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Pediatric-Onset Rehabilitation

Neuropathic and Nociceptive Pain

Pain after SCI can appear in many forms and is commonly divided into nociceptive or neuropathic pain.  The International Association for the Study of Pain defines nociceptive pain as “pain that arises from actual or threatened damage to non-neural tissue and is due to the activation of nociceptors” (International Association for the Study of Pain, 2021). In contrast, the International Association for the Study of Pain defines neuropathic pain as “caused by a lesion or disease of the somatosensory nervous system” that can be related to either the central or peripheral nervous system (International Association for the Study of Pain, 2021). In SCI, neuropathic pain may arise from the territory below the level of neurologic injury, or from the border zone (the region of transition between normal and abnormal sensation).  There is no question that pain of all types is a frequent occurrence for adults with SCI, and the institution of effective management strategies remains a challenge.

There has been little formal research regarding pain in pediatric SCI.  The literature does provide some insights into the high prevalence of pain for this population, which serves to clearly highlight a need for more information about effective pain management in children with SCI.

Author, Year

Country

Study Design

Sample Size

Population

Intervention

Outcome Measure

Results

(Jan & Wilson, 2004)

USA

Observational

N=31

Population: Age at interview: 13.9 (3-46) yr; Age at Injury: 5 mo-18 yr (n=20 <8 yr); Gender: males=15, females=16; Level of Injury: cervical=12 (n=4 ventilator dependent), thoracic=16, lumbar=3. Severity of injury: complete=18, incomplete=13.

Intervention: None.

Outcome Measures: Type (i.e., nocioceptive [musculoskeletal or visceral] versus neuropathic), quantity and severity of pain, Lansky Play Performance

Scale, Adolescent Pediatric Pain Tool.

1.         ln the entire group, 35% reported no pain and 65% reported pain.

2.        Nocioceptive pain was identified in 48% (15/31) and neuropathic pain was identified in 19% (6/31).

3.        The nocioceptive cohort was subclassified as having 10 with musculoskeletal and 8 with visceral pain.

4.        For group injured at a very early age (<3 yr old) 72% reported pain and 28% were pain free.

5.        The nocioceptive-musculoskeletal group had 60% complete and 40% incomplete injuries.

6.        All the nocioceptive-visceral group had complete injuries.

7.        The neuropathic group had 50% complete and 50% incomplete injuries.

8.        Lansky Play Scale scores ranged 50-100 with a mean of 92 and mode of 90.

For word-graphic ratings, the range of scores was 0.5-8.0 with a mean of 4.9.

(Defresne et al., 2003)

France

Observational

N=24

 

Population: Age: 2-14 yr; Gender: males=11, females=13.

Intervention: None.

Outcome Measures: Motor outcomes; urinary sequelae; Paine’s scale; cerebrospinal fluid obtained by lumbar puncture; spinal MRI.

Initial phase

1.         88% (21/24) of the patients had severe pain, which was usually symmetric (18/21) and located in one or more spinal segments (11/21), although 6 patients had leg pain and 5 had headaches.

2.        Fever was a presenting symptom in 14 of 24 patients and was not more common in the patients with a prior infection.

3.        Neurologic symptoms were present initially in only 50% of the patients and consisted of motor loss (10/12) or sphincter dysfunction (4/12).

4.        Sudden onset of a severe motor deficit was noted in 8 patients, of whom 7 became unable to walk within 12 hours and one had respiratory insufficiency.

5.        The motor symptoms antedated the sphincter dysfunction in two thirds of the patients (16/24) and were asymmetric in 71%.

 

Plateau

6.        Back pain was noted in 75% (18/24) of patients, more commonly in the neck (56%, 10/18); all but 1 patient had flaccid paralysis.

7.        The motor deficit became symmetric in 67% (16/24) of the patients.

8.        The lower limb weakness was severe in 18 (75%) patients, and one or more deep tendon reflexes in the lower limbs were abolished in 20 (83%) patients.

9.        Weakness in the upper limbs was present in 10 (42%) patients and was consistently moderate.

10.      A single patient had tetraplegia.

11.       Abnormalities in sensation were found in 19 (83%) patients, being asymmetric in 20 (82%) patients and consistent with a thoracic lesion in 21 (88%) patients and with a cervical lesion in 3 (12%) patients.

12.      Sphincter dysfunction was present in 20 patients: in 12 patients, whereas in 8 patients, the dysfunction was moderate; 11 patients had anal sphincter dysfunction.

13.      Optic neuritis was present in 4 patients.

 

Recovery phase (n=16)

14.      2 (13%) children had severe motor sequelae.

15.      8 (50%) patients recovered normal motor function. 4 (25%) patients had moderate motor sequelae and 2 (13%) had mild motor sequelae.

16.      Sensory abnormalities resolved completely in 7 children (54%).

17.      Of the 15 patients with sphincter dysfunction, 5 (33%) recovered normal sphincter function within a mean of 7 months (15 days-2 yr), 5 (33%) had mild sequelae, and 5 (33%) had severe sequelae.

18.      According to Paine’s scale, 5 (31%) children achieved a full recovery, 4 (25%) had minimal sequelae, and 7 (44%) had mild or severe sequelae.

19.      Supraspinal symptoms recovered fully in all 16 patients.

 

Prognostic factors

20.     An unfavorable outcome was more common among patients with complete paraplegia (p=0.03) and/or a time to maximal deficit shorter than 24 hours (p=0.005).

21.      A favorable outcome was associated with a plateau shorter than 8 days (p=0.03), the presence of supraspinal symptoms (p=0.01), and a time to independent walking shorter than 1 month (p=0.01).

 

Cerebrospinal fluid

22.     Abnormalities were found in 15 (62%) patients. The white blood cell count ranged from 0 to 1800/µL (mean 210/µL), and lymphocytes were the main cell type in 12 of 15 patients.

23.     The protein level was slightly elevated (up to 120 mg/dL) in 3 (20%) patients.

24.     Cerebrospinal fluid protein electrophoresis was normal in 9 of 13 patients and showed a pattern consistent with a transudate (no oligoclonal bands) in the remaining 4 patients.

25.     5 of 24 patients had serologic evidence of a recent viral infection (herpes simplex virus, n1=; Epstein-Barr virus, n=1, varicella-zoster virus, n=1; and measles, n=2).

 

MRI data

26.     2 of 6 children who underwent spinal MRI had normal findings; among the 4 other children, 1 had multiple lesions in the cervical region and conus medullaris and 3 each had a single lesion in the thoracic spinal cord.

27.     Edema of the spinal cord was visible on T1-weighted sequences in 2 children.

28.     On T2-weighted sequences, all lesions exhibited high signal intensity and extended along two or more vertebral segments.

29.     In 3 children, the high-signal lesions on the T2-weighted sequences of the initial MRI showed postgadolinium enhancement in a nodular (n=1), diffuse (n=1), or peripheral (n=1) pattern.

30.     Among the 4 patients who underwent cerebral MRI, only 1 had abnormal findings, which consisted of multiple areas of high signal in the cortex and basal ganglia on T2-weighted sequences.

31.      Follow-up MRI was performed in 3 patients and showed a return to normal in 2; the remaining patient had cervical and conus lesions on the initial MRI and persistent high signal in the conus on T2-weighted sequences after 4 months.

 

(Vogel et al., 2002b)

Part II

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 pain.

Ankle Pain and Contractures

1.         Ankle pain or contractures affected 53 subjects, with 29 individuals having contractures alone, 18 reported pain only, and 6 had complaints of both contractures and pain.

2.        Ankle pain was significantly associated with older age at injury (p=0.018) and tetraplegia (p=0.005).

3.        Ankle contractures were not significantly associated with any of the study variables.

 

Elbow Pain and Contractures

4.        Elbow pain or contractures affected 43 subjects with 27 experiencing elbow pain alone, 10 had elbow contractures alone, and 6 had both.

5.        Those with elbow pain were significantly older at follow-up (p=p=0.026) and had a longer duration of their SCI (p=0.041).

6.        As expected, elbow contractures were significantly more common in those with tetraplegia (p=0.040) and were significantly associated with lower ASIA motor scores (p=0.016) and lower total FIM (p=0.010) and motor FIM scores (p=0.009).

 

Shoulder, Back, and General Pain

7.        Shoulder pain was reported by 48% of subjects and limited activities in 21% of those affected.

8.        Shoulder pain was significantly associated with older age at interview (p=p=0.045), and longer duration of injury (p=0.034).

9.        Shoulder pain was not significantly associated with degree of neurological impairment or FIM scores.

10.      Of the 216 participants, 22% complained of back pain, which was not associated with any of the demographic, impairment or functional limitation variables.

11.       Overall, 69% complained of pain.

12.      In addition to shoulder, back, elbow, and ankle, pain involved other areas including leg (19), neck (14), hip (12), wrist (1 0), knee (9), feet (8), hands (8), and miscellaneous sites (12).

13.      Race/ethnicity was the only study variable that was significantly associated with pain, with pain more common in whites (p=0.016).

14.      There was no significant difference in the prevalence of pain among those with violent injuries (71%) in comparison to those with nonviolent injuries (69%); however, pain was significantly more common in subjects with spasticity compared to those without spasticity (p=0.001).

Author, Year

Country

Study Design

Sample Size

Objective Statement

(Shiwlochan et al., 2020)

USA

Case Report

N=1

Population: 12 yr, male, T1-2 AIS A SCI.

Intervention: Ketamine infusion.

Outcome Measures: Pain intensity.

(Armstrong et al., 1992)

Canada

Case Report

N=2

Population: Case I: 13 yr, male, complete tetraplegia SCI, severe spasticity; Case II: 5 yr, female, C1 complete tetraplegia SCI, severe spasticity.

Intervention: Intrathecal baclofen.

Outcome Measures: Spasticity, pain, transfers, dressing, catheterizations, sleep, potential adverse effects.

Discussion

As is the case with SCI in adults, the prevalence of chronic pain appears to be high in those with SCI incurred in childhood.  Among the three studies described above, prevalence of pain in various cohorts of youths with SCI ranged from 65 to 88%. In the observational study by Jan and Wilson (2004), the majority of participants with childhood-onset SCI reported nociceptive rather than neuropathic pain, though this was not specifically delineated in other studies. Therefore, it’s unclear if that observation would apply broadly to the pediatric SCI population.

Factors influencing pain prevalence are difficult to conclusively identify. Jan and Wilson (2004) reported that pain prevalence did not vary significantly with age at time of injury, level of injury, or completeness of injury, though patient numbers were small. The one notable exception to this was for visceral pain, which was reported exclusively by individuals with complete injuries. Vogel et al. (2002c) noted that pain in some body regions (shoulder and elbow in particular) was reported more frequently in those with longer duration of injury, while others (ankle and back pain) were not. They also noted that pain overall was more common in Caucasians and those with concomitant spasticity.

Reports of pain interference are somewhat conflicting. Defresne et al. (2003) found that 70% of participants with transverse myelitis who reported pain also described pain interfering with their daily activities. Jan and Wilson (2004), on the other hand, noted that very few patients with pain identified limitations in daily activities or play.  Notably, the data presented is limited by small sample sizes and incomplete information regarding SCI characteristics.

There has been no published systematic research regarding specific rehabilitative, pharmacologic, or interventional approaches to pain management for pediatric patients with SCI, representing a significant opportunity for future directions.  There is a single case report  (Shiwlochan et al. 2020) demonstrating improvement in acute allodynic pain in the immediate post-injury and post-operative stages with ketamine infusion, but the applicability of this information to the overall pediatric SCI population is minimal without further study.

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