Publications by authors named "Kelsey M Smith"

12 Publications

  • Page 1 of 1

Hyperammonemia in Patients With Status Epilepticus Treated With or Without Valproic Acid.

Neurologist 2021 May 5;26(3):80-82. Epub 2021 May 5.

Department of Neurology, Mayo Clinic, Rochester, MN.

Background: Hyperammonemia is a common side effect of valproic acid (VPA) and can occur after generalized seizures, but the clinical significance is unclear. The aim of this study was to better understand the clinical practice and utility of ammonia testing in status epilepticus (SE) treated with or without VPA.

Methods: Charts of adult patients with SE from St. Mary's Hospital Intensive Care Units (ICUs) (Mayo Clinic, Rochester, MN) from 2011 to 2016 were reviewed. Clinical factors were compared between patients who had ammonia checked versus those who did not, and those with normal ammonia versus hyperammonemia (>50 µg/dL). Charts were reviewed to determine if hyperammonemia changed clinical management and if it was felt to be symptomatic.

Results: There were 304 patients identified: 94 received VPA, 142 had ammonia checked and receiving VPA was associated with ammonia testing (P<0.001). Hyperammonemia was identified in 32 and associated with younger age, being in a non-neurological intensive care unit, and liver disease, but was not statistically associated with VPA. Only one patient had valproate-induced hyperammonemic encephalopathy; however, many patients received treatment for hyperammonemia such as lactulose, levocarnitine, or VPA dose reductions.

Conclusions: This study demonstrated variability in ammonia testing and management changes in SE but does not support the routine monitoring of ammonia levels and showed that hyperammonemic encephalopathy was rare in this clinical setting.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1097/NRL.0000000000000335DOI Listing
May 2021

Alterations to the gut microbiome impair bone tissue strength in aged mice.

Bone Rep 2021 Jun 8;14:101065. Epub 2021 Apr 8.

JM-USDA Human Nutrition Research Center on Aging, Tufts University, Boston, MA 02111, United States of America.

Whole bone strength and resistance to fracture are determined by a combination of bone quantity and bone quality - key factors in determining risk for osteoporosis and age-related fractures. Recent preclinical studies have shown that alterations to the gut microbiome can influence bone quantity as well as bone tissue quality. Prior work on the gut microbiome and bone has been limited to young animals, and it is unknown if the gut microbiome can alter bone tissue strength in aged animals. Here we ask if alterations to the constituents of the gut microbiome influence bone strength in older mice (12-24 months of age). Male C57BL/6J mice raised on a standard chow diet until 12 months of age were assigned to one of three diets: high glycemic, low glycemic, or low glycemic diet containing antibiotics (ampicillin and neomycin) to modify the constituents of the gut microbiome. The group fed the low glycemic diet containing antibiotics showed reductions in whole bone strength that could not be explained by geometry, indicating reduced bone tissue strength ( < 0.007). The high glycemic diet group had larger bone cross-sectional area and moment of inertia and a corresponding greater bone strength as compared to the low glycemic groups, however tissue strength did not noticeably differ from that of the low glycemic group. These findings demonstrate that modifying the gut microbiome in aged mice can alter bone tissue quality.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.bonr.2021.101065DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8079457PMC
June 2021

Musicogenic epilepsy: Expanding the spectrum of glutamic acid decarboxylase 65 neurological autoimmunity.

Epilepsia 2021 May 25;62(5):e76-e81. Epub 2021 Mar 25.

Department of Neurology, Mayo Clinic, Rochester, Minnesota, USA.

The objective of this study was to describe serological association of musicogenic epilepsy and to evaluate clinical features and outcomes of seropositive cases. Through retrospective chart review, musicogenic epilepsy patients were identified. Among 16 musicogenic epilepsy patients, nine underwent autoantibody evaluations and all had high-titer glutamic acid decarboxylase 65-immunoglobulin G (GAD65-IgG; >20 nmol·L , serum, normal ≤ .02 nmol·L , eight women). Median GAD65-IgG serum titer was 294 nmol·L (20.3-3005 nmol·L ), and median cerebrospinal fluid titer (n = 4) was 14.7 nmol·L . All patients had temporal lobe epilepsy, and bitemporal epileptiform abnormalities were common. Right temporal lobe seizures were most frequently captured when seizures were induced by music on electroencephalogram (3/4; 75%). Intravenous (IV) methylprednisolone and/or IV Ig (IVIG) was utilized in four patients, with one having greater than 50% reduction. Rituximab (n = 2) and mycophenolate (n = 1) were ineffective. Two patients underwent right temporal lobe resections but continued to have seizures. Vagus nerve stimulation was effective at reducing seizures in one patient by 50%, and an additional patient was seizure-free by avoiding provoking music. Right temporal lobe epilepsy was more common among patients with musicogenic epilepsy when compared to nonmusicogenic GAD65 epilepsies (n = 71, 89% vs. 47%, p = .03). GAD65-IgG should be tested in patients with musicogenic epilepsy, given implications for management and screening for comorbid autoimmune conditions.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1111/epi.16888DOI Listing
May 2021

Thalamic venous infarction from trauma mimicking a glioma.

Clin Imaging 2021 May 3;73:23-25. Epub 2020 Dec 3.

Mayo Clinic, Department of Neurology, 200 1st St. SW, Rochester, MN 55905, United States of America. Electronic address:

Traumatic brain injuries (TBI) are commonly associated with motor vehicle accidents. Neuroimaging plays a crucial role in the initial management of TBIs. We present a case of a TBI related to a motor vehicle accident in an 18-year-old woman. Initial brain imaging revealed significant traumatic injuries and an enhancing mass, without restricted diffusion, in the thalamus favored to be a thalamic glioma. Subsequent imaging revealed resolution of enhancement of the thalamic lesion and reduction in size. On review of the original imaging, it was determined that the thalamic lesion was related to a tear and partial thrombosis of a large thalamic vein resulting in infarction and hemorrhage.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.clinimag.2020.11.045DOI Listing
May 2021

Dietary Patterns, Carbohydrates, and Age-Related Eye Diseases.

Nutrients 2020 Sep 18;12(9). Epub 2020 Sep 18.

JM-USDA Human Nutrition Research Center on Aging, Tufts University, Boston, MA 02111, USA.

Over a third of older adults in the U.S. experience significant vision loss, which decreases independence and is a biomarker of decreased health span. As the global aging population is expanding, it is imperative to uncover strategies to increase health span and reduce the economic burden of this age-related disease. While there are some treatments available for age-related vision loss, such as surgical removal of cataracts, many causes of vision loss, such as dry age-related macular degeneration (AMD), remain poorly understood and no treatments are currently available. Therefore, it is necessary to better understand the factors that contribute to disease progression for age-related vision loss and to uncover methods for disease prevention. One such factor is the effect of diet on ocular diseases. There are many reviews regarding micronutrients and their effect on eye health. Here, we discuss the impact of dietary patterns on the incidence and progression of age-related eye diseases, namely AMD, cataracts, diabetic retinopathy, and glaucoma. Then, we focus on the specific role of dietary carbohydrates, first by outlining the physiological effects of carbohydrates on the body and then how these changes translate into eye and age-related ocular diseases. Finally, we discuss future directions of nutrition research as it relates to aging and vision loss, with a discussion of caloric restriction, intermittent fasting, drug interventions, and emerging randomized clinical trials. This is a rich field with the capacity to improve life quality for millions of people so they may live with clear vision for longer and avoid the high cost of vision-saving surgeries.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.3390/nu12092862DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7551870PMC
September 2020

Electroencephalogram Changes During Cheyne-Stokes Respiration in Acutely Ill Hospitalized Patients.

Neurocrit Care 2020 12;33(3):829-834

Department of Neurology, Mayo Clinic, 200 1st St. SW, Rochester, MN, 55905, USA.

View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1007/s12028-020-00937-zDOI Listing
December 2020

A low glycemic diet protects disease-prone Nrf2-deficient mice against age-related macular degeneration.

Free Radic Biol Med 2020 04 14;150:75-86. Epub 2020 Feb 14.

Laboratory for Nutrition and Vision Research, JM-USDA Human Nutrition Research Center on Aging, Tufts University, Boston, MA, 02111, USA; Friedman School of Nutrition and Science Policy, Tufts University, Boston, MA, 02111, USA; Department of Ophthalmology, Tufts University School of Medicine, Boston, MA, 02111, USA. Electronic address:

Age-related macular degeneration (AMD) is a major blinding disease, affecting over 14% of the elderly. Risk for AMD is related to age, diet, environment, and genetics. Dietary modulation of AMD risk is a promising treatment modality, but requires appropriate animal models to demonstrate advantages of diet. Mice lacking the antioxidant transcription factor Nrf2 (Nfe2l2) develop age-related retinopathy relevant to human AMD. Here we evaluated the effect of consuming high glycemic (HG) or low glycemic (LG) diets until 18-months of age on development of features relevant to AMD in Nrf2-null mice. Nrf2-null mice that consumed HG diets developed atrophic AMD, characterized by photoreceptor degeneration, retinal pigment epithelium (RPE) atrophy and pigmentary abnormalities, basal laminar deposits, and loss of the choriocapillaris. In contrast, Nrf2-null-mice that consumed LG diets did not develop retinal disease phenotypes. Consumption of HG diets was associated with accumulation of advanced glycation end-products in the RPE and systemically, whereas consumption of the LG diet was associated with increased levels of anti-glycative and anti-oxidative detoxification machinery. Together our data indicate that the Nrf2-null HG mouse is a good model for atrophic AMD studies and that the LG diet can activate protective pathways to prevent AMD, even in a genetically predisposed animal.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.freeradbiomed.2020.02.010DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7747150PMC
April 2020

Tau deposition in young adults with drug-resistant focal epilepsy.

Epilepsia 2019 12 29;60(12):2398-2403. Epub 2019 Oct 29.

Department of Neurology, Mayo Clinic, Rochester, Minnesota.

Objective: To evaluate the presence of tau deposition and pathologic features of chronic traumatic encephalopathy (CTE) in young adult patients treated with focal cortical resections for drug-resistant epilepsy.

Methods: Sixty consecutive patients who had undergone surgical treatment for drug-resistant focal epilepsy between 18 and 45 years of age were identified (2010-2017). Medical records were reviewed to determine clinical factors, including history of head trauma, age at seizure onset, age at surgical resection, seizure type(s) and frequency, imaging findings, and surgical outcome. All formalin-fixed, paraffin-embedded blocks from the surgical specimens from each subject were sectioned and stained with hematoxylin and eosin and antibodies to tau (Thermo Fisher Scientific Clone AT8), and examined blindly for tau pathology, including lesions characteristic of CTE.

Results: The median age at resection was 29.5 years (range = 19-45). A history of head trauma was reported in 19 patients. Although none of the patients had pathological findings characteristic of CTE, 23 patients (38%) demonstrated tau-immunoreactive lesions, including neurites, neurofibrillary pretangles, neurofibrillary tangles, subpial tau, and/or glial tau. In 4 of the 23 patients (7% of the cohort; 17% of those with tau pathology), substantial tau burden was identified. Three of these 4 patients had no significant history of head trauma; 1 patient had multiple sports-related concussions. No specific clinical factors correlated with the presence of tau pathology.

Significance: Tau-immunoreactive lesions were found in 38% of 60 patients who underwent a focal cortical resection for drug-resistant focal epilepsy. Diagnostic features of CTE were not detected in any patient; however, the pathological evaluation for CTE was limited to a surgical specimen. The prominent and excessive tau deposition in 23 patients (38%) is abnormal in this age group and warrants further investigation.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1111/epi.16375DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6973032PMC
December 2019

Glial Fibrillary Acidic Protein (GFAP) Autoimmunity in the Setting of Seropositive Rheumatoid Arthritis Treated With Etanercept.

Neurologist 2019 Sep;24(5):152-154

Department of Neurology.

Introduction: Glial fibrillary acidic protein (GFAP) immunoglobulin G is a recently discovered biomarker of an autoimmune central nervous system disorder characterized by a steroid-responsive meningoencephalomyelitis.

Case Report: A 63-year-old man with rheumatoid arthritis on etanercept presented with steroid-responsive subacute encephalopathy and foot drop. Brain and sural nerve biopsies demonstrated a T-cell perivascular infiltrate. Cerebrospinal fluid studies 18 months into the course of the illness demonstrated a GFAP antibody on mouse tissue immunofluorescence confirmed by cell-based assay. The patient was treated with steroids and cyclophosphamide leading to resolution of his symptoms.

Conclusion: This case expands on the previously reported cases of GFAP immunoglobulin G autoimmunity by describing an associated inflammatory large fiber peripheral neuropathy.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1097/NRL.0000000000000243DOI Listing
September 2019

Late-onset Lennox-Gastaut syndrome: Diagnostic evaluation and outcome.

Neurol Clin Pract 2018 Oct;8(5):397-402

Department of Neurology, Mayo Clinic, Rochester, MN.

Background: We describe the clinical features and outcome in patients with late-onset Lennox-Gastaut syndrome (LGS).

Methods: Adult patients evaluated between January 1, 2000, and March 1, 2017, who presented with onset of LGS ≥10 years were identified. Data abstracted included age at seizure onset, seizure types, etiology, treatments, EEG and neuroimaging results, cerebrospinal fluid (CSF) findings, and autoimmune evaluation.

Results: Ten patients (8 females) were identified. The mean age at onset of seizures consistent with LGS was 16.5 years (range, 10-32 years). Seizure types included tonic, atonic, and tonic-clonic seizures (all), myoclonic seizures (n = 3), and atypical absence seizures (n = 7). Five patients had normal intellectual function at onset. Prolonged video-EEG monitoring recorded seizures and generalized interictal epileptiform discharges in all. All patients had drug-resistant epilepsy (range of antiseizure drugs tried, 7-16). Two patients had a history of intrathecal methotrexate to treat acute lymphoblastic leukemia. Two patients had malformations of cortical development. CSF analysis (n = 5) showed a mild elevation in the protein level without other abnormalities. Autoantibody determinations in the serum (n = 4) or the CSF (n = 5) and genetic testing (n = 5) were negative. At final follow-up, all but 1 patient was disabled and required a caregiver, and none were driving. One patient died of probable sudden unexpected death in epilepsy (SUDEP).

Conclusions: Late-onset LGS represents a rare, treatment-resistant generalized epilepsy that is disabling and may be associated with progressive cognitive impairment. The elevated CSF protein level in our cohort could have been due to high seizure burden but increases the possibility of an inflammatory component to the pathophysiology of this disorder.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1212/CPJ.0000000000000527DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6276343PMC
October 2018

Pseudo in Narcolepsy Type 1.

J Clin Sleep Med 2018 09 15;14(9):1625-1627. Epub 2018 Sep 15.

Mayo Clinic, Rochester, Minnesota.

Abstract: The rare constellation of multiple episodes of cataplexy that are refractory to therapy is called . We describe a young adult with known narcolepsy type 1 who reported a marked exacerbation of her symptoms that was initially suspicious for . Neurological evaluation, polysomnography, and additional history revealed that the spells were not consistent with cataplexy, but rather with a somatoform disorder. The case highlights the importance of considering psychosomatic factors in narcolepsy type 1 when presumed cataplexy remains suboptimally controlled despite adequate dosing with appropriate medications.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.5664/jcsm.7350DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6134240PMC
September 2018

Jeavons Syndrome: Clinical Features and Response to Treatment.

Pediatr Neurol 2018 09 10;86:46-51. Epub 2018 Jul 10.

Department of Neurology, Mayo Clinic, Rochester, Minnesota. Electronic address:

Background: Jeavons syndrome is an underreported epileptic syndrome characterized by eyelid myoclonia, eyelid closure-induced seizures or electroencephalography paroxysms, and photosensitivity. Drug-resistant epilepsy is common, but the prognostic factors and clinical course leading to drug resistance have not been well characterized.

Methods: We identified 30 patients who met the diagnostic criteria of Jeavons syndrome at a single institution between January 1, 2000 and December 15, 2016. Criteria for Jeavons syndrome included all of the following: (1) eyelid myoclonia with or without absences, (2) eye-closure-induced seizures or electroencephalography paroxysms, and (3) seizure onset after 12 months of age. We reviewed and described the epilepsy history, antiepileptic drug trials, and response to treatments.

Results: Mean age at seizure onset was 7.3 years, and 80% were female. Absence seizures (63%) and generalized tonic-clonic seizures (23%) were most common at onset. Diagnosis was delayed by an average of 9.6 years. After a median follow-up of two years, 80% of patients had drug resistant epilepsy and 70% experienced generalized tonic-clonic seizures. Generalized tonic-clonic seizures and seizure types other than absence seizures increased the risk of drug-resistant epilepsy (P values 0.049 and 0.03, respectively). Valproic acid, lamotrigine, ethosuximide, and levetiracetam were the most effective in reducing seizures by more than 50%.

Conclusions: The diagnosis of Jeavons syndrome is often delayed. Generalized tonic-clonic seizures and seizure types other than absence seizures may be predictors of drug-resistant epilepsy among patients with Jeavons syndrome.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.pediatrneurol.2018.06.001DOI Listing
September 2018