Case report
A Rare Association of Trigeminal Autonomic Cephalgia: Pontine Capillary Telangiectasia
The Neuroradiology Journal 2015, Vol. 28(2) 145–147 ! The Author(s) 2015 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav DOI: 10.1177/1971400915576656 neu.sagepub.com
Rahsan Gocmen, Erdal Kurt, Sabina Arslan, Isin Unal-Cevik, Kader Karli Oguz and F Irsel Tezer
Summary This report describes a case of pontine capillary telangiectasia in a 43-year-old woman with a clinical diagnosis of trigeminal autonomic cephalgia. The possible association with pontine capillary telangiectasia and trigeminal autonomic cephalgia is discussed.
Keywords capillary telangiectasia, pons, headache, trigeminal autonomic cephalgia, magnetic resonance imaging
Introduction Capillary telangiectasia (CT) consists of clusters of dilated capillaries most often located in the pons. The fact that most CTs are found incidentally on magnetic resonance imaging (MRI) or pathological examination (0.4%) confirms the clinically benign course.2 Only few CT cases with symptoms related to the brain stem have been reported.1,3,5 Trigeminal autonomic cephalgias (TACs) are a group of primary headache disorders characterized by unilateral headache especially in the trigeminal distribution that occurs in association with ipsilateral cranial parasympathetic autonomic features. Experimental and human functional imaging suggests that these syndromes activate a normal human trigeminal parasympathetic reflex, with clinical signs of cranial sympathetic dysfunction being secondary. Secondary TACs are relatively uncommon and can be associated with diverse pathologic processes at various sites (pituitary tumours, hypothalamic lesions, vascular malformations, dissections etc.), but involvement of the brain stem is very rarely reported.4 Herein we describe a case of pontine CT presenting with excruciating headache attacks with autonomic symptoms. While the patient had a poor response to appropriate medical treatments, complete pain relief was achieved with peripheral nerve blocks.
presented 20-30 times a day, lasting five to ten minutes. The headache was accompanied by ipsilateral tearing and conjunctival hyperaemia, but no phonophobia, photophobia, nausea, or vomiting. There were no preceding symptoms like visual flickering or black spots. The patient had a history of lifting heavy objects just before the start of the headache attacks. Her medical and family histories were unremarkable. Neurological examination revealed transient left-sided sixth nerve palsy for a few days. Routine laboratory tests were unremarkable. Cranial MRI showed a subtle focal hyperintensity on T2 weighted (W) images, signal drop on T2W gradient echo images, and brush-like enhancement on post-gadolinium contrast T1W images (Figure 1). These findings were consistent with pontine CT (El-Koussy et al., 2012). The patient was treated with high dose (150 mg/day) indomethacin. Due to the lack of a complete response, bilateral greater occipital nerve block with methyl prednisolone and lidocaine and left supraorbital nerve block with lidocaine was performed by one of the co-authors (IUC). At the follow-up visits, the patient reported an excellent response with no new attacks for more than eight months.
Case Report
Radiology Department, Hacettepe University Medical School; Ankara, Turkey
A 43-year-old woman was admitted with a one-month history of left-sided, stabbing headache. These severe headaches were resistant to simple analgesics and non-steroidal anti-inflammatory drugs and had
Corresponding author: Dr Rahsan Gocmen, Radiology Department, Hacettepe University Medical School, Sihhiye, Ankara 06100 Turkey. Email: [email protected]
146
The Neuroradiology Journal 28(2)
Figure 1. a) Brain MRI of the patient. Axial T2W image shows a subtle hyperintensity in the left side of the pons (arrow). b) This area has a susceptibility artefact on the SWI image (arrow). Axial (c) and sagittal (d) T1W images display brush-like enhancement better appreciated on the sagittal plane (arrows).
Discussion Herein we describe a case with trigeminal autonomic cephalgia (TAC) associated with pontine CT and complete remission of attacks with peripheral nerve blocks. CT has characteristic MRI features which enable its in vivo detection and differentiation from other brain stem diseases including multiple sclerosis, chronic ischaemic lesion, and neoplasm(2). Similar to our case, typically, the vessels enhance after contrast administration creating a mesh (or brush-like arrangement) of enhanced structures on a background of unenhanced brain parenchyma. The exact aetiology of these telangiectasias, however, remains unclear. It has been postulated that telangiectasias are acquired lesions associated with other venous anomalies, but there is also a possibility of a primary developmental lesion.2 There are few reported cases of brain stem CT presenting with transient or permanent symptoms compatible with lesion location.1,3,5 The association with symptoms remains unproven, however, including vertigo, diplopia, hearing loss, focal weakness, ataxia,
tinnitus, and monocular ptosis. There is only one reported case with secondary basilar-type migraine as a symptom of pontine CT.1 The pathophysiology underlying transient symptoms of CTs is not established. In our case, the acute-subacute presentation of severe TAC attacks and transient sixth nerve palsy may be explained by the ‘‘aggressive course’’ of pontine CT.4 The trigeminal nociceptive system in the dorsal lateral pons can be linked to this characteristic pain. In addition, the involvement of pontine sympathetic fibres, and dorsal lemniscus can lead to autonomic symptoms. It is postulated that, in the aggressive course of telangiectasia, recurrent microscopic haemorrhage into the adjacent parenchyma may produce neuronal injury.3 However, a blood-pool shift or possibly arteriovenous shunting through a CT might be suggested. By lifting heavy objects, our patient might have had a subtle increase in intracranial pressure leading to dilatation of pontine CT, and further produced regional transient disturbances in cerebral perfusion.3 In conclusion, this case report provides further evidence that pontine CT might cause a clinical picture of TACs. We suggest that cranial MRI should be
Gocmen et al. considered in atypical, severe and frequent headache attacks. Peripheral nerve blocks may be considered in medically resistant cases. Funding This research received no specific grant from any funding agency in the public, commercial or not-for-profit sectors.
Conflict of interest The authors declare no conflict of interest.
References 1. Beukers RJ and Roos YW. Pontine capillary telangiectasia as visualized on MR imaging causing a clinical picture resembling basilar-type migraine: a case report. J Neurol 2009; 256(10): 1775–1777. doi: 10.1007/s00415009-5204-5.
147 2. El-Koussy M, Schroth G, Gralla J, et al. Susceptibilityweighted MR imaging for diagnosis of capillary telangiectasia of the brain. Am J Neuroradiol 2012; 33: 715–720. doi: 10.3174/ajnr.A2893. 3. Huddle DC, Chaloupka JC and Sehgal V. Clinically aggressive diffuse capillary telangiectasia of the brain stem: a clinical radio-pathologic case study. Am J Neuroradiol 1999; 20: 1674–1677. 4. Irimia P, Arbizu J, Prieto E, et al. Activation of the brainstem but not of the hypothalamus in hemicrania continua without autonomic symptoms. Cephalalgia 2009; 29(9): 974–979. doi: 10.1111/j.1468-2982.2008.01832.x. 5. Scaglione C, Salvi F, Riguzzi P, et al. Symptomatic unruptured capillary telangiectasia of the brain stem: report of three cases and review of the literature. J Neurol Neurosurg Psychiatry 2001; 71(3): 390–393. doi: 10.1136/ jnnp.71.3.390.
A Rare Association of Trigeminal Autonomic Cephalgia: Pontine Capillary Telangiectasia
The Neuroradiology Journal 2015, Vol. 28(2) 145–147 ! The Author(s) 2015 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav DOI: 10.1177/1971400915576656 neu.sagepub.com
Rahsan Gocmen, Erdal Kurt, Sabina Arslan, Isin Unal-Cevik, Kader Karli Oguz and F Irsel Tezer
Summary This report describes a case of pontine capillary telangiectasia in a 43-year-old woman with a clinical diagnosis of trigeminal autonomic cephalgia. The possible association with pontine capillary telangiectasia and trigeminal autonomic cephalgia is discussed.
Keywords capillary telangiectasia, pons, headache, trigeminal autonomic cephalgia, magnetic resonance imaging
Introduction Capillary telangiectasia (CT) consists of clusters of dilated capillaries most often located in the pons. The fact that most CTs are found incidentally on magnetic resonance imaging (MRI) or pathological examination (0.4%) confirms the clinically benign course.2 Only few CT cases with symptoms related to the brain stem have been reported.1,3,5 Trigeminal autonomic cephalgias (TACs) are a group of primary headache disorders characterized by unilateral headache especially in the trigeminal distribution that occurs in association with ipsilateral cranial parasympathetic autonomic features. Experimental and human functional imaging suggests that these syndromes activate a normal human trigeminal parasympathetic reflex, with clinical signs of cranial sympathetic dysfunction being secondary. Secondary TACs are relatively uncommon and can be associated with diverse pathologic processes at various sites (pituitary tumours, hypothalamic lesions, vascular malformations, dissections etc.), but involvement of the brain stem is very rarely reported.4 Herein we describe a case of pontine CT presenting with excruciating headache attacks with autonomic symptoms. While the patient had a poor response to appropriate medical treatments, complete pain relief was achieved with peripheral nerve blocks.
presented 20-30 times a day, lasting five to ten minutes. The headache was accompanied by ipsilateral tearing and conjunctival hyperaemia, but no phonophobia, photophobia, nausea, or vomiting. There were no preceding symptoms like visual flickering or black spots. The patient had a history of lifting heavy objects just before the start of the headache attacks. Her medical and family histories were unremarkable. Neurological examination revealed transient left-sided sixth nerve palsy for a few days. Routine laboratory tests were unremarkable. Cranial MRI showed a subtle focal hyperintensity on T2 weighted (W) images, signal drop on T2W gradient echo images, and brush-like enhancement on post-gadolinium contrast T1W images (Figure 1). These findings were consistent with pontine CT (El-Koussy et al., 2012). The patient was treated with high dose (150 mg/day) indomethacin. Due to the lack of a complete response, bilateral greater occipital nerve block with methyl prednisolone and lidocaine and left supraorbital nerve block with lidocaine was performed by one of the co-authors (IUC). At the follow-up visits, the patient reported an excellent response with no new attacks for more than eight months.
Case Report
Radiology Department, Hacettepe University Medical School; Ankara, Turkey
A 43-year-old woman was admitted with a one-month history of left-sided, stabbing headache. These severe headaches were resistant to simple analgesics and non-steroidal anti-inflammatory drugs and had
Corresponding author: Dr Rahsan Gocmen, Radiology Department, Hacettepe University Medical School, Sihhiye, Ankara 06100 Turkey. Email: [email protected]
146
The Neuroradiology Journal 28(2)
Figure 1. a) Brain MRI of the patient. Axial T2W image shows a subtle hyperintensity in the left side of the pons (arrow). b) This area has a susceptibility artefact on the SWI image (arrow). Axial (c) and sagittal (d) T1W images display brush-like enhancement better appreciated on the sagittal plane (arrows).
Discussion Herein we describe a case with trigeminal autonomic cephalgia (TAC) associated with pontine CT and complete remission of attacks with peripheral nerve blocks. CT has characteristic MRI features which enable its in vivo detection and differentiation from other brain stem diseases including multiple sclerosis, chronic ischaemic lesion, and neoplasm(2). Similar to our case, typically, the vessels enhance after contrast administration creating a mesh (or brush-like arrangement) of enhanced structures on a background of unenhanced brain parenchyma. The exact aetiology of these telangiectasias, however, remains unclear. It has been postulated that telangiectasias are acquired lesions associated with other venous anomalies, but there is also a possibility of a primary developmental lesion.2 There are few reported cases of brain stem CT presenting with transient or permanent symptoms compatible with lesion location.1,3,5 The association with symptoms remains unproven, however, including vertigo, diplopia, hearing loss, focal weakness, ataxia,
tinnitus, and monocular ptosis. There is only one reported case with secondary basilar-type migraine as a symptom of pontine CT.1 The pathophysiology underlying transient symptoms of CTs is not established. In our case, the acute-subacute presentation of severe TAC attacks and transient sixth nerve palsy may be explained by the ‘‘aggressive course’’ of pontine CT.4 The trigeminal nociceptive system in the dorsal lateral pons can be linked to this characteristic pain. In addition, the involvement of pontine sympathetic fibres, and dorsal lemniscus can lead to autonomic symptoms. It is postulated that, in the aggressive course of telangiectasia, recurrent microscopic haemorrhage into the adjacent parenchyma may produce neuronal injury.3 However, a blood-pool shift or possibly arteriovenous shunting through a CT might be suggested. By lifting heavy objects, our patient might have had a subtle increase in intracranial pressure leading to dilatation of pontine CT, and further produced regional transient disturbances in cerebral perfusion.3 In conclusion, this case report provides further evidence that pontine CT might cause a clinical picture of TACs. We suggest that cranial MRI should be
Gocmen et al. considered in atypical, severe and frequent headache attacks. Peripheral nerve blocks may be considered in medically resistant cases. Funding This research received no specific grant from any funding agency in the public, commercial or not-for-profit sectors.
Conflict of interest The authors declare no conflict of interest.
References 1. Beukers RJ and Roos YW. Pontine capillary telangiectasia as visualized on MR imaging causing a clinical picture resembling basilar-type migraine: a case report. J Neurol 2009; 256(10): 1775–1777. doi: 10.1007/s00415009-5204-5.
147 2. El-Koussy M, Schroth G, Gralla J, et al. Susceptibilityweighted MR imaging for diagnosis of capillary telangiectasia of the brain. Am J Neuroradiol 2012; 33: 715–720. doi: 10.3174/ajnr.A2893. 3. Huddle DC, Chaloupka JC and Sehgal V. Clinically aggressive diffuse capillary telangiectasia of the brain stem: a clinical radio-pathologic case study. Am J Neuroradiol 1999; 20: 1674–1677. 4. Irimia P, Arbizu J, Prieto E, et al. Activation of the brainstem but not of the hypothalamus in hemicrania continua without autonomic symptoms. Cephalalgia 2009; 29(9): 974–979. doi: 10.1111/j.1468-2982.2008.01832.x. 5. Scaglione C, Salvi F, Riguzzi P, et al. Symptomatic unruptured capillary telangiectasia of the brain stem: report of three cases and review of the literature. J Neurol Neurosurg Psychiatry 2001; 71(3): 390–393. doi: 10.1136/ jnnp.71.3.390.
