Limbic and new onset refractory tonic status epilepticus (NORSE) in anti-NMDAR encephalitis

Aims: To present a case of anti-NMDA receptor encephalitis (anti-NMDAR) with new onset refractory non-convulsive status epilepticus (NORSE). Methods: Case report with clinical details, MRI, PET, and EEG illustrations. Results: New onset refractory status epilepticus (NORSE) may arise from anti-NMDAR, and offers diagnostic and treatment challenges for immuno-therapy and refractory status epilepticus. Non-convulsive status epilepticus with generalized fast activity, has not been reported in anti-NMDAR, in NORSE. Conclusions: A patient with anti-NMDAR and generalized status with stiffening, right focal weakness, high frequency alpha/beta on EEG, brain FDG-PET/CT changes in the left temporo-parietal regions and cerebellum was presented. We discuss the unique treatment challenges of anti-NMDAR. NORSE and generalized nonconvulsive status epilepticus. Keywords: Tonic status epilepticus, Limbic encephalitis, Limbic status epilepticus, Anti-NMDAR, NORS

Patterns and Perceptions of Smartphone Use Among Academic Neurologists in the United States: Questionnaire Survey.

BackgroundSmartphone technology is ubiquitous throughout neurologic practices, and numerous apps relevant to a neurologist's clinical practice are now available. Data from other medical specialties suggest high utilization of smartphones in routine clinical care. However, the ways in which these devices are used by neurologists for patient care-related activities are not well defined.ObjectiveThis paper aims to characterize current patterns of smartphone use and perceptions of the utility of smartphones for patient care-related activities among academic neurology trainees and attending physicians. We also seek to characterize areas of need for future app development.MethodsWe developed a 31-item electronic questionnaire to address these questions and invited neurology trainees and attendings of all residency programs based in the United States to participate. We summarized descriptive statistics for respondents and specifically compared responses between trainees and attending physicians.ResultsWe received 213 responses, including 112 trainee and 87 attending neurologist responses. Neurology trainees reported more frequent use of their smartphone for patient care-related activities than attending neurologists (several times per day: 84/112, 75.0% of trainees; 52/87, 59.8% of attendings; P=.03). The most frequently reported activities were internet use, calendar use, communication with other physicians, personal education, and health care-specific app use. Both groups also reported regular smartphone use for the physical examination, with trainees again reporting more frequent usage compared with attendings (more than once per week: 35/96, 36.5% of trainees; 8/58, 13.8% of attendings; P=.03). Respondents used their devices most commonly for the vision, cranial nerve, and language portions of the neurologic examination. The majority of respondents in both groups reported their smartphones as "very useful" or "essential" for the completion of patient care-related activities (81/108, 75.0% of trainees; 50/83, 60.2% of attendings; P=.12). Neurology trainees reported a greater likelihood of using their smartphones in the future than attending neurologists ("very likely": 73/102, 71.6% of trainees; 40/82, 48.8% of attendings; P=.005). The groups differed in their frequencies of device usage for specific patient care-related activities, with trainees reporting higher usage for most activities. Despite high levels of use, only 12 of 184 (6.5%) respondents reported ever having had any training on how to use their device for clinical care. Regarding future app development, respondents rated vision, language, mental status, and cranial nerve testing as potentially being the most useful to aid in the performance of the neurologic examination.ConclusionsSmartphones are used frequently and are subjectively perceived to be highly useful by academic neurologists. Trainees tended to use their devices more frequently than attendings. Our results suggest specific avenues for future technological development to improve smartphone use for patient care-related activities. They also suggest an unmet need for education on effectively using smartphone technology for clinical care

Oh My GAD!! Something Else?

A 43 -year-old man presented with oscillopsia, dizziness, binocular vertical diplopia, and gait difficulties. He reported a six-month history of abdominal pain, anorexia and 50-pound weight loss, and a 3-month history of mood and cognitive changes. Torsional nystagmus, a left 4th nerve palsy (NP), unsteady gait, occasional head jerks, and intermittent facial grimacing were noted onexamination, and MRI brain/orbits was normal. Over weeks his symptoms worsened and he developed labile blood pressure and syncope. Repeat MRI, single-fiber EMG, myasthenic panel, celiac panel, TSH, cyanocobalamin, thiamine, RPR, copper, ESR, CRP, ANA, RF, ceruloplasmin, anti-ASO titers were normal. CSF and serum infectious, toxic, autoimmune and paraneoplastic panels were also within normal limits. He was referred to our institution 1 month after his initial assessment. Our evaluation demonstrated full ocular motility, a 6 prism-diopter left hypertropia (worse in right gaze and left head tilt, unchanged in upgaze and slightly less in downgaze), and spontaneous upbeat-torsional nystagmus with top poles beating toward the left ear and a milder left-beating component. Saccades, smooth pursuit, horizontal vestibulo-ocular reflex (VOR), VOR suppression and optokinetic nystagmus appeared unremarkable when taking into account the nystagmus. Afferent neuro-ophthalmic examination was normal. Serum anti-GAD antibody was positive (15 IU/mL, Normal <5.0 IU/mL). MRI brain and testicular ultrasound were normal. PET-CT showed increased FDG uptake in left medial rectus (MR), right lateral rectus (LR), and bilateral inferior recti (IR) muscles. Plasma exchange (PLEX) was initiated and his symptoms improved. Several days later, serum anti-dipeptidyl aminopeptidase-like protein 6 (DPPX) IgG returned as positive (titer 1:15.360), and the diagnosis of anti-DPPX-associated encephalitis was made. Screening for an underlying neoplasm was unrevealing, and treatment with rituximab was initiated. Nystagmus improved, and repeat PET-CT demonstrated resolution of the prior extraocular muscle (EOM) avidity

Oh My GAD!! Something Else?

A 43 -year-old man presented with oscillopsia, dizziness, binocular vertical diplopia, and gait difficulties. He reported a six-month history of abdominal pain, anorexia and 50-pound weight loss, and a 3-month history of mood and cognitive changes. Torsional nystagmus, a left 4th nerve palsy (NP), unsteady gait, occasional head jerks, and intermittent facial grimacing were noted onexamination, and MRI brain/orbits was normal. Over weeks his symptoms worsened and he developed labile blood pressure and syncope. Repeat MRI, single-fiber EMG, myasthenic panel, celiac panel, TSH, cyanocobalamin, thiamine, RPR, copper, ESR, CRP, ANA, RF, ceruloplasmin, anti-ASO titers were normal. CSF and serum infectious, toxic, autoimmune and paraneoplastic panels were also within normal limits. He was referred to our institution 1 month after his initial assessment. Our evaluation demonstrated full ocular motility, a 6 prism-diopter left hypertropia (worse in right gaze and left head tilt, unchanged in upgaze and slightly less in downgaze), and spontaneous upbeat-torsional nystagmus with top poles beating toward the left ear and a milder left-beating component. Saccades, smooth pursuit, horizontal vestibulo-ocular reflex (VOR), VOR suppression and optokinetic nystagmus appeared unremarkable when taking into account the nystagmus. Afferent neuro-ophthalmic examination was normal. Serum anti-GAD antibody was positive (15 IU/mL, Normal <5.0 IU/mL). MRI brain and testicular ultrasound were normal. PET-CT showed increased FDG uptake in left medial rectus (MR), right lateral rectus (LR), and bilateral inferior recti (IR) muscles. Plasma exchange (PLEX) was initiated and his symptoms improved. Several days later, serum anti-dipeptidyl aminopeptidase-like protein 6 (DPPX) IgG returned as positive (titer 1:15.360), and the diagnosis of anti-DPPX-associated encephalitis was made. Screening for an underlying neoplasm was unrevealing, and treatment with rituximab was initiated. Nystagmus improved, and repeat PET-CT demonstrated resolution of the prior extraocular muscle (EOM) avidity

Oh My GAD!! Something Else?

A 43 -year-old man presented with oscillopsia, dizziness, binocular vertical diplopia, and gait difficulties. He reported a six-month history of abdominal pain, anorexia and 50-pound weight loss, and a 3-month history of mood and cognitive changes. Torsional nystagmus, a left 4th nerve palsy (NP), unsteady gait, occasional head jerks, and intermittent facial grimacing were noted onexamination, and MRI brain/orbits was normal. Over weeks his symptoms worsened and he developed labile blood pressure and syncope. Repeat MRI, single-fiber EMG, myasthenic panel, celiac panel, TSH, cyanocobalamin, thiamine, RPR, copper, ESR, CRP, ANA, RF, ceruloplasmin, anti-ASO titers were normal. CSF and serum infectious, toxic, autoimmune and paraneoplastic panels were also within normal limits. He was referred to our institution 1 month after his initial assessment. Our evaluation demonstrated full ocular motility, a 6 prism-diopter left hypertropia (worse in right gaze and left head tilt, unchanged in upgaze and slightly less in downgaze), and spontaneous upbeat-torsional nystagmus with top poles beating toward the left ear and a milder left-beating component. Saccades, smooth pursuit, horizontal vestibulo-ocular reflex (VOR), VOR suppression and optokinetic nystagmus appeared unremarkable when taking into account the nystagmus. Afferent neuro-ophthalmic examination was normal. Serum anti-GAD antibody was positive (15 IU/mL, Normal <5.0 IU/mL). MRI brain and testicular ultrasound were normal. PET-CT showed increased FDG uptake in left medial rectus (MR), right lateral rectus (LR), and bilateral inferior recti (IR) muscles. Plasma exchange (PLEX) was initiated and his symptoms improved. Several days later, serum anti-dipeptidyl aminopeptidase-like protein 6 (DPPX) IgG returned as positive (titer 1:15.360), and the diagnosis of anti-DPPX-associated encephalitis was made. Screening for an underlying neoplasm was unrevealing, and treatment with rituximab was initiated. Nystagmus improved, and repeat PET-CT demonstrated resolution of the prior extraocular muscle (EOM) avidity

EEG Differences in Two Clinically Similar Rapid Dementias: Voltage-Gated Potassium Channel Complex-Associated Autoimmune Encephalitis and Creutzfeldt-Jakob Disease

Distinguishing treatable causes for rapidly progressive dementia from those that are incurable is vital. Creutzfeldt-Jakob disease (CJD) and voltage-gated potassium channel complex-associated autoimmune encephalitis (VGKC AE) are 2 such conditions with disparate outcomes and response to treatment. To determine the differences in electroencephalography between CJD and VGKC AE, we performed a retrospective review of medical records and examined clinical data, neuroimaging, and electroencephalographs performed in patients admitted for evaluation for rapidly progressive dementia diagnosed with CJD and VGKC AE at the Johns Hopkins Hospital and Bayview Medical Center between January 1, 2007 and December 31, 2015. More patients in the VGKC AE group had seizures (12/17) than those with CJD (3/14; P = .008). Serum sodium levels were lower in those with VGKC AE ( P = .001). Cerebrospinal fluid (CSF) white blood cell count was higher in VGKC AE ( P = .008). CSF protein 14-3-3 ( P = .018) was more commonly detected in CJD, and tau levels were higher in those with CJD ( P &lt; .006). On neuroimaging, diffusion restriction in the cortex ( P = .001), caudate ( P &lt; .001), and putamen ( P = .001) was more frequent in CJD. Periodic sharp wave complexes ( P = .001) and generalized suppressed activity ( P = .008) were more common on initial EEG in CJD. On serial EEGs, generalized periodic discharges ( P = .004), generalized suppressed activity (P=0.008), and periodic sharp wave complexes ( P &lt; .001) were detected more in CJD. This study shows that there are a number of differentiating features between CJD and VGKC AE, and electroencephalography can aid in their diagnoses. Performing serial EEGs better delineates these conditions