Pediatric-Onset Rehabilitation

Autonomic Dysreflexia

Autonomic dysreflexia (AD) is a physiological phenomenon in spinal cord injuries with a neurological level of lesion above T6. Damage at or above T6 interrupts descending modulation of the thoracolumbar sympathetic preganglionic neurons that regulate vasomotor tone (arising from the T5-T12) in the splanchnic vascular bed (Eldahan & Rabchevsky 2018). Aortic and carotid baroreceptors sense hypertension and inform the brain, resulting in vagal stimulation and inhibition of the sympathetic nervous system above the neurological level of lesion (Hickey & Vogel 2002).

Hypertensive episodes in AD occur as a response to noxious visceral or somatic stimulation below the neurological level of the lesion, activating a sympathetic mass reflex leading to widespread vasoconstriction. This is especially common among patients with bladder and bowel problems.  AD is defined as an increase in blood pressure of 20-40 mm Hg in adults and 15-20 mm Hg in children (Eldahan & Rabchevsky 2018). This often comes with bradycardia as the parasympathetic innervation is non-affected, though tachycardia seems as frequent. The systolic blood pressure can reach as high as above 300 mm Hg with a risk for brain injury related to the hypertensive crisis, though luckily rare (Kewalrami & Orth 1980).

Other symptoms reported in AD are related to the sympathetic mass reflex and include a pounding headache, pilomotor reflex with goosebump, paresthesia, shivering, flushing, and hyperhidrosis above the neurological level of lesion, nasal obstruction, ocular symptoms including Horner’s syndrome, desire to void, anxiety, malaise, and nausea. There may be a feeling of dullness in the head and blurring of vision. More than 50% of patients experience severe headaches, usually of occipital, bitemporal and bifrontal location, and less frequent a sensation of precordial pressure (Karlsson 1999; Kewalrami & Orth 1980).

Education and prevention are essential in the management of AD (Hickey & Vogel 2002). In addition, It is important to regularly measure blood pressure to find the baseline for comparison and individual monitoring (Zebracki et al. 2013a). Drug treatment must be considered when these measures are inadequate or in the case of an acute hypertensive episode where conservative management is inadequate. A comprehensive consensus-based recommendation involving all these steps is published by Shriner’s Hospitals for Children (McGinnis et al. 2004). A systematic review from 2009 on the management of AD as part of the Spinal Cord Injury Research Evidence project does not specifically include pediatric subjects but gives a comprehensive report of the evidence for different treatments for adults post-SCI (Krassioukov et al. 2009).

Author, Year

Country

Study Design

Sample Size

Population

Intervention

Outcome Measure

Results

(Canon et al., 2015)

USA

Observational

N=13

Population: Mean age: 12.4 yr; Gender: males=5, females=8; Injury etiology: cervical SCI=6, thoracic SCI=3, transverse myelitis=2, encephalomyelitis=2.

Intervention: None. Urodynamic study (UDS).

Outcome Measures: Autonomic dysreflexia (systolic blood pressure 15-20 mmHg above baseline and/or presence of associated symptoms.

1.         In total, 41 UDS were performed, with an average of 3.2 studies per patient.

2.        Among 13 subjects, 1 adolescent (C1/2 level injury) and 1 prepubertal child (T2/3 level injury) experienced AD; both patients experienced AD initially and on subsequent UDS, with one having a total of seven episodes of AD.

3.        Symptoms of AD for one subject included blood pressure elevation, facial flushing.

4.        Symptoms of AD and hypertension were resolved in both subjects with bladder drainage alone, without any need for pharmacological intervention; no major complications were observed.

5.        There did not appear to be noticeable correlations of AD with gender, actual-to-estimated bladder ratio, presence of uninhibited detrusor contractions, bladder compliance, presence of bacteria during UDS, or those with transverse myelitis or encephalomyelitis.

(Hwang et al., 2014a)

USA

Observational

N=351

Population: Pediatric-onset SCI: Mean age at injury: 13.8 (0-18) yr; Mean age at interview: 26.7 yr; Gender: males=226, females=125; Time since injury: 12.9 yr; Level of injury: C1-4 AIS ABC=52, C5-8 AIS ABC=126, T1-S5 AIS ABC=136, AIS D=34, unknown=3.

Intervention: None. Survey.

Outcome Measures: Incidence and prevalence of medical complications (pressure ulcers [PU], autonomic dysreflexia [AD], spasticity).

1.         In total, 1793 interviews were conducted.

2.        The prevalence of PU, AD, and spasticity were higher in those with more rostral neurologic level of injury, whereas the prevalence of most complications were lower in the AIS D group compared to the other impairment groups.

3.        At first interview, the prevalence of hypertension/cardiac disease was similar among the injury severity groups (2.0–2.9%), as was the prevalence of shoulder pain (38–50%).

4.        Over a median interval of 5.1 yr between the first and last interviews in all our participants (n=351), the prevalence of bladder accidents, hypertension/cardiac disease, and activity limiting upper extremity joint pain showed a tendency to increase.

5.        The prevalence of PUs, urolithiasis and bone fractures showed a pattern of decrease, while no patterns of change in prevalence was noted for UTI, AD, spasticity, pneumonia/respiratory failure, and bowel accidents between the two time points.

6.        Odds of complication occurrence over time varied among severity groups, with increased ORs in the C1-4 ABC group of:

·          severe urinary tract infection (1.05, CI 1.02–1.09);

·          AD (1.09, CI 1.05–1.14);

·          spasticity (1.06, CI 1.01–1.11);

·          pneumonia/respiratory failure (1.09, CI 1.03–1.16); and

·          hypertension/cardiac disease (1.07, CI 1.01–1.15);

·          Odds of complication occurrence over time varied among severity groups, with increased ORs in the C5–8 ABC group: AD (1.08, CI 1.04–1.13); and pneumonia/respiratory failure (1.09, CI 1.02–1.16).

·          Odds of complication occurrence over time varied among severity groups, with increased ORs in the T1-S5 ABC group: hypertension/cardiac disease (1.08, CI 1.02–1.14).

7.        Upper extremity joint pain had increased odds of occurrence in all injury severity groups.

(Hwang et al., 2014b)

USA

Observational

N=283

Population: Pediatric-onset SCI: Age at interview: 27.3±3.7 (21-37) yr; Age at injury: 14.5±4.3 (0-18) yr; Gender: males=182, females=101; Time since injury: 12.7±5.0 (4-30) yr; Level of injury: tetraplegia=174; Severity of injury: complete=195; C1-4 AIS ABC=46, C5-8 AIS ABC=110, T1-S5 AIS ABC=99, AIS D=28.

Intervention: None. Annual interviews.

Outcome Measures: Satisfaction with Life Scale (SWLS), Short-Form 12 Health Survey (SF-12), Patient Health Questionnaire-9 (PHQ-9), and Craig Handicap Assessment and Recording Technique (CHART).

1.         Those attaining a bachelor’s degree or higher had increased from 33.2% at the first interview to 47.0% at the last interview.

2.        There was no change in the proportion of employed versus unemployed from the first (56.8% versus 43.2%) to last interview (58.1% versus 41.9%) (less than general population estimates).

3.        At the last interview, the proportion of employed participants was significantly higher in those with a baccalaureate and post-baccalaureate degrees, whereas the proportion of unemployed individuals was higher in those with a high school diploma.

4.        Women and married participants also had higher rates of employment at the last interview than men and single participants, respectively.

5.        There was no significant change in employment status over time (OR 1.01, confidence interval (CI) 0.98-1.04).

6.        Odds of employment increased over time in participants who were women (1.04, CI 1.00-1.08), married (1.05, CI 1.02-1.08), attained a baccalaureate degree (1.03, CI 1.00-1.07), or post-baccalaureate degree (1.05, CI 1.02-1.08).

7.        Odds of employment decreased over time in participants with occurrence of autonomic dysreflexia (0.80, CI 0.65-0.99), spasticity (0.80, CI 0.59-0.99) or chronic medical condition (0.83, CI 0.71-0.98).

8.        Life satisfaction (SWLS) scores increased over time in those who remained employed (1.11, CI 1.01-1.22).

9.        Odds of depression (PHQ-9) increased over time in those who remained unemployed (1.13, CI 1.04-1.23).

(Zebracki et al., 2013b)

USA

Observational

Part I (n=279)

Population: Age: 0-5 yr=30, 6-12 yr=93, 13-15 yr=52, 16-18 yr=104; Gender: males=160, females=119; Time since injury: 38.7±44.0 mo; Level and severity of injury: C1-4 AIS ABC=29, C5-8 ABC=56, T1-S5 ABC=175, D=19; complete=174, tetraplegia=94.

Intervention: None. Chart review.

Outcome Measures: Systolic and diastolic blood pressure, heart rate.

1.         There was a stepwise increase in baseline blood pressures and decrease in heart rates with increasing age (p<0.001).

2.        Boys demonstrated higher systolic blood pressures (p<0.001) whereas girls had higher heart rates (p=0.02); this is similar to the difference observed in typically developing youths.

3.        There was no difference in diastolic blood pressure between genders.

4.        There was a significant diurnal difference in blood pressure and heart rate, with both elevated in the evening compared to morning values (p<0.001).

5.        There was no significant difference in any measures between youth with tetraplegia and those with paraplegia.

6.        A significant association was not found for duration of injury with any of the measures.

(Schottler et al., 2009)

USA

Observational

N=215

Population: Age: 9.1 yr; Gender: males=127, females=88; Level of injury: tetraplegia=116, paraplegia=99; >T6=168,

Intervention: None. Survey.

Outcome Measures: Patients and families were asked four yes/no questions: (1) Does the patient experience autonomic dysreflexia (AD)? (2) Does the patient/caregiver know what AD is? (3) Can the patient/caregiver name three signs/symptoms of an AD

episode? (4) Does the patient/caregiver know how to treat AD?

Does the patient experience AD?

1.         Overall, 40% of patients and 44% of caregivers said that the patient did experience or was symptomatic for AD.

2.        Multiple logistic regression showed that children with injury levels ≥T6 (p<0.001) and those in the oldest age (14-21 yr; p<0.001) were more likely to say that they experienced AD.

3.        Multiple logistic regression analysis showed that caregivers of people with injury levels ≥T6 (p=0.005) and those with a greater injury severity (AIS; p=0.014) were more likely to experience AD.

 

Does the patient/caregiver know what AD is?

4.        There was no association between patients’ ability to define AD with gender, race or AIS classification.

5.        Patients who were able to define AD were more likely to have traumatic etiologies (p<0.001), have ≥T6 injuries (p=0.007), have a shorter duration of injury (p<0.001) and be in the oldest age at injury group (14-21 yr; p<0.001).

6.        Caregivers of who were able to define AD were more likely to have patients with traumatic etiologies (p=0.007), have ≥T6 injuries (p=0.001), and be in the oldest age at injury group (14-21 yr; p<0.010).

 

Can the patient/caregiver name three signs/symptoms of an AD episode?

7.        There was no association between a patient’s ability to identify three signs/symptoms of AD with gender, race or AIS classification.

8.        Patients with the ability to name three signs/symptoms of AD were more likely to have traumatic injuries (p=0.014), ≥T6 injuries (p=0.006), have a shorter duration of injury (p=0.030), and be in the oldest age at injury group (14-21 yr; p<0.001).

9.        Caregivers with the ability to name three signs/symptoms of AD were more likely to have children with injuries ≥T6 (p=0.001) and who were older at interview (p=0.005).

 

Does the patient/caregiver know how to treat AD?

10.      There was no association between a patient’s knowledge of how to treat AD with gender, race or AIS.

11.       Patients who were able to express how to treat AD were more likely to have traumatic etiologies (p=0.001), have ≥T6 injuries (p=0.003), have a shorter duration of injury (p=0.003) and be in the oldest age at injury group (14-21 yr; p<0.001).

12.      Caregivers who were able to express how to treat AD were more likely to have children with traumatic etiologies (p=0.020), level of injury (p<0.001), age at injury (p=0.032) and age of patient at time at interview (p=0.008).

13.      Of the patients with a positive history of AD, 15% did not know the definition of AD, 20% could not identify three signs/symptoms of AD and 6% said they did not know how to treat an AD episode if it were to occur.

14.      For the caregivers of patients who experienced AD, 9% did not know the definition of AD, 20% could not identify three signs/symptoms and 9% said they did not know how to treat an AD episode.

(Liusuwan et al., 2007)

USA

Observational

N=215

(N=33 SCI)

Population: SCI Group (n=33): Age: 17.5±2.2 yr; Gender: males=21, females=12. SB Group (n=66): Age: 15.8±2.6 yr; Gender: males=36, females=30. Able-Bodied Overweight (OW, n=31) Group: Age: 15.6±2.6 yr; Gender: males=12, females=19. Able-Bodied Control (CTRL, n=85) Group (n=60): Age: 15.9±2.4 yr; Gender: males=44, females=16.

Intervention: None. Anthropometric testing.

Outcome Measures: Height, weight, Bone Mineral Content (BMC), Fat Tissue Mass (FTM), Total Lean Tissue Mass (TLM), Total Body Fat, Resting Energy Expenditure (REE).

1.         There was no significant difference in height between the CTRL and OW groups, but the SB group was significantly shorter (p<0.05).

2.        The OW group weighed significantly more than the SB, SCI, and CTRL groups(p<0.05).

3.        The OW group BMI was significantly higher than that of the SB group, which in turn was significantly higher than those of both the CTRL group and SCI group (p<0.05).

4.        BMI was not significantly different between CTRL and SCI groups (p<0.05).

5.        SB subjects had the lowest TLM compared to the CTRL and OW groups (p<0.05), but there was no significant difference in TLM between SB and SCI.

6.        Although the OW group had significantly higher fat mass than all other groups, there was no significant difference between the percent fat of OW versus SB group.

7.        When REE was adjusted for kg of TLM, there were no differences in REE/TLM ratio among the CTRL, OW, and SCI groups; SB had significantly higher REE/TLM ratios as compared to the REE/TLM ratios in the CTRL, OW, and SCI groups.

(Hickey et al., 2004)

USA

Observational

N=121

Population: Age: 6 yr (0-13 yr), divided into three age groups: 0-5 yr, 6-13 yr, 14-21 yr.

Intervention: None. Chart Review.

Outcome Measures: Episodes of autonomic dysreflexia (AD).

1.         Among 121 subjects, 62 (51%) experienced AD.

2.        A total of 27 AD episodes were experienced while during hospitalization and 163 episodes during an outpatient visit for which there were no significant differences in causative factors or symptoms between settings.

3.        The most common causes of AD were urologic complications (75%), primarily bladder distension (89%), and bowel impaction (18%).

4.        For episodes of AD that occurred in all three age ranges, the most common symptoms were facial flushing (43%), headache (24%), sweating (15%), and piloerection (14%).

5.        In contrast to the two older age groups, the youngest age group experienced headaches (p=0.047) and piloerection (p=0.046) uncommonly and facial flushing more commonly (p=0.016).

6.        Of the 62 affected participants, 27 AD episodes were observed in 18 individuals

·       2 episodes occurred in children <5 yr, 19 occurred among those 6-13 yr and 6 in those 14-21 yr;

·       mean increases in systolic and diastolic blood pressure was 45 mm Hg and 30 mm Hg;

·       heart rate was evaluated in just 16 episodes for which it was within 10% of baseline values for 6 episodes, bradycardic for 2 episodes (>20% below baseline), and tachycardic for 8 episodes (>20% above baseline).

·       Pharmacological management was not required for any of the observed episodes, and there were no observed or reported complications of AD.

7.        AD episodes were greater among those with:

·       complete tetraplegia compared to complete paraplegia (p=0.047);

·       traumatic SCI compared to medical or surgical causes (p=0.018; 6-13 yr age bracket only);

·       those injured at an older age (6-21 yr) compared to those injured younger (<5 yr; p=0.014);

8.        Regression analysis showed that AD was significantly associated with completeness of injury (complete versus incomplete) and older age at injury (6-13 yr versus <5 yr).

(Vogel et al., 2002b)

Part I

USA

Observational

N=216

Population: Age at injury: 14.1±4.0 yr; Age at interview: 28.6±3.4 yr; Gender: males=150, females=66; Time since injury: 14.2±4.6 yr; Level of injury: tetraplegia=123, paraplegia=93. Severity of injury: C1-4 ABC=41, C5-8 ABC=67, T1-S5 ABC=82, tetra/para D=26.

Intervention: None. Survey.

Outcome Measures: Prevalence of urinary tract infections (UTI), pressure ulcers, hemorrhoids and rectal bleeding, hospitalizations, urinary stones, orchitis or epididymitis, pneumonia, need for ventilatory

assistance, thromboembolism, and latex allergy, bladder and bowel incontinence, length of bowel

program, constipation or diarrhea, dysreflexia, hyperhidrosis frequency of smoking cigarettes or marijuana, drinking alcohol.

**Analyses of AD were limited to individuals with C1 to T61evels of injury:

1.         Within this group, 54% experienced AD; of 85 individuals, 74 had tetraplegia, and 11 had paraplegia.

2.        AD affected 62% of the subjects with tetraplegia and 30% of those with T1 to T6 paraplegia.

3.        Of the individuals with T6 or higher SCI, who did not report AD, 24% had an ASIA Impairment Scale score of D; in contrast, none with T6 or higher lesions, who experienced AD, had ASIA Impairment scores of D (p<0.001).

4.        Those with AD had significantly lower ASIA Motor scores compared with those who did not experience AD (p<0.001).

5.        A total of 31 subjects experienced hyperhidrosis (22 had tetraplegia and 9 had paraplegia).

6.        Of the 9 subjects with paraplegia and hyperhidrosis, 5 had T1 -T6 lesions and 4 had lower thoracic lesions.

7.        Individuals with hyperhidrosis had significantly lower ASIA Motor scores (p=0.007).

8.        Subjects who reported having hyperhidrosis were significantly more likely to experience AD compared with those who did not have hyperhidrosis p<0.001).

9.        Among those with C1 to T6 SCI, those who experienced hyperhidrosis were more likely to experience AD compared with those who did not experience hyperhidrosis (p=0.002).

(Vaidyanathan et al., 1998)

United Kingdom

Case Series

N=24

(N=3 pediatric patients)

(N=11 pediatric-onset SCI patients)

Population: Children with SCI (n=3):

Case CS: 3 yr male, C1-2 tetraplegia;
Case SM: 2 yr, male, ventilator-dependent tetraplegia;
Case NB: 3 yr male, ventilator-dependent tetraplegia;

 

Adults with pediatric-onset SCI (n=11)

Case WC: 32 yr female with SCI at 14 yr, C4 tetraplegia.

Case MH: 32 yr male with SCI at 21 yr, C5 tetraplegia.

Case KW: 44 yr male with SCI at 15 yr, C4 tetraplegia.

Case DM: 22 yr male with SCI at 17 yr, C6 tetraplegia.

Case GE: 30 yr male with SCI at 17 yr, C5 tetraplegia.

Case SB: 29 yr male with SCI at 17 yr, C5 tetraplegia.

Case AM: 33 yr male with SCI at 14 yr, T4 paraplegia.

Case OL: 29 yr male with SCI at 19 yr, C3 tetraplegia.

Case AG: 30 yr male with SCI at 17 yr, C5 tetraplegia.

Case DB: 16 yr male with SCI at 15 yr, C4 tetraplegia.

Case PD: 27 yr male with SCI at 19 yr, C4 tetraplegia.

Intervention: 1 mg (adults) or 0.5 mg (children) terazosin titrated up to a maximum dose, if appropriate (i.e., 10 mg in adults and 2 mg in children).

Outcome Measures: Abatement of autonomic dysreflexia and side effects.

Children (n=3):

1.         Case CS required a maximum dose of terazosin 2 mg + oxybutynin 5 mg + 2.5 mg + 5mg and had nil side affects.

2.        Case SM required a maximum dose of terazosin 1 mg + oxybutynin 2.5 mg 2x/day and had nil side affects.

3.        Case NB required a maximum dose of terazosin 1 mg + oxybutynin 2 mg 4x/day and had nil side affects.

Adults with pediatric-onset SCI (n=11)

4.        Case WC required a maximum dose of 3 mg + oxybutynin 5 mg and experienced nil side affects.

5.        Case MH required a maximum dose of 5 mg and experienced nil side affects.

6.        Case KW required a maximum dose of 6 mg and experienced nil side affects.

7.        Case DM required a maximum dose of 5 mg and experienced nil side affects.

8.        Case GE required a maximum dose of 2 mg and experienced nil side affects.

9.        Case SC required a maximum dose of terazosin 5 mg and had nil side affects.

10.      Case AM required a maximum dose of terazosin 3 mg and had nil side affects.

11.       Case OL required a maximum dose of terazosin 1 mg and had nil side affects.

12.      Case AG required a maximum dose of terazosin 4 mg and had nil side affects.

13.      Case AG required a maximum dose of terazosin 2 mg and had nil side affects.

14.      Case PD required a maximum dose of terazosin 2 mg and had nil side affects.

Author, Year

Country

Study Design

Objective/Purpose

(Lockwood et al., 2016)

USA

Case Report

N=1

Population: 11 yr, male, T4 paraplegia SCI.

Intervention: Oral oxybutynin, Botulinum toxin injections, Nifedipine,

Outcome Measures: Autonomic dysreflexia

(Bjelakovic et al., 2014)

Serbia

Case Report

N=1

Population: 12 yr, male, SCI (neck injury resulting in spastic paraplegia with CT scan showing spinal atrophy at C5-6 and T1-2).

Intervention: Midazolam, Furosemide, Mannitol, and a urinary catheter in the emergency department, followed by Xatral, Enalapril, and Presolol post AD diagnosis.

Outcome Measures: Autonomic dysreflexia.

Author, Year

Country

Study Design

Sample Size

 Study Characteristics

Results

(McLean et al., 1999)

USA

Observational

N=54

Population: Temperature Instability (n=12): Age: 11.9 (5.5-17.6) yr; Gender: males=11, females=1; Injury etiology: traumatic SCI; Time since injury: 7.3 months (10 days-11.5 mo); Level and severity of injury: paraplegia AIS A T5-6=2, tetraplegia AIS A=10. Temperature Stability (n=42): Age: 11.8 (2.4-20.3) yr; Gender: males=21, females=21; Etiology: traumatic SCI=37, tumour=4, meningitis=1, Coffin-Lowry Syndrome=1; Level and severity of injury: tetraplegia=38 (AIS A complete=36); Time since injury: 22.0 mo (13 days-160 mo).

Intervention: None. Chart Review.

Outcome Measures: Temperature.

1.         Among 54 patients, 12 (22%) subjects endured 65 episodes of environmentally responsive hypothermia and 14 events of hyperthermia; one patient accounted for 34 episodes of hypothermia and 8 episodes of hyperthermia.

2.        Average temperature of the hypothermic events was 35.28C, with a median temperature of 35.58C, and a range of 33.0±35.58 C.

3.        The average temperature of the recorded hyperthermic events was 39.08 C, with a median temperature of 39.08 C, and a range of 38.0± 0.48 C.

4.        The average duration of hypothermic events was 5.2 h, compared with an average duration of 8.5 h for the hyperthermic events.

5.        Subjects in the temperature stability group had a greater duration of time since injury (p<0.001), and shorter rehabilitation length of stay (p<0.001).

Author, Year

Country

Study Design

Sample Size

Study Characteristics

(Adams et al., 2002)

USA

Case Report

N=1

Population: 12 yr, female, epidural hematoma secondary to non-traumatic ruptured cavernous venous malformation of the spinal cord, C7-T3, 5 mo post-injury.

Problem: Hyperhydrosis.

Intervention: Received 300 mg Gabapentin, twice daily (15 mg/kg), increased to 900 mg Gabapentin, three times per day (65 mg/kg), and then increased again to 1200 mg Gabapentin (88 mg/kg), three times per day, followed by propantheline bromide (7.5 mg three times and then reduced to twice per day).

Discussion

Several observational studies and case reports, as well as one case series, examined the prevalence, etiology, and management of AD in the pediatric SCI population. Most of the studies originated from one center, Shriners Hospitals for Children (Hickey & Vogel 2002; Hickey et al. 2004; Hwang et al. 2014a, 2014b; Schottler et al. 2009; Schottler et al. 2012; Vogel et al. 2002b; Zebracki et al. 2013a, 2013b).

As highlighted by Kewalramami and Orth (1980) and  Krassioukov (2003), AD can occur, at the earliest, 8 weeks post-injury in children with SCI, but most often presents its first signs 12 to 16 weeks post-injury. Kewalramami and Orth (1980) also found that the onset of AD correlated with the occurrence of a positive bulbocavernosus reflex in their sample of 68 patients. Urologic complications, bladder distension, and bowel impaction were identified as some of the most common causes of AD (Canon et al. 2015; Hickey et al. 2004). Regarding the prevalence of AD in the pediatric SCI population, the studies revealed a rate of around 50% among those with a neurological level of the lesion at T6 and above, which is comparable to that in the adult population (Schottler et al. 2009; Schottler et al. 2012; Vogel et al. 2002b). Complete injuries, traumatic injuries, and older age seemed to be associated with a higher risk of AD (Hickey et al. 2004; Hwang et al. 2014a; Kewalrami & Orth 1980; Schottler et al. 2009; Schottler et al. 2012; Vogel et al. 2002b).

It is worth noting that the lower prevalence of AD in the younger age group may be confounded by factors such as young children’s difficulties in articulating their symptoms as well as differences in the presentation of symptoms between younger and older children. (Hickey et al. 2004). Upon reviewing the medical record of 121 children with SCI who received care at Chicago Shriner’s Hospital for Children, the researchers observed that headache and piloerection were reported less frequently in children with AD who were between ages 0-5 years compared to those between ages 6-13 and 14-21; on the other hand, facial flushing was more commonly reported in the youngest age group compared to that in the two older age groups.

As discussed previously, hyperhidrosis is a common symptom of AD. In one study, McLean et al. (1999) investigated the frequency and clinical implications of environmentally responsive temperature instability in 54 hospitalized pediatric patients with SCI. It was found that 12 (22%) patients experienced a total of 65 episodes of hypothermic events (i.e., oral temperatures less than 35.08 °C or rectal temperatures less than 35.68 °C) and 14 Hyperthermic events (i.e., oral temperatures greater than 38.08 °C or rectal temperatures greater than 38.48 °C). In addition, patients with environmentally responsive temperature instability are more likely to have shorter time post-injury, longer lengths of stay, and be ventilator-dependent than those who did not have environmentally responsive temperature instability (McLean et al. 1999). Through examining a case of a 12-year-old girl with hyperhidrosis from an SCI, Adams et al. (2002) suggested that gabapentin may be an effective therapy for SCI-related hyperhidrosis. However, larger-scale studies are needed to confirm this finding.

Schottler et al. (2009) examined knowledge of AD from the perspectives of youth with SCI and their caregivers, as part of a multicenter study including 215 participants. Participants answered questions concerning the definition, signs and symptoms, and treatment of AD. Not surprisingly, the study found that patients with greater knowledge of AD were more likely to have traumatic etiology, have T6 or higher injuries and shorter duration of injury, and be in older age groups.

The occurrence of AD has not only been linked to various medical complications but also decreased the likelihood of employment in adults with pediatric-onset SCI (Hwang et al. 2014a, 2014b). Therefore, the prevention and management of AD in this population is crucial. Zebracki (2013b) has suggested that one way of preventing and/or identifying early signs of AD in the pediatric SCI population is through the regular assessment and management of baseline blood pressure and heart rate.

The recommendations concerning the management of AD are mainly consensus-based (McGinnis et al. 2004) and/or drawn from anecdotal evidence from case reports (Bjelakovic et al. 2014; Lockwood et al. 2016; Tronnes & Berg 2012), with the exception of a study by Vaidanathan et al. (1998). Vaidyanathan et al. (1998) examined the effectiveness of Terazosin, an alpha-1 blocking agent, in managing symptoms of AD in 24 patients with SCI who had recurrent episodes of AD. Three of the patients were children with SCI who were ventilator-dependent, and 11 were adults with pediatric-onset SCI. The drug was individually titrated with a starting dose of 0.5 mg at bedtime for children and was thereafter titrated with 1.0/0.5 mg every 3-4 days until side effects or problems were resolved. One patient discontinued the medication due to dizziness. For the others, the side effects were self-limiting and noticed only during the first week of treatment and included feelings of tiredness and drowsiness. Following the terazosin therapy, the dysreflexic symptoms subsided completely for all patients. However, the authors note that Terazosin is not the first line of treatment, and should only be considered for selected patients with recurrent episodes of dysreflexia, in whom, it is not possible to identify the predisposing cause, or in whom it is not feasible to abolish the precipitating factor for the AD.

The prevalence of AD in the pediatric SCI population is around 50%, which is comparable to that in the adult SCI population.

Complete injuries, traumatic injuries, and older may be linked to a higher risk of AD in children with SCI.

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