- Email: [email protected]
A healthy man with intractable hiccups
Case Reports / Journal of Clinical Neuroscience 17 (2010) 781–783
stroke-induced acute functional dysregulation of basal ganglia motor circuits, rather than from restorative neuroplasticity.9 However, in some patients, onset of post-stroke chorea is delayed by several weeks, especially if initially there is a significant weakness of the limbs. Our patient initially presented with hemiplegia that resulted from a hemorrhagic stroke in the contralateral cerebral peduncle, with damage to the corticospinal tract. Thus, the severe muscle weakness probably overshadowed the original choreiform movements that resulted from a simultaneous hemorrhagic stroke in the subthalamus. The relatively successful recovery of muscle strength, as opposed to persistent dysregulation in the organization of the basal ganglia, resulted in worsening of the involuntary movements. Functional recovery of motor dysfunction is related to a plastic reorganization of the motor cortex.10 As a result of the persistent loss of STN neurons exciting the GPi and GPe, the abnormally patterned output from the GPi likely producing chorea identical to hemiballism. Therefore, in our patient, destruction of the GPi by pallidotomy may have disconnected these abnormal firing patterns from the GPi to the thalamus, eliciting relief from delayed-onset monochorea following recovery from hemiplegia. 4. Conclusions In our patient, a hemorrhagic stroke in the cerebral peduncle and subthalamus manifested as delayed-onset chorea after recovery from the initial limb weakness. Stereotactic pallidotomy may be a useful procedure for STN injury-related hyperkinetic move-
781
ment disorders, including the treatment of monochorea following recovery from initial hemiplegia. Appendix A. Supplementary material Supplementary material associated with this article can be found, in the online version, at doi:10.1016/j.jocn.2009.09.021. References 1. Choi SJ, Lee SW, Kim MC, et al. Posteroventral pallidotomy in medically intractable postapoplectic monochorea: case report. Surg Neurol 2003;59:486–90. 2. Krauss JK, Grossman RG, Jankovic J. Pallidal surgery for the treatment of Parkinson’s disease and movement disorders. Philadelphia, PA: LippincottRaven; 1998. 3. DeLong MR. Primate models of movement disorders of basal ganglia origin. Trends Neurosci 1990;13:281–5. 4. Ginsberg MD. Delayed neurological deterioration following hypoxia. Adv Neurol 1979;26:21–44. 5. Vitek JL, Chockkan V, Zhang JY, et al. Neuronal activity in the basal ganglia in patients with generalized dystonia and hemiballismus. Ann Neurol 1999;46: 22–35. 6. Suarez JI, Metman LV, Reich SG, et al. Pallidotomy for hemiballismus: efficacy and characteristics of neuronal activity. Ann Neurol 1997;42:807–11. 7. Slavin KV, Baumann TK, Burchiel KJ. Treatment of hemiballismus with stereotactic pallidotomy. Case report and review of the literature. Neurosurg Focus 2004;17:E7. 8. Chung SJ, Im JH, Lee MC, et al. Hemichorea after stroke: clinical-radiological correlation. J Neurol 2004;251:725–9. 9. Krauss JK, Jankovic J. Head injury and posttraumatic movement disorders. Neurosurgery 2002;50:927–39.. discussion 939–40. 10. Kim JS. Delayed onset mixed involuntary movements after thalamic stroke: clinical, radiological and pathophysiological findings. Brain 2001;124:299– 309.
doi:10.1016/j.jocn.2009.09.021
A healthy man with intractable hiccups Ioannis Pechlivanis *, Marcel Seiz, Martin Barth, Kirsten Schmieder Department of Neurosurgery, University Hospital Mannheim, University of Heidelberg, Theodor-Kutzer-Ufer 1-3, 68135 Mannheim, Germany
a r t i c l e
i n f o
Article history: Received 20 May 2009 Accepted 23 August 2009
Keywords: Cavernoma Brain stem Hiccups
a b s t r a c t We present a patient with intractable hiccups. Medical treatment decreased the frequency of the hiccups to only 5–10 per minute. After exclusion of gastrointestinal reasons for the hiccups, cranial MRI revealed a small lesion in the right rhomboid fossa, close to the vagal trigone. Microsurgical resection of the lesion was performed via a suboccipital median craniotomy. The histopathological diagnosis was a cavernoma. Promptly after surgery the patient was free of symptoms. Intractable hiccups can be associated with intracranial pathologies, including lesions in the brain stem, which we highlight with the presentation of this patient. Ó 2009 Elsevier Ltd. All rights reserved.
1. Case report A 33-year-old otherwise healthy man was admitted with intractable hiccups that had persisted for several months. The hiccups had started suddenly with a frequency of 10 to 20 hiccups per minute. The patient’s medical history included surgery for otitis media and a thoracic spine fracture due to an accident at work. No medication had been taken before the onset of the hiccups. On physical examination, the patient displayed no neurological deficit; in par* Corresponding author. Tel.: +49 621 3832360; fax: +49 621 3832004. E-mail address: [email protected] (I. Pechlivanis).
ticular, the function of the cranial nerves was normal. Blood tests, including detailed analysis of infection parameters, showed no abnormalities. To rule out gastroabdominal causes for the hiccups ultrasound of the abdomen and gastroscopy were performed, revealing no pathology. The size and the configuration of the diaphragm were normal on chest X-ray; furthermore, no tumor was detected. Since the etiology remained unclear, the patient was treated pharmacologically with gabapentin for several weeks. Although the frequency of the hiccups decreased intermittently to 5–10 per minute, paresthesia of the left side of the body developed. For this reason, cranial MRI of the brain was performed. MRI of the posterior fossa demonstrated a small lesion in the rhomboid
782
Case Reports / Journal of Clinical Neuroscience 17 (2010) 781–783
fossa, close to the right vagal trigone (Fig. 1). A small hemosiderin ring was seen on T2-weighted MRI. Digital subtraction angiography excluded an arteriovenous malformation. Minimally invasive microsurgical resection of the lesion was performed via a suboccipital median craniotomy. After incision of the rhomboid fossa directly above the lesion, a pinkish vascular tumor was removed completely. The histopathological diagnosis was cavernoma. Immediately after surgery the patient was free of symptoms without any new neurological deficit. At follow-up after 3 months, no further episodes of hiccups had occurred and no sensory paresthesia was reported. MRI of the brain showed no further lesions. 2. Discussion Hiccups, or singultus, is a polysynaptic reflex comprising a synchronous, repeated, myoclonic contraction of the diaphragm. The typical ‘‘hiccup” sound is produced by the sudden involuntary closure of the glottis at inspiration.
Fig. 1. (A) Axial and (B) sagittal T2-weighted MRI showing a small lesion in the right rhomboid fossa (arrows), irritating the ‘‘hiccup center”.
In a healthy person, hiccups is usually a self-limiting reflex of short duration. Frequent triggers are abrupt gastric extension (such as on consumption of heavy meals or sparkling drinks), alcohol, heavy smoking or psychogenic factors (stress, shock). Hiccups may continue for days, weeks, or even years, and whenever the episode extends beyond 48 hours, it is termed ‘‘persistent”. The afferent part of the reflex arc includes sensory branches of the phrenic, glossopharyngeal and vagus nerves, together with fibers of the thoracic sympathetic nervous system (T6–T12), while the main efferent output is to the phrenic nerve, resulting in a spastic contraction of the diaphragm.1,2 The central connection of this reflex in the brainstem and midbrain is between the dorsal portion of the nucleus tractus solitarius, the ventral part of the nucleus ambiguus, phrenic nerve nuclei, the medullary reticular formation and the hypothalamus. Furthermore, supratentorial areas participate in the genesis of hiccups by stimulation or inhibition of this reflex arc.3 However, the exact central genesis of hiccups is still unknown: Hassler et al.4 assumed generation of hiccups in the myoclonic triangle of Guillan-Mollaret (inferior olive, dentate nucleus, red nucleus), while others postulate a dysfunction of the inferior olivary complex, the nucleus ambiguus and the adjacent reticular formation of the medulla oblongata.5 Furthermore, in experimental animal studies the supraspinal polysynaptic center was found to be located in the medullary formation lateral to the nucleus ambiguus at the rostrocaudal level, between 1.0 mm and 2.5 mm rostral to the obex.6 The primary treatment of hiccups should comprise normal physical maneuvers such as breath-holding, drinking of cold water or application of supraorbital pressure. If hiccuping persists, pharmacological management should be considered. Many pharmacological treatments have been proposed for intractable hiccups, and there is no consensus regarding the best option. The approaches proposed include: antagonizing dopamine receptors (chlorpromazine or metoclopramide), increasing an inhibiting gamma-aminobutyric acid effect (baclofen, valproic acid or gabapentin), stabilizing neural membranes (nifedipine [Ca2+] or carbamazepine [Na+]) or relaxing muscles (orphenadrine). Other medications, including antidepressants, anticholinergics, prokinetic drugs and steroids, have also been used. Gabapentin is safe, lacks serious side effects, and has a rapid onset of action in the treatment of intractable hiccups. Nevertheless, pharmacological resolution of persistent hiccups could not be achieved in our patient. Diagnostic clarification of intractable hiccups is mandatory. The most effective (and in some cases the only possible) treatment is to eliminate the cause. Although the etiology of persistent hiccup may be metabolic (uremia, sepsis), pharmacological (methyldopa) or psychological (hysteria), factors causing compression or irritation of the phrenic or vagus nerve (such as tumor, trauma, infection or foreign bodies) have to be excluded (Table 1).7 Central causes for intractable hiccup as the predominant symptom are rare, but not unknown: multiple sclerosis in the lower brainstem is one of the most frequently mentioned reasons for persistent intractable hiccups,8 while other central inflammatory causes include neuromyelitis optica. Cerebral infarction in the dorsal and lateral medulla has been found in patients with intractable hiccups as well as primary medullary hemorrhage.3 In all of these patients hiccups is triggered by direct involvement of the central components, whereas compression may be the mechanism in patients with intracranial tumors. Intracranial neoplasms causing intractable hiccups have been reported: lowgrade glioma,9 ependymoma,10 choroid plexus papilloma of the fourth ventricle,11 hemangioblastoma12 or cerebral tuberculoma3 in the brainstem. Besides neoplastic or inflammatory diseases, Chiari I deformation or vascular anomalies such as aneurysm or
Case Reports / Journal of Clinical Neuroscience 17 (2010) 783–785
783
Table 1 Summary of potential causes of persistent hiccups Factor Irritation of reflex arc (brainstem, vagal, phrenic nerve) Tumor Trauma Ischemia Infection
Foreign body Drugs Operation/anesthesia Metabolic or toxic Psychological Idiopathic
Example
Brainstem, cervical spinal cord, mediastinum, thoracic, pulmonary, gastric, lymphadenopathies, hematoma, pancreatic pseudocysts Head, spine, abdominal trauma, intracranial hemorrhage Myocardial infarction, angina pectoris, brainstem ischemia Meningitis, encephalitis, multiple sclerosis, neuritis optica pharyngitis, laryngitis, mediastinitis, esophagitis, pneumonia, pleuritis, pleural empyema, peritonitis, pancreatitis, hepatitis, abscesses (cerebral, mediastinum, thoracic, phrenic, abdominal) Foreign bodies in acoustic canal Steroids, anesthetics, sulfonamides, barbiturates, benzodiazepine, methyldopa, morphine Mechanical or pharmacological irritation during surgery, intubation, ventilation Uremia, hypocapnia, hypokalemia, hypocalcemia, hyponatremia, carbon dioxide deficit, blood-glucose dysregulation, alcohol, fever, sepsis Sorrow, hysteria, personality disorder, anorexia nervosa No causative factor
dolichoectasia have caused hiccups by compression of the involved centers.10,13–15 To date, however, only one patient with a cavernous angioma triggering intractable hiccup has been reported to our knowledge: Musumeci et al. describe a cavernous angioma located near the obex of the medulla oblongata, with dorsal exophytic growth in the fourth ventricle. Intractable hiccups was the main symptom.16 Although the tumor compressed multiple parts of the lower rhomboid fossa, the authors postulated that in their patient, hiccups was due to the effect on the nucleus raphe magnus and the hiccup centers near the obex. However, due to the lesser size of the lesion, in our patient the ‘‘hiccup center” could be postulated to be close to the vagal trigone, interfering with these centers. We believe that MRI of the brain is mandatory in patients with intractable hiccups, if no other cause has been found. Furthermore, if treatment is possible at acceptable risk we would recommend microsurgery for small lesions, when other treatments have failed and the location implies involvement of hiccup centers.
5. 6.
7.
8. 9. 10.
11.
12.
13.
References 14. 1. Kondo T, Toyooka H, Arita H. Hiccup reflex is mediated by pharyngeal branch of glossopharyngeal nerve in cats. Neurosci Res 2003;47:317–21. 2. Lewis JH. Hiccups: causes and cures. J Clin Gastroenterol 1985;7:539–52. 3. al Deeb SM, Sharif H, al Moutaery K, et al. Intractable hiccup induced by brainstem lesion. J Neurol Sci 1991;103:144–50. 4. Hassler R. Die neuronalen systeme der extrapyramidalen myoclonien und deren stereotaktische behandlung [The neuronal systems of extrapyramidal
15. 16.
myoclonia and their stereotactic treatment]. In: Doose H, editor. Aktuelle neuropädiatrie. Stuttgart: Thieme; 1977. p. 20–46. [in German]. de la Fuente-Fernandez R. [Hiccup and dysfunction of the inferior olivary complex]. Med Clin (Barc) 1998;110:22–4. Oshima T, Sakamoto M, Tatsuta H, et al. GABAergic inhibition of hiccup-like reflex induced by electrical stimulation in medulla of cats. Neurosci Res 1998;30:287–93. Andresen V, Layer P. Schluckauf [Hiccups], In: Domschke W, Berger M, Hohenberger W, et al., Editors. Therapie-handbuch. Loseblattwerk mit onlinezugang [Handbook of therapies. Collected edition with online access]. Munich: Elsevier, Urban & Fischer; 2006. p. 1–5 [in German]. Witoonpanich R, Pirommai B, Tunlayadechanont S. Hiccups and multiple sclerosis. J Med Assoc Thai 2004;87:1168–71. Fischer AQ, McLean Jr WT. Intractable hiccups as presenting symptom of brainstem tumor in children. Childs Brain 1982;9:60–3. Amirjamshidi A, Abbassioun K, Parsa K. Hiccup and neurosurgeons: a report of 4 rare dorsal medullary compressive pathologies and review of the literature. Surg Neurol 2007;67:395–402. Marsot-Dupuch K, Bousson V, Cabane J, et al. Intractable hiccups: the role of cerebral MR in cases without systemic cause. AJNR Am J Neuroradiol 1995;16: 2093–100. Nagayama T, Kaji M, Hirano H, et al. Intractable hiccups as a presenting symptom of cerebellar hemangioblastoma. Case report. J Neurosurg 2004;100: 1107–10. Seki T, Hida K, Lee J, et al. Hiccups attributable to syringobulbia and/or syringomyelia associated with a Chiari I malformation. Neurosurgery 2004;54:224–6 [discussion 226–7]. Vanamoorthy P, Kar P, Prabhakar H. Intractable hiccups as a presenting symptom of Chiari I malformation. Acta Neurochir (Wien) 2008;150: 1207–8. Li ML, Gupta A, Thomas P, et al. Basilar artery aneurysm: an unusual cause of intractable hiccups. Hosp Med 2000;61:868–9. Musumeci A, Cristofori L, Bricolo A. Persistent hiccup as presenting symptom in medulla oblongata cavernoma: a case report and review of the literature. Clin Neurol Neurosurg 2000;102:13–7.
doi:10.1016/j.jocn.2009.08.022
Transvenous embolization of an intraorbital arteriovenous fistula using Onyx Chung-Jung Lin a,c,d,*, Raphael Blanc a,b, Frédéric Clarençon a,b, Michel Piotin a, Laurent Spelle a, Marc Williams b, Jacques Moret a a
Department of Functional and Interventional Neuroradiology, Fondation Rothschild Hospital, University of Paris 7, Bichat School of Medicine, 25–29 rue Manin, Paris 75940, France Department of Diagnostic Neuroradiology, Fondation Rothschild Hospital, University of Paris, Bichat School of Medicine, Paris, France Department of Medical Imaging, Far Eastern Memorial Hospital, Pan-Chiao City, Taiwan d National Yang Ming University, School of Medicine, Taipei, Taiwan b c
* Corresponding author. Tel.: +886 2 89514571; fax: +886 2 28676701. E-mail address: [email protected] (C.-J. Lin).
stroke-induced acute functional dysregulation of basal ganglia motor circuits, rather than from restorative neuroplasticity.9 However, in some patients, onset of post-stroke chorea is delayed by several weeks, especially if initially there is a significant weakness of the limbs. Our patient initially presented with hemiplegia that resulted from a hemorrhagic stroke in the contralateral cerebral peduncle, with damage to the corticospinal tract. Thus, the severe muscle weakness probably overshadowed the original choreiform movements that resulted from a simultaneous hemorrhagic stroke in the subthalamus. The relatively successful recovery of muscle strength, as opposed to persistent dysregulation in the organization of the basal ganglia, resulted in worsening of the involuntary movements. Functional recovery of motor dysfunction is related to a plastic reorganization of the motor cortex.10 As a result of the persistent loss of STN neurons exciting the GPi and GPe, the abnormally patterned output from the GPi likely producing chorea identical to hemiballism. Therefore, in our patient, destruction of the GPi by pallidotomy may have disconnected these abnormal firing patterns from the GPi to the thalamus, eliciting relief from delayed-onset monochorea following recovery from hemiplegia. 4. Conclusions In our patient, a hemorrhagic stroke in the cerebral peduncle and subthalamus manifested as delayed-onset chorea after recovery from the initial limb weakness. Stereotactic pallidotomy may be a useful procedure for STN injury-related hyperkinetic move-
781
ment disorders, including the treatment of monochorea following recovery from initial hemiplegia. Appendix A. Supplementary material Supplementary material associated with this article can be found, in the online version, at doi:10.1016/j.jocn.2009.09.021. References 1. Choi SJ, Lee SW, Kim MC, et al. Posteroventral pallidotomy in medically intractable postapoplectic monochorea: case report. Surg Neurol 2003;59:486–90. 2. Krauss JK, Grossman RG, Jankovic J. Pallidal surgery for the treatment of Parkinson’s disease and movement disorders. Philadelphia, PA: LippincottRaven; 1998. 3. DeLong MR. Primate models of movement disorders of basal ganglia origin. Trends Neurosci 1990;13:281–5. 4. Ginsberg MD. Delayed neurological deterioration following hypoxia. Adv Neurol 1979;26:21–44. 5. Vitek JL, Chockkan V, Zhang JY, et al. Neuronal activity in the basal ganglia in patients with generalized dystonia and hemiballismus. Ann Neurol 1999;46: 22–35. 6. Suarez JI, Metman LV, Reich SG, et al. Pallidotomy for hemiballismus: efficacy and characteristics of neuronal activity. Ann Neurol 1997;42:807–11. 7. Slavin KV, Baumann TK, Burchiel KJ. Treatment of hemiballismus with stereotactic pallidotomy. Case report and review of the literature. Neurosurg Focus 2004;17:E7. 8. Chung SJ, Im JH, Lee MC, et al. Hemichorea after stroke: clinical-radiological correlation. J Neurol 2004;251:725–9. 9. Krauss JK, Jankovic J. Head injury and posttraumatic movement disorders. Neurosurgery 2002;50:927–39.. discussion 939–40. 10. Kim JS. Delayed onset mixed involuntary movements after thalamic stroke: clinical, radiological and pathophysiological findings. Brain 2001;124:299– 309.
doi:10.1016/j.jocn.2009.09.021
A healthy man with intractable hiccups Ioannis Pechlivanis *, Marcel Seiz, Martin Barth, Kirsten Schmieder Department of Neurosurgery, University Hospital Mannheim, University of Heidelberg, Theodor-Kutzer-Ufer 1-3, 68135 Mannheim, Germany
a r t i c l e
i n f o
Article history: Received 20 May 2009 Accepted 23 August 2009
Keywords: Cavernoma Brain stem Hiccups
a b s t r a c t We present a patient with intractable hiccups. Medical treatment decreased the frequency of the hiccups to only 5–10 per minute. After exclusion of gastrointestinal reasons for the hiccups, cranial MRI revealed a small lesion in the right rhomboid fossa, close to the vagal trigone. Microsurgical resection of the lesion was performed via a suboccipital median craniotomy. The histopathological diagnosis was a cavernoma. Promptly after surgery the patient was free of symptoms. Intractable hiccups can be associated with intracranial pathologies, including lesions in the brain stem, which we highlight with the presentation of this patient. Ó 2009 Elsevier Ltd. All rights reserved.
1. Case report A 33-year-old otherwise healthy man was admitted with intractable hiccups that had persisted for several months. The hiccups had started suddenly with a frequency of 10 to 20 hiccups per minute. The patient’s medical history included surgery for otitis media and a thoracic spine fracture due to an accident at work. No medication had been taken before the onset of the hiccups. On physical examination, the patient displayed no neurological deficit; in par* Corresponding author. Tel.: +49 621 3832360; fax: +49 621 3832004. E-mail address: [email protected] (I. Pechlivanis).
ticular, the function of the cranial nerves was normal. Blood tests, including detailed analysis of infection parameters, showed no abnormalities. To rule out gastroabdominal causes for the hiccups ultrasound of the abdomen and gastroscopy were performed, revealing no pathology. The size and the configuration of the diaphragm were normal on chest X-ray; furthermore, no tumor was detected. Since the etiology remained unclear, the patient was treated pharmacologically with gabapentin for several weeks. Although the frequency of the hiccups decreased intermittently to 5–10 per minute, paresthesia of the left side of the body developed. For this reason, cranial MRI of the brain was performed. MRI of the posterior fossa demonstrated a small lesion in the rhomboid
782
Case Reports / Journal of Clinical Neuroscience 17 (2010) 781–783
fossa, close to the right vagal trigone (Fig. 1). A small hemosiderin ring was seen on T2-weighted MRI. Digital subtraction angiography excluded an arteriovenous malformation. Minimally invasive microsurgical resection of the lesion was performed via a suboccipital median craniotomy. After incision of the rhomboid fossa directly above the lesion, a pinkish vascular tumor was removed completely. The histopathological diagnosis was cavernoma. Immediately after surgery the patient was free of symptoms without any new neurological deficit. At follow-up after 3 months, no further episodes of hiccups had occurred and no sensory paresthesia was reported. MRI of the brain showed no further lesions. 2. Discussion Hiccups, or singultus, is a polysynaptic reflex comprising a synchronous, repeated, myoclonic contraction of the diaphragm. The typical ‘‘hiccup” sound is produced by the sudden involuntary closure of the glottis at inspiration.
Fig. 1. (A) Axial and (B) sagittal T2-weighted MRI showing a small lesion in the right rhomboid fossa (arrows), irritating the ‘‘hiccup center”.
In a healthy person, hiccups is usually a self-limiting reflex of short duration. Frequent triggers are abrupt gastric extension (such as on consumption of heavy meals or sparkling drinks), alcohol, heavy smoking or psychogenic factors (stress, shock). Hiccups may continue for days, weeks, or even years, and whenever the episode extends beyond 48 hours, it is termed ‘‘persistent”. The afferent part of the reflex arc includes sensory branches of the phrenic, glossopharyngeal and vagus nerves, together with fibers of the thoracic sympathetic nervous system (T6–T12), while the main efferent output is to the phrenic nerve, resulting in a spastic contraction of the diaphragm.1,2 The central connection of this reflex in the brainstem and midbrain is between the dorsal portion of the nucleus tractus solitarius, the ventral part of the nucleus ambiguus, phrenic nerve nuclei, the medullary reticular formation and the hypothalamus. Furthermore, supratentorial areas participate in the genesis of hiccups by stimulation or inhibition of this reflex arc.3 However, the exact central genesis of hiccups is still unknown: Hassler et al.4 assumed generation of hiccups in the myoclonic triangle of Guillan-Mollaret (inferior olive, dentate nucleus, red nucleus), while others postulate a dysfunction of the inferior olivary complex, the nucleus ambiguus and the adjacent reticular formation of the medulla oblongata.5 Furthermore, in experimental animal studies the supraspinal polysynaptic center was found to be located in the medullary formation lateral to the nucleus ambiguus at the rostrocaudal level, between 1.0 mm and 2.5 mm rostral to the obex.6 The primary treatment of hiccups should comprise normal physical maneuvers such as breath-holding, drinking of cold water or application of supraorbital pressure. If hiccuping persists, pharmacological management should be considered. Many pharmacological treatments have been proposed for intractable hiccups, and there is no consensus regarding the best option. The approaches proposed include: antagonizing dopamine receptors (chlorpromazine or metoclopramide), increasing an inhibiting gamma-aminobutyric acid effect (baclofen, valproic acid or gabapentin), stabilizing neural membranes (nifedipine [Ca2+] or carbamazepine [Na+]) or relaxing muscles (orphenadrine). Other medications, including antidepressants, anticholinergics, prokinetic drugs and steroids, have also been used. Gabapentin is safe, lacks serious side effects, and has a rapid onset of action in the treatment of intractable hiccups. Nevertheless, pharmacological resolution of persistent hiccups could not be achieved in our patient. Diagnostic clarification of intractable hiccups is mandatory. The most effective (and in some cases the only possible) treatment is to eliminate the cause. Although the etiology of persistent hiccup may be metabolic (uremia, sepsis), pharmacological (methyldopa) or psychological (hysteria), factors causing compression or irritation of the phrenic or vagus nerve (such as tumor, trauma, infection or foreign bodies) have to be excluded (Table 1).7 Central causes for intractable hiccup as the predominant symptom are rare, but not unknown: multiple sclerosis in the lower brainstem is one of the most frequently mentioned reasons for persistent intractable hiccups,8 while other central inflammatory causes include neuromyelitis optica. Cerebral infarction in the dorsal and lateral medulla has been found in patients with intractable hiccups as well as primary medullary hemorrhage.3 In all of these patients hiccups is triggered by direct involvement of the central components, whereas compression may be the mechanism in patients with intracranial tumors. Intracranial neoplasms causing intractable hiccups have been reported: lowgrade glioma,9 ependymoma,10 choroid plexus papilloma of the fourth ventricle,11 hemangioblastoma12 or cerebral tuberculoma3 in the brainstem. Besides neoplastic or inflammatory diseases, Chiari I deformation or vascular anomalies such as aneurysm or
Case Reports / Journal of Clinical Neuroscience 17 (2010) 783–785
783
Table 1 Summary of potential causes of persistent hiccups Factor Irritation of reflex arc (brainstem, vagal, phrenic nerve) Tumor Trauma Ischemia Infection
Foreign body Drugs Operation/anesthesia Metabolic or toxic Psychological Idiopathic
Example
Brainstem, cervical spinal cord, mediastinum, thoracic, pulmonary, gastric, lymphadenopathies, hematoma, pancreatic pseudocysts Head, spine, abdominal trauma, intracranial hemorrhage Myocardial infarction, angina pectoris, brainstem ischemia Meningitis, encephalitis, multiple sclerosis, neuritis optica pharyngitis, laryngitis, mediastinitis, esophagitis, pneumonia, pleuritis, pleural empyema, peritonitis, pancreatitis, hepatitis, abscesses (cerebral, mediastinum, thoracic, phrenic, abdominal) Foreign bodies in acoustic canal Steroids, anesthetics, sulfonamides, barbiturates, benzodiazepine, methyldopa, morphine Mechanical or pharmacological irritation during surgery, intubation, ventilation Uremia, hypocapnia, hypokalemia, hypocalcemia, hyponatremia, carbon dioxide deficit, blood-glucose dysregulation, alcohol, fever, sepsis Sorrow, hysteria, personality disorder, anorexia nervosa No causative factor
dolichoectasia have caused hiccups by compression of the involved centers.10,13–15 To date, however, only one patient with a cavernous angioma triggering intractable hiccup has been reported to our knowledge: Musumeci et al. describe a cavernous angioma located near the obex of the medulla oblongata, with dorsal exophytic growth in the fourth ventricle. Intractable hiccups was the main symptom.16 Although the tumor compressed multiple parts of the lower rhomboid fossa, the authors postulated that in their patient, hiccups was due to the effect on the nucleus raphe magnus and the hiccup centers near the obex. However, due to the lesser size of the lesion, in our patient the ‘‘hiccup center” could be postulated to be close to the vagal trigone, interfering with these centers. We believe that MRI of the brain is mandatory in patients with intractable hiccups, if no other cause has been found. Furthermore, if treatment is possible at acceptable risk we would recommend microsurgery for small lesions, when other treatments have failed and the location implies involvement of hiccup centers.
5. 6.
7.
8. 9. 10.
11.
12.
13.
References 14. 1. Kondo T, Toyooka H, Arita H. Hiccup reflex is mediated by pharyngeal branch of glossopharyngeal nerve in cats. Neurosci Res 2003;47:317–21. 2. Lewis JH. Hiccups: causes and cures. J Clin Gastroenterol 1985;7:539–52. 3. al Deeb SM, Sharif H, al Moutaery K, et al. Intractable hiccup induced by brainstem lesion. J Neurol Sci 1991;103:144–50. 4. Hassler R. Die neuronalen systeme der extrapyramidalen myoclonien und deren stereotaktische behandlung [The neuronal systems of extrapyramidal
15. 16.
myoclonia and their stereotactic treatment]. In: Doose H, editor. Aktuelle neuropädiatrie. Stuttgart: Thieme; 1977. p. 20–46. [in German]. de la Fuente-Fernandez R. [Hiccup and dysfunction of the inferior olivary complex]. Med Clin (Barc) 1998;110:22–4. Oshima T, Sakamoto M, Tatsuta H, et al. GABAergic inhibition of hiccup-like reflex induced by electrical stimulation in medulla of cats. Neurosci Res 1998;30:287–93. Andresen V, Layer P. Schluckauf [Hiccups], In: Domschke W, Berger M, Hohenberger W, et al., Editors. Therapie-handbuch. Loseblattwerk mit onlinezugang [Handbook of therapies. Collected edition with online access]. Munich: Elsevier, Urban & Fischer; 2006. p. 1–5 [in German]. Witoonpanich R, Pirommai B, Tunlayadechanont S. Hiccups and multiple sclerosis. J Med Assoc Thai 2004;87:1168–71. Fischer AQ, McLean Jr WT. Intractable hiccups as presenting symptom of brainstem tumor in children. Childs Brain 1982;9:60–3. Amirjamshidi A, Abbassioun K, Parsa K. Hiccup and neurosurgeons: a report of 4 rare dorsal medullary compressive pathologies and review of the literature. Surg Neurol 2007;67:395–402. Marsot-Dupuch K, Bousson V, Cabane J, et al. Intractable hiccups: the role of cerebral MR in cases without systemic cause. AJNR Am J Neuroradiol 1995;16: 2093–100. Nagayama T, Kaji M, Hirano H, et al. Intractable hiccups as a presenting symptom of cerebellar hemangioblastoma. Case report. J Neurosurg 2004;100: 1107–10. Seki T, Hida K, Lee J, et al. Hiccups attributable to syringobulbia and/or syringomyelia associated with a Chiari I malformation. Neurosurgery 2004;54:224–6 [discussion 226–7]. Vanamoorthy P, Kar P, Prabhakar H. Intractable hiccups as a presenting symptom of Chiari I malformation. Acta Neurochir (Wien) 2008;150: 1207–8. Li ML, Gupta A, Thomas P, et al. Basilar artery aneurysm: an unusual cause of intractable hiccups. Hosp Med 2000;61:868–9. Musumeci A, Cristofori L, Bricolo A. Persistent hiccup as presenting symptom in medulla oblongata cavernoma: a case report and review of the literature. Clin Neurol Neurosurg 2000;102:13–7.
doi:10.1016/j.jocn.2009.08.022
Transvenous embolization of an intraorbital arteriovenous fistula using Onyx Chung-Jung Lin a,c,d,*, Raphael Blanc a,b, Frédéric Clarençon a,b, Michel Piotin a, Laurent Spelle a, Marc Williams b, Jacques Moret a a
Department of Functional and Interventional Neuroradiology, Fondation Rothschild Hospital, University of Paris 7, Bichat School of Medicine, 25–29 rue Manin, Paris 75940, France Department of Diagnostic Neuroradiology, Fondation Rothschild Hospital, University of Paris, Bichat School of Medicine, Paris, France Department of Medical Imaging, Far Eastern Memorial Hospital, Pan-Chiao City, Taiwan d National Yang Ming University, School of Medicine, Taipei, Taiwan b c
* Corresponding author. Tel.: +886 2 89514571; fax: +886 2 28676701. E-mail address: [email protected] (C.-J. Lin).