British Journal of Neurosurgery, 2014; Early Online: 1–2 © 2014 The Neurosurgical Foundation ISSN: 0268-8697 print / ISSN 1360-046X online DOI: 10.3109/02688697.2014.957155
SHORT REPORT
The treatment of combined trigeminal and glossopharyngeal neuralgia by glycerol rhizolysis of the trigeminal ganglion Niovi Papalexopoulou1, Harutomo Hasegawa1, Richard Selway1, Sam Chong2 & Keyoumars Ashkan1,3 1Department of Neurosurgery, King’s College Hospital, London, UK, 2Department of Neurology, King’s College Hospital, London,
Br J Neurosurg Downloaded from informahealthcare.com by Dicle Univ. on 11/09/14 For personal use only.
UK, and 3Department of Clinical Neurosciences, Institute of Psychiatry, King’s College London, London, UK
pain across the left side of the face into the ear. Shaving and brushing the teeth also triggered the pain. The pain episodes lasted for 7–10 days and occurred every 2–3 months. He continued to have glossopharyngeal neuralgia, but by late 2010 the trigeminal neuralgia was much worse. Further medical therapy was limited due to intolerable side effects, leading to the consideration of surgical options. Glycerol rhizolysis was chosen due to its relatively smaller risks, and although it was not expected to improve the glossopharyngeal neuralgia, the relief of trigeminal neuralgia was considered to afford a substantial improvement in the patient’s quality of life. In May 2011, 0.6 ml of glycerol was injected into the left trigeminal ganglion using bi-planar X-ray imaging. Postoperatively, the patient reported a significant improvement in both neuralgias and was able to eat and drink immediately. At 1-year follow-up he was pain free, and had reduced his medication requirements to gabapentin 300 mg/day. Two years postoperatively, he experienced a recurrence of the tongue pain, which was managed by increasing the dose of gabapentin to 1800 mg/day and the addition of lacosamide 150 mg/day. He remains pain free almost 3 years after surgery.
Abstract A 78-year-old man with combined trigeminal and glossopharyngeal neuralgia underwent glycerol rhizolysis of the trigeminal ganglion. The treatment led to the immediate relief of both neuralgias. We discuss the potential mechanism of this unexpected therapeutic effect with reference to the pathophysiology of trigeminal and glossopharyngeal neuralgia. Keywords: glossopharyngeal neuralgia; glycerol rhizolysis; trigeminal neuralgia
Clinical details In 2006, a previously fit 78-year-old man began experiencing mild recurrent paroxysmal pain described as clusters of sharp stabs on the left side of the posterior tongue and throat, which radiated to the left ear. There were at least 6 clusters of pain per day. Episodes of pain lasted several weeks and occurred a few months apart. The pain was triggered by eating, swallowing, talking, and laughing. Eating was particularly difficult as he had to gulp his food down quickly in between attacks of pain. The neurological examination was normal. Magnetic resonance imaging of the brain was unremarkable and showed no neurovascular contact. He fulfilled the International Headache Society diagnostic criteria for glossopharyngeal neuralgia and was managed by the facial pain multidisciplinary team at King’s College Hospital. In 2008, the intensity and frequency of the pain increased to up to 20 clusters per day, leading to progressive problems with eating, weight loss and malnutrition. His pain was managed using a combination of medications including carbamazepine, gabapentin, oxcarbazepine, lacosamide and lamotrigine, which were partially effective in suppressing the relapses. In 2010, the neuralgia changed to incorporate elements of trigeminal neuralgia. He began to experience clusters of pain affecting the left jaw and cheek; the forehead was spared. Tactile stimulation of a spot on the lower lip produced a severe shooting
Discussion Combined trigeminal and glossopharyngeal neuralgia is a rare, but recognized condition. It has been reported that 0.3–0.5% of patients with trigeminal neuralgia also have glossopharyngeal neuralgia,1 and the management has traditionally involved the surgical treatment of both neuralgias separately. The neural basis of trigeminal neuralgia, including the mechanism of allodynia, the periodicity of symptoms and the conscious perception of pain, remains poorly understood. The existence of both peripheral and central pathways in the trigeminal nociceptive system is, however, well established, and has been supported by functional imaging studies demonstrating the involvement of multiple
Correspondence: Harutomo Hasegawa, Department of Neurosurgery, King ’s College Hospital, Denmark Hill, London SE5 9RS, UK. Tel: ⫹ 020-3299-4737. E-mail: [email protected] Received for publication 16 February 2014; accepted 16 August 2014
1
Br J Neurosurg Downloaded from informahealthcare.com by Dicle Univ. on 11/09/14 For personal use only.
2
N. Papalexopoulou et al.
cortical and subcortical structures in trigeminal neuralgia.2 The therapeutic effect of glycerol rhizolysis is attributed to the toxic effect (demyelination and axonal destruction) of glycerol on peripheral nerves. How this leads to the pain relief is not completely understood, but is presumably related to a reduction in afferent input. Peripheral nerve injury results in plastic changes in second-order (and higher) neurons and these adaptive changes may be involved in both the pathogenesis of trigeminal neuralgia and glycerol-mediated pain relief. A possible explanation for the relief of glossopharyngeal neuralgia following glycerol rhizolysis of the trigeminal ganglion may involve interactions between the glossopharyngeal and trigeminal sensory systems. The extensive convergence of trigeminal nociceptive afferents with those from the lower cranial and upper cervical nerves in the brainstem trigeminal nucleus, and experimental evidence for the modulation of nociceptive processing in this area by peripheral afferent and higher brain centres, provides an anatomical basis for such an interaction.3 The functional and therapeutic implications of this are illustrated, in addition to the present case,
by the emerging success in treating headache disorders with occipital and vagus nerve stimulation. Such findings demonstrating clinically relevant modulation of one pain pathway by another open new therapeutic avenues, and encourage research towards novel treatment strategies.
Declaration of interest: The authors report no declarations of interest. The authors alone are responsible for the content and writing of the paper.
References 1. Kobata H, Kondo A , Iwasaki K, Nishioka T. Combined hyperactive dysfunction syndrome of the cranial nerves: trigeminal neuralgia, hemifacial spasm, and glossopharyngeal neuralgia: 11-year experience and review. Neurosurgery 1998;43:1351–61. 2. Borsook D, Moulton EA , Pendse G, et al. Comparison of evoked vs. spontaneous tics in a patient with trigeminal neuralgia (tic doloureux). Mol Pain 2007;3:34. 3. Sessle BJ. Acute and chronic craniofacial pain: Brainstem mechanisms of nociceptive transmission and neuroplasticity, and their clinical correlates. Crit Rev Oral Biol Med 2000;11:57–91.
SHORT REPORT
The treatment of combined trigeminal and glossopharyngeal neuralgia by glycerol rhizolysis of the trigeminal ganglion Niovi Papalexopoulou1, Harutomo Hasegawa1, Richard Selway1, Sam Chong2 & Keyoumars Ashkan1,3 1Department of Neurosurgery, King’s College Hospital, London, UK, 2Department of Neurology, King’s College Hospital, London,
Br J Neurosurg Downloaded from informahealthcare.com by Dicle Univ. on 11/09/14 For personal use only.
UK, and 3Department of Clinical Neurosciences, Institute of Psychiatry, King’s College London, London, UK
pain across the left side of the face into the ear. Shaving and brushing the teeth also triggered the pain. The pain episodes lasted for 7–10 days and occurred every 2–3 months. He continued to have glossopharyngeal neuralgia, but by late 2010 the trigeminal neuralgia was much worse. Further medical therapy was limited due to intolerable side effects, leading to the consideration of surgical options. Glycerol rhizolysis was chosen due to its relatively smaller risks, and although it was not expected to improve the glossopharyngeal neuralgia, the relief of trigeminal neuralgia was considered to afford a substantial improvement in the patient’s quality of life. In May 2011, 0.6 ml of glycerol was injected into the left trigeminal ganglion using bi-planar X-ray imaging. Postoperatively, the patient reported a significant improvement in both neuralgias and was able to eat and drink immediately. At 1-year follow-up he was pain free, and had reduced his medication requirements to gabapentin 300 mg/day. Two years postoperatively, he experienced a recurrence of the tongue pain, which was managed by increasing the dose of gabapentin to 1800 mg/day and the addition of lacosamide 150 mg/day. He remains pain free almost 3 years after surgery.
Abstract A 78-year-old man with combined trigeminal and glossopharyngeal neuralgia underwent glycerol rhizolysis of the trigeminal ganglion. The treatment led to the immediate relief of both neuralgias. We discuss the potential mechanism of this unexpected therapeutic effect with reference to the pathophysiology of trigeminal and glossopharyngeal neuralgia. Keywords: glossopharyngeal neuralgia; glycerol rhizolysis; trigeminal neuralgia
Clinical details In 2006, a previously fit 78-year-old man began experiencing mild recurrent paroxysmal pain described as clusters of sharp stabs on the left side of the posterior tongue and throat, which radiated to the left ear. There were at least 6 clusters of pain per day. Episodes of pain lasted several weeks and occurred a few months apart. The pain was triggered by eating, swallowing, talking, and laughing. Eating was particularly difficult as he had to gulp his food down quickly in between attacks of pain. The neurological examination was normal. Magnetic resonance imaging of the brain was unremarkable and showed no neurovascular contact. He fulfilled the International Headache Society diagnostic criteria for glossopharyngeal neuralgia and was managed by the facial pain multidisciplinary team at King’s College Hospital. In 2008, the intensity and frequency of the pain increased to up to 20 clusters per day, leading to progressive problems with eating, weight loss and malnutrition. His pain was managed using a combination of medications including carbamazepine, gabapentin, oxcarbazepine, lacosamide and lamotrigine, which were partially effective in suppressing the relapses. In 2010, the neuralgia changed to incorporate elements of trigeminal neuralgia. He began to experience clusters of pain affecting the left jaw and cheek; the forehead was spared. Tactile stimulation of a spot on the lower lip produced a severe shooting
Discussion Combined trigeminal and glossopharyngeal neuralgia is a rare, but recognized condition. It has been reported that 0.3–0.5% of patients with trigeminal neuralgia also have glossopharyngeal neuralgia,1 and the management has traditionally involved the surgical treatment of both neuralgias separately. The neural basis of trigeminal neuralgia, including the mechanism of allodynia, the periodicity of symptoms and the conscious perception of pain, remains poorly understood. The existence of both peripheral and central pathways in the trigeminal nociceptive system is, however, well established, and has been supported by functional imaging studies demonstrating the involvement of multiple
Correspondence: Harutomo Hasegawa, Department of Neurosurgery, King ’s College Hospital, Denmark Hill, London SE5 9RS, UK. Tel: ⫹ 020-3299-4737. E-mail: [email protected] Received for publication 16 February 2014; accepted 16 August 2014
1
Br J Neurosurg Downloaded from informahealthcare.com by Dicle Univ. on 11/09/14 For personal use only.
2
N. Papalexopoulou et al.
cortical and subcortical structures in trigeminal neuralgia.2 The therapeutic effect of glycerol rhizolysis is attributed to the toxic effect (demyelination and axonal destruction) of glycerol on peripheral nerves. How this leads to the pain relief is not completely understood, but is presumably related to a reduction in afferent input. Peripheral nerve injury results in plastic changes in second-order (and higher) neurons and these adaptive changes may be involved in both the pathogenesis of trigeminal neuralgia and glycerol-mediated pain relief. A possible explanation for the relief of glossopharyngeal neuralgia following glycerol rhizolysis of the trigeminal ganglion may involve interactions between the glossopharyngeal and trigeminal sensory systems. The extensive convergence of trigeminal nociceptive afferents with those from the lower cranial and upper cervical nerves in the brainstem trigeminal nucleus, and experimental evidence for the modulation of nociceptive processing in this area by peripheral afferent and higher brain centres, provides an anatomical basis for such an interaction.3 The functional and therapeutic implications of this are illustrated, in addition to the present case,
by the emerging success in treating headache disorders with occipital and vagus nerve stimulation. Such findings demonstrating clinically relevant modulation of one pain pathway by another open new therapeutic avenues, and encourage research towards novel treatment strategies.
Declaration of interest: The authors report no declarations of interest. The authors alone are responsible for the content and writing of the paper.
References 1. Kobata H, Kondo A , Iwasaki K, Nishioka T. Combined hyperactive dysfunction syndrome of the cranial nerves: trigeminal neuralgia, hemifacial spasm, and glossopharyngeal neuralgia: 11-year experience and review. Neurosurgery 1998;43:1351–61. 2. Borsook D, Moulton EA , Pendse G, et al. Comparison of evoked vs. spontaneous tics in a patient with trigeminal neuralgia (tic doloureux). Mol Pain 2007;3:34. 3. Sessle BJ. Acute and chronic craniofacial pain: Brainstem mechanisms of nociceptive transmission and neuroplasticity, and their clinical correlates. Crit Rev Oral Biol Med 2000;11:57–91.
