Objective: To describe the clinical characteristics and treatment response of a patient with coronavirus disease (COVID-19) associated myelitis with predominant corticospinal tract involvement. Background: Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection-related neurological manifestations have been described. Evidence of individuals with COVID-19 associated acute myelitis continue to grow. Various mechanisms have been proposed by which COVID-19 could lead to spinal cord manifestations; an immune-mediated pathogenesis has been considered. However, there is still not a clear distinction between para- and post- infectious presentation of COVID-19 associated myelitis and there are still no specific evidence-based management strategies. For these reasons, it is important to identify and report unusual presentations. Design/Methods: We identified a male with few risk factors for severe COVID-19 complications, with sudden onset impaired gait particularly while walking downstairs nine days after the initial presentation of SARS-CoV-2 respiratory symptoms and after receiving a course of nirmatrelvir/ritonavir plus oral steroids. Gait impairment rapidly progressed to leg stiffness and pain of thighs. A second FDA approved COVID-19 booster had been administered months prior to onset. Results: Neurologic examination showed lower limbs spasticity, clonus, and corticospinal signs with myalgia of thighs muscles in the absence of weakness, sensory loss, or abnormal sphincter control. CSF showed lymphocytic pleocytosis with elevated proteins; serum inflammatory markers were elevated; viral serology positive for SARS-CoV-2 antigen. Thoraco-lumbar gadolinium MRI was normal. High dose IV steroids were administered with near full recovery. Conclusions: SARS-CoV-2 has been associated to several neurological complications. We report a case of pure motor acute-onset rapidly progressing, MRI-negative myelitis, nine days after COVID-19 onset in a vaccinated patient who responded to immunotherapy. To the best of our knowledge, this is the first report of COVID-19 associated myelitis with predominant corticospinal tract involvement without weakness. These findings suggest an immune-mediated pathogenesis and support a combined treatment strategy that needs further investigation. Disclosure: Dr. Reyes Rivera has nothing to disclose. Dr. Tait has nothing to disclose. The institution of Dr. Serrano has received research support from Eli Lilly. The institution of Dr. Serrano has received research support from Abbvie.

Objective: To present a case of a Puerto Rican woman with Chorea-Acanthocytosis positive for VSP13A gene variant presenting with severe pelvic movements. Background: Chorea-Acanthocytosis is an autosomal recessive inherited neurodegenerative disorder originated from variants of the VPS13A gene. It is characterized by clinical features that may include chorea, tics, parkinsonism, dystonia, speech disturbances, and epilepsy. Most case reports of Puerto Rican descent have been reported in the United States. Design/Methods: NA Results: We present the case of a 41-year-old Puerto Rican woman with unusual involuntary movements and mutism. At onset she developed irritability and generalized choreiform movements that worsened over the years. There is family history of undiagnosed involuntary movements and family consanguinity. Clinical symptoms worsened to non-verbal communication, dysphagia, additional behavioral changes, and involuntary movements. Neurological examination showed choreiform movements, orofacial movements and blepharospasm. She presented forceful, brisk and disabling movements of the pelvis and neck present while lying down and worsened with activity. Genetic test for Huntington disease was negative, repeated blood smear did not show acanthocytes. CPK was mildly elevated, liver enzymes and serum ceruloplasmin levels were normal. CSF analysis was unremarkable. Autoimmune encephalitis was suspected and she received a course of IVIG without benefit. EEG was normal. Brain MRI showed abnormal signal intensity of bilateral gray matter involving putamen and caudate nuclei with associated ex vacuo dilation of frontal horns and lateral ventricles. Genetic testing was positive for autosomal recessive homozygous VSP13A gene variant. Conclusions: This case illustrates the variable presentation of chorea-acanthocytosis and emphasizes the importance of early recognition and timely genetic testing among Hispanic patients presenting with involuntary movements. Our patient had an uncommon initial presentation with predominant severe pelvic and neck movements making the diagnosis challenging and delayed. An accurate and early diagnosis is important to offer symptomatic treatment, identify potential future complications, and provide genetic counseling. Disclosure: Dr. Enriquez-Gonzalez has nothing to disclose. The institution of Dr. Serrano has received research support from Eli Lilly. The institution of Dr. Serrano has received research support from Abbvie.

Wednesday, April 29April 14, 2020Free AccessYoung Woman with weakness progressing to quadriplegia: Is this multiple sclerosis or something else? A Case Report (2887)Maria E. Garcia Ayala, Carmen Serrano, and Eduardo LabatAuthors Info & AffiliationsApril 14, 2020 issue94 (15_supplement)https://doi.org/10.1212/WNL.94.15_supplement.2887 Letters to the Editor

Objective: To assess if a concise and specific electronic template for Parkinson’s patients improves documentation of the 10 practice parameters for Parkinson’s disease published by the American Academy of Neurology (AAN). Background: Parkinson’s disease is a clinical priority in Neurology. Managing the motor and non-motor complications in these patients may be time consuming. Therefore, use of an electronic template may prevent missing documentation of the recommended quality measures Methods: Pre-template implementation, clinical notes from 15 patients were screened to assess the appropriate documentation of the 10 quality parameters for Parkinson’s disease in the Movement Disorders Clinic at the University of Puerto Rico. Poor documentation was found, thus, an electronic template outlining the 10 quality measures as recommended by the AAN was created for use at follow up visits. Neurology and non-neurology residents were educated about the 10 quality measures and on how to use the template. Post-template implementation, clinical notes from 33 Parkinson’s disease patients were randomly selected and documentation rates of quality measures were compared pre- and post-template use. Results: Most quality measures for Parkinson’s disease were not documented appropriately before template implementation. Following template implementation, documentation of most quality measures improved. Documentation of falls improved significantly (P=0.0007). Unexpectedly, fall documentation rate was similar between adult neurology vs non-adult neurology residents (P= 0.6927), demonstrating the ease of use of the template. Conclusions: A Parkinson’s disease template improved adherence to documenting the 10 quality measures for Parkinson’s disease at follow up visits. The similar fall documentation rate between neurology and non-neurology residents suggests that consistent documentation depends on the template itself rather than the training of the provider. Disclosure: Dr. Vargas Perez has nothing to disclose. Dr. Serrano has nothing to disclose.

Objective: To study the prevalence and characterize mild cognitive impairment (MCI) in patients with early Parkinson's disease (PD), and to investigate a possible relation between disturbances in glucose/insulin metabolism, and MCI in these patients. Background: There is a well-documented high prevalence of diabetes mellitus and glucose metabolism problems in Puertorrican population. Multiple clinical, pathological, and experimental evidences suggest a relationship between insulin signaling abnormalities, neurodegeneration and cognitive impairment in various conditions. In fact common intracellular pathways have been identified linking the neurodegenerative process in PD with insulin resistance. Additionally elevated levels of certain ceramides, part of the intracellular cascade of the insulin receptor, have been found in associated with MCI. Methods: We are studying cognitive function, glucose metabolism, parameters of metabolic syndrome, and plasma levels of sIR in patients with mild to moderate PD without subjective memory complains, and age-matched non-PD controls. Extensive neuropsychological evaluation is performed including two tests in five cognitive domains. To the date 21 patients and 7 controls have been studied. Insulin resistance was determined using the HOMA equation. Results: Analysis of the current group revealed a 77[percnt] prevalence of multi-domain MCI. Both frontal and posterior cognitive functions were affected in most patients. Women were significantly more prone to develop posterior, non-dopamine dependent cognitive problems. Cognitive problems were significantly associated with lower insulin and higher glucose levels, and there was a tendency of HOMA values to be reduced in patients with PD and MCI. Conclusions: Prevalence of multi-domain MCI affecting both frontal and posterior cognitive domains was unexpectedly high in our population. Patients with PD and MCI had significant changes in glucose/insulin levels and an unexpected tendency to have less insulin resistance compared with cognitively intact PD patients.

ABSTRACT Background Few studies have systematically investigated the association between PARKIN genotype and psychiatric co‐morbidities of Parkison's disease (PD). PARKIN ‐associated PD is characterized by severe nigral dopaminergic neuronal loss, a finding that may have implications for behaviors rooted in dopaminergic circuits such as obsessive‐compulsive symptoms (OCS). Methods The Schedule of Compulsions and Obsessions Patient Inventory (SCOPI) was administered to 104 patients with early‐onset PD and 257 asymptomatic first‐degree relatives. Carriers of one and two PARKIN mutations were compared with noncarriers. Results Among patients, carriers scored lower than noncarriers in adjusted models (one‐mutation: 13.9 point difference, P = 0.03; two‐mutation: 24.1, P = 0.001), where lower scores indicate less OCS. Among asymptomatic relatives, a trend toward the opposite was seen: mutation carriers scored higher than noncarriers (one mutation, P = 0.05; two mutations, P = 0.13). Conclusions First, a significant association was found between PARKIN mutation status and obsessive‐compulsive symptom level in both PD and asymptomatic patients, suggesting that OCS might represent an early non‐motor dopamine‐dependent feature. Second, irrespective of disease status, heterozygotes were significantly different from noncarriers, suggesting that PARKIN heterozygosity may contribute to phenotype. © 2014 International Parkinson and Movement Disorder Society

IMPORTANCE:Data on the long-term cognitive outcomes of patients with PARKIN-associated Parkinson disease (PD) are unknown but may be useful when counseling these patients. OBJECTIVE:Among patients with early-onset PD of long duration, we assessed cognitive and motor performances, comparing homozygotes and compound heterozygotes who carry 2 PARKIN mutations with noncarriers. DESIGN, SETTING, AND PARTICIPANTS:Cross-sectional study of 44 participants at 17 different movement disorder centers who were in the Consortium on Risk for Early-Onset PD study with a duration of PD greater than the median duration (>14 years): 4 homozygotes and 17 compound heterozygotes (hereafter referred to as carriers) and 23 noncarriers. MAIN OUTCOMES AND MEASURES:Unified Parkinson Disease Rating Scale Part III (UPDRS-III) and Clinical Dementia Rating scores and neuropsychological performance. Linear regression models were applied to assess the association between PARKIN mutation status and cognitive domain scores and UPDRS-III scores. Models were adjusted for age, education, disease duration, language, and levodopa equivalent daily dose. RESULTS:Carriers had an earlier age at onset of PD (P < .001) and were younger (P = .004) at time of examination than noncarriers. They performed better than noncarriers on the Mini-Mental State Examination (P = .010) and were more likely to receive lower scores on the Clinical Dementia Rating (P = .003). In multivariate analyses, carriers performed better than noncarriers on the UPDRS-III (P = .02) and on tests of attention (P = .03), memory (P = .03), and visuospatial (P = .02) cognitive domains. CONCLUSIONS AND RELEVANCE:In cross-sectional analyses, carriers demonstrated better cognitive and motor performance than did noncarriers with long disease duration, suggesting slower disease progression. A longitudinal follow-up study is required to confirm these findings.