Comparative assessment of health-related quality of life with and without anticonvulsant therapy in patients with childhood epilepsy with centrotemporal spikes.

OBJECTIVE: To estimate anti-seizure medication (ASM) treatment burden and its effects on health-related quality of life (HRQOL) in new-onset childhood epilepsy with centrotemporal spikes (CECTS) using different treatment approaches in Kazakhstan. METHODS: Forty-three patients were followed prospectively during 2015 to 2020 for at least 2 years. Patients were divided into three groups: (1) history of ≤3 seizures (n = 32); (2) ≥4 seizures (n = 6); (3) cerebral palsy coexisting with CECTS (n = 5). The first group was subdivided into treated (n = 8) and observed (n = 24) subgroups. The shortened Quality of Life in Childhood Epilepsy Questionnaire (QOLCE-55) was completed by parents after 6 months of follow-up. RESULTS: At the end of the study, all children had a sustained remission from seizures for at least 2 years. Differences were identified in emotional, social, and physical subscales between patients in the low seizure frequency group. Signs of low self-esteem, anxiety, depression, limited social interaction owing to pharmacotherapy, painful medical procedures, and stigma were reasons for decreased HRQOL in the treated subgroup. Overall HRQOL in treated (89.2 ± 5.2) patients was significantly decreased compared with observed children with low seizure frequency (98.0 ± 3.0). CONCLUSION: ASM therapy does not necessarily improve and may decrease HRQOL in children with low seizure frequency CECTS.

A Self-Limited Childhood Epilepsy as Co-Incidental in Cerebral Palsy.

BACKGROUND: Cerebral palsy is the most frequent motor disability in childhood and is associated with a higher incidence of seizure disorders. In many instances, it is recognized that motor difficulties, as well as seizures, are from the same underlying brain lesion. However, self-limited childhood epilepsies, being a common group of epilepsy syndromes, would be expected to occur in patients with cerebral palsy merely on chance association and be unrelated to the structural brain imaging abnormality causing the motor impairment. Differential diagnosis in this case is important determining the long-term prognosis and need for anticonvulsant treatment. CASE PRESENTATION: Here, we report two patients with cerebral palsy combined with epilepsy, whose age at onset, seizure semiology and electroclinical features were similar to children with self-limited childhood-specific seizure disorders (childhood epilepsy with centrotemporal spikes and Panayiotopoulos syndrome). CONCLUSION: These cases highlight the importance of comprehensive differential diagnosis of seizures in cerebral palsy. Co-existence of age-dependent focal epilepsies with an underlying brain pathology as white matter injury, not affecting the cerebral cortex, might take place in the case of children with impaired motor skills. With health systems increasingly utilizing clinical pathways, it is important to consider the possibility of a self-limited childhood epilepsy and avoid aggressive and unnecessary medication treatment in children with cerebral palsy.

A Clinical Paradigm for Classifying Neurologic Symptoms to Screen for Emergent Large Vessel Occlusions.

BACKGROUND: With newly-extended treatment windows for endovascular therapy in emergent large vessel occlusions, it is increasingly important to identify thrombectomy-eligible patients without overwhelming resources dedicated to acute stroke care. We devised a simple paradigm to classify patient's presenting neurologic symptoms to screen for large vessel occlusions. METHODS: We reviewed the presenting symptoms, imaging findings, and final diagnoses of consecutive emergency department stroke alert cases. Patients were classified based on their neurologic exams as focal objective, focal subjective, or nonfocal. Outcomes of final diagnoses of acute ischemic stroke and large vessel occlusions were compared across groups. Comparisons were made to other large vessel occlusion prediction scales. RESULTS: Of 521 patients, 342 (65.6%) were categorized as focal objective, 142 (27.2%) as focal subjective, and 37 (7.1%) as nonfocal. Ischemic stroke and large vessel occlusions were diagnosed in 114 (21.9%) and 27 (5.2%) of patients, respectively. Classification as focal objective significantly predicted stroke (odds ratio 3.77; 95% confidence interval 2.17-6.55) and captured all large vessel occlusions (P = .0001). The focal objective categorization was the only tool which achieved 100% sensitivity for large vessel occlusions (with a specificity of 36%) compared to other large vessel occlusion prediction tools. CONCLUSIONS: Patients who presented as stroke alerts without focal neurologic symptoms were unlikely to have large vessel occlusions. With high sensitivity, classifying patients' neurologic exams into focal objective versus subjective or nonfocal categories may serve as a useful tool to screen for large vessel occlusions and prevent unnecessary emergent workup in patients unlikely to be endovascular candidates.

Isolated focal dystonia phenotypes are associated with distinct patterns of altered microstructure.

Objective: Isolated adult-onset focal dystonia is considered a network disorder with disturbances to the motor basal ganglia and cerebellar circuits playing a pathophysiological role, but why specific body regions become affected remains unknown. We aimed to use diffusion tensor imaging to determine if the two most common phenotypes of focal dystonia are associated with distinguishing microstructural changes affecting the motor network. Methods: Fifteen blepharospasm patients, 20 cervical dystonia patients, and 30 age- and sex-matched healthy controls were recruited. Maps of fractional anisotropy and mean diffusivity were analyzed using a voxel-based approach and an automated region-of-interest technique to evaluate deep gray matter nuclei. Correlations between diffusion measures and dystonia severity were tested, and post hoc discriminant analyses were conducted. Results: Voxel-based analyses revealed significantly reduced fractional anisotropy in the right cerebellum and increased mean diffusivity in the left caudate of cervical dystonia patients compared to controls, as well as lower fractional anisotropy in the right cerebellum in cervical dystonia patients relative to blepharospasm patients. In addition to reduced fractional anisotropy in the bilateral caudate nucleus of cervical dystonia patients relative to controls and blepharospasm patients, region-of-interest analyses revealed significantly reduced fractional anisotropy in the right globus pallidus internus and left red nucleus of blepharospasm patients compared to both controls and cervical dystonia patients. Diffusivity measures in the red nucleus of blepharospasm patients correlated with disease severity. In a three-group discriminant analysis, participants were correctly classified with only modest reliability (67-75%), but in a two-group discriminant analysis, patients could be distinguished from each other with high reliability (83-100%). Conclusions: Different focal dystonia phenotypes are associated with distinct patterns of altered microstructure within constituent regions of basal ganglia and cerebellar circuits.

Curative and palliative MRI-guided laser ablation for drug-resistant epilepsy.

Epilepsy is a common neurological disorder occurring in 3% of the US adult population. It is characterised by seizures resulting from aberrant hypersynchronous neural activity. Approximately one-third of newly diagnosed epilepsy cases fail to become seizure-free in response to antiseizure drugs. Optimal seizure control, in cases of drug-resistant epilepsy, often requires neurosurgical intervention targeting seizure foci, such as the temporal lobe. Advances in minimally invasive ablative surgical approaches have led to the development of MRI-guided laser interstitial thermal therapy (LITT). For refractory epilepsy, this surgical intervention offers many advantages over traditional approaches, including real-time lesion monitoring, reduced morbidity, and in some reports increased preservation of cognitive and language processes. We review the use of LITT for epileptic indications in the context of its application as a curative (seizure freedom) or palliative (seizure reduction) measure for both lesional and non-lesional forms of epilepsy. Furthermore, we address the use of LITT for a variety of extratemporal lobe epilepsies. Finally, we describe clinical outcomes, limitations and future applications of LITT for epilepsy.

The Addition of Endovascular Intervention for Dural Venous Sinus Thrombosis: Single-Center Experience and Review of Literature.

BACKGROUND: Dural venous sinus thrombosis (DVST) is a cause of infarction and intracranial hemorrhage (ICH) that can lead to significant morbidity. Endovascular therapy has emerged as an adjunctive therapy in select cases but has been associated with increased hemorrhagic complications. We present our experience with a large single-center cohort of DVST cases treated with current-generation thrombectomy devices. MATERIALS AND METHODS: In this retrospective cohort study, a chart review was performed to compare presentations and outcomes of patients treated with anticoagulation alone with those treated with additional interventional therapy, using the modified Rankin Scale (mRS) score at discharge and at 90 days' follow-up. RESULTS: A total of 66 patients were included; 37 were treated with anticoagulation alone, and 29 underwent additional interventional therapy. Patients presenting with ICH or infarction had a significantly greater likelihood of disability at the time of discharge (odds ratio [OR] of 64.5 and 45.8, respectively; P < .0001) and at 90 days (OR of 28.4 and 22.8, respectively; P < .0001). Patients presenting with ICH or infarction were more likely to be selected for endovascular therapy (P < .05). Endovascular therapy was typically performed within 24 hours of admission; 9 patients (31%) had post-treatment hemorrhage, with 2 being (6.9%) symptomatic. There were fewer patients with slight disability (mRS score ≤1) in the endovascular group compared with the anticoagulation group at discharge (P = .05), but outcomes were not significantly different at 90 days (P = .19). CONCLUSIONS: Despite a higher rate of ICH or infarction at presentation in the endovascular group and an increased risk of postprocedural ICH, both treatment groups had similarly good functional outcomes at 90 days.

Imaging of Laser Therapy in Epilepsy.

BACKGROUND AND PURPOSE: MR-guided laser interstitial thermal therapy (MRgLITT) is an increasingly popular neuroablative method for the surgical treatment of epilepsy patients. This article intends to demonstrate the utility and pitfalls of imaging in the context of patient care with MRgLITT. MATERIAL AND METHODS: A retrospective review of the medical records and imaging database with six illustrative cases selected to demonstrate the use of imaging throughout patient management with MRgLITT in diverse clinical situations and pathologies. A review of the knowledge in the literature was applied to the relevant points discussed. RESULTS: Imaging findings were described in the setting of laser therapy in nonlesional epilepsy, mesial temporal sclerosis, dual pathology, periventricular nodular heterotopia, and schizencephaly. Discussion of imaging principles, potential pitfalls, as well as its use in the patient work-up and follow-up, is shown. CONCLUSIONS: MRgLITT is an alternative minimally invasive therapy for refractory epilepsy, which is becoming widely sought for. Imaging plays a crucial role prior to, during, and after the procedure.

Optic Radiation Tractography and Visual Field Deficits in Laser Interstitial Thermal Therapy for Amygdalohippocampectomy in Patients with Mesial Temporal Lobe Epilepsy.

BACKGROUND/AIMS: Laser interstitial thermal therapy (LITT) has become an alternative to open-resective surgery for refractory mesial temporal lobe epilepsy (MTLE). Occurrence of visual field defects (VFDs) following open surgery for MTLE is reported at 52-100%. We examined the rate of VFDs following LITT for amygdalohippocampectomy (AHE) and correlated the occurrence of VFDs with damage to the optic radiations, assessed by diffusion tensor tractography (DTI). METHODS: We performed a retrospective analysis of 5 patients who underwent LITT-AHE for medically refractory MTLE. We examined the association between VFDs and optic radiation damage by correlating postprocedural visual field testing with qualitative assessment of optic radiation fiber tracts. RESULTS: Postoperative assessments showed that 4 patients had normal visual field testing, and 1 had a right superior quadrantanopsia (20%). We performed 3-dimensional reconstruction of the optic radiation, laser probe trajectory, and ablation volume. Damage to Meyer's loop was determined consistent with the VFD. CONCLUSIONS: Short-term follow-up in our series suggests that laser ablation AHE may be associated with a lower rate of VFD than has been reported for open AHE. Our results suggest that incorporating optic radiation mapping through DTI may preoperatively help to minimize the risk of VFD following laser ablation AHE.

Successful antiviral treatment after 6years of chronic progressive neurological disease attributed to VZV brain infection.

We describe an extraordinary case of an immunocompetent patient who developed sacral-distribution zoster, followed 3months later by neurological disease that progressed for 6years and was attributed to varicella zoster virus (VZV) infection of the brain. Despite the prolonged infection, neurologic symptoms and signs resolved rapidly and completely after treatment with intravenous acyclovir.

Arterial spin labeling: Pathologically proven superiority over conventional MRI for detection of high-grade glioma progression after treatment.

BACKGROUND: Standard of care for high-grade gliomas (HGGs) includes surgical debulking and adjuvant chemotherapy and radiation. Patients under treatment require frequent clinical and imaging monitoring for therapy modulation. Differentiating tumor progression from treatment-related changes can be challenging on conventional MRI, resulting in delayed recognition of tumor progression. Arterial spin labeling (ASL) may be more sensitive for tumor progression. MATERIALS AND METHODS: ASL and associated conventional MR images obtained in patients previously treated for HGGs and before biopsy or re-resection were reviewed by three neuroradiologists who were blinded to the histopathologic results. Regions of interest (ROIs) of greatest perfusion were chosen by consensus, and mirror-image contralateral ROIs were also placed. Sensitivity of ASL for tumor progression was compared with those of contrast-enhanced T1-weighted (T1W-CE) and fluid-attenuated inversion recovery (FLAIR) images using McNemar's test. We tested for an association between cerebral blood flow (CBF) and apparent diffusion correlation (ADC) using a Hotelling-Lawley trace. Finally, we used a Pearson's analysis to test for a correlation between CBF and percentage of tumor within each resection. RESULTS: Twenty-two patients were studied. ASL demonstrated hyperperfusion in all cases with mean CBF ratio 3.37 (±1.71). T1W-CE and FLAIR images were positive in 15 (p = 0.0233) and 16 (p = 0.0412) cases, respectively. There was no association between ADC and CBF (p = 0.687). There was a correlation between CBF and percentage of tumor (p = 0.048). CONCLUSION: ASL may be more sensitive than conventional MR sequences for the detection of tumor progression in patients treated for HGGs.

Diagnostic, treatment, and surgical imaging in epilepsy.

Dedicated epilepsy centers are growing in hospitals throughout the USA and abroad, with a continuously increasing role of imaging in multidisciplinary meetings. Imaging is paramount in diagnosis, treatment, and surgical decision-making in lesional and nonlesional epileptic disease. Besides being up-to-date with technical developments in imaging that may make an impact in patient care, familiarity with clinical and surgical aspects of epilepsy is fundamental to better understanding of patient management. The present article intends to revisit diagnostic, therapeutic, and surgical imaging in epilepsy. Finally, with the increase in frequency of epilepsy management-related procedures and their hardware, MRI safety issues are discussed.

Neuroimaging of Concussion.

In this review, we discuss the literature regarding both concussion and mild traumatic brain injury. We focus on the role for neuroimaging in the concussed patient and describe the recommended practices related to imaging in concussion. This discussion first focuses on the exclusion of severe injuries and is followed by a discussion of the potential utility of various advanced imaging techniques in research and clinical practice.

Microstructural Changes within the Basal Ganglia Differ between Parkinson Disease Subtypes.

Diffusion tensor imaging (DTI) of the substantia nigra has shown promise in detecting and quantifying neurodegeneration in Parkinson disease (PD). It remains unknown, however, whether differences in microstructural changes within the basal ganglia underlie PD motor subtypes. We investigated microstructural changes within the basal ganglia of mild to moderately affected PD patients using DTI and sought to determine if microstructural changes differ between the tremor dominant (TD) and postural instability/gait difficulty (PIGD) subtypes. Fractional anisotropy, mean diffusivity, radial, and axial diffusivity were obtained from bilateral caudate, putamen, globus pallidus, and substantia nigra of 21 PD patients (12 TD and 9 PIGD) and 20 age-matched healthy controls. T-tests and ANOVA methods were used to compare PD patients, subtypes, and controls, and Spearman correlations tested for relationships between DTI and clinical measures. We found our cohort of PD patients had reduced fractional anisotropy within the substantia nigra and increased mean and radial diffusivity within the substantia nigra and globus pallidus compared to controls, and that changes within those structures were largely driven by the PIGD subtype. Across all PD patients fractional anisotropy within the substantia nigra correlated with disease stage, while in PIGD patients increased diffusivity within the globus pallidus correlated with disease stage and motor severity. We conclude that PIGD patients have more severely affected microstructural changes within the substantia nigra compared to TD, and that microstructural changes within the globus pallidus may be particularly relevant for the manifestation of the PIGD subtype.

Neuroimaging of Natalizumab Complications in Multiple Sclerosis: PML and Other Associated Entities.

Natalizumab (Tysabri) is a monoclonal antibody (α4 integrin antagonist) approved for treatment of multiple sclerosis, both for patients who fail therapy with other disease modifying agents and for patients with aggressive disease. Natalizumab is highly effective, resulting in significant decreases in rates of both relapse and disability accumulation, as well as marked decrease in MRI evidence of disease activity. As such, utilization of natalizumab is increasing, and the presentation of its associated complications is increasing accordingly. This review focuses on the clinical and neuroimaging features of the major complications associated with natalizumab therapy, focusing on the rare but devastating progressive multifocal leukoencephalopathy (PML). Associated entities including PML associated immune reconstitution inflammatory syndrome (PML-IRIS) and the emerging phenomenon of rebound of MS disease activity after natalizumab discontinuation are also discussed. Early recognition of neuroimaging features associated with these processes is critical in order to facilitate prompt diagnosis, treatment, and/or modification of therapies to improve patient outcomes.

Migraine with aura is associated with an incomplete circle of willis: results of a prospective observational study.

OBJECTIVE: To compare the prevalence of an incomplete circle of Willis in patients with migraine with aura, migraine without aura, and control subjects, and correlate circle of Willis variations with alterations in cerebral perfusion. METHODS: Migraine with aura, migraine without aura, and control subjects were prospectively enrolled in a 1∶1∶1 ratio. Magnetic resonance angiography was performed to examine circle of Willis anatomy and arterial spin labeled perfusion magnetic resonance imaging to measure cerebral blood flow. A standardized template rating system was used to categorize circle of Willis variants. The primary pre-specified outcome measure was the frequency of an incomplete circle of Willis. The association between circle of Willis variations and cerebral blood flow was also analyzed. RESULTS: 170 subjects were enrolled (56 migraine with aura, 61 migraine without aura, 53 controls). An incomplete circle of Willis was significantly more common in the migraine with aura compared to control group (73% vs. 51%, p = 0.02), with a similar trend for the migraine without aura group (67% vs. 51%, p = 0.08). Using a quantitative score of the burden of circle of Willis variants, migraine with aura subjects had a higher burden of variants than controls (p = 0.02). Compared to those with a complete circle, subjects with an incomplete circle had greater asymmetry in hemispheric cerebral blood flow (p = 0.05). Specific posterior cerebral artery variants were associated with greater asymmetries of blood flow in the posterior cerebral artery territory. CONCLUSIONS: An incomplete circle of Willis is more common in migraine with aura subjects than controls, and is associated with alterations in cerebral blood flow.