Left Pons Research Articles

Recurrent Brainstem Lesions as a Manifestation of MOGAD

Objective NA. Background MOG antibody-associated disease (MOGAD) is an inflammatory and demyelinating disease of the central nervous system. Brainstem and cerebellar involvement in MOGAD is relatively common and typically occurs in conjunction with lesions in other parts of the central nervous system such as the optic nerve, supratentorial white matter, or spine. Isolated attacks of the brainstem and cerebellum are rare–especially in a relapsing form. Design/Methods A case report. Results We present the case of a 60 year-old female who developed new onset weakness and incoordination of her left arm and leg. Her brain MRI revealed restricted diffusion and edema involving the left cerebellar peduncle. She was diagnosed with a stroke and discharged home on anti-platelet therapy. One month later, she developed a left facial droop and was diagnosed with a Bell's palsy. She received oral steroids and had improvement of her facial droop, though it returned a few weeks after completion of her oral steroids. A repeat brain MRI at that time revealed enlargement of the left cerebellar lesion with extension into the left pons. She was again treated with oral steroids and her symptoms improved. She presented again 3 months later with dysarthria and pseudobulbar affect with her brain MRI revealing diffuse T2/FLAIR hyperintensities and edema throughout the bilateral pons and cerebellar peduncles. She received 3 days of high-dose IV methylprednisolone with marked improvement. She ultimately had serum MOG antibody testing performed which returned positive (titer of 1:1000). She was initiated on rituximab, and was clinically stable for over 24 months. Conclusions Although brainstem involvement in MOGAD usually occurs in conjunction with other lesions, our case demonstrates that relapsing attacks isolated to the brainstem and cerebellum are within the clinical spectrum of MOGAD.

Spectrum of Brain MRI Features in CASPR2 Associated Autoimmune Encephalitis: A Case Report With Parietal Lobe Involvement

Objective Contactin-associated-protein-like 2 (CASPR2)-antibody-mediated autoimmune encephalitis (AE) is characterized by diverse clinical manifestations with involvement of both central and peripheral nerve systems. Background Furthermore, approximately 50-55% of the patients with this condition present with an abnormal brain MRI including bilateral/unilateral T2-hyperintensity in the mesial temporal lobes, thalami, basal ganglia, brainstem, or cerebellum and hippocampal atrophy/sclerosis. To our knowledge this is the first case of CASPR-Ab-related AE reporting concurrent parieto-occipital lobes enhancing and non-enhancing brain lesions. Design/Methods A 71-year-old male presented with 3-months of cognitive decline, generalized tonic-clonic seizures, and parasomnias. Initial neurological examination showed diminished attention with neuropsychology evaluation revealed episodic memory deficits and executive dysfunction, otherwise no focal abnormality. Results Brain MRI with and without contrast from showed subtle cortical enhancement in the anterior left parietal lobe, additionally T2 FLAIR hyperintensities in the bilateral hippocampi, left centrum semiovale and left dorsal pons. CSF analysis revealed 5 nucleated cells, protein and glucose were normal. Serum and CSF paraneoplastic panel was positive for CASPR-2 auto antibodies. His memory and executive function improved after plasma exchange and long-term steroids. One year later the patient developed ataxia and gait instability. Repeated examination revealed diminished vibration in bilateral lower extremities, prominent left upper extremity ataxia compared to the right and a wide based gait. Updated brain MRI demonstrated progression of T2 FLAIR hyperintensities in the cortical and subcortical areas of the left parieto-occipital fissure. IVIG was started with stabilization of the progression. Conclusions CASPR2-autoantibody is associated to a wide range of neurological manifestations and diagnosing this condition could be challenging. This case demonstrates the possible involvement of the parietal and occipital cortex as a target of the antibodies directed against this membrane protein. Recognition of this spectrum of symptoms and imaging findings are important for early diagnosis of this disorder and appropriate treatment.

LEFT ATRIAL SEPTAL POUCH IN A PATIENT WITH RECURRENT STROKE

LEFT ATRIAL SEPTAL POUCH IN A PATIENT WITH RECURRENT STROKE

P26 Challenging Scenario: Scleroderma/Sjogren overlap with dysautonomia

Introduction/BackgroundScleroderma Overlap conditions are well defined and often behave differently than their limited or diffuse counterparts. The treatment for such patients is usually tailored around the organ system involvement and severity. We present a challenging case of Scleroderma/Sjogren overlap with refractory Raynaud's phenomenon who posed treatment challenges due to severe autonomic dysfunction.Description/Method76-year-old Caucasian lady, non-smoker, who presented in 1990’s with dry eyes, dry mouth and severe Raynaud's phenomenon with recurrent digital ulceration which became infected on multiple occasions. ANA was 1:400 with Extractable nuclear antigen Ro and La positive. Anti DsDNA was negative and C4 was low. Antiphospholipid antibodies and cryoglobulins are negative. She failed calcium channels blockers and phosphodiesterase 5 inhibitors and was intolerant to intravenous (IV) Iloprost which was tried on multiple occasions. IV latanoprost was tried as well. This treatment was complicated when she developed postural hypotension and multiple fall episodes during vasodilator use. This was attributed to autonomic dysfunction secondary to her Sjogren syndrome. She was initiated on Injection Fludrocortisone, however her hypotensive episodes continued. She received monthly intravenous immunoglobulins 20gm (over two days) infusion for a few months for autonomic dysfunction with some response. She eventually developed recurrent diarrhoea and abdominal pain and bloating which was attributed to small bowel overgrowth with rapid bowel transit on a gastric emptying scan. She had a blocked bile duct as well which was unblocked using Endoscopic Retrograde Cholangiopancreatography. Over the next 8 years, she continued struggling with refractory Raynaud’s, having failed IV regional guanethidine blocks and digital sympathectomy. Her postural hypotension decompensated on multiple occasions necessitating plasmapheresis which provided some benefit. In 2020, she was detected to have a PET avid slowly progressing left upper lobe lung lesion. She underwent left video assisted thoracoscopy surgery with upper lobectomy. Histology was suggestive of adenocarcinoma. In 2021, she had another fall episode due to an acute/subacute infarct of left pons. She continues having postural hypotension and requires regular plasmapheresis sessions to manage her symptoms. She is also presently on Bosentan for her raynaud’s phenomenon.Discussion/ResultsThis is a case of overlap of Sjogren syndrome with scleroderma features. Management of Refractory Raynaud’s as part of her scleroderma phenotype was challenging. This case gave us the opportunity to explore all the treatment options available for Raynaud’s phenomenon albeit with poor outcome. She also struggled with autonomic dysfunction leading to postural hypotension and gastrointestinal symptoms which can be seen in upto 50% of patients with Sjogren syndrome. Autonomic failure is often thought to be immune mediated in Sjogren syndrome. This makes way for the use of intravenous immunoglobulin and plasmapheresis to treat these conditions as was done in this case. Increased risk of lung carcinoma with scleroderma is well documented. Autonomic dysfunction can also present as paraneoplastic syndrome with lung carcinoma, however it is more common with small cell lung carcinoma and our patient was diagnosed with Adenocarcinoma. We would have to wait and see if the resection of the tumour has any demonstrable effect on her dysautonomia.Key learning points/ConclusionOverlap connective tissue diseases are often difficult to manage in view of extensive disease heterogeneity. Clinicians need to be aware of challenges in managing Difficult Raynaud’s with Autonomic dysfunction. Treating physicians need to be aware of the high risk of lung malignancy and regular close monitoring is required. This case-based conference gives us the opportunity to discuss further management approaches for her.

DIPG-33. Treating Pediatric Diffuse Intrinsic Pontine Glioma with Brain Radiation and Temozolomide: A Case Report

Abstract Diffuse Intrinsic Pontine Gliomas (DIPG) is a rare and highly aggressive central nervous system tumour arising from glial cells which occur mostly in children. It remains a potentially deadly cancer despite treatment, with the highest mortality rate, in which most children die within 1-year of diagnosis. Radiation therapy might improve the clinical symptoms, but the results are temporary, with tumor progression typically occurring months post radiation, whereas chemotherapy remains controversial. Here we present a case report of a patient with DIPG Indonesia and how we diagnose and currently treat it. An eight years-old boy presented with decrease level of consciousness with headache, limb weakness, slurred speech, double vision, hemifacial weakness, paraplegia, and disequilibrium which worsened two weeks prior to admission. He was initially noted to have a medially inverted left eye two months prior to admission. On physical examination, there was a N.VI and N.VII palsy, hyperreflexia, paraplegia, and nystagmus. Head MRI demonstrated a solid with minimal enhancement after contrast administration in the left pons region extending into mesencephalon with cystic component and flat floor of fourth ventricle sign, causing hydrocephalus, suggestive diffuse intrinsic pontine glioma. He was given fractionated focal intensity modulated radiation therapy (IMRT) to the tumor along with 5 mm margins with a total dose of 54 Gy. 1.8 Gy fractions, given once daily for 5 days per week over a period of 6 weeks. Supportive care in the form of corticosteroids is used to treat the peri-tumoral edema. There was a short period of motoric improvement. But then the disease progressed with the latest head CT showed hydrocephalus formation with increased size of mass. He is treated with chemotherapy using Temozolomide, and currently alive after 3 months post diagnosis. Keywords: childhood brain tumour, Diffuse Intrinsic Pontine Glioma, radiation therapy

AB1095 POSTERIOR REVERSIBLE ENCEPHALOPATHY SYNDROME IN A PATIENT WITH SYSTEMIC LUPUS ERYTHEMATOSUS AFTER COVID-19 VACCINATION: A CASE REPORT

BackgroundThe COVID-19 pandemic has infected millions of people around the world and there has been a new surge of virulent strains in many parts of the world[2]. Patients with Systemic Lupus Erythematosus (SLE) were reported to be at higher risk of SARS-CoV-2 infection and worse outcomes from COVID-19, possibly due to their intrinsic immune dysfunction, demographics, disease activity, medications, associated organ damage, comorbidities and as such, have been among the first to receive the vaccines [3]. The most common reason for vaccine refusal in patients with SLE is fear of SLE disease flare. Additionally, SARS-CoV-2 mRNA vaccines could potentially induce interferon production, associated with increased SLE disease activity[1].Objectiveswe report a case of SLE presented with lupus flare after receiving the 1st dose of phizer vaccine.MethodsA 30-year-old female patient, kown case of SLE since 2011 well controlled on low dose steroids, hydroxychloroquine and azathioprine. Upon receiving her 1st shot of Pfizer-BioNTech COVID-19 Vaccine, she developed high grade fever associated with generalized tender papulovesicular skin eruption mainly on the back of the trunk and the outer surface of both thighs, then she developed generalized tonic-clonic convulsions and transferred for Intensive Care Unit (ICU), intubated, mechanically ventilated and received intravenous anti-epileptic medications. During her admission, Cerebrospinal fluid (CSF) examination and Magnetic Resonance Imaging (MRI) brain were done.she regained her consciousness, extubated after 48 hours.ResultsThe initial laboratory invwstigations revealed COVID19-PCR: negative,ESR: 35 mm/hr,CRP: 78,C3: 70 mg/dL (90 – 180) and C4: 8 mg/dL (10 – 40).CSF examination revealed proteins: 116.9 mg/dL (15 – 45),glucose: 46.3 mg/dL (50-60% of serum),LDH: 49.1 U/L (10% of serum) and no cells.Emergency MRI brain was performed revealed multiple bilateral symmetrical mainly cortical and subcortical abnormal signal with cortical swelling are seen mainly involving both occipito-temporo-parietal lobes with patchy enhancement of left cerebellar hemisphere, cerebellar vermis, both thalami, medulla and pons,Picture suggestive of Posterior Reversible Encephalopathy Syndrome (PRES).Accordingly the patient received received pulse steroid therapy for 3 days under cover of oral acyclovir.She also received levetiracetam and Oxcarbazepine.the condition markedly improved and discharged from the hospital for follow up after one month.Conclusion1)The mRNA COVID Vaccine may rarely cause CNS affection, or even SLE flare so, SLE patients must be well controlled before giving the Vaccine. 2) SLE patients must be monitored closely by clinical examination and laboratory investigations after taking mRNA COVID Vaccine.

Lacunar Infarction In Old Stroke: A Case Report

Introduction: Lacunar stroke accounts for nearly a quarter of all ischemic strokes and is an important cause of vascular cognitive impairment and dementia. Case Presentation: A 52-year-old man with lacunar infarct presented with weakness in his left thumb and forefinger since one day ago when he was sitting down. The patient’s fingers appear bent as if holding a pencil and could not be straightened. Physical examination showed an high blood pressure, and paresis of the right facial and hypoglossal nerve. A non-contrast CT scan of head using the parenchymal window showed a hypodense lesion on the left pons, interpreted as a lacunar infarct. Discussion: Lacunar strokes represent approximately 25% of ischemic strokes and most are associated with good outcomes. However, in 20% to 30% of patients with lacunar stroke, early neurologic damage occurs within the first days after stroke onset. Lacunar stroke was defined as acute lacunar syndrome with infarction <15 mm in diameter in the basal ganglia, deep white matter, thalamus or pons that is not associated with large vessel atherosclerosis or a cardioembolic source. Conclusion: Lacunar infarction is a potentially reversible. These conditions can potentially be well managed with adequate treatment.

Double Trouble: Biventricular Thrombi And Embolic Complications

<h3>Case Summary</h3> A 49 year old with peripheral vascular disease presented to his vascular surgeon with leg pain and it was found cold and pulseless. He was diagnosed with critical limb ischemia and was directly admitted to the vascular surgery. CT found a femoral artery occlusion, cardiomegaly and bi-ventricular clots. Cardiology was consulted and a TTE noted bi-ventricular thrombi and a severely dilated LV with EF=20%. Palliative care was consulted. He was taken to the OR for BKA. Post-operatively he had a PEA arrest. He was in shock and given inotropes. Invasive hemodynamics, mechanical support, and coronary angiography were limited by renal failure, vascular access and thrombi. His shock improved, but he was not a candidate for advanced therapies. Coronary angiography showed multi-vessel disease. Cardiac MR showed viability in most regions but while awaiting revascularization, he became unresponsive. CT showed R ICA occlusion, R MCA occlusion, L M1 occlusion, and R watershed infarct. He was taken for mechanical thrombectomy. MRI showed multifocal infarcts in the L MCA, left pons, and left PCA. Fluids were trialed to improve perfusion, but he became hypoxic and repeat MRI did not show any improvement. He was unresponsive. The family enrolled the patient in hospice. <h3>Discussion</h3> Our patient had no history of HFrEF. While he had risk factors, symptoms were masked by severe peripheral vascular disease. Bi-ventricular thrombi are uncommon and place the patient at risk for systemic and pulmonary embolization. Aside from anticoagulation, there is little consensus on the management of intracardiac thrombi and the risk varies significantly within published data. In severe HF, complications and inability to tolerate GDMT should warrant advanced HF consultation. Early involvement of palliative care helped guide family during end of life care. More research is needed to better inform management options in heart failure complicated by intracardiac thrombi.

Demyelinating Neurological Adverse Events following the Use of Anti-TNF-α Agents: A Double-Edged Sword

Background Tumor necrosis factor antagonists (anti-TNF-α) are an established therapeutic option for several autoimmune and inflammatory bowel diseases. Despite their clinical effectiveness, neurological adverse events have been reported, and literature data suggest a potential role of anti-TNF-α in the induction of demyelination. Case Presentation. In this series, we present three cases of demyelination after the use of anti-TNF-α agents. The first case involved a 21-year-old man with HLA-B27 negative peripheral spondylarthritis who had been taking adalimumab for 2 years. He developed headache, urinary incontinence, and bilateral lower extremity numbness that progressed to the middle of the trunk for 2 days. Magnetic resonance imaging (MRI) showed multiple hyperintense enhancement lesions in the left paramedian anterior pons consistent with multiple sclerosis (MS). The second case included a 17-year-old woman who was on 2 years of adalimumab treatment for juvenile idiopathic arthritis and chronic anterior uveitis and developed new-onset dizziness and tremors. The clinical examination showed signs of cerebellar dysfunction. MRI findings were consistent with multiple sclerosis. The third case was a 34-year-old male who was on 5 years of infliximab treatment for ankylosing spondylitis when he developed left hand numbness and weakness. Cerebrospinal fluid (CSF) analysis and MRI findings were consistent with demyelination. Discontinuation of tumor necrosis factor antagonists (anti-TNF-α) resulted in resolution of the symptoms with no recurrence in the first case, but there was evidence of recurrence in the other 2 cases, where one was managed with rituximab and the second one improved with pulse steroid therapy. Conclusion Despite the small number of patients, our series adds to the growing body of evidence supporting a causal link between anti-TNF-α agents and demyelination. Thus, we can conclude that on suspicion of any neurological side effects, early discontinuation of the TNF-α blockers and requesting urgent MRI scan to confirm the diagnosis is of utmost importance.

A CASE OF FOVILLE SYNDROME

60 year old diabetic and hypertensive female presented to casualty with right hemiplegia and hemianesthesia with lower motor neuron type of facial nerve palsy and loss of pain, temperature on left side of the face. She had difculty adduction and abduction on left eye with horizontal nystagmus in the contralateral eye when abducted while vertical movements were preserved. MRI brain was done which showed acute focal non hemorrhagic infarct in the left hemi pons along with partial basilar artery thrombosis. Hence a diagnosis of foville syndrome with inter nuclear opthalmoplegia was made. Classically foville syndrome is characterised by ipsilateral sixth nerve palsy, lower motor neuron facial nerve palsy, fth sensory nuclear palsy along with contralateral hemiparesis and hemisensory loss. The patient was started on single antiplatelet along with high intensity statin. On follow up after 3 months patients power improved.

Vertebrobasilar artery dolichoectasia manifesting as Millard-Gubler syndrome in a young acute ischemic stroke

Introduction: Vertebrobasilar dolichoectasia (VBD) is an arterial disease characterized by dilatation, elongation, and tortuosity of the vertebral and basilar arteries. VBD can generate intracranial hemorrhage, compression of the brainstem, and ischemic stroke as the most common presentation. Millard-Gubler syndrome (MGS) is a pontine-crossed syndrome affecting the pons at the level of the facial nerve nucleus. We present an unusual case of posterior circulation ischemic stroke presenting as MGS due to VBD. Observation: A 50-year-old man, with a medical history of high blood pressure, was hospitalized after 20 hours of transient dizziness, left facial numbness, and diplopia. On examination, he had a right hemiparesis with right Babinski. Deep tendon reflexes were present. Cranial nerves examination showed left abducens paresis with left peripheral facial nerve palsy. Cranial computed tomography (CT) imaging showed left pontine infarct with dilated basilar artery. Brain MRI showed a tortuous and dilated vertebrobasilar system touching the left acoustico-facial complex nerves with a high signal intensity involving the left anterolateral pons with a small right cerebellar hemorrhage. Paraclinical tests showed high blood total cholesterol and bilateral carotid artery atherosclerosis without stenosis. The patient received aspirin, atorvastatin with anti-hypertensive agents. Two weeks after his admission, there was a resolution of the left abducens nerve paresis with mild residual left peripheral facial paresis and left hemiparesis. Discussion: VBD is a complex arteriopathy with unknown etiology. Clinical symptoms in patients with VBD are various including brainstem and cranial nerve compression, intracranial hemorrhage, and ischemic stroke. MGS is an uncommon pontine-crossed syndrome occurring due to a lesion in the pons at the level of the facial nerve nucleus. MGS can be caused by brainstem tumor, cavernous angioma, neurocysticercosis, and cerebral infarction. The coexistence of MGS and VBD is rarer. Conclusion: Our case provides evidence that VBD may be presented as MGS secondary to a posterior circulation ischemic stroke. Therapeutic management of patients with VBD requires a careful balancing of the benefits and risks.

ML-7 Liquid biopsy for MYD88 mutation in cerebrospinal fluid in patients with suspected primary CNS lymphoma

Background: Treatment intervention for central nervous system lymphoma (CNSL) requires pathological diagnosis by surgical biopsy. However, there are some cases in which the risk of surgery is high due to age, comorbidities, localization of lesions, etc. We are developing a CNSL diagnostic method based on the detection of MYD88 L265P mutation by digital PCR (dPCR) using CSF-DNA, and a high accuracy with a sensitivity of 92.9% and a specificity of 100% has been reported. Here, we report two cases with suspected brain stem CNSL, whose treatment strategy was determined by integrated clinico-laboratory information including neurological presentations, imaging, and the result of liquid biopsy. Result: Case 1. A 63-year-old woman visited our hospital with a complaint of right hemiplegia, which deteriorated in two months. MR images revealed a contrast-enhancing lesion in the left midbrain-ventral pons, suggesting CNSL. Biopsy was not considered because of its location, while dPCR using CSF-DNA showed a cluster of MYD88 mutation signals. Based on these work-ups, she was treated with high-dose methotrexate-based chemotherapy, resulting in a complete response with marked improvement of symptoms. Case 2. An 83-year-old man was referred for a history of diplopia and ataxic gait lasting for a month. MR images revealed an invasive lesion on his right midbrain-dorsal pons. Biopsy was declined due to the location, and liquid biopsy using CSF-DNA was performed to assist the diagnosis. In the first test, the CSF-DNA yield was too insufficient to determine the mutation signal by dPCR. The second dPCR using sufficient amount of CSF-DNA resulted in the Target/Total value of 0.049% which was lower than the threshold, suggesting the absence of MYD88 mutation. The patient underwent radiation therapy accordingly.Conclusions: CSF MYD88 mutation analysis by dPCR may have clinical utility and requires sufficient amount of CSF-DNA for exclusion of noise signals.

Toxocariasis affecting brain stem and skull base

BackgroundToxocariasis is a helminthic infection caused by a nematode that mainly affects populations in tropical and subtropical latitudes. Humans are potential paratenic hosts, and clinical disease occurs as a result of parasite migration through intestinal tissue. We present a clinical case of otorhinolaryngological affectation by Toxocara canis.Case presentationA 60-year-old male from Ecuador, resident in Spain for 5 years, evaluated in the emergency department for presenting headache, otorrhea and left ear pain. Computed tomography (CT) and magnetic resonance imaging (MRI) reported a large mass of the nasopharynx with infiltration of the skull base, intracranial extension and a lesion in the left pons without being able to exclude metastases. Two Functional Endoscopic Sinus Surgery (FESS) biopsies were negative for malignancy. Despite not meeting the diagnostic criteria established by the existing literature, the clinical and radiological presentation, the presence of risk factors, a positive serology for Toxocara canis (IgGELISA) and the absence of alternative diagnosis were considered sufficient criteria to establish toxocariasis with inflammatory lesions in the nasopharynx and pons as the most probable diagnosis. Treatment with albendazole (400 mg / 12 h) and corticosteroids (1 mg / kg for 5 days) was started and continued for one month. Post treatment negative serology, and MRI and CT post treatment controls were performed after one year, both showing a decrease in lesion of the clivus as well as the pons.ConclusionsWith the appropriate personal history, toxocariasis should be included in the differential diagnosis ofinfiltrating lesions of the skull base with a negative study of tumor histology. Albendazole treatment has been shown tocontrol and cure the disease.

Migraine in Multiple Sclerosis Patients Affects Functional Connectivity of the Brain Circuitry Involved in Pain Processing.

Migraine is particularly common in patients with multiple sclerosis (MS) and has been linked to the dysfunction of the brain circuitry modulating the peripheral nociceptive stimuli. Using MRI, we explored whether changes in the resting state-functional connectivity (RS-FC) may characterize the occurrence of migraine in patients with MS. The RS-FC characteristics in concerned brain regions were explored in 20 MS patients with migraine (MS+M) during the interictal phase, and compared with 19 MS patients without migraine (MS-M), which served as a control group. Functional differences were correlated to the frequency and severity of previous migraine attacks, and with the resulting impact on daily activities. In MS+M, the loss of periaqueductal gray matter (PAG) positive connectivity with the default mode network and the left posterior cranial pons was associated with an increase of migraine attacks frequency. In contrast, the loss of PAG negative connectivity with sensorimotor and visual network was linked to migraine symptom severity and related daily activities impact. Finally, a PAG negative connection was established with the prefrontal executive control network. Migraine in MS+M patients and its impact on daily activities, underlies RS-FC rearrangements between brain regions involved in pain perception and modulation.

A patient with probable neuro‐Behçet's disease presenting a unique linear pontine trigeminal root lesion and a linear subcortical white matter lesion

AbstractBackgroundNeuro‐Behçet's disease (NBD) is typically characterized by inflammatory lesions with vasculitis in the brainstem and basal ganglia. Subcortical white matter lesions and linear lesions along the intramedullary trigeminal nerve are rare, and these lesions make NBD difficult to differentiate from other demyelinating diseases, such as multiple sclerosis.Case presentationA 48‐year‐old woman with an 11‐year history of intestinal Behçet's disease was referred to Department of Neurology, Tohoku Medical and Pharmaceutical University Hospital, Sendai, Japan owing to numbness on the left side of her face and oral cavity for 12 days. Neurological examination revealed left facial hypoesthesia. Brain magnetic resonance imaging showed a linearly shaped T2/fluid‐attenuated inversion recovery hyperintense magnetic resonance imaging lesion in the left pons, which corresponded neuroanatomically with the intramedullary trigeminal root. Based on the presumptive diagnosis of NBD, a course of high‐dose intravenous methylprednisolone was administered on the 14th hospital day. Thereafter, the patient's neurological symptoms improved. However, on the 18th hospital day, the patient developed a linear subcortical white matter lesion with vasogenic edema in the right hemisphere that resolved after the re‐administration of high‐dose intravenous methylprednisolone.ConclusionThe case of a patient with NBD presenting with atypical brain lesions is reported. The NBD in this case might have a pathogenetic mechanism that is different from that of typical NBD.

840 Obstructive to Central: A switch in sleep apnea type after arteriovenous malformation rupture

Abstract Introduction Sleep apnea and stroke have long been shown to be linked, with sleep apnea increasing the risk for stroke and stroke leading to sleep apnea. When the latter occurs, it can present as central sleep apnea (CSA), often in the form of Cheyne-Stokes breathing (CSB), and has been shown to resolve over time. We present a patient with persistent CSA after severe hemorrhagic stroke secondary to rupture of a temporal/thalamic arteriovenous malformation (AVM). Report of case(s) A 33-year-old man with a history of obstructive sleep apnea (OSA) presented to our clinic for re-evaluation of his disease. He was diagnosed with OSA in 2006 at which time he was 270 pounds with a body mass index (BMI) of 36.7, thus the OSA was thought to be secondary to obesity. When he presented to our clinic 10 years later, he had lost approximately 80 pounds after suffering multiple strokes. In 2014, he had a left temporal lobe hemorrhage due to rupture of a left temporal/thalamic AVM and required decompressive hemicraniectomy. In 2015, he had a re-bleed of this AVM, with new hemorrhage extending inferiorly into the left cerebral peduncle and pons, and superiorly into the left parietal periventricular white matter anteriorly along the optic tract. Ultimately, he was treated with stereotactic radiotherapy to the AVM nidus with no residual AVM. However, he has chronic encephalomalacia of the left basal ganglia, thalamus, temporal, parietal, and occipital lobes with extension into the left cerebral peduncle and changes consistent with radiation necrosis. His residual symptoms are aphasia and right-sided hemiplegia and although his snoring resolved with weight loss, his mother noticed pauses in his breathing overnight. A repeat sleep study done in 2016 showed 27 central apneas and 0 obstructive apneas with an AHI of 5.4 events/hour. He was subsequently studied on ASV with residual AHI of 0.4 events/hour. Conclusion Although patients with OSA may be at higher risk for stroke, it is important to re-evaluate their sleep apnea after such an event to ensure appropriate diagnosis and treatment going forward. Support (if any):

Structural and Functional Changes in the Cerebellum and Brainstem in Patients with Benign Paroxysmal Positional Vertigo.

Benign paroxysmal positional vertigo (BPPV) is one of the most common peripheral vestibular diseases. Since the peripheral vestibular system connects with the cerebellum via the brainstem, repeated episodic vertigo may result in progressive structural and functional changes in the cerebellum and brainstem. In the present work, voxel-based morphometry (VBM) of T1-weighted images and resting-state functional magnetic resonance imaging (fMRI) in 32 patients with BPPV and 32 matched healthy controls were used to assess cerebellar and brainstem anatomical and spontaneous resting-state brain activity alterations associated with BPPV. We used a spatially unbiased infratentorial template toolbox in combination with VBM to analyze cerebellar and brainstem gray matter volume (GMV), fractional amplitude of low-frequency fluctuations (fALFF), and regional homogeneity (ReHo). Patients with BPPV showed decreased GMV in the right cerebellum posterior lobe/cerebellar tonsil extending to the cerebellum anterior lobe and pons relative to healthy controls. BPPV patients also exhibited significantly higher fALFF values in the right pons and left pons and higher ReHo values in the left cerebellum posterior lobe/Crus2 than the controls. Furthermore, the fALFF z-scores in the pons were positively correlated with the duration of vertigo at baseline and dizziness visual analog scale scores 1 week after canalith repositioning procedures (CRPs). BPPV patients exhibited structural and functional changes in the cerebellum and pons, which may reflect the adaptation and plasticity of these anatomical structures after repeated attacks of episodic vertigo. These results indicate that the changes in pons function may be closely related to residual dizziness after CRPs.

Longitudinal change in fine motor skills after brain radiotherapy and in vivo imaging biomarkers associated with decline.

We explored fine motor skills (FMS) before and after brain radiotherapy (RT), analyzing associations between longitudinal FMS and imaging biomarkers of cortical and white matter (WM) integrity in motor regions of interest (ROIs). On a prospective trial, 52 primary brain tumor patients receiving fractionated brain RT underwent volumetric brain MRI, diffusion tensor imaging, and FMS assessments (Delis-Kaplan Executive Function System Trail Making Test Motor Speed [DKEFS-MS], Grooved Pegboard Dominant Hands [PDH], and Grooved Pegboard Nondominant Hands [PNDH]) at baseline and 3-, 6-, and 12-month post-RT. Motor ROIs autosegmented included: sensorimotor cortices and superficial WM, corticospinal tracts, cerebellar cortices and WM, and basal ganglia. Volume (cc) was measured in all ROIs at each timepoint. Diffusion biomarkers (FA [fractional anisotropy] and MD [mean diffusivity]) were additionally measured in WM ROIs. Linear mixed-effects models assessed biomarkers as predictors of FMS scores. P values were corrected for multiple comparisons. Higher RT dose was associated with right paracentral cortical thinning (β = -2.42 Gy/(month × mm), P = .03) and higher right precentral WM MD (β = 0.69 Gy/(month × µm2/ms), P = .04). Higher left (β = 38.7 points/(month × µm2/ms), P = .004) and right (β = 42.4 points/(month × µm2/ms), P = .01) cerebellar WM MD, left precentral cortical atrophy (β = -8.67 points/(month × mm), P = .02), and reduced right cerebral peduncle FA (β = -0.50 points/month, P = .01) were associated with worse DKEFS-MS performance. Left precentral cortex thinning was associated with worse PDH scores (β = -17.3 points/(month × mm), P = .02). Left (β = -0.87 points/(month × cm3), P = .001) and right (β = -0.64 points/(month × cm3), P = .02) cerebellar cortex, left pons (β = -19.8 points/(month × cm3), P = .02), and right pallidum (β = -10.8 points/(month × cm3), P = .02) atrophy and reduced right internal capsule FA (β = -1.02 points/month, P = .03) were associated with worse PNDH performance. Biomarkers of microstructural injury in motor-associated brain regions were associated with worse FMS. Dose avoidance in these areas may preserve FMS.

Torsipulsion, a useful diagnostic sign in lateral medullary infarction

A number of ocular motility disturbances such as skew deviation, saccadic/smooth pursuit abnormalities, Horner's syndrome, and nystagmus have been reported in lateral medullary infarction. We report 3 patients who had torsipulsion as the main ocular movement abnormality directly attributable to lateral medullary infarction. Patients 1 and 2 had a clockwise torsional movement that was diagnosed on slit lamp. The movement was elicited on asking the patients to make a horizontal saccade. Both patients were noted to have dorsolateral medullary infarction on MRI of the brain. Neither patient had symptoms directly attributable to torsipulsion. Patient No. 3 was referred for non-resolving left 6th nerve palsy. He had infarcts in the left pons, cerebellum and dorsolateral medulla. Torsipulsion was the only ocular motility disorder caused by medullary infarction. Different pathways in the dorsolateral medulla have been implicated in the various ocular motility and neurological abnormalities seen in lateral medullary syndrome. It is possible that sparing of some and involvement of others could result in varied clinical presentations reported in literature. It is important for the ophthalmologist to be aware of torsipulsion as a diagnostic sign of lateral medullary infarction.

Stroke presenting as a rare complication of neurosarcoidosis in a young adult male: A case report

A 29-year-old Filipino-Chinese man with pathologically diagnosed neurosarcoidosis experienced right-sided weakness and slurring of speech. Neuroimaging showed a subacute infarction at the left paramedian pons. Pharmacotherapy included steroids, immunosuppressants, and anticoagulants. Three-month follow-up showed no progression of focal neurologic deficits. Stroke is rare in neurosarcoidosis and typically relates to granulomatous inflammation with a predilection for the perforator arteries. Neurosarcoidosis generally responds well to corticosteroids; however, combination therapy with additional immunosuppression is recommended to reduce the risk of relapse on steroid weaning. This report aims to highlight the occurrence of stroke as a rare complication of an equally rare disease entity in neurosarcoidosis.