Faculty Bibliography

Background: Paroxysmal tonic spasms, also known as tonic seizures or paroxysmal dystonia, can be an atypical presenting or complicating feature of multiple sclerosis (MS). They entail sudden, stereotyped episodes of abnormal posture. They are often brief, occurring many times a day, either spontaneously or provoked by movement, touch, or other maneuvers. We describe five such patients, all of whom had a demyelinating lesion in the contralateral corticospinal tract in the posterior limb of the internal capsule. Objective: To present a case series of five patients with MS who experienced tonic spasms and their localisation. Methods: Chart review and literature search. Results: Five cases of tonic spasms in MS with correlating corticospinal tract lesions: Case 1: A 29-year-old female presented with cramping in her right arm and leg with abnormal posturing of the hand and foot with clenching and overlapping of digits. Case 2: A 30-year-old female with frequent episodes of right arm and leg dystonic movements with fanning of the fingers and toes. Case 3: A 39-year-old male with left facial twitching and spams of the left hand elicited by yawning. Case 4: A 29-year-old female with MS with involuntary clenching of the left hand. Case 5: A 34-year-old female with tingling of the right arm and leg followed by involuntary flexion of the right wrist and fingers. In all cases, a brain MRI showed a demyelinating lesion in the contralateral corticospinal tract in the posterior limb of the internal capsule, as well as other lesions consistent with MS. Conclusions: Tonic spasms in MS can be localised to the contralateral posterior limb of the internal capsule. While non-epileptic in origin, they often respond to anti-epileptic drugs. Prompt recognition of this phenomenon can expedite work up and facilitate treatment

BACKGROUND: Optical coherence tomography (OCT) and magnetic resonance imaging (MRI) can provide complementary information on visual system damage in multiple sclerosis (MS). OBJECTIVES: The objective of this paper is to determine whether a composite OCT/MRI score, reflecting cumulative damage along the entire visual pathway, can predict visual deficits in primary progressive multiple sclerosis (PPMS). METHODS: Twenty-five PPMS patients and 20 age-matched controls underwent neuro-ophthalmologic evaluation, spectral-domain OCT, and 3T brain MRI. Differences between groups were assessed by univariate general linear model and principal component analysis (PCA) grouped instrumental variables into main components. Linear regression analysis was used to assess the relationship between low-contrast visual acuity (LCVA), OCT/MRI-derived metrics and PCA-derived composite scores. RESULTS: PCA identified four main components explaining 80.69% of data variance. Considering each variable independently, LCVA 1.25% was significantly predicted by ganglion cell-inner plexiform layer (GCIPL) thickness, thalamic volume and optic radiation (OR) lesion volume (adjusted R2 0.328, p = 0.00004; adjusted R2 0.187, p = 0.002 and adjusted R2 0.180, p = 0.002). The PCA composite score of global visual pathway damage independently predicted both LCVA 1.25% (adjusted R2 value 0.361, p = 0.00001) and LCVA 2.50% (adjusted R2 value 0.323, p = 0.00003). CONCLUSION: A multiparametric score represents a more comprehensive and effective tool to explain visual disability than a single instrumental metric in PPMS.

Objective: To present clinical and radiographic descriptions of conditions that may feature corticospinal tract abnormalities observed on magnetic resonance imaging (MRI). Background: Corticospinal tract lesions have a broad differential diagnosis, including neurodegenerative diseases, toxic/metabolic derangements, malignancies, autoimmune diseases, infectious diseases, and neurogenetic conditions. Design/Methods: Review of clinical presentations and brain MRIs. Results: Conditions that have been associated with corticospinal tract hyperintensities on brain MRI include: amyotrophic lateral sclerosis, primary lateral sclerosis, heroin leukoencephalopathy, brainstem glioma, neuroBehcets, HIV infection, neuromyelitis optica, Krabbe A disease, adult polyglucosan body disorder, Xlinked Charcot-Marie-Tooth disease, Behr syndrome, Whipple disease, and sequela of liver transplantation. We present representative images and discuss clinical and radiographic features that distinguishing these conditions. Conclusions: Corticospinal tract lesions have a heterogenous etiology, with widely different treatments and prognoses. An understanding of these potential etiologies will assist neurologists confronted with this imaging finding

Introduction Physicians often struggle with the intricacies of brain death determination and communication about end-of-life care. In an effort to remedy this situation, we introduced an educational initiative at our medical school to improve student comprehension and comfort dealing with brain death. Methods Beginning in July 2017, students at our medical school were required to attend a 90-minute brain death didactic and simulation session during their neurology clerkship. Students completed a test immediately before and after participating in the initiative. Results Of the 145 students who participated in this educational initiative between July 2016 and June 2017, 124 (86%) consented to have their data used for research purposes. Students correctly answered a median of 53% of questions (IQR 47-58%) on the pretest and 86% of questions (IQR 78-89%) on the posttest (p<0.001). Comfort with both performing a brain death evaluation and talking to a family about brain death improved significantly after this initiative (18% of students were comfortable performing a brain death evaluation before the initiative and 86% were comfortable doing so after the initiative, p<0.001; 18% were comfortable talking to a family about brain death before the initiative and 76% were comfortable doing so after the initiative, p<0.001). Conclusions Incorporation of simulation in undergraduate medical education is high-yield. At our medical school, knowledge about brain death and comfort performing a brain death exam or talking to a family about brain death was poor prior to development of this initiative, but awareness and comfort dealing with brain death improved significantly after this initiative. This initiative was clearly a success and can serve as a model for brain death education at other medical schools

The epidemiology of multiple sclerosis (MS) includes a consideration of genetic and environmental factors. Comparative studies of different populations have revealed prevalence and incidence rates that vary with geography and ethnicity. With a prevalence ranging from 2 per 100,000 in Japan to greater than 100 per 100,000 in Northern Europe and North America, the burden of MS is similarly unevenly influenced by longevity and comorbid disorders. Well-powered genome-wide association studies have investigated the genetic substrate of MS, providing insight into autoimmune mechanisms involved in the etiopathogenesis of MS and elucidating possible avenues of biological treatment.