There were 109 children with basal ganglia and/or thalamus ischaemic stroke, accounting for 14.2% (109/768) of all children with ischaemic stroke in our centre all together. In this group, 64/109 male patients accounted for 58.7%, and 45/109 cases were female, accounting for 41.3% of the cases. The onset age ranged from 1 month 17 days to 14 years, with a median age of 4 years (Table 1). There were 17 infants, 45 children in early childhood, 23 preschool children and 24 school-age children. All patients were full term, with a normal birth history and past medical history.
Ninety-nine patients had major complaints of unilateral body weakness, 6 had focal seizures, 3 had facial paralysis, 3 had headache and vomiting, and 1 had aphasia.
The time from onset to diagnosis ranged from 1 hour to 300 days, with a mean of 4 days. There were 13 cases in which the time from onset to diagnosis was less than 12 hours and 29 cases in which the time period was less than 24 hours. All cases underwent a professional physical examination. A total of 107 cases experienced different severities of weakness in the unilateral limbs when diagnosed, often reaching the peak level within 24 hours after onset. All 107 patients had lower strength in their upper limbs compared to that in their legs (0-V level muscle strength). Two patients had headache, vomiting and focal seizures but no limb weakness. Forty-six patients had central facial paralysis. Twenty-seven patients had aphasia; these patients were able to understand words and presented speechlessness or slow speech speed. Nine patients exhibited headaches and vomiting. Eighteen patients had focal seizures.
A total of 109 cases were examined by CT, MRI, MRA, video electroencephalography (VEEG), echocardiography, electrocardiography, routine blood and biochemical examination, homocysteine evaluation, protein S evaluation, protein C evaluation, routine cerebral spinal fluid (CSF) evaluation, CSF biochemistry evaluation and virus antibody evaluation (Epstein–Barr virus[EBV]/ herpes simplex virus[HSV]/measles).
MRI of the brain could be normal within 6 hours, with a low T1 signal and a high T2 signal in fluid‐attenuated inversion recovery (FLAIR) and diffusion-weighted imaging (DWI), and the high FLAIR and DWI signals could fade out with time. All 109 cases had no lesions in brain regions other than the basal ganglia and thalamus. All 109 cases had lentiform nucleus lesions: 50 cases only had lesions in the lenticular nucleus (lenticular nucleus group), as shown in Figure 1 A; 21 cases had lesions in both the lenticular nucleus and caudate head (caudate head group), as shown in Figure 1 B; and 38 cases had lesions in both the lenticular nucleus and thalamus (thalamus group), as shown in Figure 1 C. MRA results were unremarkable, except for some occasional mild vascular malformations; for example, some lesions were slightly thin on one side of the anterior cerebral artery, as shown in Figure 1 D (Figure 1).
CT of the brain identified bilateral punctate calcification of the basal ganglia in 21 cases.
VEEG results showed bilateral voltage asymmetry, such as the affected side was associated with a lower voltage, or the advantage of the occipital region was not obvious. Children with focal seizures exhibited discharge in the EEG. Through routine echocardiography detection, we found 2 cases of congenital heart disease after intervention, 1 case of tetralogy of Fallot, and 2 cases of atrial myxoma. The acute phase coincided with increased homocysteine in 8 cases, and the level of homocysteine was 50 µmol/l in one 8-year-old patient. This patient had a second basal ganglia ischaemic stroke 1 year later, and at the acute phase of the second time, the level of homocysteine was 22 µmol/l. We administered folate 2 mg qd, vitamin B6 10 mg qd and vitamin B12 5 mg qd; later, the patient’s homocysteine level was normal, and subsequent stroke did not occur. The other 7 patients had a mild level of hyperhomocysteinemia during the acute phase, ranging from 15.58 to 22.5 µmol/l (mean±SD, 18.09±2.6); upon examination one week later, all patients exhibited normal levels. All the cases had a blood and urine metabolism screening with normal results. All children had blood pressure in the normal range for their age.
A total of 53/109 children (48.6%) had a definite history of trauma within 12 hours before the onset of the disease, but the injuries were always very mild, such as that acquired by falling from the bed. A total of 21/109 children (18.3%) had bilateral basal ganglia calcification; blood and urine metabolic screening and homocysteine tests were conducted to exclude genetic metabolic diseases. Five patients had congenital vascular malformations. Eight/109 (7.3%) patients had increased homocysteine in the acute phase, and levels returned to normal one week later. A total of 4/109 children (3.7%) had varicella infection 2 weeks before the disease, and 4/109 children (3.7%) had allergic purpura before the disease. Also, 2/109 children (1.8%) had an interventional therapy for congenital heart disease before stroke, 1/109 children (0.9%) had tetralogy of Fallot, 2/109 children (1.8%) had atrial myxoma, and 19/109 children (17.4%) cases had unknown aetiology. There were 10/109 (9.2%) patients with more than one stroke: 4/38 (10.5%) were in the thalamus group, 4/50 (8%) were in the lenticular nucleus group, and 2/21 (9.5%) were in the caudate head group. There was no significant difference in the incidence rate of each group.
Twenty-seven patients received aspirin antiplatelet therapy, 25 patients received low molecular weight heparin calcium anticoagulant therapy, 37 patients received antiplatelet and anticoagulant therapy, and 20 patients received only symptomatic and supportive therapy.
None of the 109 cases were lost to follow-up, and the follow-up rate was 100%. The follow-up time was 1-15 years, with a median time of 6.5 years.
Limb paralysis: The patients with the fastest recovery of muscle strength recovered to grade 5 within one day, while those with slow recovery reached grade 5 within 1 month after the onset of disease.
There was no significant difference in the time required for muscle strength to recover to grade 5.
Fine motor dysfunction: Most of the children exhibited different degrees of fine motor disorder and dystonia on the affected side limbs. As time passed, the children’s fine motor disorder and dystonia symptoms gradually eased, and the time required to recover their fine motor skills was approximately 2-3 years or longer. Fine motor disorders and dystonia may exist for a long time. By the end of the follow-up period, all the children had no obvious dystonia, but 5/109 (4.6%) children had changed their handedness, manifesting as a child with a right-hand advantage changing to having a left-hand advantage. Regarding children who changed their handedness, a total of 3/21 (14.2%) patients were in the caudate head group, 1/50 (2.0%) were in the lenticular nucleus group, and 1/38 (2.6%) were in the thalamus group.
Facial paralysis: Most of the children with basal ganglia ischaemic stroke recovered completely from facial paralysis, with the shortest recovery time being one week and the longest recovery time being three months. There was no significant difference in the recovery time regarding facial paralysis among the groups.
Convulsion and aphasia: Children recovered quickly from focal convulsion, and those with abnormal EEG results were controlled within 1 week after treatment with oxcarbazepine or levetiracetam. Patients without abnormal EEG results recovered within 1 week without targeted treatment. Similarly, children recovered from aphasia within 2 weeks to 1 month without special treatment.
Overall prognosis: To evaluate the overall prognosis, the mRS score was calculated. The results showed that the scores of all 109 cases were less than or equal to 2 points, with an average of 0.62 points. The mean score was 0.62 points in the thalamus group, 0.66 points in the caudate head group, and 0.63 points in the lenticular nucleus group. The details are shown in Table 2. Statistical analysis of the relationship between the scores and the affected sites or treatment methods showed no statistical correlation.
Anxiety: To evaluate the anxiety of children in each group, the SCAS-P was administered to parents with children older than 6 years during follow-up in each group. There were 15 children over 6 years old in the caudate head group, 43 in the lenticular nucleus group and 28 in the thalamus group. Sex, age, parents' age, parents' education and parents' work conditions affected the children's SCAS-P score. To exclude the influence of the above factors, we performed statistical analysis, and no significant differences among groups in our sample were found in sex, age, parents' age, parents' education or parents' work conditions among the groups (Table 3, one-way ANOVA, p> 0.05).
The SCAS-P scores were 14.00 ± 1.181 (n = 12) in the caudate head group, 12.08 ± 0.877 (n = 43) in the lenticular nucleus group, and 19.12 ± 1.185 (n = 25) in the thalamus group. The SCAS-P score of the thalamus group was higher than that of the lenticular nucleus group and caudate head group, and the difference was statistically significant (as shown in Figure 2).