Focal Weakness Research Articles

EPEN-05. SURGERY FOR CHILDHOOD EPENDYMOMA: THE EPENDYMOMA MULTIDISCIPLINARY ADVISORY GROUP (EMAG) CLINICAL TRIAL EXPERIENCE

Abstract INTRODUCTION The National Ependymoma Multidisciplinary Advisory Group (EMAG) is the central review mechanism for the SIOP Ependymoma II clinical trial in the UK. EMAG is a multiprofessional group which meets weekly to support decision making. We report detailed surgical outcomes for children with confirmed intracranial ependymoma, discussed through EMAG between January 2020 and November 2022. METHODS Data was collected on resection status, surgical outcomes, reasons for not achieving Gross Total Resection (GTR), number of surgeries and adjuvant therapies. Data was analysed descriptively. RESULTS After first surgery, GTR or near total resection (NTR) rate was 69/105 (66%) rising to 83/105 (79%) following repeated surgeries. 17 patients underwent second-look surgery prior to further therapy, whilst 9 underwent second-look surgery after chemotherapy but before radiotherapy. Eight (17%) posterior fossa (PF) surgeries were not completed due to factors including patient instability and tumour attachments. Most post-operative problems were seen in PF tumours with 26/60 (43%) bulbar palsy, 13/60 (22%) focal weakness and 12/60 (20%) cerebellar mutism. Incomplete PF resections were associated with increased bulbar palsy risk (p=0.02, Chi-Square test). 73% of PF patients required hydrocephalus management with external ventricular drainage, endoscopic third ventriculostomy (ETV) or shunt. Longer term hydrocephalus management with ETV or shunt was required in 29% and was more common in those with subtotal resection (R3-RX, 8/16, 50%) compared to GTR or NTR (R0-R2, 9/43, 21%, p=0.03). 27/60 (45%) received post-op NG feeding and 2/60 (3%) needed tracheostomies. Second surgery was associated with lower complication rates. DISCUSSION Our National MDT approach supports collaborative surgical decision making without a need for centralisation of the surgery itself. There was no evidence of increased complication rates compared to historical trials despite a more aggressive surgical approach. We argue for continued centralisation of EPN evaluation to support optimal care for children with this devastating disease.

Focal motor weakness and recovery following chronic subdural hematoma evacuation.

The incidence of chronic subdural hematomas (cSDHs) is expected to climb precipitously in the coming decades because of the aging populous. Neurological weakness is one of the most common presenting neurological symptoms of cSDH. Yet, the recovery rates of motor strength recovery are seldom documented, as neurological outcomes have predominantly focused on broader functional assessment scores or mortality. In this study, the authors performed one of the first detailed analyses on functional motor weakness and recovery in patients who underwent cSDH evacuation. Patients who underwent evacuation of a cSDH at a tertiary academic medical center between November 2013 and December 2021 were retrospectively identified using ICD-9 and ICD-10 billing codes. The presence of focal motor weakness was subcategorized by location as upper extremity (UE) or lower extremity (LE). Postoperative improvement, worsening, or resolution of weakness was recorded at the time of discharge. Statistical analysis included univariate and backward stepwise multivariable logistic regression modeling. A total of 311 patients were included in the analysis. Patients were significantly more likely to experience UE weakness than LE weakness (29% vs 18%, p < 0.001). Forty-one percent (43/104) had both UE and LE weakness present. Risk factors for the development of focal motor weakness at the time of presentation were older age (OR 1.02, p = 0.03), increased cSDH size (OR 1.04, p = 0.02), and the presence of a unilateral cSDH (OR 2.32, p = 0.008). The majority of patients (68%, 71/104) experienced motor strength improvement following cSDH evacuation, with 58% (60/104) having complete resolution of weakness. Multivariable logistic regression analysis revealed that longer symptom duration was associated with lower rates of improvement (OR 0.96, p = 0.024). Older age was also associated with reduced resolution of weakness (OR 0.96, p = 0.02). This study represents one of the first in-depth analyses investigating the rates of motor strength weakness and recovery following cSDH evacuation. Nearly two-thirds of all patients had complete resolution of their weakness by the time of discharge, and more than three-quarters had partial improvement. Risk factors for impaired neurological recovery were longer symptom duration prior to treatment and older age.

Motor band sign is specific for amyotrophic lateral sclerosis and corresponds to motor symptoms.

Magnetic resonance imaging can detect neurodegenerative iron accumulation in the motor cortex, called the motor band sign. This study aims to evaluate its sensitivity/specificity and correlations to symptomatology, biomarkers, and clinical outcome in amyotrophic lateral sclerosis. This prospective study consecutively enrolled 114 persons with amyotrophic lateral sclerosis and 79 mimics referred to Karolinska University Hospital, and also 31 healthy controls. All underwent 3-Tesla brain susceptibility-weighted imaging. Three raters independently assessed motor cortex susceptibility with total and regional motor band scores. Survival was evaluated at a median of 34.2 months after the imaging. The motor band sign identified amyotrophic lateral sclerosis with a sensitivity of 59.6% and a specificity of 91.1% versus mimics and 96.8% versus controls. Higher motor band scores were more common with genetic risk factors (p = 0.032), especially with C9orf72 mutation, and were associated with higher neurofilament light levels (std. β 0.22, p = 0.019). Regional scores correlated strongly with focal symptoms (medial region vs. gross motor dysfunction, std. β -0.64, p = 0.001; intermediate region vs. fine motor dysfunction, std. β -0.51, p = 0.031; lateral region vs. bulbar symptoms std. β -0.71, p < 0.001). There were no associations with cognition, progression rate, or survival. In a real-life clinical setting, the motor band sign has high specificity but relatively low sensitivity for identifying amyotrophic lateral sclerosis. Associations with genetic risk factors, neurofilament levels and somatotopic correspondence to focal motor weakness suggest that the motor band sign could be a suitable biomarker for diagnostics and clinical trials in amyotrophic lateral sclerosis.

Predictors of Clinically Important Neuroimaging Findings in Children Presenting Pediatric Emergency Department.

The aim of the study is to evaluate predictors of clinically important neuroimaging results, that is, computed tomography and magnetic resonance imaging in children in an academic pediatric emergency department (PED) from 2015 to 2019. This study was conducted in an academic PED. The patient's demographic and clinical characteristics of PED visits and neuroimaging findings requested at the PED were recorded for January 1, 2015, to December 31, 2019. In addition, descriptive statistics and logistic regression analyses were conducted. We described and determined the predictors of clinically important neuroimaging findings in children. Clinically important neuroimaging findings were detected in patients with blurred vision ( P = 0.001), ataxia ( P = 0.003), unilateral weakness ( P = 0.004), and altered level of consciousness ( P = 0.026). Clinically important neuroimaging was found 9.4 times higher in patients with altered level of consciousness, 7.4 times higher in patients with focal weakness, 4.6 times higher in patients with blurred vision, and 3.5 times more in patients presenting with ataxia. Advanced neuroimaging, especially for selected patients in PED, can improve the quality of health care for patients. On the other hand, irrelevant neuroimaging findings can lead physicians away from prompt diagnosis and accurate management. According to our study, advanced neuroimaging can be performed in the early period for both diagnosis and early treatment, especially in selected patients with ataxia, blurred vision, altered consciousness, and unilateral weakness. In other cases, clinicians may find more supporting evidence.

Isolated Bilateral Triceps Weakness in Myasthenia Gravis

Purpose: Myasthenia gravis (MG) is the most common autoimmune disease that affects the neuromuscular junction and can cause weakness in various muscle groups. The most commonly affected muscles are the eye, facial, and neck flexors. Focal or dominant weakness of the triceps muscle is rare. In this case, we aimed to describe a rare form of MG consisting of selective or dominant triceps muscle weakness. Case report: We present a 45-year-old male patient whose initial complaints were diplopia and ptosis. Acetylcholine receptor antibody was positive. After 10 years of well-being following thymectomy, bilateral triceps weakness was added to his ocular symptoms despite regular medication (pyridostigmine and prednisone). Repetitive nerve stimulation (RNS) showed decremental responses in the right triceps muscles. Conclusion: It is important to recognize this type of myasthenia gravis to facilitate diagnosis and appropriate treatment and to avoid unnecessary investigations and treatments.

Zoster-associated Limb Paralysis: Clinical and Electrophysiological Data of 15 Cases with Segmental Zoster Paresis with the Literature Review

ABSTRACT Background: Herpes zoster (HZ) is a viral disease characterized by skin eruptions and pain in specific dermatomes. Focal motor weakness that appears in the segment of skin eruptions is a rare complication of this infection. Because of the difficulty in clinically diagnosing thoracic or upper cervical motor weakness and due to the pain that causes overlooking of weakness, the true incidence of this complication is not known. In this study, we want to review the clinical presentation of segmental zoster paresis and also discuss electrophysiological findings with the involvement of nerve, plexus parts, or spinal roots. Methods: Electromyography records were examined retrospectively from 2010 to 2023 for patients who had HZ-associated limb paresis. Clinical data were reviewed and abstracted, and patients whose clinical limb weakness and sensory deficits conformed to the distribution of peripheral nerve (s) or spinal root and whose electrodiagnostic evaluation corroborated the localization, were included in the series. Results: Fifteen patients were included in our study. Ten of them were men and five of them were women. Paresis and neuropathic pain are the main complaints. In all patients, the distribution of paresis was consistent with the area of skin eruptions. C8–T1 in the upper extremity and L5–S1 in the lower extremity are the most commonly involved segments. Electrophysiological findings showed radicular involvement in three cases, brachial plexus in six cases, ulnar nerve in one case, sciatic nerve in two cases, and fibular nerve in three cases. Axonopathy is the main detected pathology in all cases. Conclusion: Segmental zoster paresis is a rare complication of cutaneous zona zoster and it affects different nerve segments. Clinicians should be aware of clinical and electrophysiological findings of this disease for the appropriate management of the patients.

Focal finger palsy and wrist pain due to cortical infarction: a case report

Abstract Background Hand knob infarction induced focal weakness of contralateral hand or distal arm, only accounts for less than 1% of all ischemic strokes. To date, there is no case with pain during sleep as the onset symptoms being reported. The atypical symptoms of hand knob infarction might increase the risk of delaying treatment especially in the hyperacute phase of stroke. Case presentation A 70-year-old man awoke from sleep due to sudden pain of his right medial wrist and presented to the emergency department with difficulty extending his right index and middle fingers within the time window of intravenous thrombolysis. But the intravenous thrombolysis was not given based on the NIHSS score (0) and atypical symptoms. MRI showed multiple DWI hyperintense lesions, including partial left hand knob area and left posterior central gyrus. CTA showed a severe focal stenosis of proximal left internal carotid artery. Conclusions The hand knob infarction might onset with unusual pain and should be carefully inspected in patients combined with acute onset of focal hand paresis.

Keratoconus cone location influences ocular biomechanical parameters measured by the Ocular Response Analyzer.

Keratoconus is characterized by asymmetry in the biomechanical properties of the cornea, with focal weakness in the area of cone formation. We tested the hypothesis that centrally-measured biomechanical parameters differ between corneas with peripheral cones and corneas with central cones. Fifty participants with keratoconus were prospectively recruited. The mean ± standard deviation age was 38 ± 13 years. Axial and tangential corneal topography were analyzed in both eyes, if eligible. Cones in the central 3mm of the cornea were considered central, and cones outside the central 3mm were considered peripheral. Each eye was then measured with the Ocular Response Analyzer (ORA) tonometer. T-tests compared differences in ORA-generated waveform parameters between cohorts. Seventy-eight eyes were analyzed. According to the axial topography maps, 37 eyes had central cones and 41 eyes had peripheral cones. According to the tangential topography maps, 53 eyes had central cones, and 25 eyes had peripheral cones. For the axial-topography algorithm, wave score (WS) was significantly higher in peripheral cones than central cones (inter-cohort difference = 1.27 ± 1.87). Peripheral cones had a significantly higher area of first peak, p1area (1047 ± 1346), area of second peak, p2area (1130 ± 1478), height of first peak, h1 (102 ± 147), and height of second peak, h2 (102 ± 127), than central cones. Corneal hysteresis (CH), width of the first peak, w1, and width of the second peak, w2, did not significantly differ between cohorts. There were similar results for the tangential-topography algorithm, with a significant difference between the cohorts for p1area (855 ± 1389), p2area (860 ± 1531), h1 (81.7 ± 151), and h2 (92.1 ± 131). Cone location affects the biomechanical response parameters measured under central loading of the cornea. The ORA delivers its air puff to the central cornea, so the fact that h1 and h2 and that p1area and p2area were smaller in the central cone cohort than in the peripheral cone cohort suggests that corneas with central cones are softer or more compliantcentrally than corneas with peripheral cones, which is consistent with the location of the pathology. This result is evidence that corneal weakening in keratoconus is focal in nature and is consistent with localized disruption of lamellar orientation.

Exoscope-assisted resection of a recurrent left frontal pilocytic astrocytoma.

An exoscope strengthens the armamentarium of a neurosurgeon by improving visualization and surgeon ergonomics, reducing surgeon discomfort, and improving coordination among the surgical team. A 23-year-old male patient developed focal seizures and weakness affecting his right arm that was attributable to a recurrent left frontal lesion. Despite two craniotomies at an 8-year interval, chemotherapy, and radiation, the tumor continued to progress. In this video, the authors demonstrate resection of a recurrent left frontal pilocytic astrocytoma with the assistance of an exoscope, neuronavigation, and neuromonitoring. The exoscope can enhance surgical resectability while smoothening the surgical workflow. The video can be found here: https://stream.cadmore.media/r10.3171/2023.10.FOCVID23158.

Dental Management of Patients With Amyotrophic Lateral Sclerosis.

Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease that affects the upper and lower motor neurons with upper and lower motor neuron manifestations. It is divided into two variants: a spinal onset and a bulbar onset. The first starts as focal muscle weakness and wasting that spreads with disease progression, while the secondphenotype presents with dysarthria, dysphonia, and dysphagia. Moreover, an extra-motor manifestation could be reported with the most commonly reported symptoms being the change in cognition and sleep disorder. Oral manifestations include increased salivation, limited mouth opening, and dysphagia. Patients with ALS have difficulty maintaining oral hygiene, and it is important for the practitioner and the caregiver to take care of this group of population. We herein provide a short review of the disease with a focus on the oral manifestations and dental considerations for management for this group.

Palliative Care and Care Partner Support in Neuro-oncology.

The journey for a patient with a brain tumor and their loved ones can be extremely challenging due to the high burden of physical symptoms and the emotional distress caused by the diagnosis. Optimizing quality of life by addressing symptoms and reducing this emotional distress can improve treatment tolerance and outcomes and alleviate care partner distress and burden. Symptoms in patients with central nervous system (CNS) tumors can vary in onset and intensity, ranging from headaches, seizures, and focal weakness to emotional distress and cognitive dysfunction. Additionally, care partners may demonstrate distress due to the high burden of care and need appropriate support structures and access to resources to alleviate this stress. Evidence-based recommendations are unfortunately limited given the lack of high-quality research in this area, but patients living with CNS tumors and their loved ones can benefit from early and routine symptom identification and management, compassionate and transparent communication, and practical guidance for the future. These principles are part of palliative care, a field of medicine focused on alleviating suffering in patients with serious, chronic illness. Clinicians involved in the care of patients with CNS tumors must be educated in these important primary palliative care principles. This article focuses on key symptom management, strategies for high-quality communication, a discussion of advance care planning, and an overview of end-of-life care.

Diffusion-Restricted Lesions of the Splenium: Clinical Presentation, Radiographic Patterns, and Patient Outcomes.

Diffusion-restricted (DR) lesions of the splenium are encountered in a wide variety of pathologies, and their significance is often unclear. We sought to report the spectrum of clinical presentations, neuroimaging patterns, and the predictors of radiographic and clinical outcomes from DR splenial lesions. This was a single-center, retrospective cohort study from January 1, 2009, to August 1, 2020. A consecutive sample of 3,490 individuals who underwent brain MRI with reported corpus callosum lesions during the study period were evaluated for DR lesions in the corpus callosum. DR lesions were defined as increased signal intensity on diffusion-weighted imaging sequences with decreased signal intensity on apparent diffusion coefficient. Patients with prior neurosurgical procedures, hemorrhage-associated DR, anoxic brain injury, and chronic or previously known or characterized disease processes in the corpus callosum were excluded. Clinical and radiologic outcomes were ascertained, including readmissions within 1 year, in-hospital mortality rates, and resolution of DR at first follow-up imaging. Outcomes were defined a priori. Two hundred patients met criteria for inclusion. The average age was 57 years (standard deviation 19 years). Near half of the patients were women (47%). Encephalopathy (55%), focal weakness (46.5%), and cortical signs (44%) were the most common presenting clinical features. Thirty-five cases (17.5%) had features consistent with cytotoxic lesions of the corpus callosum (CLOCCs). Vascular causes were most frequent (61%), followed by malignancy-related (15%) and trauma (8%). In-hospital mortality occurred in 8.5% of cases, 46.5% were readmitted to the hospital within 1 year, and 49.1% of patients had resolution of the splenial DR at the next scan. Backward stepwise regression models showed that mass effect was negatively associated with splenial DR resolution (odds ratio [OR]: 0.12, confidence interval [CI] 0.03-0.46, p = 0.002). Encephalopathy was significantly associated with in-hospital mortality (OR: 4.50, CI 1.48-17.95, p = 0.007). Patients with a CLOCC had less frequent readmissions at 1-year compared with patients without a CLOCC, p = 0.015. Vascular DR lesions of the splenium were more common than CLOCCs and other etiologies in this cohort. While splenial DR lesions can present a clinical challenge, their associated clinical and radiographic characteristics may predict outcome and guide prognosis.

Efficacy and safety of abobotulinum injection in patients with focal dystonia

Objectives: To investigate the clinical features of focal dystonia and evaluate the efficacy and safety of Abobotulinum injection on these patients. Subjects and methods: A prospective cross-sectional descriptive study on 59 patients and 114 injection sessions of focal dystonia at the Functional Exploration Unit, Hue University of Medicine and Pharmacy Hospital from July 2020 to June 2023. We recorded the dystonia severity by corresponding scale and followed up every one month after the injection to document the maximal subjective improvement and side effects of the injection. Results: The number of patients with hemifacial spasm: blepharospasm: cervical dystonia: limb dystonia: oromandibular dystonia are 17: 12: 20: 8: 2, respectively. Regarding to hemifacial spasm, the primary cause accounted for 82.4%, the average grading scale was 6.1 ± 0.5; the average dose was 80.9 ± 29.9 U Abobotulinum; the proportion of complete and &gt; 50% improvement was 83.9% of injections; the most common adverse effect was facial weakness in 47.0% of patients and 41.9% of injections. In terms of blepharospasm, the mean severity was 5.5 ± 0.9 on the Jankovic’s scale; the average therapeutic dose of 68.1 ± 27.6 helped 90.5% improve &gt; 50% and completely; 1/3 of patients had lagophthalmos, only 2 patient had bruising but no one had ptosis. Cervical dystonia patients had mean Toronto Western Spasmodic Torticollis Rating Scale of 26.3 ± 9.6; using an average dose of 406.1 ± 225.2, which improved 97.8% of injections. The most notable adverse events reported were 1 patient with vasovagal syncope and 1 patient with flu-like symptoms after injection. Limb dystonia data recorded subjective improvement in all patients, and only 1 patient had focal weakness. In 2 cases of closed jaw LTLC, both improved and had no side effects. All of the above side effects were transient and mild. Conclusion: Dystonia involves predominantly the facial and neck muscles, mainly of idiopathic origin. Research has demonstrated its effectiveness in improving symptoms of muscle dystonia, similar to studies conducted worldwide in hemifacial spasm, blepharospasm, and cervical dystonia, with less effectiveness in arm dystonia. Local side effects are quite common in hemifacial spasm and blepharospasm, while they are rare in other forms. Key words: focal dystonia, Abobotulinum, Botulinum toxin injection, cervical dystonia, blepharospasm, hemifacial spasm, oromandibular dystonia, limb dystonia.

SDPS-36 CLINICAL PRESENTATION IS A USEFUL PROGNOSTIC MARKER IN PATIENTS WITH BRAIN METASTASES

Abstract BACKGROUND Brain metastases are the most common malignant intracranial tumors and a leading cause of cancer-associated death. The influence on survival of their clinical presentation is scarce. AIM To determine if the clinical presentation of patients with brain metastases is associated with survival. METHODS A retrospective database of patients with brain metastases seen at a single center from 2010 to 2022 was analyzed. Clinical presentation and its association with survival were measured individually and adjusted for other prognostic variables. RESULTS From 822 patients, the most common symptoms were headache 53%, focal weakness 35%, visual disorders 24%, nausea/vomiting 23%, seizures 22%, and altered mental status 18%. Clinical presentations associated with survival after multivariate analysis were no symptoms [HR 0.55 (95%CI 0.39-0.79), P = 0.001], focal weakness [HR 1.21 (95%CI 1.01-1.44), P = 0.032], altered mental status [HR 1.24 (95%CI 1.01 – 1.52), P = 0.038], and visual disorders [HR 1.29 (95%CI 1.07-1.46), P = 0.007. Adding the clinical information to the graduated prognostic score improved its prognostic performance measured by both the C-statistic and the Akaike information criteria. CONCLUSION Clinical symptoms should be considered in all patients with brain metastases for they are associated with outcomes.

Immunotherapies in MuSK-positive Myasthenia Gravis; an IgG4 antibody-mediated disease.

Muscle-specific kinase (MuSK) Myasthenia Gravis (MG) represents a prototypical antibody-mediated disease characterized by predominantly focal muscle weakness (neck, facial, and bulbar muscles) and fatigability. The pathogenic antibodies mostly belong to the immunoglobulin subclass (Ig)G4, a feature which attributes them their specific properties and pathogenic profile. On the other hand, acetylcholine receptor (AChR) MG, the most prevalent form of MG, is characterized by immunoglobulin (Ig)G1 and IgG3 antibodies to the AChR. IgG4 class autoantibodies are impotent to fix complement and only weakly bind Fc-receptors expressed on immune cells and exert their pathogenicity via interfering with the interaction between their targets and binding partners (e.g. between MuSK and LRP4). Cardinal differences between AChR and MuSK-MG are the thymus involvement (not prominent in MuSK-MG), the distinct HLA alleles, and core immunopathological patterns of pathology in neuromuscular junction, structure, and function. In MuSK-MG, classical treatment options are usually less effective (e.g. IVIG) with the need for prolonged and high doses of steroids difficult to be tapered to control symptoms. Exceptional clinical response to plasmapheresis and rituximab has been particularly observed in these patients. Reduction of antibody titers follows the clinical efficacy of anti-CD20 therapies, a feature implying the role of short-lived plasma cells (SLPB) in autoantibody production. Novel therapeutic monoclonal against B cells at different stages of their maturation (like plasmablasts), or against molecules involved in B cell activation, represent promising therapeutic targets. A revolution in autoantibody-mediated diseases is pharmacological interference with the neonatal Fc receptor, leading to a rapid reduction of circulating IgGs (including autoantibodies), an approach already suitable for AChR-MG and promising for MuSK-MG. New precision medicine approaches involve Chimeric autoantibody receptor T (CAAR-T) cells that are engineered to target antigen-specific B cells in MuSK-MG and represent a milestone in the development of targeted immunotherapies. This review aims to provide a detailed update on the pathomechanisms involved in MuSK-MG (cellular and humoral aberrations), fostering the understanding of the latest indications regarding the efficacy of different treatment strategies.

Agitation and somnolence by bilateral paramedian thalamic infarct

Key Clinical MessageBilateral thalamic infarction in paramedian artery territory may present with severe acute illness, confusion, coma and memory impairment. However, subtle clinical presentation as in our case should alert the clinician to consider such a diagnosis as it can be associated with good prognosis.AbstractBilateral thalamic infarct is a rare form of stroke. Mostly thalamic infarcts are unilateral. In most cases, bilateral thalamic infarction leads to cognitive dysfunction, opthalmoparesis, conscious impairment, behavioral disturbance, and corticospinal dysfunction. Here, we describe the case of a 75‐year‐old male patient who presented to the emergency department of our hospital with agitation and somnolence for one day. He had poorly controlled hypertension. There was no previous history of stroke, diabetes mellitus, hyperlipidemia, known cardiac disease, or smoking history. There was no seizure, recent headache, or visual disturbance. The patient was somnolent and not oriented to time, person, or place. Neurological examination did not show any focal weakness or vertical eye movement restrictions. Other systemic examinations, including those of the respiratory and cardiovascular systems, were unremarkable. Extensive laboratory investigations excluded potential metabolic, infectious, endocrine, or toxic etiologies. The patient did not have any recent history of drug misuse, including benzodiazepines. Brain MRI with diffusion‐weighted imaging showed an acute bilateral thalamic infarct. Cerebral angiography was unremarkable. The patient was treated with low molecular weight heparin 60 mg subcutaneously, aspirin 300 mg daily, and haloperidol 5 mg twice daily for agitation. After two weeks of intrahospital treatment, his condition improved (consciousness and orientation massively improved).

An Uncommon Disease with a Common Presentation: Severe Back pain and Diffuse Neuropathy in Two Cases of Hereditary Neuropathy with Liability to Pressure Palsy

Introduction: Hereditary Neuropathy with liability to Pressure Palsy (HNPP) is an autosomal dominant genetic disorder characterized by multiple episodes of focal weakness and sensory loss caused by compression or trauma. HNPP is rare disorder and often exhibits cranial nerve involvement, sensorineural deafness and scoliosis. Individuals diagnosed with this condition typically exhibit a primary deficit of a single nerve palsy that can result in numbness, paresthesias, or weakness. Case Presentation: Two unrelated individuals with HNPP were evaluated with clinical, laboratory, electrophysiological and genetic testing. Case 1 was a 32-year-old man who presented with an episode of transient right arm weakness and severe back pain. Case 2 was a 46-year-old woman who presented with severe back pain and diffuse paresthesias, which was diagnosed as HNPP. Discussion: These cases present common symptoms that are uncommon for the typical HNPP patient. This report serves to contribute new information to the body of evidence describing HNPP. Conclusion: Although HNPP typically presents with transient compressive mononeuropathies, this study suggests that HNPP can present with various broad clinical presentations, including severe back pain. Neurologists need to broaden their spectrum of clinical presentations to help diagnose these rare conditions.

0977 Bilateral thalamic strokes presenting as acute hypersomnia

Abstract Introduction Acute hypersomnia is a rare presentation of bilateral paramedian thalamic strokes (PTS). The typical symptoms of vertical gaze palsy, memory impairment, and even coma, do not include acute hypersomnia. Hypersomnia can be normal in appropriate settings. However, acute hypersomnia from a wake state is abnormal and should alert observers to a potential stroke, making timely intervention consequential to patient’s outcome. Report of case(s) A 59-year-old right-handed female, who was a passenger in a car, suddenly fell asleep and started snoring. Her sister, as the driver, was not able to arouse her. The patient was taken to the Emergency Department for further evaluation. Initial head CT was normal. Follow up MRI of the brain demonstrated bilateral PTS with extension to rostral midbrain (Axial images are available with DWI/ADC windows). Throughout her five-day-hospitalization, she remained somnolent without improvement. Her neurological exam was significant for decreased alertness, impaired short-term memory recall, vertical gaze palsy, and difficulty with two-step commands. She had no focal weakness or numbness. Electroencephalogram (EEG) showed generalized theta slowing background without seizure or epileptiform discharges. Cerebral and neck vessel images, cerebrospinal fluid studies, echocardiogram, and hypercoagulable tests were normal. Outpatient workup revealed atrial fibrillation, which was the strokes’ presumed etiology. She was also diagnosed with obstructive sleep apnea (Apnea-Hypopnea Index of 9.6) and was treated with CPAP. Despite compliance, her hypersomnia (Epworth Sleepiness Scale of 24) and cognitive dysfunction persisted, making a resumption of her prior employment not feasible. Bilateral PTS commonly stem from the artery of Percheron (AOP), which is a rare vascular variation where a single artery arises from the posterior cerebral artery to supply both thalami and midbrain.1 Ischemic disruption of the thalami’s dual role in sleep-wake regulation can lead to decreased daytime arousal and increased nighttime sleep disturbance with subsequent hypersomnia.2,3 Vertical gaze palsy is due to disruption of descending pathway of vertical gaze center. Conclusion Bilateral PTS have deleterious long-term clinical consequences. Increased recognition of acute hypersomnia as the presenting symptom for PTS can enhance acute stroke treatment with tPA and/or mechanical thrombectomy, and thereby improve the odds for recovery. Support (if any)

Abstract #1402832: Doege-Potter Syndrome Due to Solitary Fibrous Tumor of the Kidney

Solitary fibrous tumor (SFT) is a rare mesenchymal tumor which can be benign or malignant. A small subset (<5%) of patients with SFT present with refractory hypoglycemia, known as Doege-Potter syndrome. This is due to increased production of insulin-like growth factor (IGF-2), its precursor pro-IGF-2, and is associated with large tumor size or aggressive clinical behavior. A 77-year-old female was brought to the ER after was found down at home with hypoglycemia down to 20 mg/dL during a wellness check. She had slurred speech and right-sided weakness. MRI of the brain and MRA of head and neck were unremarkable. She was found to have a urinary tract infection which was treated with antibiotics. Due to concern for stroke, aspirin was started. Subsequently she developed hematuria. A CT abdomen and pelvis was performed that revealed a large 17 x 12 x 11 cm heterogenous exophytic mass from the anterior margin of the right kidney. Patient's encephalopathy and focal weakness resolved with glucose administration, but she continued to have recurrent hypoglycemic episodes. A repeat MRI of the brain showed no evidence of metastatic disease and no evidence of CVA. Thyroid, adrenal and liver function tests were within normal range, and insulin and C-peptide levels were grossly suppressed during hypoglycemic episodes. IGF-1 and IGF-2 levels were normal. In the absence of any underlying endocrine pathology or the administration of any hypoglycemic agent, clinically Whipple’s triad was positive (symptoms attributable to hypoglycemia, plasma hypoglycemia at time of symptoms and resolution of symptoms with treatment of hypoglycemia). Tumor induced hypoglycemia was suspected and she underwent right radical nephrectomy. Pathology demonstrated a fully resected large (21 x 11.5 x 11 cm) spindle cell neoplasm, positive for CD34 and STAT6, consistent with solitary fibrous tumor. Post-surgically her hypoglycemia resolved. Our case highlights the importance of early diagnosis and intervention in Doege-Potter syndrome. Hypoglycemia with low levels of serum insulin, C-peptide, and beta-hydroxybutyrate in a healthy-appearing person should prompt evaluation for a tumor with cross-sectional imaging of the chest, abdomen, and pelvis. Although our patient had normal IGF-2 levels, pro-IGF-2 levels were not checked. In SFT the hypoglycemia may be due to elevated IGF-2 or pro-IGF-2 levels which leads to inappropriate activation of systemic insulin receptors and inhibition of glucose release from the liver leading to fatal hypoglycemia. Tumor induced hypoglycemia should be considered as the primary differential diagnosis when hypoglycemia is without an alternative cause.

Geriatric-8 Assessment is Predictive of Hospitalization in Patients with Glioblastoma (P7-13.005)

<h3>Objective:</h3> To describe predictors of adverse outcomes in older patients with glioblastoma (GBM). <h3>Background:</h3> Older adults with GBM have poor survival and are vulnerable to treatment toxicities. Optimal therapy is not defined and decision making depends on identifying risk factors associated with poor outcomes. <h3>Design/Methods:</h3> We prospectively enrolled patients over age 65 with newly-diagnosed GBM to undergo a comprehensive geriatric assessment (CGA) and geriatric-8 (G8) prior to disease-directed therapy. The G8 is a brief, valid and feasible tool that consists of eight items assessing age, food intake, weight loss, mobility, neuropsychology, body mass index, prescription drug, and self-perception of health. CGA consisted of several tests including Short Physical Performance Battery (SPPB), Geriatric depression scale (GDS-15), and Montreal Cognitive Assessment (MoCA). Patients were followed for adverse events, hospitalizations, and survival. Logistic regression modeled contributions of MGMT methylation status, extent of resection (EOR), G8 score and elements of CGA. Cox regression was also used to model impact of these factors on survival time. <h3>Results:</h3> 27 patients were enrolled with median age of 73.5. 20 patients were male and 11 were methylated. 21 patients are deceased (median survival=11.6 months). 44% of patients (n=12) had unplanned hospitalizations. Most common causes were for seizures (n=3), focal weakness (n=5), and cognitive decline (n=3). Poor G8 score (low) predicted unplanned hospitalizations (OR=3.38, p=0.0386). Age, EOR, MGMT methylation, GDS-15, and CGA elements were not significantly associated with hospitalization. Only MoCA score was significantly associated with improved survival (HR=0.7, p=0.02). <h3>Conclusions:</h3> Nearly half of older patients with GBM had unplanned hospitalizations. Poor G8 score predicted hospitalizations, suggesting that G8 may reveal underlying vulnerability to treatment toxicity and should be studied prospectively to validate these findings. Future studies should evaluate if treatment decisions based on G8 can reduce hospitalizations, improve patient quality of life, and decrease healthcare cost. <b>Disclosure:</b> Mr. Whitt has nothing to disclose. Dr. Hemminger has nothing to disclose. Miss Cawley has nothing to disclose. Dr. Mohile has nothing to disclose. Dr. Wasilewski has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Novocure. Dr. Hardy has received research support from Del Monte Institute for Neuroscience.