- Email: [email protected]
A patient with Wallenberg’s syndrome induced by severe cough
Case report
A patient with Wallenberg’s syndrome induced by severe cough Masahiro Nomura1, Seiji Kannuki2, Kazuyuki Kuwayama2, Yukio Kohyama2, Yoshinori Hayashi3, Erika Yamamoto4, Tadayoshi Kaji4, Kohzou Uehara1, Akiyoshi Nishikado1, Susumu Ito1, Yutaka Nakaya5, Sinji Nagahiro6 1 Department of Digestion and Cardiovascular Medicine, University of Tokushima, Tokushima, Japan, 2Department of Neurosurgery, Takamatsu City Hospital, Takamatsu, Japan, 3Department of Radiology, Takamatsu City Hospital, Takamatsu, Japan, 4Department of Rehabilitation, Takamatsu City Hospital, Takamatsu, Japan, 5Department of Nutrition, University of Tokushima, Tokushima, Japan, 6Department of Neurosurgery, School of Medicine, University of Tokushima, Tokushima, Japan
Summary A 45-year-old man developed severe cough with cervical pain. The patient was unable to hold an upright position. The origin of the right posterior inferior cerebellar artery was not enhanced by angiography. MRI showed a high signal intensity string-like structure of the right vertebral artery. In young patients, Wallenberg’s syndrome related to mild head trauma has been reported. However, none of the previous studies related to vertebral arterial dissection was induced by severe cough. When cervical pain is present in young patients with severe cough, MRI should be performed to evaluate the possibility of vertebral arterial dissection. ª 2003 Elsevier Ltd. All rights reserved. Journal of Clinical Neuroscience (2004) 11(2), 179–182 0967-5868/$ - see front matter ª 2003 Elsevier Ltd. All rights reserved. doi:10.1016/S0967-5868(03)00139-5
Keywords: Wallenberg syndrome, cough, MRI, posterior inferior cerebellar artery Received 18 February 2003 Accepted 25 April 2003 Correspondence to: Masahiro Nomura MD, PHD, Department of Digestion and Cardiovascular Medicine, University of Tokushima, 2-50 Kuramoto-cho, Tokushima 770-8503, Japan. Tel.: +81-88-633-7124; Fax: +81-88-633-9235; E-mail: [email protected]
CASE REPORT A 45-year-old man was admitted to the Department of Neurosurgery, Emergency Outpatient Unit, Takamatsu City Hospital, with gait disturbance, right posterior cervical pain, dysphagia, and hoarseness of voice. The patient was a medical doctor who had been well until 2 weeks earlier, when he was infected with influenza and had a high fever (>38 C) and pharyngalgia. The symptoms subsided in a few days. However, a dry cough persisted for 2 weeks. In addition, right posterior cervical pain was noted. The patient was coughing severely, with the head rotated to the left. As right posterior cervical pain persisted, the patient received an anti-inflammatory agent (diclofenac sodium). In the evening of the day of admission, dysphagia and voice hoarseness suddenly developed during meals. The patient was unable to hold an upright position. Therefore, the patient was brought by ambulance to the Department of Neurosurgery, Emergency Outpatient Unit, Takamatsu City Hospital. He had suffered from rib fracture related to cough at the age of 29 years. There was no history of hypertension or diabetes mellitus. On physical examination, consciousness was intact, pulse was 84/min regular, and blood pressure was 149/91 mmHg. On ausculation, no vascular murmurs were heard in the cervical or thoracic regions. Cerebellar ataxia of the right upper and lower limbs, paralysis of the right pharyngeal muscle, mild muscular weakness of the right upper and lower limbs, constant-direction nystagmus in the left direction, and hoarseness of voice were observed. A finger-nose test and a knee-heel test showed dysmetria on the right side. Body lateropulsion, in which the head and trunk were inclined or fell down to the right, was noted. In addition, right Horner sign and sensory dissociation of the right face and the left half of the body were noted, suggesting Wallenberg’s syndrome. There were no abnormalities of the biochemical parameters, as follows: WBC (leukocyte count) 7700/ll, RBC (erythrocyte count) 426 104 /ll, PL (platelet count) 19.4 104 /ll. Glutamic oxaloacetic transaminase (GOT) 20 IU/L, glutamic pyruvic transaminase (GPT) 21 IU/L, creatine kinase (CK) 121 IU/L, blood urea nitrogen (BUN) 15 IU/L, creatinine 0.8 mg/dl, uric acid 7.7 mg/dl, Na 143 mEq/L, K 4.4 mEq/L, total cholesterol 196 mg/ dl, HDL-cholesterol 36 mg/dl, and fasting blood sugar level 96 mg/dl. Moreover, there were no abnormalities of coagulation or fibrinolysis systems, and immunological examinations revealed no abnormal findings. Neither electrocardiography nor chest X-ray revealed any abnormalities.
Cerebral angiography
INTRODUCTION It has been reported that vertebral arterial dissection is an important etiological factor for juvenile stroke in patients of 50 years old or younger.1 In Japan, Wallenberg’s syndrome related to dissection of the intracranial vertebral basilar artery is frequently detected.2 Cerebral arterial dissection is etiologically classified into traumatic dissection and non-traumatic (idiopathic) dissection. It has been reported that in many cases of idiopathic dissection without underlying diseases, arterial dissection is associated with mild head and neck injury (chiropractic treatment, cervical massage, yoga, sports, such as swimming, golf, and skiing, and painting work-related cervical retroflexion and hyperextension) prior to onset.3–8 In the present study, we encountered a patient in whom severe cough persisted after influenza that caused dissection of the vertebral artery, inducing Wallenberg’s syndrome.
A four-vessel study was performed immediately after admission. The origin of the right posterior inferior cerebellar artery was not detected. However, the anterior inferior cerebellar artery on the right side had developed well. Furthermore, the intracranial right vertebral artery did not show any findings suggesting arterial dissection, such as pearl and string sign, or double lumen (Fig. 1). In other vascular systems, there were no arteriosclerotic changes such as stenosis or occlusion. MRI The FLAIR MRI obtained 2 h after onset did not show any high intensity areas suggesting infarcted foci (Fig. 2A). Ten hours after onset, the infarcted focus was unclear, and only a slightly high intensity was detected (Fig. 2B). On the 7th hospital day, both FLAIR and T2-weighted images revealed an infarcted focus with high intensity on the right lateral side of the lower medulla 179
180 Nomura et al.
Fig. 1 Angiography of the right vertebral artery (A: lateral view; B: A-P view). The origin of the right posterior inferior cerebellar artery was not detected. However, the ipsilateral anterior inferior cerebellar artery had developed well. Furthermore, there were no findings suggesting dissection, such as pearl and string or double lumen, in the vertebral artery. A, anterior; P, posterior; R, right; L, left.
oblongata (Fig. 2C). Furthermore, T1- and T2-weighted images showed a string-like structure, visualized as a high intensity area, on the vascular wall between the pachymeninx-penetrating lateral retrobulbar side of the right vertebral artery and the region confluent with the contralateral right vertebral artery (Fig. 3). These findings suggested that in this present patient, dissection of the right vertebral artery involving the intracranial pachymeninxpenetrating region and confluence region led to occlusion of the right posterior inferior cerebellar artery and hence Wallenberg’s syndrome. We investigated the possibility of cardiogenic cerebral embolism, which may cause non-arteriosclerotic cerebral infarction, and autoimmune disease- or angitis-related cerebral arterial occlusion. However, these disorders were ruled out.
Clinical course after admission Based on brain computed tomography (CT) and MRI findings, the presence of subarachnoid hemorrhage related to arterial dissection was ruled out. Intravenous heparin (9000 U/day), sodium ozagrel (80 mg/day), and edaravone (60 mg/day) was started. Two weeks after onset, intravenous therapy was switched to oral administration of aspirin (81 mg/day). Cervical pain disappeared 10 days after onset. After admission, blood pressure had increased (160–170/80–100 mmHg), and amlodipine besilate (5 mg/ day) and perindopril erbumine (4 mg/day) were orally administered. Two weeks or more after onset, blood pressure was controlled, and administration of these hypertensive agents was discontinued. Rehabilitation improved cerebellar ataxia of the right upper and lower limbs and the mild muscular weakness of the right upper and lower limbs gradually, over 3 months, and these disorders no longer affected his daily life. However, a sensory dissociation of the left half of the body persisted. A videofluoroscopic examination of swallowing (March 29, 2002) was performed to evaluate dysphagia related to paralysis of the right pharyngeal muscle. Dilatation of the right ring-like pharyngeal muscle was insufficient. Subsequently, Mendelzone’s procedure, tongue protrusion-swallowing training, and the balloon catheter training method facilitated taking standard diets. In addition, speech therapy, paramedian fixation of the vocal cord on the affected side, and compensatory displacement of the unaffected-side vocal cord toward the affected side improved hoarseness of voice related to right vocal cord paralysis. Journal of Clinical Neuroscience (2004) 11(2)
DISCUSSION In the present study, we report a patient in whom a continuous external force related to cough caused vertebral arterial dissection, who showed symptoms typical of Wallenberg’s syndrome. Previous studies have reported that mild trauma or a cervical rotation movement-related external force can cause arterial dissection in the cervical blood vessels in young patients. However, none of the previous studies have reported cough-related intracranial vertebral arterial dissection. The etiology of vertebral basilar arterial dissection has been reported to involve underlying diseases characterized by vascular fragility (e.g., fibromuscular dysplasia, SLE, atherosclerosis, Marfan’s syndrome, Ehler’s Danlos syndrome) or mild head and neck trauma.9 The pathogenesis of vertebral basilar arterial dissection is assumed to be as follows: (1) cervical rotation movement extends the vertebral artery between the V2 and V3 portions of the atlas contralateral to the rotation direction, and the external force readily induces dissection and (2) in the structure of the vertebral artery, the muscle layer is thin in the media of the intradural artery, and the volume of elastic fibers is small. Furthermore, media thickness rapidly becomes thin at the pachymeninx-penetrating region. Therefore, the media of the intracranial vertebral artery readily dissects.10;11 In addition, the cervical vertebrae-supporting muscle and ligaments are insufficient, although extension, flexion, and rotation are possible in the cervical vertebrae. Therefore, an external force may easily affect this region, and rupture of the intima and thrombus formation may be induced when the cervix is rapidly twisted or strongly flexed.12 The patient had a past history of cough-related rib fracture. The cough-related external force to the cervix might have been relatively great. When the cervix was rotated to the left and severe cough repeatedly occurred, a very slight mechanical external force may have been repeatedly added to the right vertebral artery, causing vascular dissection along with an instant increase in blood pressure related to cough. Vertebral basilar arterial dissection frequently causes headache and cervical pain. However, the reported interval between dissection and the appearance of neurological symptoms has varied among previous studies. Most patients develop neurological symptoms immediately or within 24 h after onset. However, some patients develop these symptoms a few weeks after onset.9;13–17 Our patient had a 2-week history of cervical pain. Repeated cough-related mild vascular injury may have gradually promoted vascular wall disorder and thrombus attachment, resulting in the appearance of the symptoms. ª 2003 Elsevier Ltd. All rights reserved.
Wallenberg’s syndrome following cough 181
Fig. 2 Serial changes in the infarcted lesion (FLAIR MRI images). (A) (2 h after onset): On a T2-weighted MRI image, there were no high intensity areas suggesting infarction. (B) (10 h after onset, March 22, 2002): On the right lateral side of the lower medulla oblongata, a small high intensity area was suggested, but was not clear. (C) (March 28, 2002): Seven days after onset, a high intensity area was clearly detected on the right lateral side of the lower medulla oblongata, suggesting an infarcted focus. L: left.
It is difficult to diagnose vertebral basilar arterial dissection. Direct findings, such as string sign, pearl reaction, string and pearl sign, double lumen, intimal flap, and intramural pooling sign, are rarely obtained, although cerebral angiography is useful. In the presented patient, there were no findings suggesting arterial dissection other than the finding that the right posterior inferior cerebellar artery was not visualized. Blood vessels branching to the cerebellum between the vertebral artery and the basilar artery, include the superior cerebellar artery, anterior inferior cerebellar artery, and posterior inferior cerebellar artery. The branching morphology of these vessels varies, and the possibility of normal variation cannot be ruled out based on the finding that only the right posterior inferior cerebellar artery is not visualized, as observed in the presented patient. Noninvasive MRI may be useful for definitively diagnosing cerebral arterial dissection.6;18–21 It has been reported that an intramural thrombus suggesting vertebral basilar arterial dissection on T1-weighted images and an intimal flap/double lumen on T2weighted images are more frequently detected by MRI than by cerebral angiography. Cerebral infarction is generally visualized as a high signal intensity area on T2-weighted MRI images 6 h after onset. However, in the presented patient, cerebral infarction was unclear on MRI images even 10 h after onset. In the presented patient, a relatively large volume of residual blood flow on the lateral side of the medulla oblongata, in which the infarcted focus developed, may have caused infarction to progress more slowly than is typical, considering that the anterior inferior cerebellar artery was advanced, and that the reflux area in the posterior inferior cerebellar artery region, other than the area on the lateral side of medulla oblongata where the infarcted focus developed, was compensated by collateral circulation from the anterior inferior cerebellar artery. Since the volume of residual blood flow in the ischemic focus influences the rate of infarcted focus formation, repeated follow-up MRI examinations may be important to clarify pathogenesis at the corresponding site, where there is a marked difference in development between the left and right vertebral arteries, the branching morphology markedly varies, and infarcted foci are localized. The diagnosis of infarction and arterial dissection was not difficult if the diffusion weighted image and 3-D rotational angiography were performed. Unfortunately, we could not perform the study by diffusion weighted image and 3-D rotational angiography, because of no available equipment in our hospital. When the vertebral artery dissection is revealed, the external force to the neck, rotation of the neck and the rise of blood pressure should be
Fig. 3 MRI of right vertebral arterial dissection (T1-weighted MRI coronary sections, 10 h after onset, March 22, 2002). T1-weighted images showed a string-like structure, visualized as a high signal intensity area (arrow), on the vascular wall of the right vertebral artery between the lateral side of medulla oblongata and the contralateral confluence region. L: left.
ª 2003 Elsevier Ltd. All rights reserved.
Journal of Clinical Neuroscience (2004) 11(2)
182 Morgan et al.
avoided as much as possible. Moreover, the serial observation of clinical course by MRI is recommended. Some patients with intracranial vertebral arterial dissection develop subarachnoid hemorrhage, while others develop cerebral infarction. Even in patients with cerebral infarction, headache, and cervical pain are frequently observed prior to onset, as demonstrated in our patient. MRI detects vertebral arterial dissection more frequently than does cerebral arterial angiography. When headache and cervical pain are observed in young people with severe cough, MRI should be performed to evaluate the presence or absence of vertebral artery dissection. REFERENCES 1. Hart RG. Vertebral artery dissection. Neurology 1988; 38: 987–989. 2. Hosoya T, Nagahata M, Yamaguchi K. Prevalence of vertebral artery dissection in Wallenberg syndrome: neuroradiological analysis of 93 patients in the Tohoku District, Japan. Radiat Med 1996; 14: 241–246. 3. Pratt-Thomas HR, Berger KE. Cerebellar and spinal injuries after chiropractic manipulation. JAMA 1947; 133: 600–603. 4. Krueger BR, Okazaki H. Vertebral-basilar distribution infarction following chiropractic cervical manipulation. Mayo Clin Proc 1980; 55: 322–332. 5. Levy RL, Dugan TM, Bernat JL, Keating J. Lateral medullary syndrome after neck injury. Neurology 1980; 30: 788–790. 6. Kitanaka C, Tanaka J, Kuwahara M, Teraoka A. Magnetic resonance imaging study of intracranial vertebrobasilar artery dissection. Stroke 1994; 25: 571–575. 7. Mokri B, Houser OW, Sandok BA, Piepgras DG. Spontaneous dissections of the vertebral arteries. Neurology 1988; 38: 880–885. 8. Hosoya T, Watanabe N, Yamaguchi K, Kubota H, Onodera Y. Intracranial vertebral artery dissection in Wallenberg syndrome. Am J Neuroradiol 1994; 15: 1161–1165. 9. Mas JL, Bousser MG, Hasboun D, Laplane D. Extracranial vertebral artery dissections: a review of 13 cases. Stroke 1987; 18: 1037–1047. 10. Wiikinson IMS. The vertebral artery: Extracranial and intracranial structure. Arch Neurol 1972; 27: 392–396. 11. Yonas H, Agamanolis D, Takaoka Y, White RJ. Dissecting intracranial aneurysms. Surg Neurol 1977; 8: 407–415. 12. Carpenter S. Injury of neck as cause of vertebral artery thombosis. J Neurosurg 1961; 18: 849–853. 13. Sturzenegger M. Headache and neck pain: The warning symptoms of vertebral artery dissection. Headache 1994; 34: 187–193. 14. Silbert PL, Mokri B, Schievink WI. Headache and neck pain in spontaneous internal carotid and vertebral artery dissection. Neurology 1995; 45: 1517–1522. 15. Caplan LR, Zarins CK, Hemmati M. Spontaneous dissection of the extracranial vertebral arteries. Stroke 1985; 16: 1030–1038. 16. Caplan LR. In: Posterior circulation disease: clinical findings, diagnosis, and management. Blackwell Science, Cambridge 1996; 262–323. 17. Silbert PL, Mokri B, Schievink WI. Headache and neck pain in spontaneous internal carotid and vertebral artery dissections. Neurology 1995; 45: 1517–1522. 18. Quint DJ, Spicker EM. Magnetic resonance demonstration of vertebral artery dissection. Report of two cases. J Neurosurg 1990; 72: 965–967. 19. Provenzale JM. Dissection of the internal carotid and vertebral arteries: Imaging features. Am J Radiol 1995; 165: 1099–1104. 20. Zuber M, Meary E, Meder JF, Mas JL. Magnetic resonance imaging and dynamic CT scan in cervical artery dissections. Stroke 1994; 25: 576–581. 21. Yoshimoto Y. Unruptured intracranial vertebral artery dissection: clinical course and serial radiographic imagings. Stroke 1997; 28: 370–374.
A case study of the resolution of paediatric dysphagia following brainstem injury: clinical and instrumental assessment Angela Morgan1, Elizabeth Ward2, Bruce Murdoch3 Journal of Clinical Neuroscience (2004) 11(2)
1
Department of Speech Pathology and Audiology, University of Queensland, St. Lucia, Brisbane, Qld. 4072, Australia, 2Department of Speech Pathology and Audiology, University of Queensland, St. Lucia, Brisbane, Qld. 4072, Australia, 3Head of School of Health and Rehabilitation Sciences, University of Queensland, St. Lucia, Brisbane, Qld. 4072, Australia
Summary The coexistance of a swallowing impairment can severely impact upon the medical condition and recovery of a child with traumatic brain injury [ref.1 : Journal of Head Trauma Rehabilitation 9 (1) (1994) 43]. Limited data exist on the progression or outcome of dysphagia in the paediatric population with brainstem injury. The present prospective study documents the resolution of dysphagia in a 14-year-old female post-brainstem injury using clinical, radiological and endoscopic evaluations of swallowing. The subject presented with a pattern of severe oral-motor and oropharyngeal swallowing impairment post-injury that resolved rapidly for the initial 12 weeks, slowed to gradual progress for weeks 12–20, and then plateaued at 20 weeks post-injury. Whilst a clinically functional swallow was present at 10 months post-injury, radiological examination revealed a number of residual physiological impairments, reduced swallowing efficiency, and reduced independence for feeding, indicating a potential increased risk for aspiration. The data highlight the need for early and continued evaluation and intensive treatment programs, to focus on the underlying physiological swallowing impairment postbrainstem injury, and to help offset any potential deleterious effects of aspiration that may affect patient recovery, such as pneumonia. ª 2003 Elsevier Ltd. All rights reserved. Journal of Clinical Neuroscience (2004) 11(2), 182–190 0967-5868/$ - see front matter ª 2003 Elsevier Ltd. All rights reserved. doi:10.1016/S0967-5868(03)00195-4
Received 27 March 2003 Accepted 11 June 2003 Correspondence to: Angela Morgan, Department of Speech Pathology and Audiology, University of Queensland, St. Lucia 4072, Brisbane, Qld., Australia. Tel.: +61-7-3365-6149; Fax: +61-7-3365-1877; E-mail: [email protected]
INTRODUCTION Oral intake can be markedly reduced by dysphagia following traumatic brain injury (TBI), subsequently impacting upon the patient’s nutritional status and compromising the recovery process.2 The impact of dysphagia on recovery in children post-TBI is of particular concern given that children already have additional energy requirements in order to ensure continued growth, and thus have added difficulties maintaining nutrition in the hypermetabolic state.3;4 The extent to which the recovery process post-TBI is impacted upon by dysphagia will also depend largely upon the degree or severity of the swallowing impairment. The nonhomogenous neuropathology of traumatic brain injury results in variability within the degree or severity of swallowing deficits noted post-injury. Rowe5 suggested that a general knowledge of the underlying pathology of the neurological injury can assist in management decisions following dysphagia post-TBI. One particular neuropathology that has been found to commonly result in dysphagia is brainstem injury.6 Furthermore, brainstem injury has been reported to result in more debilitating and persistent swallowing deficits than other forms of brain injury.7;8 Despite this knowledge, there is very little data available documenting the resolution of dysphagia subsequent to brainstem injury in the paediatric population.5 There have been a number of paediatric studies reporting the general characteristics and resolution of dysphagia in neurological ª 2003 Elsevier Ltd. All rights reserved.
A patient with Wallenberg’s syndrome induced by severe cough Masahiro Nomura1, Seiji Kannuki2, Kazuyuki Kuwayama2, Yukio Kohyama2, Yoshinori Hayashi3, Erika Yamamoto4, Tadayoshi Kaji4, Kohzou Uehara1, Akiyoshi Nishikado1, Susumu Ito1, Yutaka Nakaya5, Sinji Nagahiro6 1 Department of Digestion and Cardiovascular Medicine, University of Tokushima, Tokushima, Japan, 2Department of Neurosurgery, Takamatsu City Hospital, Takamatsu, Japan, 3Department of Radiology, Takamatsu City Hospital, Takamatsu, Japan, 4Department of Rehabilitation, Takamatsu City Hospital, Takamatsu, Japan, 5Department of Nutrition, University of Tokushima, Tokushima, Japan, 6Department of Neurosurgery, School of Medicine, University of Tokushima, Tokushima, Japan
Summary A 45-year-old man developed severe cough with cervical pain. The patient was unable to hold an upright position. The origin of the right posterior inferior cerebellar artery was not enhanced by angiography. MRI showed a high signal intensity string-like structure of the right vertebral artery. In young patients, Wallenberg’s syndrome related to mild head trauma has been reported. However, none of the previous studies related to vertebral arterial dissection was induced by severe cough. When cervical pain is present in young patients with severe cough, MRI should be performed to evaluate the possibility of vertebral arterial dissection. ª 2003 Elsevier Ltd. All rights reserved. Journal of Clinical Neuroscience (2004) 11(2), 179–182 0967-5868/$ - see front matter ª 2003 Elsevier Ltd. All rights reserved. doi:10.1016/S0967-5868(03)00139-5
Keywords: Wallenberg syndrome, cough, MRI, posterior inferior cerebellar artery Received 18 February 2003 Accepted 25 April 2003 Correspondence to: Masahiro Nomura MD, PHD, Department of Digestion and Cardiovascular Medicine, University of Tokushima, 2-50 Kuramoto-cho, Tokushima 770-8503, Japan. Tel.: +81-88-633-7124; Fax: +81-88-633-9235; E-mail: [email protected]
CASE REPORT A 45-year-old man was admitted to the Department of Neurosurgery, Emergency Outpatient Unit, Takamatsu City Hospital, with gait disturbance, right posterior cervical pain, dysphagia, and hoarseness of voice. The patient was a medical doctor who had been well until 2 weeks earlier, when he was infected with influenza and had a high fever (>38 C) and pharyngalgia. The symptoms subsided in a few days. However, a dry cough persisted for 2 weeks. In addition, right posterior cervical pain was noted. The patient was coughing severely, with the head rotated to the left. As right posterior cervical pain persisted, the patient received an anti-inflammatory agent (diclofenac sodium). In the evening of the day of admission, dysphagia and voice hoarseness suddenly developed during meals. The patient was unable to hold an upright position. Therefore, the patient was brought by ambulance to the Department of Neurosurgery, Emergency Outpatient Unit, Takamatsu City Hospital. He had suffered from rib fracture related to cough at the age of 29 years. There was no history of hypertension or diabetes mellitus. On physical examination, consciousness was intact, pulse was 84/min regular, and blood pressure was 149/91 mmHg. On ausculation, no vascular murmurs were heard in the cervical or thoracic regions. Cerebellar ataxia of the right upper and lower limbs, paralysis of the right pharyngeal muscle, mild muscular weakness of the right upper and lower limbs, constant-direction nystagmus in the left direction, and hoarseness of voice were observed. A finger-nose test and a knee-heel test showed dysmetria on the right side. Body lateropulsion, in which the head and trunk were inclined or fell down to the right, was noted. In addition, right Horner sign and sensory dissociation of the right face and the left half of the body were noted, suggesting Wallenberg’s syndrome. There were no abnormalities of the biochemical parameters, as follows: WBC (leukocyte count) 7700/ll, RBC (erythrocyte count) 426 104 /ll, PL (platelet count) 19.4 104 /ll. Glutamic oxaloacetic transaminase (GOT) 20 IU/L, glutamic pyruvic transaminase (GPT) 21 IU/L, creatine kinase (CK) 121 IU/L, blood urea nitrogen (BUN) 15 IU/L, creatinine 0.8 mg/dl, uric acid 7.7 mg/dl, Na 143 mEq/L, K 4.4 mEq/L, total cholesterol 196 mg/ dl, HDL-cholesterol 36 mg/dl, and fasting blood sugar level 96 mg/dl. Moreover, there were no abnormalities of coagulation or fibrinolysis systems, and immunological examinations revealed no abnormal findings. Neither electrocardiography nor chest X-ray revealed any abnormalities.
Cerebral angiography
INTRODUCTION It has been reported that vertebral arterial dissection is an important etiological factor for juvenile stroke in patients of 50 years old or younger.1 In Japan, Wallenberg’s syndrome related to dissection of the intracranial vertebral basilar artery is frequently detected.2 Cerebral arterial dissection is etiologically classified into traumatic dissection and non-traumatic (idiopathic) dissection. It has been reported that in many cases of idiopathic dissection without underlying diseases, arterial dissection is associated with mild head and neck injury (chiropractic treatment, cervical massage, yoga, sports, such as swimming, golf, and skiing, and painting work-related cervical retroflexion and hyperextension) prior to onset.3–8 In the present study, we encountered a patient in whom severe cough persisted after influenza that caused dissection of the vertebral artery, inducing Wallenberg’s syndrome.
A four-vessel study was performed immediately after admission. The origin of the right posterior inferior cerebellar artery was not detected. However, the anterior inferior cerebellar artery on the right side had developed well. Furthermore, the intracranial right vertebral artery did not show any findings suggesting arterial dissection, such as pearl and string sign, or double lumen (Fig. 1). In other vascular systems, there were no arteriosclerotic changes such as stenosis or occlusion. MRI The FLAIR MRI obtained 2 h after onset did not show any high intensity areas suggesting infarcted foci (Fig. 2A). Ten hours after onset, the infarcted focus was unclear, and only a slightly high intensity was detected (Fig. 2B). On the 7th hospital day, both FLAIR and T2-weighted images revealed an infarcted focus with high intensity on the right lateral side of the lower medulla 179
180 Nomura et al.
Fig. 1 Angiography of the right vertebral artery (A: lateral view; B: A-P view). The origin of the right posterior inferior cerebellar artery was not detected. However, the ipsilateral anterior inferior cerebellar artery had developed well. Furthermore, there were no findings suggesting dissection, such as pearl and string or double lumen, in the vertebral artery. A, anterior; P, posterior; R, right; L, left.
oblongata (Fig. 2C). Furthermore, T1- and T2-weighted images showed a string-like structure, visualized as a high intensity area, on the vascular wall between the pachymeninx-penetrating lateral retrobulbar side of the right vertebral artery and the region confluent with the contralateral right vertebral artery (Fig. 3). These findings suggested that in this present patient, dissection of the right vertebral artery involving the intracranial pachymeninxpenetrating region and confluence region led to occlusion of the right posterior inferior cerebellar artery and hence Wallenberg’s syndrome. We investigated the possibility of cardiogenic cerebral embolism, which may cause non-arteriosclerotic cerebral infarction, and autoimmune disease- or angitis-related cerebral arterial occlusion. However, these disorders were ruled out.
Clinical course after admission Based on brain computed tomography (CT) and MRI findings, the presence of subarachnoid hemorrhage related to arterial dissection was ruled out. Intravenous heparin (9000 U/day), sodium ozagrel (80 mg/day), and edaravone (60 mg/day) was started. Two weeks after onset, intravenous therapy was switched to oral administration of aspirin (81 mg/day). Cervical pain disappeared 10 days after onset. After admission, blood pressure had increased (160–170/80–100 mmHg), and amlodipine besilate (5 mg/ day) and perindopril erbumine (4 mg/day) were orally administered. Two weeks or more after onset, blood pressure was controlled, and administration of these hypertensive agents was discontinued. Rehabilitation improved cerebellar ataxia of the right upper and lower limbs and the mild muscular weakness of the right upper and lower limbs gradually, over 3 months, and these disorders no longer affected his daily life. However, a sensory dissociation of the left half of the body persisted. A videofluoroscopic examination of swallowing (March 29, 2002) was performed to evaluate dysphagia related to paralysis of the right pharyngeal muscle. Dilatation of the right ring-like pharyngeal muscle was insufficient. Subsequently, Mendelzone’s procedure, tongue protrusion-swallowing training, and the balloon catheter training method facilitated taking standard diets. In addition, speech therapy, paramedian fixation of the vocal cord on the affected side, and compensatory displacement of the unaffected-side vocal cord toward the affected side improved hoarseness of voice related to right vocal cord paralysis. Journal of Clinical Neuroscience (2004) 11(2)
DISCUSSION In the present study, we report a patient in whom a continuous external force related to cough caused vertebral arterial dissection, who showed symptoms typical of Wallenberg’s syndrome. Previous studies have reported that mild trauma or a cervical rotation movement-related external force can cause arterial dissection in the cervical blood vessels in young patients. However, none of the previous studies have reported cough-related intracranial vertebral arterial dissection. The etiology of vertebral basilar arterial dissection has been reported to involve underlying diseases characterized by vascular fragility (e.g., fibromuscular dysplasia, SLE, atherosclerosis, Marfan’s syndrome, Ehler’s Danlos syndrome) or mild head and neck trauma.9 The pathogenesis of vertebral basilar arterial dissection is assumed to be as follows: (1) cervical rotation movement extends the vertebral artery between the V2 and V3 portions of the atlas contralateral to the rotation direction, and the external force readily induces dissection and (2) in the structure of the vertebral artery, the muscle layer is thin in the media of the intradural artery, and the volume of elastic fibers is small. Furthermore, media thickness rapidly becomes thin at the pachymeninx-penetrating region. Therefore, the media of the intracranial vertebral artery readily dissects.10;11 In addition, the cervical vertebrae-supporting muscle and ligaments are insufficient, although extension, flexion, and rotation are possible in the cervical vertebrae. Therefore, an external force may easily affect this region, and rupture of the intima and thrombus formation may be induced when the cervix is rapidly twisted or strongly flexed.12 The patient had a past history of cough-related rib fracture. The cough-related external force to the cervix might have been relatively great. When the cervix was rotated to the left and severe cough repeatedly occurred, a very slight mechanical external force may have been repeatedly added to the right vertebral artery, causing vascular dissection along with an instant increase in blood pressure related to cough. Vertebral basilar arterial dissection frequently causes headache and cervical pain. However, the reported interval between dissection and the appearance of neurological symptoms has varied among previous studies. Most patients develop neurological symptoms immediately or within 24 h after onset. However, some patients develop these symptoms a few weeks after onset.9;13–17 Our patient had a 2-week history of cervical pain. Repeated cough-related mild vascular injury may have gradually promoted vascular wall disorder and thrombus attachment, resulting in the appearance of the symptoms. ª 2003 Elsevier Ltd. All rights reserved.
Wallenberg’s syndrome following cough 181
Fig. 2 Serial changes in the infarcted lesion (FLAIR MRI images). (A) (2 h after onset): On a T2-weighted MRI image, there were no high intensity areas suggesting infarction. (B) (10 h after onset, March 22, 2002): On the right lateral side of the lower medulla oblongata, a small high intensity area was suggested, but was not clear. (C) (March 28, 2002): Seven days after onset, a high intensity area was clearly detected on the right lateral side of the lower medulla oblongata, suggesting an infarcted focus. L: left.
It is difficult to diagnose vertebral basilar arterial dissection. Direct findings, such as string sign, pearl reaction, string and pearl sign, double lumen, intimal flap, and intramural pooling sign, are rarely obtained, although cerebral angiography is useful. In the presented patient, there were no findings suggesting arterial dissection other than the finding that the right posterior inferior cerebellar artery was not visualized. Blood vessels branching to the cerebellum between the vertebral artery and the basilar artery, include the superior cerebellar artery, anterior inferior cerebellar artery, and posterior inferior cerebellar artery. The branching morphology of these vessels varies, and the possibility of normal variation cannot be ruled out based on the finding that only the right posterior inferior cerebellar artery is not visualized, as observed in the presented patient. Noninvasive MRI may be useful for definitively diagnosing cerebral arterial dissection.6;18–21 It has been reported that an intramural thrombus suggesting vertebral basilar arterial dissection on T1-weighted images and an intimal flap/double lumen on T2weighted images are more frequently detected by MRI than by cerebral angiography. Cerebral infarction is generally visualized as a high signal intensity area on T2-weighted MRI images 6 h after onset. However, in the presented patient, cerebral infarction was unclear on MRI images even 10 h after onset. In the presented patient, a relatively large volume of residual blood flow on the lateral side of the medulla oblongata, in which the infarcted focus developed, may have caused infarction to progress more slowly than is typical, considering that the anterior inferior cerebellar artery was advanced, and that the reflux area in the posterior inferior cerebellar artery region, other than the area on the lateral side of medulla oblongata where the infarcted focus developed, was compensated by collateral circulation from the anterior inferior cerebellar artery. Since the volume of residual blood flow in the ischemic focus influences the rate of infarcted focus formation, repeated follow-up MRI examinations may be important to clarify pathogenesis at the corresponding site, where there is a marked difference in development between the left and right vertebral arteries, the branching morphology markedly varies, and infarcted foci are localized. The diagnosis of infarction and arterial dissection was not difficult if the diffusion weighted image and 3-D rotational angiography were performed. Unfortunately, we could not perform the study by diffusion weighted image and 3-D rotational angiography, because of no available equipment in our hospital. When the vertebral artery dissection is revealed, the external force to the neck, rotation of the neck and the rise of blood pressure should be
Fig. 3 MRI of right vertebral arterial dissection (T1-weighted MRI coronary sections, 10 h after onset, March 22, 2002). T1-weighted images showed a string-like structure, visualized as a high signal intensity area (arrow), on the vascular wall of the right vertebral artery between the lateral side of medulla oblongata and the contralateral confluence region. L: left.
ª 2003 Elsevier Ltd. All rights reserved.
Journal of Clinical Neuroscience (2004) 11(2)
182 Morgan et al.
avoided as much as possible. Moreover, the serial observation of clinical course by MRI is recommended. Some patients with intracranial vertebral arterial dissection develop subarachnoid hemorrhage, while others develop cerebral infarction. Even in patients with cerebral infarction, headache, and cervical pain are frequently observed prior to onset, as demonstrated in our patient. MRI detects vertebral arterial dissection more frequently than does cerebral arterial angiography. When headache and cervical pain are observed in young people with severe cough, MRI should be performed to evaluate the presence or absence of vertebral artery dissection. REFERENCES 1. Hart RG. Vertebral artery dissection. Neurology 1988; 38: 987–989. 2. Hosoya T, Nagahata M, Yamaguchi K. Prevalence of vertebral artery dissection in Wallenberg syndrome: neuroradiological analysis of 93 patients in the Tohoku District, Japan. Radiat Med 1996; 14: 241–246. 3. Pratt-Thomas HR, Berger KE. Cerebellar and spinal injuries after chiropractic manipulation. JAMA 1947; 133: 600–603. 4. Krueger BR, Okazaki H. Vertebral-basilar distribution infarction following chiropractic cervical manipulation. Mayo Clin Proc 1980; 55: 322–332. 5. Levy RL, Dugan TM, Bernat JL, Keating J. Lateral medullary syndrome after neck injury. Neurology 1980; 30: 788–790. 6. Kitanaka C, Tanaka J, Kuwahara M, Teraoka A. Magnetic resonance imaging study of intracranial vertebrobasilar artery dissection. Stroke 1994; 25: 571–575. 7. Mokri B, Houser OW, Sandok BA, Piepgras DG. Spontaneous dissections of the vertebral arteries. Neurology 1988; 38: 880–885. 8. Hosoya T, Watanabe N, Yamaguchi K, Kubota H, Onodera Y. Intracranial vertebral artery dissection in Wallenberg syndrome. Am J Neuroradiol 1994; 15: 1161–1165. 9. Mas JL, Bousser MG, Hasboun D, Laplane D. Extracranial vertebral artery dissections: a review of 13 cases. Stroke 1987; 18: 1037–1047. 10. Wiikinson IMS. The vertebral artery: Extracranial and intracranial structure. Arch Neurol 1972; 27: 392–396. 11. Yonas H, Agamanolis D, Takaoka Y, White RJ. Dissecting intracranial aneurysms. Surg Neurol 1977; 8: 407–415. 12. Carpenter S. Injury of neck as cause of vertebral artery thombosis. J Neurosurg 1961; 18: 849–853. 13. Sturzenegger M. Headache and neck pain: The warning symptoms of vertebral artery dissection. Headache 1994; 34: 187–193. 14. Silbert PL, Mokri B, Schievink WI. Headache and neck pain in spontaneous internal carotid and vertebral artery dissection. Neurology 1995; 45: 1517–1522. 15. Caplan LR, Zarins CK, Hemmati M. Spontaneous dissection of the extracranial vertebral arteries. Stroke 1985; 16: 1030–1038. 16. Caplan LR. In: Posterior circulation disease: clinical findings, diagnosis, and management. Blackwell Science, Cambridge 1996; 262–323. 17. Silbert PL, Mokri B, Schievink WI. Headache and neck pain in spontaneous internal carotid and vertebral artery dissections. Neurology 1995; 45: 1517–1522. 18. Quint DJ, Spicker EM. Magnetic resonance demonstration of vertebral artery dissection. Report of two cases. J Neurosurg 1990; 72: 965–967. 19. Provenzale JM. Dissection of the internal carotid and vertebral arteries: Imaging features. Am J Radiol 1995; 165: 1099–1104. 20. Zuber M, Meary E, Meder JF, Mas JL. Magnetic resonance imaging and dynamic CT scan in cervical artery dissections. Stroke 1994; 25: 576–581. 21. Yoshimoto Y. Unruptured intracranial vertebral artery dissection: clinical course and serial radiographic imagings. Stroke 1997; 28: 370–374.
A case study of the resolution of paediatric dysphagia following brainstem injury: clinical and instrumental assessment Angela Morgan1, Elizabeth Ward2, Bruce Murdoch3 Journal of Clinical Neuroscience (2004) 11(2)
1
Department of Speech Pathology and Audiology, University of Queensland, St. Lucia, Brisbane, Qld. 4072, Australia, 2Department of Speech Pathology and Audiology, University of Queensland, St. Lucia, Brisbane, Qld. 4072, Australia, 3Head of School of Health and Rehabilitation Sciences, University of Queensland, St. Lucia, Brisbane, Qld. 4072, Australia
Summary The coexistance of a swallowing impairment can severely impact upon the medical condition and recovery of a child with traumatic brain injury [ref.1 : Journal of Head Trauma Rehabilitation 9 (1) (1994) 43]. Limited data exist on the progression or outcome of dysphagia in the paediatric population with brainstem injury. The present prospective study documents the resolution of dysphagia in a 14-year-old female post-brainstem injury using clinical, radiological and endoscopic evaluations of swallowing. The subject presented with a pattern of severe oral-motor and oropharyngeal swallowing impairment post-injury that resolved rapidly for the initial 12 weeks, slowed to gradual progress for weeks 12–20, and then plateaued at 20 weeks post-injury. Whilst a clinically functional swallow was present at 10 months post-injury, radiological examination revealed a number of residual physiological impairments, reduced swallowing efficiency, and reduced independence for feeding, indicating a potential increased risk for aspiration. The data highlight the need for early and continued evaluation and intensive treatment programs, to focus on the underlying physiological swallowing impairment postbrainstem injury, and to help offset any potential deleterious effects of aspiration that may affect patient recovery, such as pneumonia. ª 2003 Elsevier Ltd. All rights reserved. Journal of Clinical Neuroscience (2004) 11(2), 182–190 0967-5868/$ - see front matter ª 2003 Elsevier Ltd. All rights reserved. doi:10.1016/S0967-5868(03)00195-4
Received 27 March 2003 Accepted 11 June 2003 Correspondence to: Angela Morgan, Department of Speech Pathology and Audiology, University of Queensland, St. Lucia 4072, Brisbane, Qld., Australia. Tel.: +61-7-3365-6149; Fax: +61-7-3365-1877; E-mail: [email protected]
INTRODUCTION Oral intake can be markedly reduced by dysphagia following traumatic brain injury (TBI), subsequently impacting upon the patient’s nutritional status and compromising the recovery process.2 The impact of dysphagia on recovery in children post-TBI is of particular concern given that children already have additional energy requirements in order to ensure continued growth, and thus have added difficulties maintaining nutrition in the hypermetabolic state.3;4 The extent to which the recovery process post-TBI is impacted upon by dysphagia will also depend largely upon the degree or severity of the swallowing impairment. The nonhomogenous neuropathology of traumatic brain injury results in variability within the degree or severity of swallowing deficits noted post-injury. Rowe5 suggested that a general knowledge of the underlying pathology of the neurological injury can assist in management decisions following dysphagia post-TBI. One particular neuropathology that has been found to commonly result in dysphagia is brainstem injury.6 Furthermore, brainstem injury has been reported to result in more debilitating and persistent swallowing deficits than other forms of brain injury.7;8 Despite this knowledge, there is very little data available documenting the resolution of dysphagia subsequent to brainstem injury in the paediatric population.5 There have been a number of paediatric studies reporting the general characteristics and resolution of dysphagia in neurological ª 2003 Elsevier Ltd. All rights reserved.