Neurocrit Care (2012) 17:265–267 DOI 10.1007/s12028-012-9729-6
PRACTICAL PEARL
Thumbs Up Sign in Brain Death Manoj K. Mittal • Grace M. Arteaga Eelco F. M. Wijdicks
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Published online: 7 July 2012 Ó Springer Science+Business Media, LLC 2012
Abstract Introduction Spinal reflexes can be seen in the setting of brain death. We present a new spinal reflex. We also review spinal movements in pediatric brain death and provide suggestions to distinguish them from movements generated by the brain. Case Report We report a five-year old girl admitted after an asystolic cardiac arrest and was soon declared brain death as a result of bilateral cerebellar hematoma. She had spinal movements including a ‘‘Thumbs up sign’’. These findings delayed organ procurement. Conclusion ‘‘Thumbs up sign’’ should be added to the list of spinal reflexes seen with brain death. Spinal reflexes in brain death can be clinically recognized and should explained to all involved parties to avoid unnecessary testing, confusion for family members, and delay or refusal of organ donation. Keywords Spinal reflexes Child Organ donation Brain death
Introduction Brain death is a result of a massive brain injury leading to loss of all brainstem function. Brain death is usually associated with no spontaneous movements in extremities, trunk, or neck. Movements generated from the spinal cord are very uncommon and were found in only 4 of 228 patients at Mayo Clinic (2 %) [1]. Others have found a much higher prevalence of spinal reflexes, but its presence may depend on whether the response is provoked or is considered relevant [1, 2]. In one study, 80 % of physicians and nursing staff reported spinal reflexes to be a potential concern if relatives are present during brain death determination [3]. The presence of these movements may cause delay in brain death determination or may even halt organ retrieval. We present here a new movement after noxious stimulation and review the literature. We also use this opportunity to further describe general characteristics of these movements.
Case Report
Electronic supplementary material The online version of this article (doi:10.1007/s12028-012-9729-6) contains supplementary material, which is available to authorized users. M. K. Mittal (&) E. F. M. Wijdicks Department of Neurology, Mayo Clinic, 200 First Street SW, Rochester, MN 55905, USA e-mail:
[email protected] G. M. Arteaga Department of Pediatrics, Mayo Clinic, Rochester, MN, USA
A five-year-old girl had acute onset of headache and vomiting. Ten minutes later, she was unresponsive. Emergency medical services were called and patient developed asystolic cardiac arrest on her way to the hospital. Cardiopulmonary resuscitation (CPR) was started immediately. She received CPR for 20 min including a dose of atropine before presentation to a local hospital. CPR was continued for another 25 min including chest compressions, atropine, epinephrine, sodium bicarbonate, and calcium chloride. CPR resulted in return of spontaneous circulation after a total resuscitation time of 45 min. She was comatose with non-reactive pupils and absent
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corneal reflexes, oculocephalic reflexes, gag and cough reflexes. Hypothermia was not considered as patient had asystolic cardiac arrest and was moribund. The computed tomography (CT) of head showed acute bilateral cerebellar hemorrhagic lesions associated with surrounding mass effect with complete effacement of the fourth ventricle. CT of head also revealed generalized effacement of the sulci (Fig. 1). She was transferred to Saint Marys Hospital for possible neurosurgical intervention. Her heart rate was 155 beats/min, blood pressure was 102/69 mmHg, oxygen saturation was 96 % with 100 % FiO2, and temperature was 35.0 °C. On initial neurological examination performed 4 h after she was resuscitated, she had non-reactive pupils; absent corneal reflex, oculocephalic reflexes, and oculovestibular reflexes; no facial movements to noxious stimuli at the supraorbital nerve; and absent gag and cough reflexes. Patient was seen by neurosurgery, pediatric neurology, and pediatric intensivist and they collectively decided not to pursue any surgical options as there were no brainstem reflexes and apnea for several hours. No confounding factors were present before a formal brain death examination. Her temperature was corrected to 37.0 °C using warm intravenous fluids, forced air warming, and warm blankets. There was no history of use of any illicit drugs, neuromuscular blocking agents, or sedative drugs. She was in a euvolemic state. At the time of first brain death evaluation, her laboratory testing revealed serum sodium 139 mmol/L, potassium 3.3 mmol/L, calcium 5.6 mg/dl, creatinine 0.4 mg/dl, blood urea nitrogen 9 mg/dl, magnesium 1.7 mg/dl, glucose 118 mg/dl, arterial blood gas with pH 7.33, PaCO2 31 mmHg, PaO2 234 mmHg, and bicarbonate 25 mmol/L. First brain death examination was performed 6 h after admission and adequate resuscitation. Neurological examination was unchanged from the initial findings. Apnea test was performed and her PaCO2 increased Fig. 1 CT of head findings in a five-year-old patient with brain death. Non-contrasted CT (window width and window level adjusted to 45 each for better delineation of gray-white junction and sulci) head axial cut showing preserved graywhite differentiation and generalized effacement of the sulci (a). Axial cut through the cerebellar hemispheres showing bilateral cerebellar hematomas and surrounding mass effect with complete effacement of the fourth ventricle (b)
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from 31 to 71 mmHg within 8 min. She had a repeat brain death examination after 24 h by a different physician with identical results. Repeat apnea testing showed increase in PaCO2 from 46 to 99 mmHg within ten minutes. She was noted to have flexion and extension movements in both upper and lower extremities in response to turning in bed and transfer from bed to the cart initially and later only with noxious stimuli at nailbed in fingers and toes. These movements were consistent with spinal reflexes and included extensor plantar response with noxious stimuli at the plantar aspect of foot, triple flexion response after noxious stimuli at the toe nailbed, undulating toe flexion sign with flexion at the great toe, and quadriceps contraction on the right associated with triple flexion on the left with noxious stimuli at the left toe nailbed. She was noted to have thumb extension in response to painful stimuli applied to the nailbed of the middle finger of the right hand (Video). All the above-mentioned movements in our patient were consistent with spinal movements. Following death notification, the family opted for organ donation. However several nurses in the operating room questioned whether the movements were not ‘a sign of life’ and the transplant surgeon postponed organ retrieval. An EEG in the operating room was inconclusive due to artifact. Donation proceeded after reexamination by an independent neurologist. Autopsy showed acute hemorrhages in both the cerebellar hemispheres and vermis with extension into fourth ventricle, dependent basilar cisterns, and spinal subarachnoid space. The etiology of hemorrhages was undetermined in the absence of any vascular malformation, vasculitis, or tumor. In addition, diffuse cerebral edema with gyral flattening, bilateral uncal prominence, and cerebellar tonsillar herniation was seen. There was cerebellar tonsillar fragmentation and caudal displacement into spinal subarachnoid space.
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Review of Literature Multiple databases (Embase 1988 to 2012 Week 07, Ovid MEDLINE(R) 1946 to February Week 2, 2012, PsycINFO 1967 to February Week 2, 2012, and EBM ReviewsCochrane Database of Systematic Reviews 2005 to January 2012) were searched for the key words ‘‘brain death’’ and ‘‘spinal reflex’’. A total of 84 articles were obtained. Twenty one relevant articles were studied in detail and their references were searched to identify relevant papers.
Discussion The recent pediatric guidelines states ‘‘if abnormal movements are present, clinical assessment to determine whether or not these are spinal cord reflexes should be done.’’ [4] The following five aspects of the movements seen after brain death may assist in the differentiation of spinal responses from postural motor responses: (1)
(2)
(3)
(4)
(5)
There is no resemblance of a spinal response to the classic postural motor responses. These responses are recognized by synchronized decorticate (thumb folded under flexed fingers in a fist, pronated forearm, flexed elbow, and extended lower extremity with inverted foot) or decerebrate responses (pronated and extended upper and lower extremity). Most often, the spinal responses are slow and short in duration. However, there can be some exceptions as follows: finger flexion can be seen as quick jerks with minimal excursions; and lower extremity responses are often more complex and can be wavy or shock like. The most common spinal response is triple flexion response (flexion in foot, knee, and hip) which may have variations such as undulating toe sign or a Babinski sign. Most movements are provoked and not spontaneous. The provocation can be movement during nursing care procedures of the patient such as turning in bed or transfer from bed to a transport cart. In some patients, spinal responses can be elicited by forceful neck flexion and by noxious stimuli below cervicomedullary junction. They are not seen with pressure at the supraorbital ridge or temporomandibular joint.
Thumb extension mimicking a ‘‘thumbs up sign’’ seen in our patient has not been reported previously. The exact pathophysiology of this movement is not clear; however, it may be secondary to disinhibition of spinal cord generators following corticoreticular disconnection or it is a stretchrelated polysegmental spinal reflex. Spinal movements in upper extremities have been previously described in brain
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death, including Lazarus sign (neck flexion causing elevation of one or both arms and flexion at the elbows followed by return to primary position), pronation-extension reflex, and spontaneous jerks of the fingers [5]. Spinal reflexes have not been reported in any of the 11 infants less than 1 year of age in a study of 45 children. Spinal reflexes were seen in 27 % of children 1–5 years of age and 44 % of children age more than 5 years of age [6]. Various spinal responses, mostly single cases, have been observed in children including Lazarus sign [7], plantar flexion response, pronation-extension reflex, triple flexion reflex [5], decerebrate posturing, [8] viscero-somatic reflex including contraction of the abdominal musculature after cutting parietal peritoneum [9]. Spinal reflexes are seen within the first 24 h of brain death and may last up to 72 h. Spinal reflexes have been known for many years but continue to cause concern and demand explanation to the family and education of the intensive care unit staff. The presence of spinal reflexes, when incorrectly attributed to brainstem function, may lead to considerable confusion and ultimately delay the brain death diagnosis with unnecessary ancillary tests and prolonged observation periods. Financial Support and Disclosure Conflict of interest
None.
None.
References
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