- Email: [email protected]
Short-lasting unilateral neuralgiform headache with conjunctival injection and tearing caused by a pituitary adenoma
1180
Case Reports / Journal of Clinical Neuroscience 20 (2013) 1180–1181
5. Maurya RP, Mishra D, Bhushan P, et al. Orbital Myiasis: Due to Invasion of Larvae of Flesh Fly (Wohlfahrtia magnifica) in a Child; rare presentation. Case Rep Ophthalmol Med 2012;2012:371498. 6. Terterov S, Taghva A, MacDougall M, et al. Posttraumatic human cerebral myiasis. World Neurosurg 2010;73:557–9. 7. Mumcuoglu KY. Clinical applications for maggots in wound care. Am J Clin Dermatol 2001;2:219–27. 8. Antunes AA. Santos Tde S, Avelar RL, Martins Neto EC, Macedo Neres B, Laureano Filho JR: Oral and maxillofacial myiasis: a case series and literature review. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2011;112:e81–5.
9. Cestari TF, Pessato S, Ramos-e-Silva M. Tungiasis and myiasis. Clin Dermatol 2007;25:158–64. 10. Victoria J, Trujillo R, Barreto M. Myiasis: a successful treatment with topical ivermectin. Int J Dermatol 1999;38:142–4. 11. Blejter J. Tracheostomy wound myiasis in a child: case report and review of the literature. Case Rep Pediatr 2012;2012:317862. 12. Kumar SL, Manuel S, John TV, et al. Extensive gingival myiasis - Diagnosis, treatment, and prevention. J Oral Maxillofac Pathol 2011;15:340–3.
http://dx.doi.org/10.1016/j.jocn.2012.10.014
Short-lasting unilateral neuralgiform headache with conjunctival injection and tearing caused by a pituitary adenoma T.D. Musuka a,⇑, R.H. Edis b, A.G. Kermode a a b
Department of Neurology, Sir Charles Gairdner Hospital, Hospital Avenue, Nedlands, WA 6008, Australia Department of Neurology, Royal Perth Hospital, Perth, Western Australia, Australia
a r t i c l e
i n f o
Article history: Received 26 March 2012 Accepted 31 October 2012
Keywords: Head pain SUNCT Trigeminal autonomic cephalalgias
a b s t r a c t Short-lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT) syndrome is a rare primary headache syndrome first described in 1978. We report on a 43-year-old man with a 10 year history of SUNCT in whom a pituitary macroadenoma was eventually detected. His pain rapidly improved with medical treatment of the prolactinoma and we propose that this is a case of symptomatic SUNCT. Crown Copyright Ó 2012 Published by Elsevier Ltd. All rights reserved.
1. Introduction Short-lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT) syndrome is defined as a rare primary headache first described in 1978.1 The characteristics of the syndrome are ultra-short paroxysms (lasting seconds) of pain in the first division of the trigeminal nerve accompanied by conjunctival injection and tearing. Based on the classical presentation of unilateral head pain and activation of cranial autonomic efferents, it has been classified together with cluster headache and paroxysmal hemicrania as the trigeminal autonomic cephalalgias.2–4 At the time of this classification by the International Headache Society, there was no known cause for SUNCT, hence its classification as a primary headache. However, as this entity is becoming more recognised, there have been case reports where SUNCT has been secondary to a structural cause.
2. Case report A 43-year-old previously healthy man had been afflicted by multiple daily episodes of brief left-sided periorbital sharp, stabbing pain of varying severity for more than 10 years. The episodes had become more problematic and resulted in this current presentation. The episodes lasted about 10 seconds and had become more debilitating because of the severity, frequency of attacks and associated severe brief lethargy lasting about 10 minutes after the pain subsided. Neck movement triggered some episodes and others had ⇑ Corresponding author. Tel.: +61 8 9346 3088 (Home). E-mail address: [email protected] (T.D. Musuka).
no apparent trigger. Some episodes woke him from sleep. The head pain was instantly associated with tearing of the left eye and rhinorrhoea. The frequency of attacks was extremely variable, from 1 per day during ‘‘good’’ weeks to more than 10 per day during ‘‘bad’’ weeks. His past medical history was unremarkable. He had seen a neurologist about 10 years prior when the diagnosis of SUNCT had been made. He had undergone a trial of sodium valproate with no success. MRI scan of the head with contrast was normal. On current presentation, he had no systemic symptoms. Clinical neurologic examination was normal and specifically, ocular examination including confrontation visual fields and colour vision was normal. A repeat MRI scan of the head showed a 15 mm left sellar mass with extension into the cavernous sinus and suprasellar cistern. Biochemical assessment revealed a markedly elevated prolactin concentration of more than 12,000 mIU/L, consistent with a prolactin-secreting pituitary macroadenoma. Other anterior pituitary hormone levels were within normal ranges. Further questioning revealed that his libido had decreased in the 12 months prior to presentation. He, however, denied erectile dysfunction, galactorrhoea, gynaecomastia or hot flushes. He was started on a dopamine agonist with excellent response in his serum prolactin. His headaches also improved dramatically after only a few weeks. Eighteen months after starting the dopamine agonist, he is headache free. Serum prolactin remains elevated at 1600 mU/L. A repeat MRI scan (Fig. 1) shows stable appearances of the pituitary mass. A surgical option was explored but was deemed to be unnecessary at this stage as the prolactinoma was predominantly in the sella, there was no visual compromise, and there had been good response to medical therapy. A conservative approach was adopted with a plan for follow-up MRI.
Case Reports / Journal of Clinical Neuroscience 20 (2013) 1181–1183
1181
complete resolution of the pain when the pituitary lesion was treated. The others’ symptoms resolved almost completely with treatment of the prolactinoma. In the patients described the headache symptoms pre-dated the pituitary manifestations by 3 to 10 years; they noted that the headaches may continue after the treatment of the pituitary tumour. This coupled with the fact that the headache symptoms may occur with microprolactinomas argues against a compressive aetiology for the head pain. Matharu et al.5 raised the possibility of a neurohormonal cause for SUNCT in their case report. Our patient had symptoms for about 10 years with the initial MRI being normal. Moreover, on direct questioning, symptoms of reduced libido likely due to the elevated prolactin level had only been present for 1 year. Whether a pituitary microadenoma was present 10 years before, but was too small to detect on MRI, is a moot point.
4. Conclusion
Fig. 1. Sagittal T1-weighted MRI showing stable appearance of the pituitary mass.
3. Discussion
When the history is suggestive of SUNCT, it is worth searching for a structural cause, especially pituitary lesions, either macro or microadenomas. The headache symptoms may precede detection of pituitary lesions by up to 10 years and repeat clinical and MRI surveillance over time is warranted. Our patient is likely an example of this. References
SUNCT is considered to be a rare primary headache disorder whose hallmarks are recurrent bouts of unilateral head pain accompanied by autonomic features.2–4 The prominent autonomic features and lack of response to carbamazepine serve to distinguish this disorder from trigeminal neuralgia. Most cases of SUNCT have no known cause.2–4 There are increasing numbers of published patient reports of potential causes of this syndrome and therefore it is not always a primary headache disorder. Cohen described 52 patients with SUNCT2, which included three patients with SUNCT associated with pituitary lesions, two macroadenomas and one microadenoma. One patient with a macroadenoma had
1. Sjaastad O, Russell D, Horven I, et al. Multiple neuralgiform unilateral headache attacks associated with conjunctival injection and appearing in clusters. A nosological problem. In: Proceedings of the Scandinavian Migraine Society. Arhus; 1978. p. 31l. 2. Cohen AS. Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing. Cephalalgia 2007;27:824–32. 3. Leone M, Bussone G. Pathophysiology of trigeminal autonomic cephalalgias. Lancet Neurol 2009;8:755–64. 4. International Headache Society Classification ICHD-II, Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT). http://ihsclassification.org/en/02_klassifikation/02_teil1/03.03.00_cluster.html. Accessed 22 January 2012. 5. Matharu M, Levy M, Merry R, et al. SUNCT syndrome secondary to prolactinoma. J Neurol Neurosurg Psychiatry 2003;74.
http://dx.doi.org/10.1016/j.jocn.2012.10.014
Leptomeningeal metastasis of an intradural malignant peripheral nerve sheath tumor Andreas M. Stark ⇑, H. Maximilian Mehdorn Klinik für Neurochirurgie, Universitätsklinikum Schleswig-Holstein, Campus Kiel, Arnold-Heller-Str. 2, Haus 41, 24105 Kiel, Germany
a r t i c l e
i n f o
Article history: Received 3 September 2012 Accepted 8 September 2012
Keywords: Leptomeningeal Malignant peripheral nerve sheath tumor Metastases MPNST
a b s t r a c t Malignant peripheral nerve sheath tumors (MPNST) are defined as any malignant tumor arising from or differentiating towards the peripheral nerve sheath. Intradural MPNST metastases are very rare. We report, to our knowledge, the first case of leptomeningeal metastasis of a MPNST to the spine and intracranial space. A 56-year-old woman with primary intradural MPNST of the S1 nerve root developed leptomeningeal metastases as well as brain metastases 19 months after diagnosis. The patient had a history of non-Hodgkins lymphoma for which she had received irradiation to the spine 15 years prior to this presentation. She had no stigmata of neurofibromatosis type 1. Patients with MPNST may also develop leptomeningeal metastases as demonstrated in this patient with intradural post-radiation MPNST. Ó 2013 Elsevier Ltd. All rights reserved.
⇑ Corresponding author. Tel.: +49 0431 597 8550; fax: +49 0431 597 4918. E-mail address: [email protected] (A.M. Stark).
Case Reports / Journal of Clinical Neuroscience 20 (2013) 1180–1181
5. Maurya RP, Mishra D, Bhushan P, et al. Orbital Myiasis: Due to Invasion of Larvae of Flesh Fly (Wohlfahrtia magnifica) in a Child; rare presentation. Case Rep Ophthalmol Med 2012;2012:371498. 6. Terterov S, Taghva A, MacDougall M, et al. Posttraumatic human cerebral myiasis. World Neurosurg 2010;73:557–9. 7. Mumcuoglu KY. Clinical applications for maggots in wound care. Am J Clin Dermatol 2001;2:219–27. 8. Antunes AA. Santos Tde S, Avelar RL, Martins Neto EC, Macedo Neres B, Laureano Filho JR: Oral and maxillofacial myiasis: a case series and literature review. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2011;112:e81–5.
9. Cestari TF, Pessato S, Ramos-e-Silva M. Tungiasis and myiasis. Clin Dermatol 2007;25:158–64. 10. Victoria J, Trujillo R, Barreto M. Myiasis: a successful treatment with topical ivermectin. Int J Dermatol 1999;38:142–4. 11. Blejter J. Tracheostomy wound myiasis in a child: case report and review of the literature. Case Rep Pediatr 2012;2012:317862. 12. Kumar SL, Manuel S, John TV, et al. Extensive gingival myiasis - Diagnosis, treatment, and prevention. J Oral Maxillofac Pathol 2011;15:340–3.
http://dx.doi.org/10.1016/j.jocn.2012.10.014
Short-lasting unilateral neuralgiform headache with conjunctival injection and tearing caused by a pituitary adenoma T.D. Musuka a,⇑, R.H. Edis b, A.G. Kermode a a b
Department of Neurology, Sir Charles Gairdner Hospital, Hospital Avenue, Nedlands, WA 6008, Australia Department of Neurology, Royal Perth Hospital, Perth, Western Australia, Australia
a r t i c l e
i n f o
Article history: Received 26 March 2012 Accepted 31 October 2012
Keywords: Head pain SUNCT Trigeminal autonomic cephalalgias
a b s t r a c t Short-lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT) syndrome is a rare primary headache syndrome first described in 1978. We report on a 43-year-old man with a 10 year history of SUNCT in whom a pituitary macroadenoma was eventually detected. His pain rapidly improved with medical treatment of the prolactinoma and we propose that this is a case of symptomatic SUNCT. Crown Copyright Ó 2012 Published by Elsevier Ltd. All rights reserved.
1. Introduction Short-lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT) syndrome is defined as a rare primary headache first described in 1978.1 The characteristics of the syndrome are ultra-short paroxysms (lasting seconds) of pain in the first division of the trigeminal nerve accompanied by conjunctival injection and tearing. Based on the classical presentation of unilateral head pain and activation of cranial autonomic efferents, it has been classified together with cluster headache and paroxysmal hemicrania as the trigeminal autonomic cephalalgias.2–4 At the time of this classification by the International Headache Society, there was no known cause for SUNCT, hence its classification as a primary headache. However, as this entity is becoming more recognised, there have been case reports where SUNCT has been secondary to a structural cause.
2. Case report A 43-year-old previously healthy man had been afflicted by multiple daily episodes of brief left-sided periorbital sharp, stabbing pain of varying severity for more than 10 years. The episodes had become more problematic and resulted in this current presentation. The episodes lasted about 10 seconds and had become more debilitating because of the severity, frequency of attacks and associated severe brief lethargy lasting about 10 minutes after the pain subsided. Neck movement triggered some episodes and others had ⇑ Corresponding author. Tel.: +61 8 9346 3088 (Home). E-mail address: [email protected] (T.D. Musuka).
no apparent trigger. Some episodes woke him from sleep. The head pain was instantly associated with tearing of the left eye and rhinorrhoea. The frequency of attacks was extremely variable, from 1 per day during ‘‘good’’ weeks to more than 10 per day during ‘‘bad’’ weeks. His past medical history was unremarkable. He had seen a neurologist about 10 years prior when the diagnosis of SUNCT had been made. He had undergone a trial of sodium valproate with no success. MRI scan of the head with contrast was normal. On current presentation, he had no systemic symptoms. Clinical neurologic examination was normal and specifically, ocular examination including confrontation visual fields and colour vision was normal. A repeat MRI scan of the head showed a 15 mm left sellar mass with extension into the cavernous sinus and suprasellar cistern. Biochemical assessment revealed a markedly elevated prolactin concentration of more than 12,000 mIU/L, consistent with a prolactin-secreting pituitary macroadenoma. Other anterior pituitary hormone levels were within normal ranges. Further questioning revealed that his libido had decreased in the 12 months prior to presentation. He, however, denied erectile dysfunction, galactorrhoea, gynaecomastia or hot flushes. He was started on a dopamine agonist with excellent response in his serum prolactin. His headaches also improved dramatically after only a few weeks. Eighteen months after starting the dopamine agonist, he is headache free. Serum prolactin remains elevated at 1600 mU/L. A repeat MRI scan (Fig. 1) shows stable appearances of the pituitary mass. A surgical option was explored but was deemed to be unnecessary at this stage as the prolactinoma was predominantly in the sella, there was no visual compromise, and there had been good response to medical therapy. A conservative approach was adopted with a plan for follow-up MRI.
Case Reports / Journal of Clinical Neuroscience 20 (2013) 1181–1183
1181
complete resolution of the pain when the pituitary lesion was treated. The others’ symptoms resolved almost completely with treatment of the prolactinoma. In the patients described the headache symptoms pre-dated the pituitary manifestations by 3 to 10 years; they noted that the headaches may continue after the treatment of the pituitary tumour. This coupled with the fact that the headache symptoms may occur with microprolactinomas argues against a compressive aetiology for the head pain. Matharu et al.5 raised the possibility of a neurohormonal cause for SUNCT in their case report. Our patient had symptoms for about 10 years with the initial MRI being normal. Moreover, on direct questioning, symptoms of reduced libido likely due to the elevated prolactin level had only been present for 1 year. Whether a pituitary microadenoma was present 10 years before, but was too small to detect on MRI, is a moot point.
4. Conclusion
Fig. 1. Sagittal T1-weighted MRI showing stable appearance of the pituitary mass.
3. Discussion
When the history is suggestive of SUNCT, it is worth searching for a structural cause, especially pituitary lesions, either macro or microadenomas. The headache symptoms may precede detection of pituitary lesions by up to 10 years and repeat clinical and MRI surveillance over time is warranted. Our patient is likely an example of this. References
SUNCT is considered to be a rare primary headache disorder whose hallmarks are recurrent bouts of unilateral head pain accompanied by autonomic features.2–4 The prominent autonomic features and lack of response to carbamazepine serve to distinguish this disorder from trigeminal neuralgia. Most cases of SUNCT have no known cause.2–4 There are increasing numbers of published patient reports of potential causes of this syndrome and therefore it is not always a primary headache disorder. Cohen described 52 patients with SUNCT2, which included three patients with SUNCT associated with pituitary lesions, two macroadenomas and one microadenoma. One patient with a macroadenoma had
1. Sjaastad O, Russell D, Horven I, et al. Multiple neuralgiform unilateral headache attacks associated with conjunctival injection and appearing in clusters. A nosological problem. In: Proceedings of the Scandinavian Migraine Society. Arhus; 1978. p. 31l. 2. Cohen AS. Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing. Cephalalgia 2007;27:824–32. 3. Leone M, Bussone G. Pathophysiology of trigeminal autonomic cephalalgias. Lancet Neurol 2009;8:755–64. 4. International Headache Society Classification ICHD-II, Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT). http://ihsclassification.org/en/02_klassifikation/02_teil1/03.03.00_cluster.html. Accessed 22 January 2012. 5. Matharu M, Levy M, Merry R, et al. SUNCT syndrome secondary to prolactinoma. J Neurol Neurosurg Psychiatry 2003;74.
http://dx.doi.org/10.1016/j.jocn.2012.10.014
Leptomeningeal metastasis of an intradural malignant peripheral nerve sheath tumor Andreas M. Stark ⇑, H. Maximilian Mehdorn Klinik für Neurochirurgie, Universitätsklinikum Schleswig-Holstein, Campus Kiel, Arnold-Heller-Str. 2, Haus 41, 24105 Kiel, Germany
a r t i c l e
i n f o
Article history: Received 3 September 2012 Accepted 8 September 2012
Keywords: Leptomeningeal Malignant peripheral nerve sheath tumor Metastases MPNST
a b s t r a c t Malignant peripheral nerve sheath tumors (MPNST) are defined as any malignant tumor arising from or differentiating towards the peripheral nerve sheath. Intradural MPNST metastases are very rare. We report, to our knowledge, the first case of leptomeningeal metastasis of a MPNST to the spine and intracranial space. A 56-year-old woman with primary intradural MPNST of the S1 nerve root developed leptomeningeal metastases as well as brain metastases 19 months after diagnosis. The patient had a history of non-Hodgkins lymphoma for which she had received irradiation to the spine 15 years prior to this presentation. She had no stigmata of neurofibromatosis type 1. Patients with MPNST may also develop leptomeningeal metastases as demonstrated in this patient with intradural post-radiation MPNST. Ó 2013 Elsevier Ltd. All rights reserved.
⇑ Corresponding author. Tel.: +49 0431 597 8550; fax: +49 0431 597 4918. E-mail address: [email protected] (A.M. Stark).