Arch Neurol Research Articles

Psychiatric brain gain in Switzerland. Competency-based onboarding.

IntroductionIn the last 30 years, Switzerland has been established as a destination country for psychiatric trainees. The needed competences for the work as a trainee deviate regarding colleagues from foreign countries though, hindering a viable solid development professional without specific on-boarding program. A similar approach to the figure of tutor anchored in the Spanish postgraduate medical training is still missing in the Swiss medical System. Hereby we performed a survey in the new colleagues who are part from the medical team in an observer status before beginning with the responsibilities as a trainee.ObjectivesRecognizing competences and needs of the onboarding in current trainees that are still allocating because of the work conditions as stated in the following paper, (Bischof et al. Swiss Arch Neurol Psychiatr Psychother. 2021;172:w03198)MethodsSurvey with open questions collecting needs and competences expected to fulfil in Switzerland were distributed in 5 different medical colleagues in an observer status between August 2022 and September 2023.ResultsResponse rate was 62,5 %. Main reasons for the migration were considering better perspectives in education and professional development in the goal country, coming push factors as the current work situation in the original country to the fore. Support regarding the local language and an overview of the interprofessional communication were outlined as the advantage of the internship prior to the duties as a psychiatric trainee.ConclusionsAn structured on-boarding program is a demand for the newcomers - majority of trainees from foreign countries - to step in better in the Swiss health system. Elements of the Spanish trainee system could be adapted for a suitable allocation and integration process in the goal country.Disclosure of InterestNone Declared

18 Automatically calculated lexical and sentential context features of connected speech predict cognitive impairment

Objective:Early detection is critical to the effective management of Alzheimer’s Disease (AD) and other forms of dementia. Existing screening assessments are often costly, require substantial expertise to administer, and may be insensitive to mild changes in cognition. A promising alternative is automatically measuring features of connected speech (c.f., Ostrand & Gunstad, 2021, Journal Geriatric Psych & Neurol) to predict impairment. Here, we built on prior work examining how well speech features predicted cognitive impairment. Unique to the current work, we attempted to capture more holistic effects of cognitive impairment by examining the relevance of linguistic features that measure sentential or discourse context properties of speech, including the context in which filler words (e.g., um) occur, and the predictability of individual words within their sentence context, computed from a large computational language model (GPT-2).Participants and Methods:Participants completed the Cookie Theft picture description task, with data available in the DementiaBank corpus (Becker et al., 1994, Arch Neurol). Descriptions that contained at least 50 words (N = 214) were submitted to an automatic feature calculation pipeline written in Python to calculate various part-of-speech counts, lexical diversity metrics, and mean lexical frequency, as well as multiple metrics related to lexical surprisal (i.e., how surprising a word is given its context).Surprisal of individual words was computed using the pre-trained GPT-2 transformer language model (Radford et al., 2019, Comput. Sci.) by computing word probability given the previous 12 words. Multiple linear regression was performed using 17 linguistic features jointly as predictors, and Mini-Mental State Examination (MMSE) score as the outcome variable. Simple regressions were calculated between each feature and MMSE scores to examine the predictability of each linguistic feature on cognitive decline.Results:A multiple linear regression model containing all linguistic features plus demographic information (age, sex, education) significantly predicted MMSE scores (Adjusted R2 = 0.41, F20, 193 = 8.37, p < .001), and explained significantly more variance in MMSE scores than did demographic variables alone (F17193 = 6.85, p < .001). Individual predictors that were significantly correlated with MMSE score included: how unexpected an individual’s word choices were, given the preceding context (median surprisal: r = -0.33, p < 0.001; interquartile range: r = 0.18, p < 0.02), mean lexical frequency (r = -0.50, p < .001), and usage of definite articles (r = 0.31, p < 0.001), nouns (r = 0.26, p < .001), and empty words (r = -0.25, p < 0.001).Conclusions:Participants with lower MMSE scores, indicating greater impairment, used more frequent, yet more surprising, words, and produced more empty words and fewer definite articles and nouns. These results suggest that measures of semantic specificity and coherence of speech could be meaningful predictors of cognitive decline, and can be computed automatically from speech transcriptions. The results also provide novel evidence that computational approaches to estimating lexical predictability may have value in predicting the degree of decline, motivating future work in other speech elicitation tasks and differing clinical groups.

The Case Files

The Case Files

Palato-ocular synchrony: Seeing what is behind closed eyelids in palatal tremor (P5-11.010)

<h3>Objective:</h3> To call attention to palato-ocular synchrony, a little-known manifestation of palatal tremor. <h3>Background:</h3> Palatal tremor due to a lesion of the dentato-rubro-olivary pathway (Guillain-Mollaret triangle) may spread to contiguous or remote muscles. When it involves the eyes it is known as oculopalatal tremor (OPT) manifesting as pendular nystagmus, typically vertical and/or torsional, causing oscillopsia. Nystagmus may be minimal or absent with eyes open and appear only under closed eyelids, a phenomenon described in 1976 by Jacobs and Bender, and called palato-ocular synchrony (Arch Neurol;33:289). These authors observed that palato-ocular synchrony was caused by eyelid closure rather than simply the removal of vision. <h3>Design/Methods:</h3> Two illustrative video case reports. <h3>Results:</h3> Following brainstem hemorrhage (patient 1 – midbrain; patient 2 – pons) involving the Guillain-Mollaret triangle, two patients developed hypertrophic olivary degeneration and asymptomatic palatal tremor. In patient 1, there was no nystagmus with eyes open, but under closed eyelids, vertical oscillations were readily apparent. In patient 2, there was very subtle torsional pendular nystagmus without oscillopsia, but large vertical oscillations were apparent with eyelid closure that were synchronous with PT. The mechanism of palato-ocular synchrony is unknown but may be related to the observation that palatal tremor, due to a lesion of the Guillain-Mollaret triangle, is one of the few movement disorders that persist during sleep, and this may simply reflect eyelid closure. <h3>Conclusions:</h3> A lesion, typically stroke, involving the Guillain-Mollaret triangle often leads to delayed development of palatal tremor. Such patients should undergo a careful ocular motor examination looking for oculopalatal tremor; if absent, then the globes should be observed during active eye closure. A visible or palpable ocular oscillation under closed eyelids is known as palato-ocular synchrony, an underappreciated phenomenon that extends the spectrum of manifestations of palatal tremor. <b>Disclosure:</b> Dr. Reich has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Best Doctors. Dr. Reich has received personal compensation in the range of $500-$4,999 for serving as a Consultant for UpToDate. Dr. Reich has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Enterin. Dr. Reich has received personal compensation in the range of $0-$499 for serving on a Scientific Advisory or Data Safety Monitoring board for NIH. Dr. Reich has received personal compensation in the range of $10,000-$49,999 for serving as an Expert Witness for Various law firms. The institution of Dr. Reich has received research support from ninds. Dr. Reich has received publishing royalties from a publication relating to health care. Dr. Reich has received publishing royalties from a publication relating to health care. Dr. Gold has received personal compensation in the range of $500-$4,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Springer .

Chinese expert consensus on the treatment of MMD

Moyamoya disease (MMD), also known as spontaneous occlusion of the circle of Willis, is defined by progressive stenosis or occlusion of the internal carotid arteries, and it can progress to the anterior, middle, and posterior cerebral arteries. As these arteries are gradually stenosed, a collateral network of capillaries develops at the base of the brain, producing the characteristic reticulate appearance (“puff of smoke”) on angiography. Therefore, it was named by Suzuki and Takaku in 1969 after the Japanese term “moyamoya” (Suzuki and Takaku, Arch Neurol 20:288–299, 1969). MMD is most common in East Asian countries such as Japan and Korea, and it shows a slight female predominance. MMD is mainly characterized by ischemia and hemorrhage. Hemorrhagic MMD is very rare in children, and most cases occur in adults due to the rupture of the compensatory blood vessels, which often leads to hemorrhagic symptoms (Scott and Smith, N Engl J Med 360:1226–1237, 2009). In recent years, the diagnosis rate has increased with the popularization of imaging techniques. However, the pathogenesis of MMD is still not completely understood, and there is currently no evidence to suggest that drug treatment can delay or even reverse the progression of MMD. The current drug treatment for in MMD only targets its clinical symptoms, including ischemia and hemorrhage. The main choice of treatment for MMD is surgical revascularization. As an increasing number of hospitals have developed surgical treatment for MMD, our compiling group has jointly discussed the formulation of a consensus among Chinese experts on the treatment of MMD.

DEALING WITH DOUBLE TROUBLE: EMERGENT CARDIAC CATHETERIZATION COMPLICATED BY PERIPROCEDURAL ISCHEMIC STROKE

DEALING WITH DOUBLE TROUBLE: EMERGENT CARDIAC CATHETERIZATION COMPLICATED BY PERIPROCEDURAL ISCHEMIC STROKE

A RARE CAUSE OF PARAPLEGIA: DISTRIBUTIVE SHOCK CAUSING BORDER ZONE STROKES

TOPIC: Critical Care TYPE: Medical Student/Resident Case Reports INTRODUCTION: Simultaneous bilateral cerebrovascular infarction is relatively uncommon and is an entity seen rarely in watershed cerebral infarctions which occur at the border between cerebral vascular territories in the setting of severe hypotension. CASE PRESENTATION: 84-year-old male with history of CAD, cerebellar stroke, peripheral arterial disease was admitted to the ICU with distributive shock and intubated for toxic metabolic encephalopathy. Initial CT head on admission was unremarkable. On day 2 of hospitalization his neuro assessment was notable for bilateral lower extremity paresis with preservation of sensation, proprioception and temperature but had sluggish reflexes and loss of motor strength. Repeat CT head did not show a bleed and subsequently, MRI brain with MRA head and neck revealed bilateral acute anterior cerebral artery strokes. Given the location and duration of symptoms he was not a TPA candidate and was treated with medical management with aspirin and statin. Echocardiogram and carotid ultrasounds were done that were unremarkable for embolic cause of stroke. DISCUSSION: The anterior cerebral artery is a major vessel supplying blood to the interhemispheric region. ACA territory infarction accounts for only 0.3% to 4.4% of cerebral infarctions. Bilateral ACA infarction is even rarer. Twenty-seven cases of ACA territory infarction were reported among 1490 cases of cerebral infarction in the Lausanne Stroke Registry; among them, only two cases had bilateral ACA territory infarction. Bilateral ACA infarction can occur due to vasospasm in the setting of subarachnoid hemorrhage caused by a rupture of aneurysms of the anterior communicating arteries or distal ACAs, thrombosis or embolism in case of anomaly of circle of Willis or bilateral anaplastic ACAs. In our patient the area of infarction was in a watershed zone bilaterally, unfortunately affected by hypotension which is rare in the absence of any of the above-mentioned anomalies as predisposing factors. The true incidence of bilateral ACA infarction is unknown, with few cases reported in the literature. CONCLUSIONS: This case report highlights the possibility of developing simultaneous bilateral cerebral infarctions in the setting of profound systemic hypotension and shock, especially if the patient has baseline chronic small vessel changes. REFERENCE #1: Orlandi G, Moretti P, Fioretti C, Puglioli M, Collavoli P, Murri L. Bilateral medial frontal infarction in a case of azygous anterior cerebral artery stenosis. Ital J Neurol Sci. 1998;19:106–108. doi: 10.1007/BF02427567. [PubMed] [CrossRef] [Google Scholar] REFERENCE #2: Gacs G, Fox AF, Barnett HJ, Vinuela F. Occurrence and mechanisms of occlusion of the anterior cerebral artery. Stroke. 1983;14:952–959. [PubMed] [Google Scholar] REFERENCE #3: Bogousslavsky J, Regli F. Anterior cerebral artery territory infarction in the Lausanne Stroke Registry. Clinical and etiologic patterns. Arch Neurol. 1990;47:144–150. [PubMed] [Google Scholar] DISCLOSURES: No relevant relationships by Imama Ahmad, source=Web Response No relevant relationships by Christine Blaski, source=Web Response No relevant relationships by Sneha Lakshman, source=Web Response

UNEXPECTED OPPORTUNISTIC INFECTION IN PATIENT WITH SEVERE COVID-19 PNEUMONIA

TOPIC: Chest Infections TYPE: Medical Student/Resident Case Reports INTRODUCTION: The pathogenesis of the novel coronavirus, Covid-19, was characterized by an overdrive of the immune system that lead to some patients to a life-threatening cytokine storm with severe lung inflammation and multiorgan failure. Considering this pathogenic pathway, the use of high dose systemic steroids, and other immunomodulatory therapy was part of the main therapy. As management evolves, complications have risen, including cardiac, thromboembolic, neurologic and secondary infections. Multiple clinical reports have described complications with bacteria, and fungal infections in different organ systems, most commonly the respiratory track. However, systemic and multiorgan infections have been reported due to severe immunosuppression after viral infection and the use of anti-inflammatory therapy. CASE PRESENTATION: We present the case of an 86-year-old male with a past medical history of benign prostate hypertrophy, hyperlipidemia and hypertension who developed respiratory failure due to severe COVID-19 pneumonia, managed with antiviral therapy and high dose dexamethasone. After initial improvement from the respiratory standpoint, the patient developed persistent fungemia with Candida parapsilosis. There was no evidence of endocarditis. The patient presented with persistent altered mental status after cessation of sedation. Brain imaging revealed a right posterior parietal parafalcine hematoma with surrounding vasogenic edema. Angiogram revealed a saccular aneurysm measuring approximately 5.7 mm in largest dimension located at midline posterior frontal in location in relation to pericallosal arteries. Cerebral arterial aneurysm secondary to systemic fungal infection, knowns as mycotic aneurysms, have been reported in medical literature. DISCUSSION: Aneurysms are an abnormal arterial focal dilation which can become infected, but also aneurysmal degeneration of the arterial wall can occur due to bacteremia or a septic embolus;as seen in mycotic aneurisms. Despite its name, most mycotic aneurysms are caused by bacteria from associated endocarditis. This is a serious complication associated to an elevated morbidity and mortality. Several risk factors predispose to infected aneurysm, these include arterial injury, infection, immunocompromised status, atherosclerosis and pre-existing aneurysms. Fungal infections tend to be rare but may occur in immune suppressed patients or as sequelae of treatment of disseminated fungal disease. CONCLUSIONS: This is an unexpected complication of immunosuppression with systemic steroids in management of COVID-19 pneumonia. Treatment with systemic steroid and thromboembolic complication prevention have helped improve COVID-19 pneumonia patient prognosis, however physicians should be careful as it predisposes patients, especially those with advanced age, to opportunistic infections in unexpected organ and settings. REFERENCE #1: WHO Rapid Evidence Appraisal for COVID-19 Therapies (REACT) Working Group, Sterne JAC, Murthy S, Diaz JV, Slutsky AS, Villar J, Angus DC, Annane D, Azevedo LCP, Berwanger O, Cavalcanti AB, Dequin PF, Du B, Emberson J, Fisher D, Giraudeau B, Gordon AC, Granholm A, Green C, Haynes R, Heming N, Higgins JPT, Horby P, Juni P, Landray MJ, Le Gouge A, Leclerc M, Lim WS, Machado FR, McArthur C, Meziani F, Moller MH, Perner A, Petersen MW, Savovic J, Tomazini B, Veiga VC, Webb S, Marshall JC. Association Between Administration of Systemic Corticosteroids and Mortality Among Critically Ill Patients With COVID-19: A Meta-analysis. JAMA. 2020 Oct 6;324(13):1330-1341. doi: 10.1001/jama.2020.17023. PMID: 32876694;PMCID: PMC7489434. REFERENCE #2: Wu FZ, Lai PH. Evolution and regression of intracranial infectious aneurysm diagnosed by brain computed tomographic angiography. Arch Neurol. 2010 Sep;67(9):1147. doi: 10.1001/archneurol.2010.216. PMID: 20837863. REFERENCE #3: World Health Organization. Corticosteroids for COVID-19: Living guidance. https://www.who.int/publications/i/item/WHO-2019-nCoV-Cor icosteroids-2020.1 DISCLOSURES: No relevant relationships by Onix Cantres-Fonseca, source=Web Response No relevant relationships by Porfirio Diaz-Rodriguez, source=Web Response No relevant relationships by Hector Nieves, source=Web Response No relevant relationships by Luis Vazquez, source=Web Response

Invited Perspective: Air Pollution and Dementia: Challenges and Opportunities.

Invited Perspective: Air Pollution and Dementia: Challenges and Opportunities.

Short and Long-Term Risk of Major Cardiovascular Events after Ischemic Stroke or Transient Ischemic Attack in Myeloproliferative Neoplasms

BACKGROUND. Very few previous studies have estimated the risk of recurrence after cerebral transient ischemic attack (TIA) or ischemic stroke (IS) in patients with myeloproliferative neoplasms (MPN). PRISM (Preventing Ischemic Stroke in Myeloproliferative Neoplasms) is an international investigator-driven study with three purposes: (1) to describe the clinical profile at presentation of ischemic TIA/IS in contemporary patients with polycythemia vera (PV), essential thrombocythemia (ET) and primary myelofibrosis (PMF); (2) to evaluate the short and long-term outcomes beyond the acute phase and to explore their comparability with non MPN population; (3) to refine the risk assessment for recurrences and cardiovascular mortality.METHODS. From January 2005 through December 2015, 597 patients with objectively proven TIA (n= 270) or IS (n=327) at 22 sites of 5 countries were enrolled. We estimated the 1- and 5-year risk of composite cardiovascular (CV) outcome, including recurrent TIA, IS, myocardial infarction (AMI) and CV deaths, and analyzed the incidence of each single outcome by competing risk method.RESULTS. The distribution of single MPN and baseline blood counts were similar in IS and TIA groups; atrial fibrillation was observed in a minority of cases (4% TIA and 7% IS) as compared with the general population (11-18% and 20%-30%, respectively); other comorbidities were approximately half of those of non-MPN patients [ Weimar C et al, Arch Neurol 2002;59:1584; Buchwald F et al, Stroke 2016;47:2456; Amarenco P et al, N Engl J Med 2016;374:1533 ]; a substantial proportion of TIA cases had a higher history of microvascular disturbances while arterial hypertension was more frequent in IS than TIA. Secondary prophylaxis included aspirin, oral anticoagulants and cytoreductive therapy.After one year since the TIA index event, the incidence of cumulative outcome was 4.21%. However, no IS happened in the first 2 years after TIA and this event after 5 years happened in only 1.24% of cases. The corresponding incidence in the general population was 4.4% to 5.1% after one year and 12-16% after 5 years [ Weimar C et al, Arch Neurol 2002;59:1584; Buchwald F et al, Stroke 2016;47:2456; Amarenco P et al, N Engl J Med 2016;374:1533 ]. In contrast, new TIA episodes progressively increased year by year reaching 12% incidence after 5 years.After the IS index event, new strokes occurred with cumulative incidence lower than in the general population: after 1 and 5 years, the incidence was 2.03% and 6.52% vs 11.1%-12% and 26.4% of non-MPN patients [ Mohan KM et al, Stroke 2011;42:1489; Bergstrom L et al, Stroke 2017 Jul 13, AOP ]. The rate of deep venous thrombosis (DVT) in the first year, was higher after index IS than TIA, but was the same by 5 years (3.68% and 4.60%, respectively; p=0.47).Predictors of recurrent TIA and IS were the same index events (HR=2.41 and 4.41), and for recurrent TIA the remote history of cerebrovascular TIA episodes (HR=3.40) and microvascular disturbances (HR=2.30). Other prognostic factors of recurrent IS were arterial hypertension (HR=4.24) and occurrence of IS during the course of MPN (HR=4.27). On the contrary, cytoreductive therapy emerged as a strong protective factor able to reduce the probability of new strokes in 76% of patients (HR=0.24). The history of peripheral thrombosis, both venous and arterial events, independently predicted the occurrence of AMI (HR=6.30), while age over 60 years (HR=3.98), index IS (HR=3.61), occurrence after MPN diagnosis (HR=2.62), were all prognostically significant for CV mortality. During median follow-up of 4.2 years, major bleeding occurred with a frequency similar to that observed in the general population receiving long-term antiplatelet secondary prophylaxis (0.90 % pts/year) [ Li L et al, Lancet 2017 Jun 13, AOP ].CONCLUSION. Current antithrombotic therapy and cytoreductive drugs after index TIA and IS led to a reduction of cardio-vascular recurrences and mortality in a proportion of MPN patients greater than in general population. This would indicate that ischemic cerebral vascular events recognize a specific pathogenesis of thrombosis linked to the clonal disorder. Although the benefit-risk profile of secondary prophylaxis appears favorable, some high risk categories need new intervention strategies to be defined in prospective studies. DisclosuresIurlo:Novartis: Consultancy, Honoraria; Bristol Myers Squibb: Consultancy, Honoraria; Pfizer: Consultancy, Honoraria. Finazzi:Italfarmaco S.p.A.: Consultancy. Bonifacio:Incyte: Membership on an entity's Board of Directors or advisory committees; Novartis: Membership on an entity's Board of Directors or advisory committees, Research Funding; Pfizer: Membership on an entity's Board of Directors or advisory committees; Bristol-Myers Squibb: Membership on an entity's Board of Directors or advisory committees. Gaidano:Gilead: Consultancy, Honoraria; Roche: Consultancy, Honoraria; Amgen: Consultancy, Honoraria; AbbVie: Consultancy, Honoraria; Janssen: Consultancy, Honoraria. Vannucchi:Shire: Speakers Bureau; Novartis: Honoraria, Speakers Bureau.

The Smile Sign

The neurologist's diagnostic process is often held in reverence among academic clinicians. We neurologists, it is often said, “understand the brain” and identify obscure patterns through arcane physical diagnostics. Yet we have had our difficulties speaking plainly when addressing the question of functional neurological disorders. 1 Friedman J.H. Curt LaFrance Jr W. Psychogenic disorders--the need to speak plainly. Arch Neurol. 2010; 67: 753-755 Crossref PubMed Scopus (35) Google Scholar

CSF TO ACE THE DIAGNOSIS

SESSION TITLE: Tuesday Medical Student/Resident Case Report Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: 10/22/2019 01:00 PM - 02:00 PM INTRODUCTION: Sarcoidosis is a granulomatous disorder characterized by noncaseating granulomas affecting involved organs. Although the majority of sarcoidosis is diagnosed as pulmonary sarcoidosis incidentally on chest imaging, extrapulmonary sarcoidosis is the presenting symptom for approximately 30% of all cases, with 5-10% affecting the nervous system. Angiotensin Converting Enzyme can be produced by granulomas, with 55% of cases demonstrating positive ACE levels within cerebrospinal fluid. CASE PRESENTATION: A 28 year old man with no past medical history presented with bilateral lower extremity weakness and numbness, gradually worsening over four weeks, starting in the pelvic area causing urinary urgency and painful defecation with distal spread. Over the few days prior to admission his family had also noticed dysarthria. Physical exam was notable for anisocoria, dysarthria, ataxic gait, diminished lower extremity reflexes, and saddle anesthesia. Serum was unremarkable except a sed rate of 39 and a positive ANA. Lumbar puncture showed an elevated white count of 43 cells/uL, predominantly lymphocytes, and an elevated protein at 336mg/dL. CSF ACE level was elevated (7.0 units/L). An MRI of the brain and spine showed diffuse nodular leptomeningeal enhancement of the entire spinal cord and brain. A CT of chest abdomen and pelvis was obtained which demonstrated innumerable pulmonary nodules and masses in a perilymphatic distribution with upper lobe predominance and subcarinal, pelvic and abdominal lymphadenopathy. Endobronchial biopsies of right mainstem bronchus nodularity and EBUS guided transbronchial needle aspiration of the subcarinal lymph node revealed granulomatous inflammation. He was treated with IV pulsed Solumedrol for 3 days with improvement in his neurologic symptoms. He was discharged on prednisone 1 mg/kg and methotrexate 15 mg weekly. Repeat MRI 5 days post treatment showed improvement of leptomeningeal enhancement. DISCUSSION: In this case, we observed a young man who developed neurologic symptoms as his first sign of sarcoidosis. Neurosarcoidosis can effect any portion of the peripheral or central nervous system. Cranial mononeuropathy, cognitive deficits, radiculopathy, and aseptic meningitis are common manifestations, wherever a granuloma can obstruct a nerve. A helpful diagnostic cue is angiotensin I-converting enzyme within the CSF itself, as granulomas synthesize this enzyme which can diffuse into various fluid compartments. The mainstay of treatment for any neurologic symptom is to treat the underlying sarcoidosis with steroids, as was done on our patient. CONCLUSIONS: In a younger patient with a pulmonary nodules and masses with associated neurologic complaints, neurosarcoidosis is a unique diagnosis that physicians should consider on their differential, while excluding malignancy. Angiotensin Converting Enzyme in CSF can be a helpful diagnostic cue. Reference #1: "Neurosarcoidosis” by Burns, Arch Neurol. 2003 Aug;60(8):1166-8. Reference #2: "Clinical characteristics of patients in a case control study of sarcoidosis” by Baughman, Teirstein, Et Al. Am J Respir Crit Care Med. 2001 Nov 15;164(10 Pt 1):1885-9. Reference #3: "Cerebrospinal fluid angiotensin-converting enzyme for diagnosis of central nervous system sarcoidosis.” By Khoury, Wellik, Et Al. Neurologist. 2009 Mar;15(2):108-11. https://doi.org/10.1097/NRL.0b013e31819bcf84. DISCLOSURES: No relevant relationships by Elena Burke, source=Web Response no disclosure on file for Christine Fleury; No relevant relationships by Kellen Mulhern, source=Web Response

STROKE AND MYASTHENIA GRAVIS LEAD TO CRISIS IN THE ELDERLY

SESSION TITLE: Tuesday Medical Student/Resident Case Report Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: 10/22/2019 01:00 PM - 02:00 PM INTRODUCTION: Myasthenia gravis (MG) is frequently underdiagnosed especially in the elderly population with comorbid illness [1]. Late-onset MG is defined as onset after the age of 50 [2]. MG is especially difficult to identify in patients with recent stroke given overlapping signs and symptoms. We present here a case of late-onset MG preceded by an acute stroke that pursued a rapid downhill course leading to myasthenic crisis and acute respiratory failure. CASE PRESENTATION: The patient was a 75 year old male who presented for inpatient rehabilitation after an acute medullary stroke. During rehab, the patient developed new onset diplopia and bilateral ptosis which was initially thought to be due to progression of his stroke. His symptoms continued to worsen, however, and infection, thyroid dysfunction, electrolyte abnormalities, and stroke progression was ruled out by appropriate laboratory and radiographic studies. The patient was subsequently found to have positive AChR antibodies with a positive pyridostigmine test confirming the diagnosis of MG. A CT thorax was negative for thymoma. Despite aggressive treatment with IVIG and systemic steroids, the patient’s disease progressed to the development of a myasthenic crisis. Even with the initiation of plasmapheresis the patient developed acute hypercapneic respiratory failure necessitating endotracheal intubation and mechanical ventilation. The patient failed to respond to therapy and was ultimately withdrawn from life support at the request of the DPOA. DISCUSSION: We present an unusual scenario of late onset MG confounded by the presence of an acute medullary stroke. Acute stroke and MG rarely coincide in the same patient; in fact only a single case is reported that had both conditions concurrently [3]. To our knowledge, this is the first case report of a rare presentation of combined acute stroke and MG progressing to acute myasthenic crisis. With our aging population, prevalence of late-onset MG is on the rise [2]. Although difficult due to physiological changes that occur with aging, prompt diagnosis is crucial especially in the elderly population. Delayed diagnosis can be catastrophic as seen in our patient with abrupt decline. We postulate that the rapid progression of the patient’s respiratory failure and inability to successfully liberate from mechanical ventilation was due to the simultaneous insults to the medullary respiratory center and respiratory musculature from the two neurologic disorders. CONCLUSIONS: MG should remain in the differential diagnosis especially in elderly patients presenting with sudden onset of ocular symptoms even in post stroke patients. Patients can have rapid progression to myasthenia crisis when the respiratory system is already compromised by medullary stroke. Reference #1: Aarli JA. Myasthenia gravis in the elderly: is it different? Ann N Y Acad Sci 2008;1132:238-43. Reference #2: Aarli JA. Late-onset myasthenia gravis: a changing scene. Arch Neurol. 1999;56:25–27. Reference #3: Kim SJ, Park GY, Choi YM, Sohn DG, Kang SR, Im S. Bulbar Myasthenia Gravis Superimposed in a Medullary Infarction Diagnosed by a Fiberoptic Endoscopic Evaluation of Swallowing With Simultaneous Tensilon Application. Ann Rehabil Med. 2017;41(6):1082-1087. DISCLOSURES: No relevant relationships by Nemer Dabage-Forzoli, source=Web Response No relevant relationships by Sana Khan, source=Web Response No relevant relationships by Nick Meyerkord, source=Web Response No relevant relationships by Charles Schroeder, source=Web Response No relevant relationships by Yunna Sinskey, source=Web Response No relevant relationships by Shashank Varakantam, source=Web Response

062 Alcohol associated toxic myelopathy with normal hepatic function

IntroductionAlcohol related neurological dysfunction affects the central and peripheral nervous system. We present a rare consequence of excessive alcohol consumption. A 42-year-old female consuming 2 bottles of wine daily for the preceding 5 years presented with 12 months of progressive gait disturbance. Examination revealed a spastic paraparesis with prominent dorsal column signs. Extensive work up found no alternate cause for her presentation. The final diagnosis was direct alcohol mediated toxic myelopathy. We reviewed the current literature on this rare condition.MethodsWe searched PubMED/OVID databases with the terms ‘alcohol AND myelopathy’.ResultsThere is a paucity of research on this clinical entity. Sage et al.1 described 5 well-nourished alcoholics presenting with a progressive myelopathy. As with our patient, routine laboratory tests, including nutritional screen, were normal. CT Myelogram revealed no structural cause. CSF was normal. In contrast, a myelopathic syndrome can occur in alcoholic hepatic failure, attributed to portosystemic shunting of blood, with resultant hyper-ammonaemia and demyelination of corticospinal tracts.2–4ConclusionAlcohol associated toxic myelopathy is a rare entity, distinct from hepatic myelopathy. It is thought to result from direct toxic effects of ethanol on corticospinal and large myelinated sensory tracts. The sensory involvement is unique for this entity, which distinguishes it from hepatic myelopathy, where corticospinal involvement predominates. It is prudent to look for other potentially treatable secondary causes of myelopathy. Abstinence from alcohol results in modest improvement in symptoms. Minimal literature exists on this clinical entity and further research is required.ReferencesSage JI, et al. Alcoholic myelopathy without substantial liver disease. A syndrome of progressive dorsal and lateral column dysfunction. Arch Neurol. 1984 Sep;41(9):999–1001.Premkumar M, et al. Hepatic Myelopathy in a Patient with Decompensated Alcoholic Cirrhosis and Portal Colopathy. Volume 2012, Article ID 735906, 4 pages.Koo JE, et al. Hepatic myelopathy as a presenting neurological complication in patients with cirrhosis and spontaneous splenorenal shunt. Korean J Hepatol. 2008 Mar;14(1):89–96.Kori P, et al. Hepatic myelopathy: an unusual neurological complication of chronic liver disease presenting as quadriparesis. BMJ Case Rep. 2013; 2013: bcr2013009078.

Brainstem morphology and movement disorders

The control of movement involves complex neural circuits that span several brain regions. One brain region that is important for movement is a brainstem structure called the substantia nigra. The importance of this brain region is evidenced by the motor signs that accompany Parkinson's disease, a movement disorder characterised by degeneration of neurons in the substantia nigra. Substantia nigra morphology is difficult to assess in humans with standard clinical MRI but it can be readily viewed with transcranial sonography. Use of this technique has shown that the substantia nigra is abnormally bright and enlarged in patients with Parkinson's disease, and the abnormality (hyperechogencity) is present in 78–90% of patients. Because of this, the technique is now being used in some countries as a diagnostic aid for Parkinson's disease. Substantia nigra hyperechogenicity has also been observed in ∼9% of healthy adults. The presence of this abnormality in healthy older adults is a significant risk factor for Parkinson's disease. Healthy older adults with this abnormality are 17 times more likely to be diagnosed with Parkinson's disease over a 3-year period than people without the abnormality (Berg et al 2011 Arch Neurol 68:932-7). Our research team has demonstrated that substantia nigra hyperechogenicity in healthy older adults is associated with altered neuronal circuitry in a functionally connected brain region (motor cortex) and altered fine motor function. The changes share similarities with de novo patients diagnosed with Parkinson's disease. Our results support use of transcranial ultrasound as a diagnostic aid and early disease marker for Parkinson's disease. Our research has also identified a young-adult population that has a high prevalence of substantia nigra hyperechogenicity – individuals that have used the drug methamphetamine or ‘ice’. Methamphetamine is toxic to the same neurons that undergo neurodegeneration in Parkinson's disease. Our research shows that two thirds of young adults that have used methamphetamine, as little as five times, have substantia nigra hyperechogenicity and use of the drug is associated with clinical signs of parkinsonism. This observation is alarming because methamphetamine is a significant health problem in many countries and there are no reports of the substantia nigra abnormality returning to normal in the literature (in any population), despite tens of thousands of people having undergone the procedure world-wide. Our research suggests that substantia nigra hyperechogenicity in healthy adults is associated with changes characteristic of Parkinson's disease and supports its role as a marker of vulnerability for this disorder.

Running-down phenomenon captured with chronic electrocorticography.

SummaryThe running‐down phenomenon refers to 2 analogous but distinct entities that may be seen after epilepsy surgery. The first is clinical, and denotes a progressive diminution in seizures after epilepsy surgery in which the epileptogenic zone could not be completely removed (Modern Problems of Psychopharmacology 1970;4:306, Brain 1996:989). The second is electrographic, and refers to a progressive deactivation of a secondary seizure focus after removal of the primary epileptogenic zone. This progressive decrease in epileptiform activity may represent a reversal of secondary epileptogenesis, where a primary epileptogenic zone is postulated to activate epileptiform discharges at a second site and may become independent.3 The electrographic running‐down phenomenon has been reported in only limited numbers of patients, using serial postoperative routine scalp electroencephalography (EEG) (Arch Neurol 1985;42:318). We present what is, to our knowledge, the most detailed demonstration of the electrographic running‐down phenomenon in humans, made possible by chronic electrocorticography (ECoG). Our patient's left temporal seizure focus overlapped with language areas, limiting the resection to a portion of the epileptogenic zone, followed by implantation of a direct brain‐responsive neurostimulator (RNS System, NeuroPace Inc.) to treat residual epileptogenic tissue. Despite the limited extent of the resection, the patient remains seizure‐free more than 2 years after surgery, with the RNS System recording ECoG without delivering stimulation. We reviewed the chronic recordings with automated spike detection and inspection of electrographic episodes marked by the neurostimulator. These recordings demonstrate progressive diminution in spiking and rhythmic discharges, consistent with an electrographic running‐down phenomenon.

The Association of Schizophrenia and Narcolepsy in Adolescents

As a rare and disabling neurological sleep disorder that is often diagnosed during adolescence, narcolepsy type 1 (NT1) has been described as a relatively homogeneous clinical syndrome of hypothalamic deficiency in hypocretin-1 (orexin) producing neurons. 1 Longstreth W.T. Koepsell T.D. Ton T.G. Hendrickson A.F. Belle G. The epidemiology of narcolepsy. Sleep. 2007; 30: 13-26 Crossref PubMed Scopus (328) Google Scholar , 2 Mignot E. Lammers G.J. Ripley B. et al. The role of cerebrospinal fluid hypocretin measurement in the diagnosis of narcolepsy and other hypersomnias. Arch Neurol. 2002; 59: 1553-1562 Crossref PubMed Scopus (961) Google Scholar As up to 98% of patients are human leukocyte antigen DQB1*06:02 positive, an autoimmune-mediated hypothesis has been supported. 3 Bourgin P. Zeitzer J.M. Mignot E. CSF hypocretin-1 assessment in sleep and neurological disorders. Lancet Neurol. 2008; 7: 649-662 Abstract Full Text Full Text PDF PubMed Scopus (110) Google Scholar The core features include excessive daytime sleepiness (EDS), cataplexy, fragmented nocturnal sleep with vivid dreams, and hallucinations and sleep paralysis during sleep-wake transitions. Commonly, adolescents experience concurrent depression, stigmatization from peers, and their body mass index is higher than 98th percentile. 4 Ohayon M.M. Narcolepsy is complicated by high medical and psychiatric comorbidities: a comparison with the general population. Sleep Med. 2013; 14: 488-492 Abstract Full Text Full Text PDF PubMed Scopus (160) Google Scholar

How mild is the outcome of mild neonatal encephalopathy?

Over 4 decades ago, Sarnat defined stage I, or mild, neonatal encephalopathy following fetal distress as a postanoxic state characterized by a hyperalert level of consciousness with normal muscle tone on exam, no clinical seizures, and a normal EEG (Arch Neurol 1976;33:696-705). In that report of just 21 patients >36 weeks' gestation with fetal distress and investigator-defined stage I mild to stage III severe encephalopathy, those neonates with <5 days in stage II moderate encephalopathy and a predominance of stage I findings were all developmentally normal subsequently at age 12 months. The derivative conclusion was that newborns with mild neonatal encephalopathy are largely developmentally normal months later. Since then, many changes in neonatal care have taken place, including technology to rescue younger and sicker term and preterm babies, use of surfactant for lung immaturity, and implementation of neonatal hypothermia for babies with neonatal encephalopathy, acidosis, and gestational age 34 to 35 weeks. But is mild neonatal encephalopathy truly associated with mildly abnormal or normal outcomes when larger samples of newborns are examined, especially after the application of therapeutic hypothermia for concerns of distress, acidosis, or seizures with decreasing gestational age? In this volume of The Journal, Walsh et al report a limited, single institution, historical cohort study of 89 neonates >34 weeks' gestation with neonatal encephalopathy and pH <7.1, all treated with hypothermia. The investigators sought to determine whether magnetic resonance imaging detected brain abnormalities after hypothermia were any different among those with mild, moderate, or severe neonatal encephalopathy, based upon more modern grading criteria. They studied 48 infants with mild encephalopathy, with 35 moderate, yet just 6 with severe neonatal encephalopathy. Although this team did find in their sample proportionately more acute basal ganglia and thalamic abnormalities in those neonates with severe encephalopathy after therapeutic hypothermia, they could not detect any differences in the rate of overall brain abnormalities demonstrated by brain magnetic resonance imaging after hypothermia. Their finding is provocative but based on an early exploration. Their conclusions derive from a small, retrospective sample with limited power to detect a difference and the potential for a type II error. The findings of Walsh et al are intriguing and should spur further research into both the radiographic and clinical outcomes of mild encephalopathy after therapeutic hypothermia. More comprehensive, long-term data are needed to delineate the neurological and developmental outcome of newborns with mild neonatal encephalopathy, with and without therapeutic hypothermia. Article page 26 ▶ The Frequency and Severity of Magnetic Resonance Imaging Abnormalities in Infants with Mild Neonatal EncephalopathyThe Journal of PediatricsVol. 187PreviewTo assess and contrast the incidence and severity of abnormalities on cerebral magnetic resonance imaging (MRI) between infants with mild, moderate, and severe neonatal encephalopathy who received therapeutic hypothermia. Full-Text PDF

Cognitive Screening Model Expands Health Care Delivery

Cognitive Screening Model Expands Health Care Delivery

Seeing is believing: a demonstration of critical fusion frequency and its multidimensional nature.

critical fusion frequency (CFF), or the flicker fusion threshold, is defined as the critical frequency above which a flickering light source appears steady to the observer (see [Fig. 1][1]). It is known to depend on a number of parameters, namely, intensity of the light source ([5][2], [15][3]),