Neurocrit Care (2012) 17:265–267 DOI 10.1007/s12028-012-9729-6

PRACTICAL PEARL

Thumbs Up Sign in Brain

Manoj K. Mittal • Grace M. Arteaga • Eelco F. M. Wijdicks

Published online: 7 July 2012 Ó Springer Science+Business Media, LLC 2012

Abstract Introduction Introduction Spinal reflexes can be seen in the setting of . We present a new spinal reflex. We also Brain death is a result of a massive brain injury leading to review spinal movements in pediatric brain death and loss of all brainstem function. Brain death is usually provide suggestions to distinguish them from movements associated with no spontaneous movements in extremities, generated by the brain. trunk, or neck. Movements generated from the spinal cord Case Report We report a five-year old girl admitted after are very uncommon and were found in only 4 of 228 an asystolic cardiac arrest and was soon declared brain patients at Mayo Clinic (2 %) [1]. Others have found a death as a result of bilateral cerebellar hematoma. She had much higher prevalence of spinal reflexes, but its presence spinal movements including a ‘‘Thumbs up sign’’. These may depend on whether the response is provoked or is findings delayed organ procurement. considered relevant [1, 2]. In one study, 80 % of physicians Conclusion ‘‘Thumbs up sign’’ should be added to the list and nursing staff reported spinal reflexes to be a potential of spinal reflexes seen with brain death. Spinal reflexes in concern if relatives are present during brain death deter- brain death can be clinically recognized and should mination [3]. The presence of these movements may cause explained to all involved parties to avoid unnecessary delay in brain death determination or may even halt organ testing, confusion for family members, and delay or refusal retrieval. We present here a new movement after noxious of . stimulation and review the literature. We also use this opportunity to further describe general characteristics of Keywords Spinal reflexes Á Child Á Organ donation Á these movements. Brain death

Case Report

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 Electronic supplementary material The online version of this developed asystolic cardiac arrest on her way to the hos- article (doi:10.1007/s12028-012-9729-6) contains supplementary pital. Cardiopulmonary resuscitation (CPR) was started material, which is available to authorized users. immediately. She received CPR for 20 min including a M. K. Mittal (&) Á E. F. M. Wijdicks dose of atropine before presentation to a local hospital. Department of Neurology, Mayo Clinic, 200 First Street SW, CPR was continued for another 25 min including chest Rochester, MN 55905, USA compressions, atropine, epinephrine, sodium bicarbonate, e-mail: [email protected] and calcium chloride. CPR resulted in return of spontane- G. M. Arteaga ous circulation after a total resuscitation time of 45 min. Department of Pediatrics, Mayo Clinic, Rochester, MN, USA She was comatose with non-reactive pupils and absent 123 266 Neurocrit Care (2012) 17:265–267 corneal reflexes, oculocephalic reflexes, gag and cough from 31 to 71 mmHg within 8 min. She had a repeat brain reflexes. Hypothermia was not considered as patient had death examination after 24 h by a different physician with asystolic cardiac arrest and was moribund. The computed identical results. Repeat apnea testing showed increase in tomography (CT) of head showed acute bilateral cerebellar PaCO2 from 46 to 99 mmHg within ten minutes. hemorrhagic lesions associated with surrounding mass She was noted to have flexion and extension movements effect with complete effacement of the fourth ventricle. CT in both upper and lower extremities in response to turning of head also revealed generalized effacement of the sulci in bed and transfer from bed to the cart initially and later (Fig. 1). She was transferred to Saint Marys Hospital for only with noxious stimuli at nailbed in fingers and toes. possible neurosurgical intervention. Her heart rate was These movements were consistent with spinal reflexes and 155 beats/min, blood pressure was 102/69 mmHg, oxygen included extensor plantar response with noxious stimuli at saturation was 96 % with 100 % FiO2, and temperature the plantar aspect of foot, triple flexion response after was 35.0 °C. On initial neurological examination per- noxious stimuli at the toe nailbed, undulating toe flexion formed 4 h after she was resuscitated, she had non-reactive sign with flexion at the great toe, and quadriceps contrac- pupils; absent corneal reflex, oculocephalic reflexes, and tion on the right associated with triple flexion on the left oculovestibular reflexes; no facial movements to noxious with noxious stimuli at the left toe nailbed. She was noted stimuli at the supraorbital nerve; and absent gag and cough to have thumb extension in response to painful stimuli reflexes. Patient was seen by neurosurgery, pediatric neu- applied to the nailbed of the middle finger of the right hand rology, and pediatric intensivist and they collectively (Video). All the above-mentioned movements in our decided not to pursue any surgical options as there were no patient were consistent with spinal movements. Following brainstem reflexes and apnea for several hours. No con- death notification, the family opted for organ donation. founding factors were present before a formal brain death However several nurses in the operating room questioned examination. Her temperature was corrected to 37.0 °C whether the movements were not ‘a sign of life’ and the using warm intravenous fluids, forced air warming, and transplant surgeon postponed organ retrieval. An EEG in warm blankets. There was no history of use of any illicit the operating room was inconclusive due to artifact. drugs, neuromuscular blocking agents, or sedative drugs. Donation proceeded after reexamination by an independent She was in a euvolemic state. At the time of first brain death neurologist. showed acute hemorrhages in both evaluation, her laboratory testing revealed serum sodium the cerebellar hemispheres and vermis with extension into 139 mmol/L, potassium 3.3 mmol/L, calcium 5.6 mg/dl, fourth ventricle, dependent basilar cisterns, and spinal creatinine 0.4 mg/dl, blood urea nitrogen 9 mg/dl, magne- subarachnoid space. The etiology of hemorrhages was sium 1.7 mg/dl, glucose 118 mg/dl, arterial blood gas with undetermined in the absence of any vascular malformation, pH 7.33, PaCO2 31 mmHg, PaO2 234 mmHg, and bicar- vasculitis, or tumor. In addition, diffuse cerebral edema bonate 25 mmol/L. First brain death examination was with gyral flattening, bilateral uncal prominence, and cer- performed 6 h after admission and adequate resuscitation. ebellar tonsillar herniation was seen. There was cerebellar Neurological examination was unchanged from the initial tonsillar fragmentation and caudal displacement into spinal

findings. Apnea test was performed and her PaCO2 increased subarachnoid space.

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 gray- white 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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Review of Literature death, including Lazarus sign (neck flexion causing ele- vation of one or both arms and flexion at the elbows followed Multiple databases (Embase 1988 to 2012 Week 07, Ovid by return to primary position), pronation-extension reflex, MEDLINE(R) 1946 to February Week 2, 2012, PsycINFO and spontaneous jerks of the fingers [5]. 1967 to February Week 2, 2012, and EBM Reviews- Spinal reflexes have not been reported in any of the 11 Cochrane Database of Systematic Reviews 2005 to January infants less than 1 year of age in a study of 45 children. 2012) were searched for the key words ‘‘brain death’’ and Spinal reflexes were seen in 27 % of children 1–5 years of ‘‘spinal reflex’’. A total of 84 articles were obtained. age and 44 % of children age more than 5 years of age [6]. Twenty one relevant articles were studied in detail and Various spinal responses, mostly single cases, have been their references were searched to identify relevant papers. observed in children including Lazarus sign [7], plantar flexion response, pronation-extension reflex, triple flexion reflex [5], decerebrate posturing, [8] viscero-somatic reflex Discussion including contraction of the abdominal musculature after cutting parietal peritoneum [9]. The recent pediatric guidelines states ‘‘if abnormal move- Spinal reflexes are seen within the first 24 h of brain ments are present, clinical assessment to determine whether death and may last up to 72 h. Spinal reflexes have been or not these are spinal cord reflexes should be done.’’ [4] known for many years but continue to cause concern and The following five aspects of the movements seen after demand explanation to the family and education of the brain death may assist in the differentiation of spinal staff. responses from postural motor responses: The presence of spinal reflexes, when incorrectly attrib- uted to brainstem function, may lead to considerable (1) There is no resemblance of a spinal response to the confusion and ultimately delay the brain death diagnosis classic postural motor responses. These responses are with unnecessary ancillary tests and prolonged observation recognized by synchronized decorticate (thumb periods. folded under flexed fingers in a fist, pronated forearm, flexed elbow, and extended lower extremity with Financial Support and Disclosure None. inverted foot) or decerebrate responses (pronated and extended upper and lower extremity). Conflict of interest None. (2) 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 References minimal excursions; and lower extremity responses are often more complex and can be wavy or shock like. 1. Wijdicks EFM, Rabinstein AA, Manno EM, Atkinson JD. (3) The most common spinal response is triple flexion Pronouncing brain death: contemporary practice and safety of response (flexion in foot, knee, and hip) which may the apnea test. Neurology. 2008;71:1240–4. have variations such as undulating toe sign or a 2. Saposnik G, Basile VS, Young GB. Movements in brain death: a Babinski sign. systematic review. Can J Neurol Sci. 2009;36:154–60. 3. Pugh J, Clarke L, Gray J, Haveman J, Lawler P, Bonner S. (4) Most movements are provoked and not spontaneous. Presence of relatives during testing for brain stem death: The provocation can be movement during nursing questionnaire study. BMJ. 2000;321:1505–6. care procedures of the patient such as turning in bed 4. Nakagawa TA, Ashwal S, Mathur M, et al. Guidelines for the or transfer from bed to a transport cart. determination of brain death in infants and children: an update of the 1987 task force recommendations. Pediatrics. 2011;128: (5) In some patients, spinal responses can be elicited by e720–40. forceful neck flexion and by noxious stimuli below 5. Saposnik G, Bueri JA, Maurino J, Saizar R, Garretto NS. cervicomedullary junction. They are not seen with Spontaneous and reflex movements in brain death. Neurology. pressure at the supraorbital ridge or temporomandib- 2000;54:221–3. 6. Fackler JC, Troncoso JC, Gioia FR. Age-specific characteristics of ular joint. brain death in children. Am J Dis Child. 1988;142:999–1003. Thumb extension mimicking a ‘‘thumbs up sign’’ seen in 7. Spittler JF, Wortmann D, Von Du¨ring M, Gehlen W. Phenome- nological diversity of spinal reflexes in brain death. Eur J Neurol. our patient has not been reported previously. The exact 2000;7:315–21. pathophysiology of this movement is not clear; however, it 8. Martı´-Fa`bregas J, Lo´pez-Navidad A, Caballero F, Otermin P. may be secondary to disinhibition of spinal cord generators Decerebrate-like posturing with mechanical ventilation in brain following corticoreticular disconnection or it is a stretch- death. Neurology. 2000;54:224–7. 9. Conci F, Procaccio F, Arosio M, Boselli L. Viscero-somatic and related polysegmental spinal reflex. Spinal movements in viscero-visceral reflexes in brain death. J Neurol Neurosurg upper extremities have been previously described in brain Psychiatry. 1986;49:695–8. 123