小儿神经病学和医学杂志

The anti-myelin oligodendrocyte glycoprotein autoantibody (anti-MOG) is recognized as a new diagnostic and prognostic markers in paediatric acquired demyelinating diseases of the central nervous system. We report a 6 years gril who developed a venous sinus thrombosis and signs of intracranial hypertension after acute otitis media. One month after this infection the patient had an episode of unilateral optic neuritis associated with anti-MOG in serum. Our patient received intravenous steroid treatment and showed a highly favourable response. This is the first case report of demyelinating disease with anti-MOG antibodies associated with thrombophilia.

Baclofen, a GABA-B agonist, reduces hypertonia by increasing spinal inhibition and improves motor function in functional children with cerebral palsy. We hypothesized that oral baclofen reduces pain and improves daily care in nonfunctional children with spastic cerebral palsy. To test this children residing in nursery homes with hypertonia causing pain or interfering with daily care were enrolled in this randomized, double blind, placebo controlled, crossover pilot study. Participants were examined before, during and after 13 weeks of baclofen (2 mg/kg/day) and placebo for pain (Brief Pain Inventory and Dalhousie Pain Survey), ease of care (care and comfort questionnaire) and overall treatment assessment (specific questionnaire, designed for this study). Two participants completed this pilot (ages 7 and 8 years). Pain was reported to interfere less on peak dose of baclofen treatment compared to placebo in one child (p=0.0001) and care tended to be easier in the other (p=0.05). Blinded caregivers recommended continuing baclofen. No significant side effects were reported. Baclofen, an inexpensive safe medication, may alleviate pain in nonfunctional spastic children.

Introduction: Choroid plexus carcinoma (CPC) is a rare central nervous system neoplasm derived from the choroid plexus epithelium. CPCs are challenging to surgically resect due to their intraventricular location and high vascularity. Tumor recurrence and dissemination are frequently seen with CPC. Due to the malignant nature of these tumors, the prognosis is poor with a five year survival rate of approximately 40%.

Case Description: The authors report a pediatric patient with a CPC (WHO grade III) with oncocytic changes, who survived for seven years after her initial diagnosis. A 14-month-old female presented to the emergency department with a sudden onset change in mental status following a fall. Imaging revealed a hemorrhagic, left intraventricular heterogeneous tumor. The patient underwent a left parietal craniotomy and gross tumor resection. Pathological findings were consistent with a malignant CPC. Her treatment regimen included four cycles of etoposide, Temodar, Cytoxan and vincristine, two cycles of oral etoposide and temozolomide with vincristine, in addition to a stem cell harvest. Recurrence of our patient’s disease led to two subsequent gross total resections. This patient was in the minority who survived greater than five years with this tumor type.

Conclusion: Gross total resection is the goal, and pharmacologic intervention including chemotherapy and radiation therapy remains controversial, as this treatment is not curative. We would like to share this unusual case with the medical community to increase clinical understanding in the management and treatment of this malignant tumor.

Background: Progressive cerebral arteriopathies are not an uncommon cause of ischemic stroke in children. The clinical and radiological distinction between various cerebral arteriopathies remains a challenge and topic of controversy for physicians.

Methods: We report an 11-year-old girl who presented with a left subcortical ischemic stroke.

Results: An 11-year-old girl presented with a progressive right hemiparesis. Her neuroimaging showed diffusion restriction and T2 signal abnormalities in the left basal ganglia with normal magnetic resonance and CT angiography. Subsequent MRA and cerebral catheter angiography at 9 days post-stroke revealed medium and large vessel stenoses of bilateral distal anterior cerebral arteries, proximal and distal segment of left middle cerebral artery, and distal left internal cerebral artery.

Conclusion: This case demonstrates the uncertainties and challenges related to ischemic stroke due to cerebral arteriopathies and the importance of early evaluation with cerebral catheter angiogram with unidentified ischemic stroke etiology and normal initial non-invasive angiography.

Background: Perinatal arterial ischemic stroke is an established risk factor for neuromotor and cognitive sequelae, but little is known about long-term neuropsychological implications. We aimed to evaluate long-term neuropsychological outcome and its effect on life quality in children with history of perinatal arterial ischemic stroke.

Methods: Seventeen children with history of perinatal arterial ischemic stroke, selected from the institutional cerebrovascular registry, were recruited for this study. Outcome was investigated using a battery of standardized neuropsychological tests and PedsQL questionnaire. A neuropsychological composite score (Cognitive Index) was calculated taking into account impaired performances.

Results: 47% of the patients had impaired Cognitive Index. Neuropsychological functions involved included: language, visuo-motor and executive functions, visual selective attention, with sustained attention as the most affected (59% of patients). Impairment in Cognitive Index was significantly associated with poor quality of life as perceived by parents.

Conclusion: In conclusion, minor impairments, such as neuropsychological deficits, can frequently occur in children with history of perinatal arterial ischemic stroke and may affect quality of life.