The use of stereotactic MRI-guided laser interstitial thermal therapy for the treatment of pediatric cavernous malformations: the SUNY Upstate Golisano Children's Hospital experience.

PURPOSE: Cavernous malformations (CM) are central nervous system lesions characterized by interlaced vascular sinusoids coated with endothelial cells without intervening parenchyma. Magnetic resonance imaging-guided laser interstitial thermal therapy (MRIgLITT) is a minimally invasive treatment modality that can precisely treat pathologic cerebral tissue, making it an effective alternative for the management of cavernomas. We describe the outcomes of a series of pediatric patients with cavernous brain malformations treated with MRIgLITT between 2014 and 2018 at our institution. METHODS: We retrospectively analyzed 11 cavernomas in 6 pediatric patients treated with MRIgLITT. Both the Visualase System® and/or Neuroblate® systems were used. A variation of the surgical technique on the application of the laser was developed. Post-ablation MRIs were obtained to assess ablated areas. RESULTS: A total of 11 cavernomas in 6 patients were treated with MRIgLITT. Median age was 15 years (12 to 17 years); 75% were males. Presenting symptoms were headache (75%) and seizures (25%). Two patients presented with multiple CMs. All lesions in this study were supratentorial (cerebral hemispheres 81.8%, corpus callosum 9.1%, basal ganglia 9.1%). Our surgical technique was well-tolerated, with no significant adverse events observed. Hospital stay for all patients was less than 48 hours. CONCLUSION: MRIgLITT is an effective minimally invasive technique for the treatment of pediatric CMs. It represents a useful and safe tool, when other therapeutic alternatives may represent a greater risk of surgical morbidity.

Role of Electroencephalogram (EEG) and Magnetic Resonance Imaging (MRI) Findings in Early Recognition and Diagnosis of Neuronal Ceroid Lipofuscinosis Type 2 Disease.

Neuronal ceroid lipofuscinosis type 2 (CLN2) disease is a very rare neurodegenerative lysosomal storage disorder. Progression is rapid and irreversible, making early diagnosis crucial for timely treatment. A group of pediatric neurologists and neuroradiologists with expertise in CLN2 convened to discuss early electroencephalogram (EEG) and magnetic resonance imaging (MRI) findings in CLN2 diagnosis. Of 18 CLN2 cases, 16 (88.9%) had background slowing and 16 (88.9%) had epileptiform discharges on initial EEG. Seven of 17 (41.2%) patients who received intermittent low-frequency photic stimulation had a photoparoxysmal response. Initial MRIs showed subtle cerebellar (n = 14, 77.8%) or cerebral (n = 9, 50.0%) atrophy, white matter abnormalities (n = 11, 61.1%), and basal ganglia T2 hypointensity (n = 6, 33.3%), which became more apparent on follow-up MRI. The recognition of even subtle cerebellar atrophy and white matter signal changes in children aged 2-5 years who present with language delay, new-onset seizures, and an EEG with epileptiform discharges and background slowing should prompt investigation for CLN2. Because these early signs are not unique to CLN2, genetic testing is essential early in the diagnostic journey.

Pediatric fibrocartilaginous spine embolism induced by trauma.

Fibrocartilaginous embolic infarction of the spinal cord is a rare cause of acute back pain and motor weakness. Most symptoms start after minor trauma that is often considered harmless and forgotten, however these minor injuries can result in lethal consequences. It is quite rare to diagnose fibrocartilaginous embolism in a timely manner and start treatment to prevent poor outcomes. We present the case of a previously healthy eight-year-old female with sudden onset neck pain and progressive bilateral upper extremity weakness following an injury while playing with her younger sister. Magnetic resonance imaging of the cervical spinal cord without contrast revealed a posterior disc protrusion suggestive of post-traumatic spinal cord infarction due to fibrocartilaginous embolism. In young, otherwise healthy, patients with acute motor deficits, radiographic imaging can help identify rare presentations like fibrocartilaginous embolism in order to rapidly diagnose and efficiently treat such patients.

COVID-19 as a rare cause of facial nerve neuritis in a pediatric patient.

COVID-19 has been noted to present with neurological symptoms in nearly 30% of patients. While children are more likely to be asymptomatic, neurological involvement has been observed. We present the case of a 23-month-old previously healthy female who was brought to the emergency room for a new-onset facial droop. The patient tested positive for COVID-19 but was otherwise asymptomatic. Magnetic resonance imaging of the brain with and without contrast revealed abnormal enhancement along the canalicular segment of the right cranial VII extending to the first genu suggestive of cranial nerve neuritis. Given that our case involves a pediatric patient with no significant comorbidities presenting with facial drop, COVID-19 should be considered on the differential when evaluating causes of new onset peripheral nerve palsies.

Central and peripheral dysmyelination in a 3-year-old girl with ring chromosome 18.

Myelin basic protein (MBP) contributes to peripheral and central nervous system myelin. Developmental myelinopathies exist on a clinical spectrum, but MBP is not included on leukodystrophy or CMT gene panels. This ring chromosome 18 case presents serial MRI and EMG/NCS, shedding light on the early clinical course of the disorder.

Magnetic resonance imaging as a predictor of submucous cleft palate severity and guide for surgical intervention.

BACKGROUND: Diagnosis of submucous cleft palate (SMCP) is frequently delayed, adversely affecting speech outcomes. Previous studies show that MRI reliably identifies structural abnormalities in velopharyngeal musculature. This information has potential to assist with diagnosis and treatment decisions. AIMS: The objectives of this study were to (1) develop a clinician-friendly MRI grading scale of SMCP anatomy, (2) identify correlations between radiographic cleft severity and clinical severity using Pittsburgh Weighted Speech Scores (PWSS), and (3) determine if MRI is a predictor of surgical efficacy in improving PWSS. DESIGN: Thirty patients presenting to our Cleft Palate-Craniofacial Clinic for evaluation of velopharyngeal insufficiency (VPI) and suspected SMCP were reviewed. VPI severity was clinically graded using PWSS. All patients underwent MRI to grade palatal abnormalities, using a novel MRI grading scale. PWSS and cleft severity on MRI were compared. A subgroup of patients (n = 19) underwent palatoplasty. Preoperative and postoperative PWSS were compared. Degree of PWSS improvement was then correlated with the preoperative MRI grade. RESULTS: Twenty-nine out of 30 MRIs demonstrated abnormal palate anatomy. Of the 30 patients evaluated, 5 clinically improved with speech therapy alone. In this subgroup, MRI severity did not correlate with PWSS (P = 0.06-0.6). Nineteen patients underwent palatoplasty. Of these, 14 demonstrated improved postoperative PWSS. There were no significant correlations between severity of cleft on imaging and preoperative PWSS or score improvement (P = 0.056-0.65). CONCLUSION: While MRI accurately identifies structural abnormalities of the soft palate, these abnormalities do not reliably correspond to clinical severity. Clinical examination including speech scores and dynamic speech testing, rather than static MRI, should guide treatment decisions and surgical indications.

Idiopathic acute transverse myelitis in children: an analysis and discussion of MRI findings.

BACKGROUND: There is lack of reported magnetic resonance imaging (MRI) studies of idiopathic acute transverse myelitis (ATM) in children. OBJECTIVE: To describe the imaging features of idiopathic ATM in children. METHODS: We retrospectively analyzed the spinal MRI findings of children diagnosed with ATM. The anatomic regions, vertebral segmental length, gray or white matter involvement, cord expansion and gadolinium enhancement were examined. RESULTS: A total of 27 children were diagnosed with isolated monophasic ATM with a mean follow-up of 5.2 years. Two children later diagnosed with neuromyelitis optica were excluded from the pediatric ATM cohort. None of the patients had a subsequent diagnosis of multiple sclerosis. The mean age of onset was 9.5 years (0.5-16.9 years). Spinal MRIs were abnormal in 21 (78%). The mean interval between symptom onset and the MRI was 1.7 days (0-19 days). Central cord hyperintensity involving gray matter was seen in all patients. A majority (67%) of the patients demonstrated long segment lesions with a mean segment length of 6.4. CONCLUSIONS: We conclude that central cord inflammation extending over three or more segments is the most common finding of idiopathic monophasic transverse myelitis in children. The risk of multiple sclerosis in children who experience isolated transverse myelitis as a first demyelinating event is low.

The MR imaging features and clinical correlates in low back pain-related syndromes.

Several distinct clinical syndromes can accompany low back pain in patients with lumbar spine abnormality. Developmental factors and any superimposed degenerative changes determine the size and configuration of the spinal canal, lateral recess, and neural foramen, and can affect the nerve roots. Somatic or referred pain may develop depending on the involved anatomic site and underlying pathology. Many times, but not always, MR imaging findings correlate with the clinical presentation. Combined analysis of the imaging and clinical findings may provide a more accurate and concise approach to the patient with low back pain.

West Nile virus meningoencephalitis: MR imaging findings.

BACKGROUND AND PURPOSE: Reports of MR imaging in West Nile virus (WNV) meningoencephalomyelitis are few and the described findings limited. The purpose of this study was to review the spectrum of MR imaging findings for WNV meningoencephalomyelitis and investigate whether any of the findings correlates with clinical presentation of flaccid paralysis. METHODS: We reviewed the MR imaging findings of 17 patients with confirmed WNV encephalitis and/or myelitis. MR imaging brain studies were evaluated for location of signal intensity abnormalities, edema, hydrocephalus, or abnormal enhancement. MR imaging spine studies were evaluated for signal intensity abnormalities in cord and/or enhancement. RESULTS: Retrospective review of the MR imaging studies of 17 patients was performed by 2 neuroradiologists. Eleven of 16 brain MR images demonstrated abnormalities. Eight (50%) patients had abnormal studies related to meningoencephalitis. All 8 patients had abnormal findings in the deep gray matter and/or brain stem; 2 had additional white matter abnormalities. Three patients with abnormal MR studies of the spine had extremity weakness on examination. The imaging findings included abnormal signal intensity more pronounced in the ventral horns and/or enhancement around the conus medullaris and cauda equina. One patient had additional abnormalities in the pons. CONCLUSION: Abnormal MR imaging findings in patients with WNV meningoencephalomyelitis are nonspecific but not uncommon. Anatomic areas commonly affected are basal ganglia, thalami, mesial temporal structures, brain stem, and cerebellum. Extremity weakness or flaccid paralysis corresponds to spinal cord/cauda equina abnormalities.

Request form history, clinical indication, and yield of brain magnetic resonance studies.

PURPOSE: To investigate whether improved clinical history allows the radiologist to better predict the pretest probability of obtaining a positive or negative result from a magnetic resonance (MR) examination. MATERIALS AND METHODS: Six neuroradiologists prospectively reviewed 100 consecutive requests for brain MR examinations and sequentially assessed 1) quality of written history, 2) degree of indication for requested study, and 3) any pertinent new information found during chart review that may have altered the degree of indication. MR yield was correlated with the degree of indication assessed before and after chart review. RESULTS: Most request form histories were judged as poor (63%), and chart review reduced the overall indications for MR examinations, as there was a tendency for high-indication requests to migrate to the low-indication category. Based on request form history alone, the yields for low- and high-indication studies were 13% and 37%, respectively. Correlations between MR yield and indication after chart review improved significantly (P < 0.05) with 2% and 61% for low and high indications, respectively. Sensitivity and specificity for a positive MR yield were 71% and 62%, respectively, for the indication judged by the request history alone, and 96% and 80%, respectively, after chart review. Positive and negative prediction rates were 37% and 87%, respectively, for the indication judged by the request history alone, and 61% and 98%, respectively, for the indication judged after chart review. CONCLUSION: Based on our limited data, most request form histories were inadequate, and essential information available in the chart before MR examinations was frequently missing from the request forms. When adequate information was provided, the indication for the studies as judged by the radiologists predicted the MR yield more accurately, particularly for those requests with low indication. Therefore, our study suggests that MR imaging (MRI) may be used more effectively when pertinent clinical history is available. However, our study is limited and further studies are needed to confirm our results.