.5 years later showed a reduction in tumor size (Fig. 3). He continues to be clinically asymptomatic with no neurologic deficits at his 3-year follow-up. 3. Discussion Paragangliomas, schwannomas and meningiomas are the common tumors seen in the jugular foramen.3,4 Adenocarcinomas in the jugular foramen are rare tumors.1–3 These tumors are usually metastatic, arising from the prostate, breast, kidney or lung.4 Primary adenocarcinomas of the jugular foramen are usually of endolymphatic sac origin. A differential diagnosis to these lesions is ceruminous gland adenocarcinoma.5 Adenocarcinomas of the endolymphatic sac (also known as Heffner’s tumors) are low grade cystic adenocarcinomas arising from the pars rugosa of the endolymphatic sac.1 They grow in one of two directions: either laterally towards the middle/external ear and jugular foramen or medially in the direction of the cerebellopontine angle.6 They are commonly associated with Von HippelLindau disease. Bambakidis et al., in their study of endolymphatic sac tumors, have concluded that patients without Von HippelLindau disease are generally older and the sexes are equally represented.7 Tumors in these patients are of a higher grade and are usually unilateral.7