Motor Exam Research Articles

Does an improvement in cord-level intraoperative neuromonitoring data lead to a reduced risk for postoperative neurologic deficit in spine deformity surgery?

To determine if an improvement in cord-level intraoperative neuromonitoring (IONM) data following data loss results in a reduced risk for new postoperative motor deficit in pediatric and adult spinal deformity surgery. A consecutive series of 1106 patients underwent spine surgery from 2015 to 2023 by a single surgeon. Cord alerts were defined by Somatosensory-Evoked Potentials (SSEP; warning criteria: 10% increase in latency or > 50% loss in amplitude) and Motor-Evoked Potentials (MEP; warning criteria: 75% loss in amplitude without return to acceptable limits after stimulation up 100V above baseline level). Timing of IONM loss and recovery, interventions, and baseline/postoperative day 1 (POD1) lower extremity motor scores were analyzed. IONM Cord loss was noted in 4.8% (53/11,06) of patients and 34% (18/53) with cord alerts had a POD1 deficit compared to preoperative motor exam. MEP and SSEP loss attributed to 98.1% (52/53) and 39.6% (21/53) of cord alerts, respectively. Abnormal descending neurogenic-evoked potential (DNEP) was seen in 85.7% (12/14) and detected 91.7% (11/12) with POD1 deficit. Abnormal wake-up test (WUT) was seen in 38.5% (5/13) and detected 100% (5/5) with POD1 deficit. Most cord alerts occurred during a three-column osteotomy (N = 23/53, 43%); decompression (N = 12), compression (N = 7), exposure (N = 4), and rod placement (N = 14). Interventions were performed in all 53 patients with cord loss and included removing rods/less correction (N = 11), increasing mean arterial pressure alone (N = 10), and further decompression with three-column osteotomy (N = 9). After intervention, IONM data improved in 45(84.9%) patients (Full improvement: N = 28; Partial improvement: 17). For those with full and partial IONM improvement, the POD1 deficit was 10.7% (3/28) and 41.2% (7/17), respectively. For those without any IONM improvement (15.1%, 8/53), 100% (8/8) had a POD1 deficit, P < 0.001. A full or partial improvement in IONM data loss after intraoperative intervention was significantly associated with a lower risk for POD1 deficit with an absolute risk reduction of 89.3% and 58.8%, respectively. All patients without IONM improvement had a POD1 neurologic deficit.

Infinity: A Prospective Trial for Safety and Accuracy of Navigated Posterior Cervical and Thoracic Instrumentation in long-segment Fusions.

Unblinded single-arm prospective clinical trial. Evaluate safety and accuracy of navigation for placement of posterior cervicothoracic instrumentation. Computer assisted stereotactic navigation for placement of spinal instrumentation has been widely studied and implemented in the thoracic and lumbar spine. However less literature exists regarding the use of computer assisted navigation for posterior cervical instrumentation, particularly with lateral mass fixation. Here we present the first prospective study of navigated cervical lateral mass screw placement for cervicothoracic fusion. Patients who met indications for posterior cervical fusion were screened, consented, and enrolled preoperatively for instrumentation with Medtronic Infinity Occipital-Cervical-Thoracic implants, with use of intraoperative O-arm and stereotactic Stealth navigation. Postoperative CTs of the instrumented levels were obtained during the same hospital admission. Primary outcome of the trial was safety. Secondary outcomes were screw accuracy assessed by Gertzbein-Robbins grade, neurologic exams, and patient reported outcomes on the PROMIS 29 questionnaire. A total of 50 patients underwent surgery, and 557 screws were placed. There were no adverse events related to the use of navigation or screw malposition. Gertzbein-Robbins grade A or B placement comprised 95% of navigated screws. There was a decrease in positive Hoffmann sign rate postoperatively, and sensory and motor exams remained stable. There was improvement in patient reported pain and sleep domains. Navigation for cervicothoracic instrumentation is safe overall and leads to high rates of accurately placed screws. Longer term follow up could provide more insight to whether the use of this technology results in durable improvement in spinal alignment parameters and patient reported outcomes. 3.

376 Pathophysiology of voluntary motor commands in patients with multiple sclerosis identified using reverse engineering of motor unit population discharge.

OBJECTIVES/GOALS: Our objective is to characterize excitatory, inhibitory, and neuromodulatory components of the voluntary motor command at the level of the spinal motoneuron in people with multiple sclerosis (MS). This information will provide insight into neural mechanisms of motor dysfunction and their heterogeneity among patients with MS. METHODS/STUDY POPULATION: Due to advances in high-density surface EMG (HDsEMG) decomposition and the recent development of a paradigm for reverse engineering of motor unit population discharge, we can feasibly estimate aspects of excitatory, inhibitory, and neuromodulatory components of the voluntary motor command in humans on a person-specific basis. We tested 11 ambulatory patients with MS and mild-moderate disability. We recorded HDsEMG from tibialis anterior (TA) and soleus (SOL) during isometric plantarflexion and dorsiflexion, performed as slow triangle contractions. EMG was decomposed into motor unit spike trains using blind source separation. We calculated a number of motor unit variables, most notably delta-F, which estimates motoneuron excitability and the balance of neuromodulatory and inhibitory inputs. RESULTS/ANTICIPATED RESULTS: There were consistent differences in MS patients vs. controls. For TA, values were decreased for delta-F (3.9 vs. 5.9 pps), initial firing rate acceleration (5.8 vs. 7.1 pps), firing rate range (9.3 vs. 11.9 pps), and max firing rate (12.3 vs. 15.0 pps). SOL had more modest decreases in delta-F (3.0 vs. 3.8 pps) and firing rate range (4.8 vs. 5.6 pps). Self-sustained firing was longer for MS patients. Within a patient, abnormalities in motor unit variables were not consistent across muscles and legs. Interestingly, there were several abnormalities in the patients with a normal clinical motor exam, indicating that perhaps our measures are sensitive to subclinical changes in processing of voluntary motor commands. DISCUSSION/SIGNIFICANCE: Excitatory, inhibitory, and neuromodulatory components of the voluntary motor command must be appropriately balanced for skilled motor output. This study is the first to characterize how they are disrupted in MS, providing foundational information to inform the development of mechanistically-based rehabilitation interventions.

Do we fix front and back of every APC pelvic injury?

HISTORY-A 47-year-old male was on a cliff when he jumped into the water below. He jumped about 50 feet. Upon landing in the water, he felt his legs separate and abduct violently. He was taken to the Emergency unit of the nearest trauma center and was found to have no injuries except to his pelvis. He could not weight bear because of pelvic pain but had normal distal sensory and motor exam and rectal exam. His-pelvis was painful to examination anteriorly with minor left-sided posterior SI pain, and he had no blood at his meatus. X-rays and CT were done, (Figures 1-5).

Cortical and substantia nigra 18F‐PI‐2620 tau PET signal in Lewy body disease

AbstractBackgroundTau PET imaging is a potential tool for measuring comorbid tau pathology in patients with Lewy body disease. Furthermore, tau PET ligands are known to bind to neuromelanin within substantia nigra (SN), with reduced binding in Parkinson’s disease relative to healthy individuals. We sought to characterize 18F‐PI‐2620, a next generation PET tracer, in patients from across the Lewy body disease spectrum.MethodA total of 126 participants from the Stanford Alzheimer’s Disease Center (ADRC) and the Stanford Aging and Memory Study (SAMS) were recruited for PET scanning with 18F‐PI‐2620. We compared 18F‐PI‐2620 uptake within medial temporal (entorhinal cortex, amygdala) and occipital (precuneus, lingual gyrus) regions as well as SN across patients with Parkinson’s disease (PD, n = 26), dementia with Lewy bodies (DLB, n = 6), and Alzheimer’s disease spectrum (AD, n = 21), in addition to cognitively normal older adults (healthy controls, HC, n = 73). Mean bilateral signal was extracted from cortical regions of interest in native space 18F‐PI‐2620 SUVRs (inferior cerebellar gray reference). Amyloid status was determined either by 18F‐florbetaben PET or CSF. To quantify SN, SUVRs were normalized to template space and an anatomical mask was used to extract mean signal. MDS‐UPDRS‐III motor exam scores, collected in an off‐medication state for all patients, were used as a measure of motor impairment.ResultEntorhinal and amygdala SUVR were significantly elevated in the AD group relative to all other groups, and in amyloid‐positive relative to amyloid‐negative HC (Fig 1). Tau burden in all regions was low across PD and DLB groups and did not differ based on amyloid status. SN SUVR was significantly lower in the PD group compared to the HC and AD group (Fig 2A). In patients with Lewy body disease (PD &amp; DLB), MDS‐UPDRS‐III scores were negatively associated with SN SUVR (Fig 2B).ConclusionThese findings suggest that cortical tau is low in Lewy body disease. Neuromelanin‐related 18F‐PI‐2620 signal within SN carries information about disease severity. Cortical tau and SN binding represent two unique signals in the same tau PET image that may be informative in the context of Lewy body disease.

Cortical and substantia nigra 18F‐PI‐2620 tau PET signal in Lewy body disease

AbstractBackgroundTau PET imaging is a potential tool for measuring comorbid tau pathology in patients with Lewy body disease. Furthermore, tau PET ligands are known to bind to neuromelanin within substantia nigra (SN), with reduced binding in Parkinson’s disease relative to healthy individuals. We sought to characterize 18F‐PI‐2620, a next generation PET tracer, in patients from across the Lewy body disease spectrum.MethodA total of 126 participants from the Stanford Alzheimer’s Disease Center (ADRC) and the Stanford Aging and Memory Study (SAMS) were recruited for PET scanning with 18F‐PI‐2620. We compared 18F‐PI‐2620 uptake within medial temporal (entorhinal cortex, amygdala) and occipital (precuneus, lingual gyrus) regions as well as SN across patients with Parkinson’s disease (PD, n = 26), dementia with Lewy bodies (DLB, n = 6), and Alzheimer’s disease spectrum (AD, n = 21), in addition to cognitively normal older adults (healthy controls, HC, n = 73). Mean bilateral signal was extracted from cortical regions of interest in native space 18F‐PI‐2620 SUVRs (inferior cerebellar gray reference). Amyloid status was determined either by 18F‐florbetaben PET or CSF. To quantify SN, SUVRs were normalized to template space and an anatomical mask was used to extract mean signal. MDS‐UPDRS‐III motor exam scores, collected in an off‐medication state for all patients, were used as a measure of motor impairment.ResultEntorhinal and amygdala SUVR were significantly elevated in the AD group relative to all other groups, and in amyloid‐positive relative to amyloid‐negative HC (Fig 1). Tau burden in all regions was low across PD and DLB groups and did not differ based on amyloid status. SN SUVR was significantly lower in the PD group compared to the HC and AD group (Fig 2A). In patients with Lewy body disease (PD &amp; DLB), MDS‐UPDRS‐III scores were negatively associated with SN SUVR (Fig 2B).ConclusionsThese findings suggest that cortical tau is low in Lewy body disease. Neuromelanin‐related 18F‐PI‐2620 signal within SN carries information about disease severity. Cortical tau and SN binding represent two unique signals in the same tau PET image that may be informative in the context of Lewy body disease.

QOL-04. CHANGES IN FUNCTIONAL CONNECTIVITY AFTER SURGERY ARE ASSOCIATED WITH DECLINE IN FUNCTIONAL INDEPENDENCE IN PATIENTS WITH ELOQUENT GLIOMA

Abstract It is unknown whether patient independence following resection of eloquent glioma is associated with postoperative functional connectomic changes. Fifteen patients with left perisylvian glioma underwent resting-state fMRI and neuropsychological evaluation before and about 1 month after resection. Functional independence was measured with the Physical Self-Maintenance Scale (PSMS) and the Instrumental Activities of Daily Living scale (IADL). Manual dexterity testing (Grooved Pegboard; N = 13), neurologic motor exam (N = 12), and performance status (KPS; N = 14) were also obtained. Graph theoretical analysis was used to calculate functional brain network properties. Postoperative need for assistance in at least 1 activity on the IADL increased from baseline in 80% of patients, most commonly in Transportation (67%), followed by Shopping (60%), Financial Management (47%), Cooking (47%), Medication Management (33%), and Phone Use (33%). Overall need for assistance (Total IADL score) significantly increased postoperatively (Mdn change = 4, p = .006). Need for assistance on the PSMS only increased in 27% of patients, with rates less than 15% across all functions. Total PSMS scores did not differ pre- and postoperatively (Mdn change = 0). KPS scores worsened in 40% of patients. None had gross motor worsening, though fine manual dexterity declined in 7% for the left and 23% for the right hand. Increased connectomic properties were significantly associated (Spearman ρ) with greater need for assistance on the IADL [Local Efficiency: Total (.72), Shopping (.71), Cooking (.64), Financial Management (.64); Clustering Coefficient: Phone Use (.63); Betweenness Centrality: Phone Use (.63); all p &amp;lt; .01]. PSMS and IADL ratings were not associated with KPS, manual dexterity, pre/postoperative FLAIR volume, or extent of resection. This study demonstrated that increases in certain functional connectomic properties after surgery are associated with greater need for assistance in instrumental activities in the postoperative period. Connectivity increases have also been associated with poorer cognitive outcome in this population, though specific properties involved may differ.

16 Relative Contributions of Motor and Non-Motor Symptoms to Caregiver Burden in Parkinson’s Disease Patients Being Evaluated for Deep Brain Stimulation

Objective:Parkinson’s disease (PD) is a neurodegenerative disorder affecting over 10 million people worldwide. PD is characterized by both motor (e.g., tremor, rigidity, and bradykinesia) and non-motor (including cognitive impairment and neuropsychiatric symptoms such as apathy, disinhibition, executive dysfunction) symptoms. Caregiver burden is prevalent in those providing care for patients with PD and can result in negative health complications. Past work shows associations between motor symptoms, cognitive impairment, neuropsychiatric symptoms, and caregiver burden in PD. However, their relative contributions are poorly understood. This study examined these relationships, hypothesizing that while motor symptoms, cognitive impairment, and neuropsychiatric symptoms would all affect caregiver burden, neuropsychiatric symptoms would predict burden above and beyond the contribution of the other factorsParticipants and Methods:Participants were 42 people living with PD who were assessed at a hospital-based tertiary movement disorders specialty clinic for deep brain stimulation (DBS) candidacy evaluation with their caregiver. Motor exam was assessed by a PD specialist using the Unified Parkinson’s Disease Rating Scale (UPDRS). The Mini Mental State Examination (MMSE) assessed global cognition. Frontal Systems Behavior Scale (FrSBe) Family Form captured caregiver ratings of neuropsychiatric symptoms under 3 subscales: apathy, disinhibition, and executive dysfunction. The Multidimensional Caregiver Strain Index (MCSI) captured caregiver burden. Linear regression analyses examined relationships between caregiver burden (MCSI) and motor symptoms (UPDRS), cognitive impairment (MMSE), and neuropsychiatric symptoms (FrSBe).Results:Using linear regression analyses, cognitive impairment (R2=0.08, F(1,41)=4.42, p=0.04) and neuropsychiatric symptoms (R2=0.35, F(1, 41)=21.0, p&lt;0.01) predicted caregiver burden but motor symptoms did not (R2=0.03, F(1,41)=1.30, p=0.26). Hierarchical linear regression revealed that neuropsychiatric symptoms predicted caregiver burden above and beyond the contribution of cognitive impairment (AR2=0.28, AF(1)=12.7, p=0.001), accounting for an additional 28% of the variance in caregiver burden. Follow-up linear regression to examine the relationships between caregiver burden and the FrSBe subscales indicated that apathy (p&lt;0.001), versus disinhibition (p=0.16) and dysexecutive behaviors (p=0.80), was the driver of the significant relationship.Conclusions:Consistent with our hypothesis, results revealed that cognitive impairment and neuropsychiatric symptoms (specifically apathy) were independent predictors of caregiver burden, with neuropsychiatric symptoms predicting caregiver burden above and beyond the contribution of cognitive impairment. Somewhat surprisingly, motor symptoms were not a predictor of caregiver burden contrary to some previous research, though findings are mixed. Results highlight the importance of assessing for neuropsychiatric symptoms in PD, which may be overlooked by care providers relative to motor or cognitive symptoms, but which appear stressful to caregivers. Future directions include reexamining results in a larger more heterogenous sample including people living with PD at different disease stages (i.e., everyone in the present sample had severe enough symptoms to be considering DBS). Cognitive measures of executive functioning (which are more specific to PD than measures of global cognition) should also be included in future works. Development of supportive caregiver interventions specifically targeting apathy in PD may be useful. Longitudinal designs would be helpful to reexamine relationships following DBS surgery, as there are some reports of increased neuropsychiatric symptoms following the procedure.

A review of pursuit and saccadic eye movements and their utility in stroke

The head impulse-nystagmus-test of skew (+ hearing) or HINTS+ exam is a well-established clinical bedside test used in evaluating whether patients with the acute vestibular syndrome have features concerning for a central etiology (e.g., stroke). There are other components of the ocular motor exam that are helpful in the acute setting, including smooth pursuit and saccades. We discuss the anatomy and physiology of the saccade and smooth pursuit pathways from the cortex to the infratentorial region in the context of anterior and posterior circulation strokes in general but with a particular emphasis on distinct vestibular stroke syndromes. For each stroke localization, we review the vascular supply and the expected findings on the HINTS+ exam and correlate this with the expected findings on the smooth pursuit and saccade exams to aid in bedside diagnosis.

Wearable Sensors for Quantitative Motor Assessments in Multiple System Atrophy (P8-9.002)

<h3>Objective:</h3> To determine the utility of quantitative wearable sensors in Multiple system atrophy (MSA). <h3>Background:</h3> Symptoms of MSA include parkinsonism, ataxia, and/or failure of the autonomic nervous system. Wearable sensors have potential to characterize motor impairment in an outpatient setting and to serve as clinical trial outcomes in MSA. <h3>Design/Methods:</h3> Participants enrolled in biomarkers of progression in MSA (bioMUSE) had early MSA (&lt;3 years of motor symptoms) by clinical assessment. All completed neurologic exam, neuroimaging and biofluid assessments. The Unified MSA Rating Scale motor exam (UMSARS II), Parkinson Plus Scale (PPSm), and Tandem Walk (TW), were completed every 3 months. PAMSys actigraphy sensors were worn continuously for up to 12-months to assess gait (step count, bouts of walking, steps per bout, cadence, cadence variability), postures (minutes of sitting, lying, standing, walking, sedentary [sitting + lying]) and postural transitions (sit-to-stand) by averaging data over 14-days. Pearson correlation coefficients between clinical and sensor variables were estimated at each time point and two-sided p-values were calculated. <h3>Results:</h3> 12 participants (6 Female, mean age 64.2) with motor symptoms of 2.X years were followed for ≥ 6 months (n=9), and 12 months (n=5). Significant (<i>P</i>&lt;0.05) correlations were observed between step count, bouts of walking, minutes walking and sit to stand transitions for UMSARS II, PPSm and TW. Significant correlations were most frequently observed at BL and months 3, 6 and 9. The strongest correlations (r&gt;0.8) were observed at month 6 and 9. The PPSm had more frequent and stronger correlation with sensor parameters than UMSARS II. <h3>Conclusions:</h3> In early MSA, continuous actigraphy is feasible and demonstrates significant correlation with motor exam. The PPSm appeared to have stronger correlation with sensor variables than UMSARS II. Continuous motor assessments via wearable sensors may be a suitable endpoint for clinical trials in MSA. <b>Disclosure:</b> Dr. Claassen has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Teva Neuroscience. Dr. Claassen has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Spark . The institution of Dr. Claassen has received personal compensation in the range of $50,000-$99,999 for serving as a Consultant for Alterity. Dr. Claassen has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Teva Neuroscience. Dr. Claassen has received personal compensation in the range of $500-$4,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for HD Insights. The institution of Dr. Claassen has received research support from NIH. The institution of Dr. Claassen has received research support from CHDI. The institution of Dr. Claassen has received research support from HDSA. The institution of Dr. Claassen has received research support from Department of Defense. The institution of Dr. Claassen has received research support from Griffin Family Foundation. The institution of Dr. Claassen has received research support from Neurocrine. The institution of Dr. Claassen has received research support from Vaccinex. The institution of Dr. Claassen has received research support from AbbVie. The institution of Dr. Claassen has received research support from CHDI. The institution of Dr. Claassen has received research support from Genentech/ Roche. The institution of Dr. Claassen has received research support from Prilenia. The institution of Dr. Claassen has received research support from Neurocrine/ HSG. Miss Iregui has nothing to disclose. Ms. Wynn has nothing to disclose. Charles Davis has nothing to disclose. Cynthia Wong has received personal compensation for serving as an employee of Alterity Therapeutics. Dr. Stamler has received personal compensation for serving as an employee of Alterity Therapeutics.

Automated Audio Analysis of Dysarthria in Huntington’s disease (Audio-HD) (S37.010)

<h3>Objective:</h3> Using an exploratory approach, our goals were 1) identify features of speech that differentiated HD from controls, 2) use key speech features to train a classifier model and 3) compare these results to clinical assessments. <h3>Background:</h3> The diagnosis of Huntington’s disease (HD) is based primarily on the motor exam component of the Unified Huntington’s Disease Rating Scale (UHDRSTM). The UHDRSTM grades dysarthria from 0–4 (normal to anarthria), however automated speech feature detection could capture subtle changes that might serve as early disease biomarkers. <h3>Design/Methods:</h3> HD participants (n=46) and healthy controls (HC) (n=32) were matched for sex, age, and education. Participants were recorded during an 8 minute tablet-based protocol (Audio-HD) that included open-ended questions, passage reading, narrative prompt, picture description, and audio recording of the Stroop Color and Word Test (SCWT). Audio data underwent automated feature detection by Canary Speech after which key features were selected and used to train a classifier model. Model performance was compared to clinical assessment. <h3>Results:</h3> HD average UHDRSTM total motor score was 16.1 (SD=15.72) and CAG repeat length 43.1 (SD=3.62). Welch’s t-test (95% CI) identified &gt;1000 features of speech that differentiated HD from HC. The 20 most significant features were entered into a Random Forest classifier to generate a trained model. Classifier accuracy (HD vs. HC) was 96% (91% sensitivity, 98% specificity) and sensitivity was 96% for detecting dysarthria in HD. Interestingly, the model also correctly labeled 40% of HD cases lacking clinical dysarthria, suggesting it was capturing speech changes not apparent to the examiner. <h3>Conclusions:</h3> Audio-HD protocol using Canary Speech has significant potential as an efficient and sensitive tool for identifying speech biomarkers in HD. Further training of the model and longitudinal follow up will be critical for determining the overall utility of this approach. <b>Disclosure:</b> Mr. Sierra has nothing to disclose. Miss Hildebrand has nothing to disclose. Miss Ullman has nothing to disclose. Miss Mwangi has nothing to disclose. Mr. O’Connell has received personal compensation for serving as an employee of Canary Speech, Inc. Dr. Frank has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Novartis. Dr. Frank has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Sage Therapeutics. Dr. Frank has received personal compensation in the range of $500-$4,999 for serving as a Consultant for uniQure. The institution of Dr. Frank has received research support from Huntington’s Disease Society of America. The institution of Dr. Frank has received research support from Roche/Genentech. The institution of Dr. Frank has received research support from CHDI Foundation. The institution of Dr. Frank has received research support from Huntington Study Group. The institution of Dr. Frank has received research support from Triplet Therapeutics. Dr. Laganiere has received research support from HDSA.

Scurvy presenting with difficulties to walk and weakness in pediatric patients; 2 case reports. (P11-9.009)

<h3>Objective:</h3> Vitamin deficiency should be considered during differential diagnosis of neurological symptoms in patients presenting with subacute progressive gait instability and weakness. <h3>Background:</h3> Despite the perception that scurvy is a rare disorder (1,2), clinicians should have a high index of suspicion in patients at risk for nutritional deficiencies including picky eater, behavioral or sensory issues. <h3>Design/Methods:</h3> We report 2 patients of ascorbic acid deficiency who presented with subacute progressive gait instability and weakness. <h3>Results:</h3> Case 1 is a 12-year-old male with autism spectrum disorder who presented with 3 weeks of progressive bilateral lower extremity weakness resulting in wheelchair dependence. Cranial nerves, deep tendon reflexes, and sensory exam were normal. Motor exam notable for proximal &gt; distal lower extremity weakness. Skin and mucosal examination revealed gingival bleeding and corkscrew hair. Detailed dietary history revealed a severely restricted diet. He was found also to have low vitamin D. Patient treated empirically with vitamin C supplementation resulted in marked improvement within 24 hours, so no additional work up was recommended. Ascorbic acid level was extremely low confirming the diagnosis. Case 2 is a 4-year-old with history of mild hypotonia who presented with subacute progressive lower extremities weakness exacerbated by physical activity. Cranial nerves, deep tendon reflexes, and sensory exam were normal. Motor exam notable for proximal &gt; distal lower extremity weakness, waddling gait, and positive Gowers maneuver. Dietary history revealed restricted diet for the last 3 months. CK normal. Supplementation with Vitamin C resulted in minimal symptoms improvement so additional neuroimaging was recommended such as magnetic resonance imaging (MRI) of brain, spine and thigh/leg as well as lumbar puncture (LP) all unremarkable. Ascorbic acid level was low confirming the diagnosis. <h3>Conclusions:</h3> Dietary restriction and subacute progressive weakness should raise concern for nutritional myopathies. Refusal to bear weight is a frequent presentation of ascorbic acid deficiency (3). <b>Disclosure:</b> Dr. Railean has nothing to disclose. Dr. Martindale has received personal compensation in the range of $0-$499 for serving on a Speakers Bureau for Tourette Association of America. The institution of Dr. Martindale has received research support from Tourette Association of America.

Protruded Rode Post Posterior Spinal Fusion in Scoliosis Patient – Case Report

Scoliosis is defined as coronal plane spinal deformity. It is known as the lateral angulation of the vertebra more than 10 degrees which most commonly presents in adolescent girls from age 10 to 18. Scoliosis is divided into two groups which are congenital and idiopathic Scoliosis. We present a case of 18 years old male patient known as mental delayed and cleft palate, complaining of protruded Hardware for three years as post-T4 to L2 Posterior Spinal Fusion who diagnosed as a neuromuscular Scoliosis. The patient lost to follow-up since staples removal and came to the clinic after three years as transferred from primary health care. Upon physical examination, intact motor and sensory exam and normal reflexes, no signs of infection. A serial radiological investigation was ordered, and blood work was done as well. Irrigation and, debridement and hardware removal were performed, the four left proximal pedicle screws were removed. The proximal cutting part of the left rod. Deep tissue culture from different sites was taken for culture and sensitivity, which was positive for staphylococcus Aureus. The patient was kept in the hospital for intravenous antibiotics and seen in the clinic for follow-up. This case report suggests noncompliance can affect the outcome and lead to severe complications. We present a case of protruded rode post scoliosis correction in which underwent removal of loose left four pedicle screws and proximal rode.

A pilot study of a 12-week community-based boxing program for Parkinson’s disease

IntroductionCommunity-based exercise programs for Parkinson’s disease (PD) have gained popularity. Our understanding of such programs on non-motor features is limited. We characterized the effect of a 12-week community-based boxing exercise program on motor and non-motor symptoms in people with Parkinson’s disease (PwPD). MethodsIn this prospective observational study, PwPD underwent a 12-week community-based boxing program (2 sessions per week, for a total of 24 sessions). The following assessments were performed by a movement disorders neurologist at baseline and after completion of the program: MDS-Unified Parkinson’s Disease Rating Scale part III (MDS-UPDRS III) in a modified version since assessments were performed virtually due to COVID-19 pandemic, MDS Non-Motor Rating Scale (MDS-NMS), Hamilton Depression Rating Scale (HDRS), Lilli Apathy Rating Scale (LARS), Parkinson’s Disease Questionaire-39 (PDQ-39), and Schwab and England Activities of Daily Living scale (SE-ADL). Pre- and post-assessments were compared using Wilcoxon signed rank test; only participants who completed the program and both assessments were analyzed. ResultsA total of 14 PwPD agreed to be a part of the study and completed assessments. All participants were ambulatory and functionally independent at baseline. Total non-motor feature severity (MDS-NMS, p = 0.0031), depression (HDRS, p = 0.015), and motor features (MDS-UPDRS PART 3 modified, p = 0.023) all improved significantly after the intervention. Scales on apathy (LARS, p = 0.29), Parkinson’s disease-specific health related quality (PDQ-39, p = 0.093), and activities of daily living (SE-ADL, p = 0.32) did not demonstrate significant change. ConclusionPwPD who participated in a community-based, pilot boxing program showed improvements in motor exam and non-motor symptoms.

ODP136 A Rare Case of Necrotizing Myopathy after Statin Use

Abstract Introduction Statin associated necrotizing autoimmune myopathy is an extremely rare side effects of stain use. The incidence is approximately 2-3/100,000 Patients treated with statins. It usually associated with significant proximal muscle weakness, strikingly elevated creatine kinase (CK) levels and persistent symptoms despitestatin discontinuation. Here, we present a case with necrotizing myopathy after statin use as a reminder to clinicians for appropriately suspicious of this phenomenon while on treatment of statins. Clinical case: A 35 year old male with a past medical history of Autism, Type 2 Diabetes Mellitus, Hyperlipidemia, and Hypertension was brought to emergency room for right leg pain. Family states he had difficulty walking for a few weeks and had a fall from couch a week ago. Initial Vitals showed HR at 134 bpm, temp 35.9, BP 157/85 mmHg, RR 19 breaths/min. Labs showed Creatinine. 3.29 mg/dL, CO2 24 mmol/L, Na 141 mmol/L, K 4.2 mmol/L, Ca 9.5 mg/dL, glucose 265 mg/dL, AST/ALT 914/919 units/L, CK&amp;gt;14,000 units/L, WBC 9.2 thou/mcL, Hb 12.6 g/dL, TSH 9.42 mcIU/mL, Free T3 1.41 pg/mL, Free T4 0.95 mcg/dL. Urinalysis showed large blood with RBC of 1. Myoglobin&amp;gt;8750 mcg/L. Neuro motor exam showed strength 4/5 in bilateral lower extremities. His home medications including atorvastatin 20 mg nightly for 2 years, metformin and olmesartan, which all had been held. He was treated for rhabdomyolysis with aggressive hydration and Creatinine was improving. However, CK continued trending up to 42,915 units/L on day 5 with unreleased symptoms of lower extremity weakness. Neurology was consulted. Differential diagnosis of myopathy were suspected. His CK was continue to trend up to 45,585 units/L on day 10. Decision was made to have muscle biopsy with pathology showed: necrotizing myopathy without any clear evidence of inflammation. Although some autoimmune necrotizing myopathy may not show inflammation like those seen with HMG-CR antibodies or signal recognition antigen antibodies. Autoantibodies against 3-hydroxy-3-methylglutaryl coenzyme A (HMG-CoA) reductase (HMGCR Ab) was above 550. Following the biopsy, he received IV Solu-medrol 1g daily×4 doses, and then PO Prednisone 80 mg daily and then tapered down to 40 mg daily. He was also promptly started on IVIG 400 mg/kg/day for 5 days and methotrexate 7.5 mg weekly. His CK started to improve, down to 5815 units/L three weeks later. No significant improvement of strength. Conclusion Our case demonstrates that the appropriate diagnosis of statin induced necrotizing autoimmune myopathy with muscle biopsy and HMGCR Ab. We also wanted to alert future clinicians to have an early suspicious of such rare side effect and initiate treatment as early as possible which may bring the maximum benefit for patients. Presentation: No date and time listed

On the Treatment of a Patient with Ataxic Syndrome by Microdoses of Levo- and Carbo-Dopa. Post COVID Treatment

The neurological evaluation and the report of the physioterapist are described for a patient who from 2018 was blocked by neurological status with different problems that concerned his different functions of the nervous systems on coordination and balance, motor exam and even speech, after the treatment with microdoses of Sinemet CR for two months. The satisfation is reported by the neurologist regarding the results on the patient recovery. Post COVID disorders are suggested to be treated with special indication for long COVID.

S1548 GI Dysmotility Symptoms Are Not Associated With Increased L-Dopa Requirements in Parkinson’s Disease Patients

Introduction: Gastrointestinal (GI) motility disorders like gastroparesis or small bowel bacterial overgrowth are thought to impair efficacy of L-dopa, which is absorbed in the proximal small intestine, resulting in motor fluctuations among Parkinson’s Disease (PD) patients that prompt dosage increases. While symptoms of dysmotility are frequently reported in PD, results are mixed concerning their effect, if any, on L-dopa pharmacokinetics. In this study, we compare L-dopa equivalent daily dose (LEDD) and motor function among patient with 5 upper GI symptoms to study the possible relationship between dysmotility symptoms and increased L-dopa requirement. Methods: Two hundred PD patients evaluated by outpatient neurology at Mass General Brigham Healthcare between 2018-2019 were included. History of 5 upper GI symptoms (dysphagia, nausea, vomiting, epigastric pain, and bloating), as well as LEDD were obtained from the medical record. Unified PD Rating Scale (UDPRS) part III motor exam scores off medication were extracted from clinical notes. Differences between LEDD and UDPRS motor scores among patients with and without a history of each of the 5 GI symptoms were calculated via Student t-tests. Results: Dysphagia was significantly associated with increased LEDD (230mg, P=0.0067) as well as increased motor severity (4.6 UDPRS, P=0.036). In contrast, nausea was associated with decreased LEDD (-187 mg, P=0.030) despite similar motor severity (1.1 UDPRS, P=0.63) to those without nausea (Table). There were no differences in LEDD and UDPRS scores among patients with and without vomiting, epigastric pain, and bloating. Conclusion: Except dysphagia, we found that GI dysmotility symptoms were not associated with increased LEDD nor increased motor severity. This suggests that symptomatic GI dysmotility may not be directly related to poor L-dopa absorption, a common clinical question for PD patients referred to GI. While dysphagia was linked with higher LEDD, this may have stemmed from higher UDPRS scores in this population. Interestingly, those with nausea required lower LEDD despite comparable motor severity. Nausea, a frequent symptom of delayed intestinal transit, may extend absorption time leading to better motor outcomes on less L-dopa. However, further studies are needed to discern any underlying mechanism. Our results nonetheless indicate that clinical management of dysmotility may not reduce motor fluctuations and the need for increased L-dopa as previously presumed. Table 1. - Changes in L-dopa Requirements and Motor Severity in the Presence of Dysmotility Symptoms GI Symptom ΔLEDD, mg (SD) P value ΔUDPRS III (SD) P value Nausea -187 (86) 0.03 1 (2) 0.633 Vomiting 11 (95) 0.91 -2 (2) 0.343 Dysphagia 230 (84) 0.007 5 (2) 0.036 Epigastric pain 101 (104) 0.33 0 (3) 0.881 Bloating 62 (99) 0.533 1 (2) 0.764 LEDD—L-dopa equivalent daily dose UDPRS—Unified Parkinson's Disease Rating Scale.

Prospective predictors of care partner burden and depression in Parkinson's disease.

Care partners who provide informal care to individuals with Parkinson's disease (PD) report higher levels of burden and depression; however, longitudinal research on these symptoms is scarce. The current study assessed changes in care partner burden and depression, and patient and care partner predictors of these symptoms over time. Such knowledge may provide important information for assessment and treatment of depression and burden in care partners of individuals with PD. Participants were 88 PD patients without dementia and their self-identified care partner (n = 88). Care partners completed the Geriatric Depression Scale and Zarit Burden Interview. PD participants completed mood questionnaires and a motor exam at baseline and 2year follow-up. Relationships among care partner burden and depression over time with patient and care partner predictors (i.e., demographic, mood, and disease characteristics) were assessed using correlations and regression analyses. Care partner burden and depression significantly increased over an approximate 2year period. Greater baseline disease severity predicted worsening of care partner burden (p = 0.028), while baseline patient depression predicted worsening of care partner depression (p = 0.002). Results highlight differential impacts of specific PD symptoms on worsening care partner burden compared to depression; increased PD disease severity predicts increased burden, while patient mood predicts worsening of depression over time. Targeting PD disease severity and mood symptoms may prevent the progression of care partner burden and depression.

Early risk stratification for progression to death by neurological criteria following out-of-hospital cardiac arrest

BackgroundSome patients resuscitated from out-of-hospital cardiac arrest (OHCA) progress to death by neurological criteria (DNC). We hypothesized that initial brain imaging, electroencephalography (EEG), and arrest characteristics predict progression to DNC. MethodsWe identified comatose OHCA patients from January 2010 to February 2020 treated at a single quaternary care facility in Western Pennsylvania. We abstracted demographics and arrest characteristics; Pittsburgh Cardiac Arrest Category, initial motor exam and pupillary light reflex; initial brain computed tomography (CT) grey-to-white ratio (GWR), sulcal or basal cistern effacement; initial EEG background and suppression ratio. We used two modeling approaches: fast and frugal tree (FFT) analysis to create an interpretable clinical risk stratification tool and ridge regression for comparison. We used bootstrapping to randomly partition cases into 80% training and 20% test sets and evaluated test set sensitivity and specificity. ResultsWe included 1,569 patients, of whom 147 (9%) had diagnosed DNC. Across bootstrap samples, >99% of FFTs included three predictors: sulcal effacement, and in cases without sulcal effacement, the combination of EEG background suppression and GWR ≤ 1.23. This tree had mean sensitivity and specificity of 87% and 81%. Ridge regression with all available predictors had mean sensitivity 91 % and mean specificity 83%. Subjects falsely predicted as likely to progress to DNC generally died of rearrest or withdrawal of life sustaining therapies due to poor neurological prognosis. Two of these cases awakened from coma during the index hospitalization. ConclusionsSulcal effacement on presenting brain CT or EEG suppression with GWR ≤ 1.23 predict progression to DNC after OHCA.

Association between knee extensor force steadiness and postural stability against mechanical perturbation in patients with Parkinson’s disease

ObjectiveLower extremity force steadiness has been shown to decrease with aging and neuromotor dysfunction and to be associated with physical function and fall. Although patients with Parkinson’s disease (PD) experience decreased force steadiness, whether the extent of force steadiness differs according to target force or whether this steadiness is associated with postural control remain unclear. Therefore, this study aimed to compare the force steadiness while steadily exerting low and moderate levels of knee extensor force between individuals with and without PD and to examine the association between force steadiness and postural instability against mechanical perturbation in PD. MethodsA total of 33 patients with PD (mean age, 71.7 years) and 33 healthy controls (72.2 years) participated in this study. Participants with PD were classified into postural stability or instability groups based on the Movement Disorder Society-sponsored revision of the Unified Parkinson’s Disease Rating Scale motor exam item 12. Participants performed steady task of the knee isometric extension at two levels (10% and 50% of maximal voluntary contraction [MVC]). ResultsForce steadiness at 10% MVC was lower in postural instability group than that in the control and postural stability groups (P < 0.05) after adjusting for age, sex, and body mass index, whereas it was not significantly different at 50% MVC among the three groups. DiscussionThese results suggest that the knee extensor force steadiness is affected in patients with PD having postural instability against mechanical perturbation during low intensity force exertion and is not affected regardless of the presence of postural instability during moderate intensity force exertion.