Neurologic Emergencies at the Extremes of Age





The diagnosis and management of neurologic conditions are more complex at the extremes of age than in the average adult. In the pediatric population, neurologic emergencies are somewhat rare and some may require emergent consultation. In older adults, geriatric physiologic changes with increased comorbidities leads to atypical presentations and worsened outcomes. The unique considerations regarding emergency department presentation and management of stroke and altered mental status in both age groups is discussed, in addition to seizures and intracranial hemorrhage in pediatrics, and Parkinson’s disease and meningitis in the geriatric population.


Key points








  • In a child with fever and altered mental status, consider anti– N -methyl- d -aspartate receptor encephalitis and acute disseminated encephalomyelitis in the differential diagnosis.



  • Nonconvulsive status epilepticus is associated with higher mortality, longer pediatric intensive care unit stays, and increased long term disability.



  • Age alone is not a contraindication for intravenous tissue-type plasminogen activator administration within 3 hours.



  • Acute worsening in Parkinson’s disease is usually due to a medication change, infection, or missed subdural hemorrhage.



  • Meningitis in older adults presents atypically and has worse outcomes than the general population.




Introduction


Neurologic conditions, both common and emergent, present to the emergency department (ED) daily. The diagnosis and management of such conditions can be more complex at the extremes of age than in the average adult. In the pediatric population, neurologic emergencies are somewhat rare and some may require emergent consultation, as summarized in Box 1 .



Box 1

Pediatric conditions requiring emergent neurology consultation





  • High suspicion for stroke, regardless of intent to use tissue-type plasminogen activator



  • Autoimmune encephalitis



  • Acute demyelinating encephalomyelitis



  • Acute peripheral neurologic deficit, that is, acute flaccid myelitis, Guillain–Barré syndrome, acute brachial neuritis



  • Transverse myelitis



  • Any suspected seizure at <1 year of age



  • Reported or observed seizure activity with reports of loss of milestones or other systemic symptoms



  • Newly documented neurologic deficits/symptoms or first time seizure with concerns regarding timely follow-up



  • Refractory status epilepticus, convulsive or nonconvulsive




On the opposite end of the age spectrum, older adults disproportionately require emergency care, and the combination of geriatric physiologic changes with increased comorbidities leads to atypical presentations and worsened outcomes. , The unique considerations regarding emergency department (ED) presentation and management of stroke and altered mental status (AMS) in both age groups, in addition to seizures and intracranial hemorrhage in pediatrics, and Parkinson’s disease and meningitis in the geriatric population are discussed.


Neurologic emergencies in the pediatric patient


Pediatric Neurologic Examination


Although not “little adults,” our pediatric patients are not aliens either. Their neurologic systems are, however, still in development and in need of a different approach for examination. The normal range for each component varies by age, environmental exposure, and any underlying congenital malformations or diseases. The physical examination begins from the moment you see the patient. Observe how alert or withdrawn they are, or how they respond to their caregiver’s voice. Note the muscle tone and posture of the infant or child, and their response to touch. Understand the child’s baseline behavior from the accompanying caregiver and compare it with your assessment to uncover any abnormalities. An effective mnemonic for a gross neurologic evaluation of the child is TICLS : T one, I nteraction, C onsolability (irritability), L ook (gaze), S peech (including jargon or cry), as one arm of the Pediatric Assessment Triangle ( Table 1 ).



Table 1

Pediatric neurologic examination: the TICLS mnemonic

Data from Horeczko T, Enriquez B, McGrath NE, Gausche-Hill M, Lewis RJ. The Pediatric Assessment Triangle: Accuracy of Its Application by Nurses in the Triage of Children. J Emerg Nurs . 2013;39(2):182-189.



























Element Exam Questions
T Tone Do they appear floppy or limp? Head lag? Response when touched?
I Interaction Resistance to exam? Smile or grin? Acknowledgment of providers?
C Consolability Are they irritable or fussy? Consoled by caregiver presence or affection? Pitch of the cry?
L Look Pupillary size? Eye contact? Fixed or roaming gaze? Conjugate or disconjugate? Tracking?
S Speech Age appropriate babble or jargon? Age appropriate sentences? Oriented or confused? Speech slurring?


Pediatric Acute Ischemic Stroke


Clinical presentation


In the pediatric population, stroke is rare and can be more difficult to recognize than in the adult population. Children younger than 5 years of age are the most likely to experience acute ischemic stroke (AIS) among pediatric patients. AIS in young children is more likely to present with global neurologic impairment, such as seizure or AMS, than in adults. Up to 50% of infants and young children with AIS may present with AMS, fever, and/or seizure. Older children and teens are more likely to present with focal neurologic deficits similar to those in adults. Considering AIS in the list of differential diagnoses of seizure or AMS, especially in infants and younger children, can improve time to diagnosis. Delay in diagnosis up to 24 hours from the time of presentation is common, even though patients often present within 6 hours from time of onset.


Examination should begin promptly with observation of the patient and quantification of neurologic deficits as soon as they are identified or reported by the caregiver. A noncontrast computed tomography (CT) scan of the brain and point-of-care glucose are just as important in children as in adults. Rapid use of the Pediatric National Institute of Health Stroke Scale should be included in patients ages 6 years and younger to quantify neurologic deficits and guide the decision to use thrombolytics, particularly if the score is greater than or equal to 6. , The Pediatric National Institute of Health Stroke Scale is partially completed by the physician’s observation of the child’s behavior and responses in the ED given that it is difficult for many younger children to follow the instructions of the examination. Other age-specific modifications are summarized in Box 2 .



Box 2

National Institutes of Health Stroke Scale Modifications in Pediatric Patients

Data from Ichord RN, Bastian R, Abraham L, et al. Interrater reliability of the Pediatric National Institutes of Health Stroke Scale (PedNIHSS) in a multicenter study. Stroke . 2011;42(3):613-617.

a Must understand baseline verbal skills.





  • Orientation – Ask age and point to family member



  • Commands – Blink eyes and touch nose



  • Gaze – Assessed with horizontal gaze only



  • Visual fields – Finger counting if >6 years old, or visual threat if <6 years old



  • Facial palsy – Scored same as adults



  • Motor strength – If unable to follow commands, score by spontaneous or elicited movement, that is, assisted weight bearing may demonstrate 1 leg weaker than the other



  • Ataxia – Ask to reach or kick toy in examiner’s hand



  • Sensation – Pinprick testing, or observe for spontaneous response to pinprick



  • Language – Assess with stated words, naming pictures a



  • Dysarthria – Repeating words or sentences, ask about baseline speech impediment



  • Extinction/inattention – Scored similarly to adults




Thrombolytics


After ruling out intracranial hemorrhage using a CT scan, tissue-type plasminogen activator (tPA) can be given in consultation with the pediatric neurologist and/or pediatric intensivist, if available, who will be accepting the patient. MRI of the brain is the preferred imaging for detailed examination of brain parenchyma; however, it is not required before tPA administration. Thrombolytics should not be delayed for an MRI if the physician’s suspicion for AIS is reasonably high, but certain institutional algorithms may require demonstration of acute vascular occlusion before giving tPA. Goal administration time is of less than 4.5 hours from the time of onset, and early administration is more likely to result in improved outcomes. The pediatric tPA dosing is the same as that in adults: 0.9 mg/kg with 10% administered as a bolus followed by 90% as an infusion over 60 minutes. In vitro data show that the thrombus composition of children’s plasma contains less fibrin as compared with adults, resulting in higher recanalization rates with tPA. A small study to review efficacy and safety of tPA in children was published in 2019; there was no clinically significant intracranial hemorrhage after tPA administration in 26 children. One patient experienced severe epistaxis requiring intubation, but details regarding epistaxis evaluation and management are of limited description. There were 2 patients with cerebral petechiae found on follow-up MRI within the area of infarction, but this did not seem to limit their recovery. Overall, the estimated risk profile of tPA in pediatric stroke is 2.1% when given within 4.5 hours from time of onset.


Thrombectomy


There is limited evidence supporting thrombectomy in patients under 18 years of age; however, it may be performed on patients under certain circumstances. Expert opinion, based on extrapolated adult data, encourages its consideration within 6 hours of symptoms onset in select patients. Technical limitations include the smaller size of peripheral and intracranial vessels, as well as increased concerns for radiation risk and contrast load.


Stroke in sickle cell disease


Sickle cell disease accounts for a large portion of disease burden in pediatric stroke, particularly in African American children. The time of greatest risk for stroke in a patient with sickle cell disease is between 2 and 5 years of age. Given that many of these strokes are silent in nature, the only clinical signs may be early hand dominance, speech delays, or learning disabilities. When AIS in sickle cell disease is symptomatic, it is theorized that, owing to the development of higher pain tolerance, headache is a less frequently reported symptom of AIS in sickle cell disease as compared with AIS patients without sickle cell disease. In patients presenting with an acute neurologic deficit, immediate treatment is needed ( Box 3 ). In patients with a baseline hemoglobin (Hgb) of less than 10 g/dL, urgent transfusion of 10 mL/kg of packed red blood cells for a goal Hgb of 10 g/dL or sickle cell percentage of less than 15% is indicated, to be given within 6 hours of symptom onset. If the baseline Hgb is greater than 10 mg/dL, exchange transfusion must be performed. Repeat transfusion may be needed but Hgb should not go above 11 g/dL, and post-transfusion Hgb measured at approximately 2 hours after to assess risk hyperviscosity syndrome, which may occur with rapid overcorrection of the Hgb. Hyperviscosity syndrome may lead to cerebral venous sinus thrombosis or multifocal infarcts. Pain and hydration status should be addressed as they would in other vasoocclusive crises. The emergency physician should also look for additional underlying precipitating factors contributing to the acute presentation, such as infection, dehydration, trauma, hypoxia, or acidosis. Ultimately, the patient will require emergent transfer to higher level care and consultation with pediatric hematology if available.



Box 3

Stroke management in pediatric patient with sickle cell disease

Data from Ferriero DM, Fullerton HJ, Bernard TJ, et al. Management of stroke in neonates and children: A scientific statement from the American Heart Association/American stroke association. Stroke . 2019;50(3):E51-E96; Guilliams KP, Kirkham FJ, Holzhauer S, et al. Arteriopathy Influences Pediatric Ischemic Stroke Presentation, but Sickle Cell Disease Influences Stroke Management. Stroke . 2019;50(5):1089-1094.

Abbreviation: Hgb, hemoglobin.





  • Special considerations in sickle cell disease



  • When AIS suspected, prompt transfusion is needed



  • Goal time to transfusion: <6 hours from onset of symptoms



  • Start with 10 mL/kg of packed red blood cells, goal Hgb 10 g/dL or <15% sickled Hgb



  • May need exchange transfusion if baseline Hgb is close to 10 g/dL



  • Avoid hypervolemia and hyperviscosity syndrome from overtransfusing



  • For Hgb >12 g/dL, consider phlebotomy to decrease total blood volume (consult with hematology for recommended volume removal)



  • Consider aggravating factors that may contribute to acute sickling or clinical presentation




Intracranial Hemorrhage


Many principles of adult care should be readily applied in the management of traumatic and nontraumatic intracranial hemorrhage in pediatric patients. Prioritization of the airway, breathing, and circulation, monitoring of vitals, and assessing blood glucose are necessary. Airway management in the setting of profound mental status change is of paramount importance. Patients with suspected intracranial hemorrhage should then undergo a CT scan of the head to assess severity of intracranial bleeding. There should be an ongoing focus to optimize oxygenation, ventilation, and blood pressure. Ideal parameters for arterial oxygen and carbon dioxide levels, as well as glucose, are similar to those for adults; however, target blood pressure is less clear. In cases of cerebral edema or impending herniation, administration of either mannitol or hypertonic saline is equally acceptable, and the physician should consider their variable effects on blood pressure ( Table 2 ). Seizure activity should be treated aggressively. Antiepileptics are commonly used prophylactically owing to concerns of transient intracranial pressure spikes during convulsions, but there is little evidence to support the safety and efficacy of this practice. After patient stabilization, identification of intracranial hemorrhage, and initiation of supportive measures, communication with nearest pediatric neurosurgery and intensive care should ensue to arrange for transfer.



Table 2

Pediatric management of elevated intracranial pressure

Data from Kliegman RM, Stanton BF, St Geme JWI, Schor NF, Behrman RE. Nelson Textbook of Pediatrics . 20th ed.; 2016; Kochanek PM, Tasker RC, Carney N, et al. Guidelines for the Management of Pediatric Severe Traumatic Brain Injury, Third Edition: Update of the Brain Trauma Foundation Guidelines, Executive Summary. Neurosurgery . 2019;84(6):1169-1178; Stopa BM, Dolmans RG, Broekman ML, et al. Hyperosmolar Therapy in Pediatric Severe Traumatic Brain Injury—A Systematic Review. Critical Care Medicine 2019; 47(12): e1022-e1031.



















Osmotic Agent Dose Frequency Adverse Effects
Mannitol 0.25–1.00 g/kg IV over 20 min Up to every 4–6 h


  • Hypovolemia



  • Acute renal failure



  • Diuresis



  • Decreased BP

Hypertonic saline (3%) 2–5 mL/kg IV given over 10–20 min Can start continuous infusion of 0.1–1.0 mL/kg/h


  • Rebound cerebral edema



  • Osmotic demyelination syndrome a



  • Increased BP


Abbreviation: BP, blood pressure.

a Formerly known as central pontine myelinosis.



Fever and Altered Mental Status


Bacterial meningitis should not be the only diagnosis considered when a pediatric patient presents with fever and AMS. Other important diagnoses are summarized in Box 4 .



Box 4

Clinical considerations for pediatric AMS and fever





  • Infectious meningitis



  • Infectious encephalitis



  • ADEM



  • Hemophagocytic lymphohistiocytosis



  • Anti-NDMAR encephalitis



  • Other autoimmune encephalitis



  • Stroke (infants and toddlers)



  • Toxic ingestions




    • Sympathomimetic



    • Anticholinergic




  • Thyrotoxicosis



  • Paraneoplastic syndromes



Not all inclusive.



Acute disseminated encephalomyelitis and anti– N -methyl- d -aspartate receptor encephalitis


Acute disseminated encephalomyelitis (ADEM) is an acute multifocal demyelinating disease presenting with fever, acute AMS, and neuromotor dysfunction, such as seizures. Patients are commonly young males, ages 5 to 8 years old, with recent viral illness or immunization. The illness can be idiopathic. , Patients often seem to be quite ill, and symptoms can progress in hours to days, lasting up to several weeks. The clinical picture is extremely similar to acute bacterial meningitis and a distinguishing feature is contrast-enhanced MRI revealing bilateral, multifocal, asymmetric enhancing lesions on T2-weighted or fluid-attenuated inversion recovery images. Lumbar puncture findings are not diagnostic in ADEM and may reveal elevated proteins and lymphocytic pleocytosis. More important, a lumbar puncture should be done to rule out infectious meningoencephalitis and xanthochromia from intracranial hemorrhage. ,


Anti– N -methyl- d -aspartate receptor (NMDAR) encephalitis is insidious in onset; symptoms progress over weeks. It is more common in prepubertal males or postpubertal females. Classified as an autoimmune encephalitis, second most common only to ADEM, it is typically preceded by a febrile viral-like syndrome. It progresses over the following days to week, and develops into light switch acutely fluctuating mental status changes, dyskinesia, seizures, fever, and autonomic instability. Caregivers may report that the patient does not remember events of the day, has a delay or decrease in expressive language, seems to be restless with tremors or repetitive movements, and may hallucinate. Young children may present with increased frequency of unprovoked tantrums. Given the overlap in symptoms between anti-NMDAR encephalitis and initial presentation of schizophrenia or other psychoses, a thorough history is critical. Early behavioral and mental status changes are often mistaken for a new behavioral or psychiatric disorder, which highlights the importance of a thorough assessment to exclude an underlying organic etiology for the presentation of such patients before psychiatry consultation. When suspected, a full assessment should include a brain MRI, to rule out other entities such as ADEM, and lumbar puncture, to test specifically for anti-NMDAR antibodies.


Both ADEM and anti-NMDAR encephalitis require a high index of suspicion, aggressive seizure control, and early initiation of steroids. Empiric treatment for bacterial and viral meningitis should be given as well. Second- and third-line therapies such as intravenous immunoglobulin, plasmapheresis, and biologic agents such as cyclophosphamide and rituximab are effective, however not indicated in acute ED management. Postpubertal girls (or girls >14 years old) suspected of having anti-NMDAR encephalitis should undergo abdomen and pelvis MRI for the evaluation of possible ovarian mass, because an ovarian teratoma is discovered in up to 40% of these patients.


Extracranial causes of altered mental status


Given the sensitivity of children’s nervous systems, the astute physician must also consider that fever and AMS may be a consequence of disease outside of the central nervous system. One of the most common to remember is hypoglycemia, which is not a direct cause of fever, but may be a consequence of febrile illness and concomitant hypermetabolic state. Point-of-care glucose testing should be considered the sixth vital sign for any ill-appearing, altered, or vomiting child. Inborn errors of metabolism such as fatty acid oxidation disorders, carnitine transport disorder, or mitochondrial disease may also present with AMS owing to the metabolic derangements of even a minor febrile illness. Endocrinopathy-like thyrotoxicosis and pheochromocytoma should also be considered, particularly in the setting of tachycardia and hypertension for age. Finally, any anticholinergic or sympathomimetic toxidrome can also present with elevated temperatures and AMS.


Seizures


Seizures are a common clinical entity that emergency physicians must be competent in identifying and managing. Febrile, post-traumatic, and rebound seizures after medication noncompliance are all quite common; however, there are some additional considerations for seizure in pediatrics of which physicians must be aware.


Neonatal seizures


Neonatal seizures occur in 1.0 to 3.5 per 1000 live births, and can have significant consequences if misdiagnosed or mismanaged. The neonatal seizure is defined as seizure activity in a patient less than 28 days old, and can be difficult to recognize owing to the immaturity of the central nervous system. Generalized seizure activity may present only with mouthing, horizontal eye deviation, blinking, decreased responsiveness, or single limb extension, not with the classic tonic–clonic activity of a more mature brain. Common causes include hypoxic–ischemic encephalopathy, ischemic and hemorrhagic perinatal stroke, electrolyte disturbance, structural brain abnormality, abusive head trauma, or infections. The first-line treatment for neonatal seizures is phenobarbital 20 mg/kg, but if there is a delay in administration, a benzodiazepine can be given. Second-line therapy includes fosphenytoin, levetiracetam, midazolam, and lidocaine ( Table 3 ). In refractory neonatal seizures, pyridoxine may be also be effective in some metabolic errors.


Jul 11, 2021 | Posted by in EMERGENCY MEDICINE | Comments Off on Neurologic Emergencies at the Extremes of Age
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