Demographic data
The pool of this study is 30 patients, and their ages ranged from 1 to 16 years. The mean age was 6.1. The most affected age was 3. There were 18 (60%) males and 12 (40%) females.
Out of the 30 patients of acute secondary inability to walk, 12 patients (40%) had confirmed diagnosis of acute disseminated encephalomyelitis, 7 patients (23.3%) with GBS, 5 patients (16.7%) with acute transverse myelitis, 3 patients (10%) with multiple, and 3 patients (10%) with other diagnoses such as spinal tumor and cerebrovascular events.
ADEM
ADEM (12 out of 30 patients) was the most common CNS abnormality found. There were 8 males and 4 females, all presented with clinical paralysis of upper motor neuron lesion (UMNL).
Definite diagnosis of those patients was achieved with clinical symptoms suggesting ADEM including encephalopathy, and MRI findings.
MRI findings
The lesions demonstrated hyperintense signal on T2W and FLAIR images and iso/hypointense signal T1W in all patients.
All patients showed white matter subcortical region affection, and no patient had gray matter affection. All patients showed bilateral involvement of cerebral areas, seven patients (58.4%) had symmetrical affection and about five patients (41.6%) had asymmetrical involvement.
Subcortical white matter hyperintense T2/ FLAIR lesions were evident in all pediatric patients with ADEM, i.e., in 100% of our patients during their initial brain MRI.
In our study, MRI showed high sensitivity in diagnosis of ADEM (100% sensitivity and 100% negative predictive value (NPV)). However, MRI had about 66% specificity, with 92% positive predictive value (PPV).
All the patients demonstrated MRI pattern as ADEM 1 pattern in six patients (50%) (Fig. 1) and ADEM 2 pattern in six patients (50%) (Fig. 2).
Most frequently affected areas were bilateral fronto-parietal areas in 6 patients (50%), parietal areas were affected in 4 patients (33.3%), and occipito-parietal areas were affected in 2 patients (16.7%).
All patients demonstrated lesion enhancement as patchy pattern (Fig. 3), seven patients (58.3%), and ring pattern (Fig. 4) in five patients (41.7%).
Clinic-pathological correlation with ADEM disease severity
The patients with ADEM were divided according to their disability at the time of presentation into 2 groups: group A with 1 or 2 Hughes scale and Glasgow Coma Scale 13 or more, and group B with 3 or 4 Hughes scale and Glasgow Coma Scale 12 or less.
In our study, group A patients, 5 of them (62.5%) showed fronto-parietal involvement, 2 of them (25%) showed occipito-parietal involvement, and one of them (12.5%) showed parietal involvement.
Among group B patients, one of them (25%) showed fronto-parietal involvement and the other three of them (75%) showed parietal involvement.
Patients with ADEM were also evaluated for their bladder affection, among patients with bladder affection, one of them (20%) showed fronto-parietal involvement, 3 of them (60%) showed parietal involvement while one of them (20%) showed occipito-parietal involvement.
Among patients with no bladder affection, three of them (75%) showed fronto-parietal involvement and one of them (25%) showed occipito-parietal involvement.
In our study, there was no significant correlation between the severity of the disease and lesion distribution in MRI (P value = 0.120 for Hughes scale and P value = 0.377 for bladder affection).
In our study, in group A patients, 4 of them (50%) showed ADEM pattern 1 in MRI while 4 of them (50%) showed ADEM pattern 2 with relatively large lesions.
In group B patients, two of them (50%) showed ADEM pattern 1 while two of them (50%) showed ADEM pattern 2.
Patients with ADEM were also evaluated for their bladder affection, among patients with bladder affection, three of them (60%) showed ADEM 1 pattern while two of them (40%) showed ADEM 2 pattern.
Among patients who showed no bladder affection, one of them (25%) showed ADEM 1 pattern while three of them (75%) showed ADEM 2 pattern.
In our study, there was no significant correlation between the severity of the disease and the pattern of the lesions in MRI (P value = 1 for Hughes scale and P value = 0.61 for bladder affection).
Among group A, 5 patients (62.5%) showed patchy pattern of enhancement and 3 of them (37.5%) showed ring pattern of enhancement.
Among group B, two patients (50%) showed patchy pattern of enhancement and the other two (50%) showed ring pattern of enhancement.
Among the patients with bladder affection, two (40%) showed patchy pattern of enhancement while three patients (60%) showed ring pattern of enhancement.
Among the patients with no bladder affection, three patients (75%) showed patchy pattern of enhancement while one patient (25%) showed ring pattern of enhancement.
In our study, there was no significant correlation between the severity of the disease and the enhancement pattern of the lesions in MRI (P value = 0.639 for Hughes scale and P value = 0. 773 for bladder affection).
Prognosis in ADEM patients
Patient with ADEM showed various prognostic sequel 2 weeks after the onset of the disease: complete recovery, walking with aid, and worsening of symptoms which needed ICU admission.
Among patients who showed complete recovery (6 patients), four of them (66.6%) showed fronto-parietal involvement, one patient (16.6%) showed parietal involvement, and one patient (16.6%) showed ADEM occipito-parietal involvement.
Four of them (66.6%) showed ADEM pattern 1 and two patients (33.3%) of them show ADEM pattern 2.
Four of these patients (66.6%) showed patchy pattern of enhancement and two of them (33.3%) showed ring pattern of enhancement.
Among the two patients who developed walking with aid after 2 weeks, one showed fronto-parietal and the other showed parietal involvement, both showed ADEM 2 pattern, one showed patchy enhancement and the other showed ring enhancement.
Among patients who showed worsening of their symptoms and needed ICU admission (4 patients), one patient (25%) showed fronto-parietal involvement, two patients (50%) showed parietal involvement, and one patient (25%) showed occipito-parietal involvement.
Two of those patients (50%) have ADEM 1, two patients (50%) ADEM 2 pattern. Two patients (50%) showed patchy enhancement and the other two (50%) showed ring enhancement.
In our study the lesion size, distribution and enhancement pattern did not correlate with the prognosis (P value = 0.740 for lesion size, P value = 0.740 for enhancement pattern and P value = 0.610 for the affected areas).
GBS
We had seven patients with GBS, five males and two females, all with lower motor neuron (LMN) paralysis.
Definite diagnosis of those patients was achieved by clinical symptoms including acute lower limb ascending paralysis, electrodiagnosis, and MRI findings.
MRI findings
All patients demonstrated thickened cauda equina nerve roots with post-contrast T1W enhancing nerve roots. All the patients demonstrated nerve root enhancement pattern as GBS 2 pattern, 4 patients (57%) (Fig. 5), and GBS 3 pattern, 3 patients (43%) (Fig. 6).
MRI proved to be very sensitive and specific in diagnosis of GBS; nerve root enhancement was evident in all patients with GBS, i.e., in 100% of our patients during their initial spinal magnetic resonance imaging.
In our study, MRI showed high sensitivity and specificity in diagnosis of GBS (100% sensitivity, 100% NPV, and specificity 100%, with 100% PPV), and larger sample size would be of value for more accurate results.
Clinico-pathological correlation with GBS
The patients with GBS are divided according to their neurological signs at the time of presentation into 2 groups: group A with 1 or 2 Hughes scale and group B with 3 or 4 Hughes scale.
Among group A patients, two of them (50%) showed GBS pattern 2 while two of them (50%) showed GBS pattern 3 enhancement.
Among group B patients, two of them (66.6%) showed GBD pattern 2 while one patient (33.3%) showed GBD pattern 3.
In patient with GBS who showed bladder affection, three of them (75%) showed GBS 2 pattern while one of them (25%) showed GBS 3 pattern.
In patient with GBS who showed no bladder affection, two of them (66.6%) showed GBD 2 pattern while one of them (33.3%) showed GBD 3 pattern.
In our study, the pattern of nerve root enhancement did not correlate with the severity of the symptoms (P value = 1 for Hughes scale, P value = 0. 486 bladder affection).
Prognosis in GBS
Patient with GBS also showed various prognostic sequel 2 weeks after the onset of the disease: completer recovery, walking with aid, and worsening of symptoms.
Patient who showed complete recovery (4 patients), two of them (50%) showed GBS pattern 2 and two patients (50%) showed GBS pattern 3.
Patients who developed walking with aid after 2 weeks (two patients), both showed GBS 2 pattern, while the patient who needed ICU admission for worsening of his symptoms showed GBS 3 pattern.
In our study, the pattern of nerve root enhancement did not correlate with the prognosis (P value = 0.429).
ATM
We had five patients with acute transverse myelitis (ATM) (three males and two females), all with LMN paralysis. Three patients had a history of upper airway infection prior to the onset of the disease.
Definite diagnosis of those patients was achieved with clinical symptoms of acute lower limb paralysis and MRI findings.
MRI findings
Affected areas of the spinal cord were multiple dorsal and cervical spinal lesions in one patient, one patient had affected cervical spinal lesion, and three patients had dorsal spinal lesion.
MRI proved to be a very important diagnostic modality of ATM; abnormal spinal hyperintense T2 signal (Fig. 7) was evident in all patients with ATM, i.e., in 100% of our patients during their initial spinal MRI.
In our study, MRI showed high sensitivity in diagnosis of ATM (100% sensitivity and 100% NPV). However, MRI had about 90% specificity, with 83% PPV. As one patient had cervical cord lesion opposite to C3,4,5 and proven to be MS by clinical assessment as the patient had a history of previous episode of neurological symptoms consistent with MS, patient had no clearly defined sensory level as well as CSF analysis was not suggestive of ATM.
Out of the five patients, one showed multiple segments of signal alteration seen within the anterior aspect of the cervical and dorsal spinal cord, the longest seen opposite C4 down to D1 levels; a patient had a C2, C3, and C4 segment of spinal cord signal alteration at the cervical spinal cord; a patient had D9 down to L1 level segment of dorsal spinal cord signal alteration; a patient had D10 to L1 level segment of dorsal spinal cord signal alteration; and a patient had D7 to D11 level segment of dorsal spinal cord signal alteration,
All the previous patients showed spinal cord signal alteration, eliciting intermediate to low T1W and high T2W signal (Fig. 8).
Clinico-pathological correlation with ATM
Three patients out of five (60%) showed grade 2 according to Hughes scale; all three patients showed dorsal cord affection and 4–5 segments by MRI.
Two patients out of five (40%) showed grade 4 according to Hughes scale, one patient showed multiple segment affection in cervical and dorsal cord, longest was 5 segments, and the other patient showed 3 segments of cervical cord affection by MRI.
In the light of our study, patients with higher level of spinal cord affection (cervical cord) demonstrated by abnormal high T2 signal by MRI, showed worse clinical symptoms. As well as the patient with majority of cord segment affection also showed worse clinical symptoms.
All five patients showed bladder affection and sensory level loss.
Prognosis in ATM
Three patients with ATM showed complete recovery, all of them showed abnormal high T2 signal and swelling of dorsal cord by MRI.
Two patients with ATM developed walking with aid after 2 weeks, one patient showed multiple segments of abnormal high T2 signal and swelling of dorsal and cervical cord and the other showed abnormal high T2 signal and swelling cervical cord by MRI.
In the light of our study, patients with cervical level of spinal cord affection and with more segment affection demonstrated by abnormal high T2 signal in MRI, showed persistent weakness as bad outcome.
MS
We had three patients with MS, all females, age ranged from 11 to 16. No patients had significant events within 4 weeks prior to the onset of the disease. Definite diagnosis of those patients was achieved with clinical symptoms suggesting MS including episodes of symptoms, MRI findings, and medical treatment response.
MRI findings
One patient demonstrated few tiny scattered foci of altered signal intensity involving bilateral fronto-parietal subcortical, high in T2W and FLAIR with no post-contrast enhancement.
One patient showed right occipital subcortical small area of signal alteration and cervical cord short segment of opposite to C3/4 disc, both areas showed altered signal eliciting low T1W signal and high T2W and FLAIR signal (Fig. 9).
Last patient showed cervical spinal cord segment of altered signal showing low T1W signal and high T2W signal opposite to C3,4,5 vertebrae mostly affecting the left half of the cord.
Clinico-pathological correlation with MS
One patient who showed cerebral bilateral fronto-parietal tiny plaques exhibited mild symptoms (grade 2 according to Hughes scale).
One patient who showed right occipital and cervical cord short segment also exhibited mild symptoms (grade 2 according to Hughes scale).
One patient who showed long cervical cord segment affection exhibited more severe symptoms (grade 3 according to Hughes scale).
Prognosis in MS
Two patients showed complete recovery, one had cerebral bilateral fronto-parietal tiny plaques and the other one had right occipital and cervical cord short segment. One patient developed walking with aid after 2 weeks, she demonstrated long cervical cord segment affection.
In the light of our study, the size and site of the lesion correlated with the symptoms and outcome as the patient with long cervical spinal cord lesion of abnormal high T2 signal by MRI, showed more severe symptoms and worse outcome.
While patients with tiny fronto-parietal plaques and short cervical cord segment lesion demonstrated by MRI showed less severe symptoms and better outcome.
Others
One patient, 11-year-old male, showed acute bilateral lower limb paralysis (LMN) with disability grade 4 according to Hughes scale and Glasgow Coma Scale score of 15 with bladder affection and sensory loss level at the umbilicus. MRI showed a D7 vertebra body and right lamina osseous lesion with soft tissue component compressing the dorsal spinal cord, eliciting high signal in T2W, isointense signal in TIW, and rather homogenous enhancement in post-contrast T1W (Fig. 10), consistent with vertebral neoplastic lesion. The patient proceeded with surgical consultation and oncology for the treatment plan.
Another patient, 15-year-old female, showed acute bilateral lower limb paralysis (LMN) with disability grade 3 according to Hughes scale and Glasgow Coma Scale score of 15 with no bladder affection and no sensory loss. MRI showed a large oval-shaped well-defined intradural extra-medullary soft tissue mass within the spinal canal, opposite to C7 down to D2 vertebral levels. It was seen at left lateral aspect of the thecal sac, compressing the adjacent cord and displacing it to the right side, with left neural foramina extension through C7/D1 and D1/2 with extra-spinal component. It elicits intermediate signal in T1W and T2W with restricted diffusion (Fig. 11), consistent with spinal schwannoma. The patient proceeded with surgical consultation and oncology for the treatment plan.
Another 3-year-old male patient showed acute bilateral lower limb paralysis (UMN) followed by dizziness and rapidly falling consciousness, with disability grade 4 according to Hughes scale and Glasgow Coma Scale score of 10 with bladder affection. He was admitted to the ICU shortly after. MRI showed multiple recent (acute) infarctions seen at the entire left middle cerebral artery (MCA) territory including the left frontal and parietal regions as well as left caudate and lentiform nuclei with mild mass effect over the ipsilateral lateral ventricle and mild midline shift to the opposite site, eliciting intermediate to low T1W signal, high T2W and FLAIR, and diffusion restriction (Fig. 12), consistent with acute infarction. The patient rapidly deteriorated and needed ICU admission.