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Predicting outcome from coma: man-in-the-barrel syndrome as potential pitfall
Clinical Neurology and Neurosurgery 102 (2000) 23 – 25 www.elsevier.com/locate/clineuro
Case report
Predicting outcome from coma: man-in-the-barrel syndrome as potential pitfall J.W. Elting *, R. Haaxma, G. Sulter, J. De Keyser Department of Neurology, Uni6ersity Hospital Groningen, PO Box 30.001, 9700 RB Groningen, The Netherlands Received 16 June 1999; received in revised form 5 October 1999; accepted 5 October 1999
Abstract The Glasgow coma scale motor score is often used in predicting outcome after hypoxic – ischemic coma. Judicious care should be exerted when using this variable in predicting outcome in patients with coma following hypotension since borderzone infarction can obscure the clinical picture. We describe a patient who underwent skull base surgery for a schwannoma of the left facial nerve. The operation, which lasted for 10 h, was conducted under controlled hypotension. After the intervention the patient remained comatose with absent arm movements upon painful stimuli. An absent motor score usually carries a poor prognosis. However, magnetic resonance inversion recovery imaging of the brain showed bilateral hyperintense lesions in the arm – hand area indicative of borderzone ischemic damage. The patient received optimal supportive care and after 17 days he regained consciousness with ‘man-in-the-barrel syndrome’, which also further improved over time. © 2000 Elsevier Science B.V. All rights reserved. Keywords: Glasgow coma scale; Borderzone ischemia; Man-in-the-barrel syndrome; Hypotension
1. Introduction Several authors have extensively studied which factors can predict outcome from hypoxic – ischemic coma [1– 3]. Elements of the Glasgow coma scale (GCS) seem to be very predictive on certain time points after the primary insult. For example, a motor score of 3 (abnormal flexor response) or less on day 3 is correlated with a very poor prognosis: 93% of the patients die or survive in a vegetative state. The motor score of the GCS is usually obtained from the arms by administering a painful stimulus on the nailbeds and/or on the supraorbital ridge. The legs should also be evaluated but interpretation is sometimes difficult since leg movements can be of spinal origin. There are several pitfalls to be considered, all of which could theoretically lead to misinterpretation of the GCS score. These include the presence of a peripheral neuropathy or myopathy, a cervical cord lesion, hypothermia, sedative drugs or muscle relaxants, and metabolic or endocrine causes of coma. Another potential pitfall to be considered, espe* Corresponding author.
cially in less acute forms of hypoxic–ischemic brain damage associated with hypotension, is the so-called ‘man-in-the-barrel syndrome’.
2. Case report A 61-year-old man presented with a slight asymmetry of the left side of the face and some instability during walking. Furthermore, the patient had experienced hearing loss and tinnitus of the left ear for 35 years. Magnetic resonance imaging (MRI) revealed a tumor close to the left os petrosum extending into the posterior and middle fossa and the left sinus cavernosus. The circle of Willis was intact. A transcranial doppler sonogram and a duplex of both carotid arteries were unremarkable. A preoperative electroencephalogram (EEG) with compression of the left carotid artery showed no signs of insufficient collateral circulation. The patient underwent surgery to remove the tumor, which turned out to be a schwannoma of the left facial nerve. During the 10-h operation systemic blood pressure was kept between 80–100 mmHg systolic and 40–50
0303-8467/00/$ - see front matter © 2000 Elsevier Science B.V. All rights reserved. PII: S 0 3 0 3 - 8 4 6 7 ( 9 9 ) 0 0 0 6 7 - 0
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J.W. Elting et al. / Clinical Neurology and Neurosurgery 102 (2000) 23–25
mmHg diastolic. After the intervention the patient did not react to stimuli and had to be reintubated. A GCS score was not obtained at all time. A computed tomographic (CT) scan of the brain showed slight diffuse edema and some residual hematoma in the left pontocerebellar region. When sedation was stopped the patient remained comatose but made spontaneous rotating movements with his body and legs. The arms did not react to painful stimuli applied to the nailbeds and the supraorbital ridges, and the eyes were closed. Brainstem reflexes were intact. An EEG showed diffuse alfa activity with little reactivity resembling an alfa coma. Five days after surgery a MRI of the brain was performed. The T2 inversion recovery images showed bilateral increased signals in the fronto – parietal cortex in the arm –hand area, especially on the left side (Fig. 1). There were no lesions in the brainstem. After 12 days an EEG was repeated which again showed diffuse alpha activity. Sensory evoked potentials derived from all extremities showed bilateral presence of N20 responses, late cortical responses were not obtained. On day 17 after surgery the patient was out of coma and responded to commands. There was distal paralysis of both arms with proximal paresis of the right arm (MRC grade-3) more than the left arm (MRC grade-4). Arm reflexes were brisk and there was a hypesthesia of both arms. Examination further showed on the left side a complete facial nerve palsy, deafness, and hypesthesia in the area of the second and third division of the trigeminal nerve. Cognition was not impaired. Over the next months the patient gradually improved to an MRC grade-3 distal paresis of the right arm and a grade-4 distal paresis of the left arm with hypesthesia in the fourth and fifth fingers on both sides,
Fig. 1. Axial inversion recovery T2-weighted images: bilateral hyperintense signal in the sensorimotor arm–hand area.
which we interpreted as a central sensory deficit. An EMG showed absence of a peripheral lesion.
3. Discussion The term ‘man-in-the-barrel syndrome’ is currently being used to describe a clinical picture resulting from a bilateral border-zone infarction between the anterior cerebral artery and middle cerebral artery junctions, which are usually located at the level of the arm representation on the motor cortex [4–6]. It implies a paresis or paralysis of both arms while the cranial nerves and motor function of the legs are preserved, giving the appearance of being confined to a barrel. The most common cause is systemic hypotension following cardiovascular arrest or cardiac surgery [6,7]. Other authors have reported the same syndrome after head injury [8] and cerebral metastases [9]. The syndrome can also occur with lesions in the central cervical myelum [10] and the bilateral brachial plexopathy. Sage and Van Uitert [6] prospectively studied 34 comatose patients who had an episode of systemic hypotension. Eleven of these patients developed ‘manin-the-barrel syndrome’ and ten of them died. There have been few reports of ‘man-in-the-barrel syndrome’ occurring in non-comatose patients, which is associated with a much better prognosis [11,12]. The patients described by Sage and Van Uitert [6] had a history of a hypotensive period of at least 5 min with systolic blood pressures of 50 mmHg or less. The authors concluded that a sudden decline in blood pressure is probably a prerequisite to develop the syndrome. Later reports in patients undergoing cardiac surgery yielded evidence that a more prolonged moderate hypotension could also produce this syndrome [7]. The duration and severity of hypotension needed to produce ‘man-in-the-barrel syndrome’ is not known. Individual contributing factors include previous hypertension and high-grade stenosis of the internal carotid artery on both sides. In our case manipulation at the skull base could have produced compression and traction on the cerebral arteries which further compromised bloodflow. Clinically, coma associated with ‘man-in-the-barrel syndrome’ remains a rare but important syndrome. Failure to recognise it can lead to misinterpretation of neurological signs, for example when using the GCS motor score as a factor to determine the neurological prognosis after prolonged cerebral ischemia. In several early studies on the prognosis after cerebral ischemia due to cardiac arrest, pupillary response, GCS motor score, and eye movements were the most important predictors of outcome [1,2]. More recently, somato– sensory evoked potentials have been used to predict poor outcome [13]. Prognostic schemes have originated from these studies and they are widely used in everyday
J.W. Elting et al. / Clinical Neurology and Neurosurgery 102 (2000) 23–25
practice. While these may be very valid for ‘pure cardiac arrest’ they could be less valid in situations in which there is a more mild and prolonged hypotension such as in our case. This probably explains the remarkable recovery in our patient, despite the initial coma and other possible unfavourable signs such as alfa coma. Injudicious use of such schemes could then lead to underestimation of the neurological status if one failed to observe other relevant data such as grimacing, spontaneous axial movements and leg movements. On the other hand, the interpretation of leg movements on stimulation should be careful, since they can also be of spinal origin in the severe comatose patient. Preferably, the diagnosis is confirmed with imaging techniques. In many patients however, there are normal CT scan findings [6], and MRI may be needed to confirm the diagnosis. Thus far reports of MRI scanning clearly demonstrating the lesions in the cortical arm –hand area like as in our case are scanty [6,11].
References [1] Levy DE, Caronna JJ, Singer BH, et al. Predicting outcome from hypoxic – ischemic coma. J Am Med Assoc 1985;253:1420.
.
25
[2] Longstreth WT Jr, Diehr P, Inui TS. Prediction of awakening after out-of-hospital cardiac arrest. N Engl J Med 1983;308:1378. [3] Rothstein TL, Thomas EM, Sumi SM. Predicting outcome in hypoxic – ischemic coma. A prospective clinical and electrophysiologic study. Electroencephalogr Clin Neurophysiol 1991;79(2):101– 17. [4] Mohr JP. Distal field infarction. Neurology 1969;12:279. [5] Van der Zwan A, Hillen B. Review of the variability of the territories of the major cerebral. Stroke 1991;22:1078 – 84. [6] Sage JI, Van Uitert RL. Man-in-the-barrel syndrome. Neurology 1986;36:1102 – 3. [7] Hurley JP, Wood AE. Man-in-the-barrel syndrome following cardiac surgery. Thorac Cardiovasc Surg 1993;41:252 –4. [8] Crisostomo EA, Suslavich FJJ. Man-in-the-barrel syndrome associated with closed head injury. Neuroimaging 1994;4:116–7. [9] Moore AP, Humphrey PRD. Man-in-the-barrel syndrome caused by cerebral metastases. Neurology 1989;39:1134–5. [10] Berg D, Mullges W, Koltzenburg M, Bendszus M, Reiners K. Man-in-the-barrel syndrome caused by cervical spinal cord infarction. Acta Neurol Scand 1998;97:417 – 9. [11] Olejniczak PG, Ellenberg MR, Eilender LM, Muszynski CT. Man-in-the-barrel syndrome in a noncomatose patient: a case report. Arch Phys Med Rehabil 1991;72:1021 – 3. [12] Benito-Leon J, Munoz A, Ruiz J, Gomez-Fuentes JR. Man-inthe-barrel syndrome: MRI and SPECT. Eur J Radiol 1997;24:260 – 2. [13] Zandbergen EG, de Haan RJ, Stoutenbeek CP, Koelman JH, Hijdra A. Systematic review of early prediction of poor outcome in anoxic – ischaemic coma. Lancet 1998;352(9143):1808–12.
Case report
Predicting outcome from coma: man-in-the-barrel syndrome as potential pitfall J.W. Elting *, R. Haaxma, G. Sulter, J. De Keyser Department of Neurology, Uni6ersity Hospital Groningen, PO Box 30.001, 9700 RB Groningen, The Netherlands Received 16 June 1999; received in revised form 5 October 1999; accepted 5 October 1999
Abstract The Glasgow coma scale motor score is often used in predicting outcome after hypoxic – ischemic coma. Judicious care should be exerted when using this variable in predicting outcome in patients with coma following hypotension since borderzone infarction can obscure the clinical picture. We describe a patient who underwent skull base surgery for a schwannoma of the left facial nerve. The operation, which lasted for 10 h, was conducted under controlled hypotension. After the intervention the patient remained comatose with absent arm movements upon painful stimuli. An absent motor score usually carries a poor prognosis. However, magnetic resonance inversion recovery imaging of the brain showed bilateral hyperintense lesions in the arm – hand area indicative of borderzone ischemic damage. The patient received optimal supportive care and after 17 days he regained consciousness with ‘man-in-the-barrel syndrome’, which also further improved over time. © 2000 Elsevier Science B.V. All rights reserved. Keywords: Glasgow coma scale; Borderzone ischemia; Man-in-the-barrel syndrome; Hypotension
1. Introduction Several authors have extensively studied which factors can predict outcome from hypoxic – ischemic coma [1– 3]. Elements of the Glasgow coma scale (GCS) seem to be very predictive on certain time points after the primary insult. For example, a motor score of 3 (abnormal flexor response) or less on day 3 is correlated with a very poor prognosis: 93% of the patients die or survive in a vegetative state. The motor score of the GCS is usually obtained from the arms by administering a painful stimulus on the nailbeds and/or on the supraorbital ridge. The legs should also be evaluated but interpretation is sometimes difficult since leg movements can be of spinal origin. There are several pitfalls to be considered, all of which could theoretically lead to misinterpretation of the GCS score. These include the presence of a peripheral neuropathy or myopathy, a cervical cord lesion, hypothermia, sedative drugs or muscle relaxants, and metabolic or endocrine causes of coma. Another potential pitfall to be considered, espe* Corresponding author.
cially in less acute forms of hypoxic–ischemic brain damage associated with hypotension, is the so-called ‘man-in-the-barrel syndrome’.
2. Case report A 61-year-old man presented with a slight asymmetry of the left side of the face and some instability during walking. Furthermore, the patient had experienced hearing loss and tinnitus of the left ear for 35 years. Magnetic resonance imaging (MRI) revealed a tumor close to the left os petrosum extending into the posterior and middle fossa and the left sinus cavernosus. The circle of Willis was intact. A transcranial doppler sonogram and a duplex of both carotid arteries were unremarkable. A preoperative electroencephalogram (EEG) with compression of the left carotid artery showed no signs of insufficient collateral circulation. The patient underwent surgery to remove the tumor, which turned out to be a schwannoma of the left facial nerve. During the 10-h operation systemic blood pressure was kept between 80–100 mmHg systolic and 40–50
0303-8467/00/$ - see front matter © 2000 Elsevier Science B.V. All rights reserved. PII: S 0 3 0 3 - 8 4 6 7 ( 9 9 ) 0 0 0 6 7 - 0
24
J.W. Elting et al. / Clinical Neurology and Neurosurgery 102 (2000) 23–25
mmHg diastolic. After the intervention the patient did not react to stimuli and had to be reintubated. A GCS score was not obtained at all time. A computed tomographic (CT) scan of the brain showed slight diffuse edema and some residual hematoma in the left pontocerebellar region. When sedation was stopped the patient remained comatose but made spontaneous rotating movements with his body and legs. The arms did not react to painful stimuli applied to the nailbeds and the supraorbital ridges, and the eyes were closed. Brainstem reflexes were intact. An EEG showed diffuse alfa activity with little reactivity resembling an alfa coma. Five days after surgery a MRI of the brain was performed. The T2 inversion recovery images showed bilateral increased signals in the fronto – parietal cortex in the arm –hand area, especially on the left side (Fig. 1). There were no lesions in the brainstem. After 12 days an EEG was repeated which again showed diffuse alpha activity. Sensory evoked potentials derived from all extremities showed bilateral presence of N20 responses, late cortical responses were not obtained. On day 17 after surgery the patient was out of coma and responded to commands. There was distal paralysis of both arms with proximal paresis of the right arm (MRC grade-3) more than the left arm (MRC grade-4). Arm reflexes were brisk and there was a hypesthesia of both arms. Examination further showed on the left side a complete facial nerve palsy, deafness, and hypesthesia in the area of the second and third division of the trigeminal nerve. Cognition was not impaired. Over the next months the patient gradually improved to an MRC grade-3 distal paresis of the right arm and a grade-4 distal paresis of the left arm with hypesthesia in the fourth and fifth fingers on both sides,
Fig. 1. Axial inversion recovery T2-weighted images: bilateral hyperintense signal in the sensorimotor arm–hand area.
which we interpreted as a central sensory deficit. An EMG showed absence of a peripheral lesion.
3. Discussion The term ‘man-in-the-barrel syndrome’ is currently being used to describe a clinical picture resulting from a bilateral border-zone infarction between the anterior cerebral artery and middle cerebral artery junctions, which are usually located at the level of the arm representation on the motor cortex [4–6]. It implies a paresis or paralysis of both arms while the cranial nerves and motor function of the legs are preserved, giving the appearance of being confined to a barrel. The most common cause is systemic hypotension following cardiovascular arrest or cardiac surgery [6,7]. Other authors have reported the same syndrome after head injury [8] and cerebral metastases [9]. The syndrome can also occur with lesions in the central cervical myelum [10] and the bilateral brachial plexopathy. Sage and Van Uitert [6] prospectively studied 34 comatose patients who had an episode of systemic hypotension. Eleven of these patients developed ‘manin-the-barrel syndrome’ and ten of them died. There have been few reports of ‘man-in-the-barrel syndrome’ occurring in non-comatose patients, which is associated with a much better prognosis [11,12]. The patients described by Sage and Van Uitert [6] had a history of a hypotensive period of at least 5 min with systolic blood pressures of 50 mmHg or less. The authors concluded that a sudden decline in blood pressure is probably a prerequisite to develop the syndrome. Later reports in patients undergoing cardiac surgery yielded evidence that a more prolonged moderate hypotension could also produce this syndrome [7]. The duration and severity of hypotension needed to produce ‘man-in-the-barrel syndrome’ is not known. Individual contributing factors include previous hypertension and high-grade stenosis of the internal carotid artery on both sides. In our case manipulation at the skull base could have produced compression and traction on the cerebral arteries which further compromised bloodflow. Clinically, coma associated with ‘man-in-the-barrel syndrome’ remains a rare but important syndrome. Failure to recognise it can lead to misinterpretation of neurological signs, for example when using the GCS motor score as a factor to determine the neurological prognosis after prolonged cerebral ischemia. In several early studies on the prognosis after cerebral ischemia due to cardiac arrest, pupillary response, GCS motor score, and eye movements were the most important predictors of outcome [1,2]. More recently, somato– sensory evoked potentials have been used to predict poor outcome [13]. Prognostic schemes have originated from these studies and they are widely used in everyday
J.W. Elting et al. / Clinical Neurology and Neurosurgery 102 (2000) 23–25
practice. While these may be very valid for ‘pure cardiac arrest’ they could be less valid in situations in which there is a more mild and prolonged hypotension such as in our case. This probably explains the remarkable recovery in our patient, despite the initial coma and other possible unfavourable signs such as alfa coma. Injudicious use of such schemes could then lead to underestimation of the neurological status if one failed to observe other relevant data such as grimacing, spontaneous axial movements and leg movements. On the other hand, the interpretation of leg movements on stimulation should be careful, since they can also be of spinal origin in the severe comatose patient. Preferably, the diagnosis is confirmed with imaging techniques. In many patients however, there are normal CT scan findings [6], and MRI may be needed to confirm the diagnosis. Thus far reports of MRI scanning clearly demonstrating the lesions in the cortical arm –hand area like as in our case are scanty [6,11].
References [1] Levy DE, Caronna JJ, Singer BH, et al. Predicting outcome from hypoxic – ischemic coma. J Am Med Assoc 1985;253:1420.
.
25
[2] Longstreth WT Jr, Diehr P, Inui TS. Prediction of awakening after out-of-hospital cardiac arrest. N Engl J Med 1983;308:1378. [3] Rothstein TL, Thomas EM, Sumi SM. Predicting outcome in hypoxic – ischemic coma. A prospective clinical and electrophysiologic study. Electroencephalogr Clin Neurophysiol 1991;79(2):101– 17. [4] Mohr JP. Distal field infarction. Neurology 1969;12:279. [5] Van der Zwan A, Hillen B. Review of the variability of the territories of the major cerebral. Stroke 1991;22:1078 – 84. [6] Sage JI, Van Uitert RL. Man-in-the-barrel syndrome. Neurology 1986;36:1102 – 3. [7] Hurley JP, Wood AE. Man-in-the-barrel syndrome following cardiac surgery. Thorac Cardiovasc Surg 1993;41:252 –4. [8] Crisostomo EA, Suslavich FJJ. Man-in-the-barrel syndrome associated with closed head injury. Neuroimaging 1994;4:116–7. [9] Moore AP, Humphrey PRD. Man-in-the-barrel syndrome caused by cerebral metastases. Neurology 1989;39:1134–5. [10] Berg D, Mullges W, Koltzenburg M, Bendszus M, Reiners K. Man-in-the-barrel syndrome caused by cervical spinal cord infarction. Acta Neurol Scand 1998;97:417 – 9. [11] Olejniczak PG, Ellenberg MR, Eilender LM, Muszynski CT. Man-in-the-barrel syndrome in a noncomatose patient: a case report. Arch Phys Med Rehabil 1991;72:1021 – 3. [12] Benito-Leon J, Munoz A, Ruiz J, Gomez-Fuentes JR. Man-inthe-barrel syndrome: MRI and SPECT. Eur J Radiol 1997;24:260 – 2. [13] Zandbergen EG, de Haan RJ, Stoutenbeek CP, Koelman JH, Hijdra A. Systematic review of early prediction of poor outcome in anoxic – ischaemic coma. Lancet 1998;352(9143):1808–12.