72-year-old man with right homonymous hemianopia and gait instability.

An elderly man presented with right homonymous hemianopia and gait instability. He was found to have a left occipital ring enhancing lesion that was resected. Neuropathologic examination demonstrated a Cladophialophora bantiana brain abscess (cerebral phaeohyphomycosis).

Craniocervical Dissociation in Pediatric Patients: Pearls and Pitfalls of Diagnosis and Management.

AIMS: The aims of this study were to document the injury pattern in pediatric traumatic craniocervical dissociation (CCD) and identify features of survivors. METHODS: Pediatric traumatic CCDs, diagnosed between January 2004 and July 2016, were reviewed. Survivors and nonsurvivors were compared. Categorical and continuous variables were analyzed with Fisher exact and t tests, respectively. RESULTS: Twenty-seven children were identified; 10 died (37%). The median age was 60 months (ranges, 6-109 months [survivors], 2-98 months [nonsurvivors]). For survivors, the median follow-up was 13.4 months (range, 1-109 months). The median time to mortality was 1.5 days (range, 1-7 days). The injury modality was motor vehicle collision in 18 (67%), pedestrian struck in 8 (30%), and 1 shaken infant (3%). For nonsurvivors, CCD was equally diagnosed by plain radiograph and head/cervical spine computed tomography scan. For survivors, CCD was diagnosed by computed tomography in 7 (41%), magnetic resonance imaging in 10 (59%), and none by radiograph. Seven diagnosed by magnetic resonance imaging (41%) had nondiagnostic initial imaging but persistent neck pain. Magnetic resonance imaging was obtained and was diagnostic of CCD in all 7 (P < 0.01). Survivors required significantly less cardiopulmonary resuscitation (P < 0.01), had lower Injury Severity Scores (P < 0.01), higher Glasgow Coma Scale scores (P < 0.01), and shorter transport times (P < 0.01). Significantly more involved in motor vehicle collisions survived (P = 0.04). Nine (53%) had no disability at follow-up evaluation. CONCLUSIONS: In pediatric CCD, high-velocity mechanism, cardiac arrest, high Injury Severity Score, and low Glasgow Coma Scale score are associated with mortality. If CCD is correctly managed in the absence of cardiac arrest or traumatic brain or spinal cord injury, children may survive intact.

Saksenaea infection masquerading as a brain tumor in an immunocompetent child.

Saksenaea species are a rare cause of mucormycosis, the majority associated with cutaneous and subcutaneous infections resulting from trauma in both immunocompromised and immunocompetent individuals. Unlike other causative agents of mucormycosis, cerebral infections are exceptionally rare. We describe the first case of isolated cerebral infection by Saksenaea in a 4-year-old previously healthy male child who presented with headaches. He had no past medical history other than an episode of febrile seizures. In addition to raising the awareness of an unusual presentation of infection by Saksenaea, this case highlights the importance of pathologic examination for the prompt diagnosis of mucormycosis as well as the specific fungal identification for treatment as Saksenaea spp. may be more susceptible to posaconazole and less susceptible to amphotericin B compared to more common causes of mucormycosis.

Epilepsy Surgery for Skull-Base Temporal Lobe Encephaloceles: Should We Spare the Hippocampus from Resection?

The neurosurgical treatment of skull base temporal encephalocele for patients with epilepsy is variable. We describe two adult cases of temporal lobe epilepsy (TLE) with spheno-temporal encephalocele, currently seizure-free for more than two years after anterior temporal lobectomy (ATL) and lesionectomy sparing the hippocampus without long-term intracranial electroencephalogram (EEG) monitoring. Encephaloceles were detected by magnetic resonance imaging (MRI) and confirmed by maxillofacial head computed tomography (CT) scans. Seizures were captured by scalp video-EEG recording. One case underwent intraoperative electrocorticography (ECoG) with pathology demonstrating neuronal heterotopia. We propose that in some patients with skull base temporal encephaloceles, minimal surgical resection of herniated and adjacent temporal cortex (lesionectomy) is sufficient to render seizure freedom. In future cases, where an associated malformation of cortical development is suspected, newer techniques such as minimally invasive EEG monitoring with stereotactic-depth EEG electrodes should be considered to tailor the surrounding margins of the resected epileptogenic zone.

Novel Pattern of Iron Deposition in the Fascicula Nigrale in Patients with Parkinson's Disease: A Pilot Study.

Background and Purpose. To determine whether the pattern of iron deposition in the fascicula nigrale in patients with Parkinson's disease would be different from age-matched controls by utilizing quantitative susceptibility mapping to measure susceptibility change. Methods. MRIs of the brain were obtained from 34 subjects, 18 with Parkinson's disease and 16 age- and gender-matched controls. Regions of interest were drawn around the fascicula nigrale and substantia nigra using SWI mapping software by blinded investigators. Statistical analyses were performed to determine susceptibility patterns of both of these regions. Results. Measurements showed significantly increased susceptibility in the substantia nigra in Parkinson's patients and an increased rostral-caudal deposition of iron in the fascicula nigrale in all subjects. This trend was exaggerated with significant correlation noted with increasing age in the Parkinson group. Conclusion. The pattern of an exaggerated iron deposition gradient of the fascicula nigrale in the Parkinson group could represent underlying tract dysfunction. Significant correlation of increasing iron deposition with increasing age may be a cumulative effect, possibly related to disease duration.

Evaluation of Telehealth for Preclinic Assessment and Follow-Up in an Interprofessional Rural and Remote Memory Clinic.

Using data from a sample of 169 patients, this study evaluates the acceptability and feasibility of telehealth videoconferencing for preclinic assessment and follow-up in an interprofessional memory clinic for rural and remote seniors. Patients and caregivers are seen via telehealth prior to the in-person clinic, and followed at 6 weeks, 12 weeks, 6 months, one year, and yearly. Patients are randomly assigned to in-person (standard care) or telehealth for the first follow-up, then alternating between the two modes of treatment, prior to 1-year follow-up. On average, telehealth appointments reduce participants' travel by 426 km per round trip. Findings show that telehealth coordinators rated 85% of patients and 92% of caregiversas comfortable or very comfortable during telehealth. Satisfaction scales completed by patient-caregiver dyads show high satisfaction with telehealth. Follow-up questionnaires reveal similar satisfaction with telehealth and in-person appointments, but telehealth is rated as significantly more convenient. Predictors of discontinuing follow-up are greater distance to telehealth, old-age patient, lower telehealth satisfaction, and lower caregiver burden.

Brain iron detected by SWI high pass filtered phase calibrated with synchrotron X-ray fluorescence.

PURPOSE: To test the ability of susceptibility weighted images (SWI) and high pass filtered phase images to localize and quantify brain iron. MATERIALS AND METHODS: Magnetic resonance (MR) images of human cadaver brain hemispheres were collected using a gradient echo based SWI sequence at 1.5T. For X-ray fluorescence (XRF) mapping, each brain was cut to obtain slices that reasonably matched the MR images and iron was mapped at the iron K-edge at 50 or 100 microm resolution. Iron was quantified using XRF calibration foils. Phase and iron XRF were averaged within anatomic regions of one slice, chosen for its range of iron concentrations and nearly perfect anatomic correspondence. X-ray absorption spectroscopy (XAS) was used to determine if the chemical form of iron was different in regions with poorer correspondence between iron and phase. RESULTS: Iron XRF maps, SWI, and high pass filtered phase data in nine brain slices from five subjects were visually very similar, particularly in high iron regions. The chemical form of iron could not explain poor matches. The correlation between the concentration of iron and phase in the cadaver brain was estimated as c(Fe) [microg/g tissue] = 850Deltavarpi + 110. CONCLUSION: The phase shift Deltavarpi was found to vary linearly with iron concentration with the best correspondence found in regions with high iron content.

Improving access to dementia care: development and evaluation of a rural and remote memory clinic.

The availability, accessibility and acceptability of services are critical factors in rural health service delivery. In Canada, the aging population and the consequent increase in prevalence of dementia challenge the ability of many rural communities to provide specialized dementia care. This paper describes the development, operation and evaluation of an interdisciplinary memory clinic designed to improve access to diagnosis and management of early stage dementia for older persons living in rural and remote areas in the Canadian province of Saskatchewan. We describe the clinic structure, processes and clinical assessment, as well as the evaluation research design and instruments. Finally, we report the demographic characteristics and geographic distribution of individuals referred during the first three years.

Iron, copper, and zinc distribution of the cerebellum.

Synchrotron rapid-scanning X-ray fluorescence (RS-XRF) is employed for the first time to simultaneously map iron, copper, and zinc in the normal cerebellum. The cerebellum is a major repository of metals that are essential to normal function. Therefore, mapping the normal metal distribution is an important first step towards understanding how multiple metals may induce oxidative damage, protein aggregation, and neurotoxicity leading to cerebellar degeneration in a wide range of diseases. We found that cerebellar white and grey matter could be sharply defined based upon the unique metal content of each region. The dentate nucleus was particularly metal-rich with copper localized to the periphery and iron and zinc abundant centrally. We discuss how RS-XRF metal mapping in the normal brain may yield important clues to the mechanisms of degeneration in the dentate nucleus.

Mapping metals in Parkinson's and normal brain using rapid-scanning x-ray fluorescence.

Rapid-scanning x-ray fluorescence (RS-XRF) is a synchrotron technology that maps multiple metals in tissues by employing unique hardware and software to increase scanning speed. RS-XRF was validated by mapping and quantifying iron, zinc and copper in brain slices from Parkinson's disease (PD) and unaffected subjects. Regions and structures in the brain were readily identified by their metal complement and each metal had a unique distribution. Many zinc-rich brain regions were low in iron and vice versa. The location and amount of iron in brain regions known to be affected in PD agreed with analyses using other methods. Sample preparation is simple and standard formalin-fixed autopsy slices are suitable. RS-XRF can simultaneously and non-destructively map and quantify multiple metals and holds great promise to reveal metal pathologies associated with PD and other neurodegenerative diseases as well as diseases of metal metabolism.

A comparison of rapid-scanning X-ray fluorescence mapping and magnetic resonance imaging to localize brain iron distribution.

The clinical diagnosis of many neurodegenerative disorders relies primarily or exclusively on observed behaviors rather than measurable physical tests. One of the hallmarks of Alzheimer disease (AD) is the presence of amyloid-containing plaques associated with deposits of iron, copper and/or zinc. Work in other laboratories has shown that iron-rich plaques can be seen in the mouse brain in vivo with magnetic resonance imaging (MRI) using a high-field strength magnet but this iron cannot be visualized in humans using clinical magnets. To improve the interpretation of MRI, we correlated iron accumulation visualized by X-ray fluorescence spectroscopy, an element-specific technique with T1, T2, and susceptibility weighted MR (SWI) in a mouse model of AD. We show that SWI best shows areas of increased iron accumulation when compared to standard sequences.

Rasmussen's encephalitis in a 58-year-old female: still a variant?

BACKGROUND: We report the case of a 58-year-old female with clinical, radiological, and histopathological evidence of Rasmussen's encephalitis, representing the oldest confirmed case to date. CASE SUMMARY: The patient presented with complex partial seizures characterized by numbness of the left face and staring spells. These progressed to a state of epilepsia partialis continua with jerking of the left face, as well as severe cognitive impairment and loss of all communication. The patient responded well to Intravenous Immunoglobulin (IVIG) therapy despite early complications and with ongoing treatment is living independently with minimal cognitive impairment. CONCLUSIONS: This represents the oldest confirmed case of Rasmussen's encephalitis and suggests that this diagnosis should be considered in patients of any age with an appropriate clinical picture. We recommend IVIG as a first line therapy for adult cases of Rasmussen's encephalitis.

Neonatal herpes encephalitis: a case series and review of clinical presentation.

OBJECTIVE: To describe the clinical and laboratory findings in cases of neonatal herpes simplex virus (HSV) encephalitis. BACKGROUND: Neonatal HSV encephalitis is a devastating infection which requires a high degree of clinical suspicion and rapid initiation of antiviral therapy. METHODS: We performed a retrospective search for all cases of HSV encephalitis within the two Saskatchewan pediatric tertiary care centers for the period of 1985-2001. Only those patients with consistent clinical presentations along with direct evidence of presence of HSV, such as positive cerebrospinal fluid (CSF) viral cultures, positive polymerase chain reaction (PCR) for HSV from CSF, or positive immunoglobulin G against HSV from neonatal blood, were selected. RESULTS: Five male and four female infant patients were identified. At a mean age of presentation of 24 +/- 20 days, seizures occurred in six neonates, lethargy in six neonates, temperature changes in five neonates, and apnea in three neonates. Examination of CSF demonstrated an initial monocytosis or lymphocytosis, elevated CSF protein and depressed CSF glucose in 100% of patients. Electroencephalography (EEG) was abnormal in 100% of patients. Initial computerized tomography was abnormal in 55% of patients. Clinical follow-up over an average of two years demonstrated developmental delay in four patients and upper motor neuron findings in four patients. No patients suffered from postencephalitic epilepsy or mortality. CONCLUSIONS: Neonatal HSV encephalitis most commonly presents with seizures, lethargy, and dysthermia. Cerebrospinal fluid testing and EEG have 100% sensitivity in cases with laboratory confirmation of HSV presence. Improvements in morbidity and mortality as compared to previous reports may relate to better recognition of this illness and acyclovir therapy. The lack of postinfection epilepsy in our series may also relate to better recognition and acyclovir therapy within this series of patients.