Publications by authors named "Nicholas L Zalewski"

36 Publications

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

Epilepsia 2021 Mar 25. 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.
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http://dx.doi.org/10.1111/epi.16888DOI Listing
March 2021

Clinical spectrum of high-titre GAD65 antibodies.

J Neurol Neurosurg Psychiatry 2021 Feb 9. Epub 2021 Feb 9.

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

Objective: To determine clinical manifestations, immunotherapy responsiveness and outcomes of glutamic acid decarboxylase-65 (GAD65) neurological autoimmunity.

Methods: We identified 323 Mayo Clinic patients with high-titre (>20 nmol/L in serum) GAD65 antibodies out of 380 514 submitted anti-GAD65 samples (2003-2018). Patients classified as having GAD65 neurological autoimmunity after chart review were analysed to determine disease manifestations, immunotherapy responsiveness and predictors of poor outcome (modified Rankin score >2).

Results: On review, 108 patients were classified as not having GAD65 neurological autoimmunity and 3 patients had no more likely alternative diagnoses but atypical presentations (hyperkinetic movement disorders). Of remaining 212 patients with GAD65 neurological autoimmunity, median age at symptom onset was 46 years (range: 5-83 years); 163/212 (77%) were female. Stiff-person spectrum disorders (SPSD) (N=71), cerebellar ataxia (N=55), epilepsy (N=35) and limbic encephalitis (N=7) could occur either in isolation or as part of an overlap syndrome (N=44), and were designated core manifestations. Cognitive impairment (N=38), myelopathy (N=23) and brainstem dysfunction (N=22) were only reported as co-occurring phenomena, and were designated secondary manifestations. Sustained response to immunotherapy ranged from 5/20 (25%) in epilepsy to 32/44 (73%) in SPSD (p=0.002). Complete immunotherapy response occurred in 2/142 (1%). Cerebellar ataxia and serum GAD65 antibody titre >500 nmol/L predicted poor outcome.

Interpretation: High-titre GAD65 antibodies were suggestive of, but not pathognomonic for GAD65 neurological autoimmunity, which has discrete core and secondary manifestations. SPSD was most likely to respond to immunotherapy, while epilepsy was least immunotherapy responsive. Complete immunotherapy response was rare. Serum GAD65 antibody titre >500 nmol/L and cerebellar ataxia predicted poor outcome.
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http://dx.doi.org/10.1136/jnnp-2020-325275DOI Listing
February 2021

Vascular Myelopathies.

Continuum (Minneap Minn) 2021 02;27(1):30-61

Purpose Of Review: Neurologists should be able to identify clinical and neuroimaging features that distinguish vascular disorders from other causes of myelopathy.

Recent Findings: Although certain clinical features suggest a vascular etiology in acute and chronic myelopathy settings, accurate MRI interpretation within the clinical context is key. Recent studies have shown vascular myelopathies are frequently misdiagnosed as transverse myelitis, and recognition of this diagnostic pitfall is important. Many different vascular mechanisms can cause myelopathy; this article provides a comprehensive review that simplifies disease categories into arterial ischemia, venous congestion/ischemia, hematomyelia, and extraparenchymal hemorrhage.

Summary: It is important to recognize and manage vascular disorders of the spinal cord as significant causes of acute, subacute, and progressive myelopathy.
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http://dx.doi.org/10.1212/CON.0000000000000905DOI Listing
February 2021

Spinal cord transient ischemic attack: Insights from a series of spontaneous spinal cord infarction.

Neurol Clin Pract 2020 Dec;10(6):480-483

Department of Neurology (SWE), Emory University, Atlanta, GA; and Department of Neurology (AAR, EPF, NLZ), Mayo Clinic, Rochester, MN.

Objective: To define the prevalence and characteristics of spinal cord transient ischemic attack (sTIA) in a large retrospective series of patients who met diagnostic criteria for spontaneous spinal cord infarction (SCI).

Methods: An institution-based search tool was used to identify patients evaluated at the Mayo Clinic in Rochester, MN, from 1997 to 2017 with spontaneous SCI (n = 133). Cases were subsequently reviewed for transient myelopathic symptoms preceding infarction that were suspected ischemic in nature. We performed a descriptive analysis of patients with sTIA before SCI.

Results: Of 133 patients with a diagnosis of spontaneous SCI, we identified 4 patients (3%) who experienced sTIA before SCI. The median age at presentation was 61.5 years (range 46-75 years), 2 (50%) were women, and 3 (75%) had traditional vascular risk factors. Localization was cervical cord in 2 cases (50%) and thoracic cord in 2 cases (50%); all patients developed SCI in the same distribution as their preceding sTIA symptoms. All patients experienced recurrent sTIA before SCI. Symptoms ranged from seconds to a few minutes before returning to baseline. No patients had pain as a feature of sTIA.

Conclusions: sTIAs are possible but rare in patients who subsequently have a SCI. Clinical features are similar to those of SCI, with rapid onset of severe myelopathic deficits, followed by prompt resolution. Vascular risk factors are common in these patients. Thus, recognition of a sTIA may represent a valuable opportunity for vascular risk factor modification and stroke prevention. However, given the rarity, physicians should explore other possible explanations when sTIA is considered.
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http://dx.doi.org/10.1212/CPJ.0000000000000778DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7837427PMC
December 2020

Serum Neurofilament to Magnetic Resonance Imaging Lesion Area Ratio Differentiates Spinal Cord Infarction From Acute Myelitis.

Stroke 2021 Jan 11;52(2):645-654. Epub 2021 Jan 11.

Departments of Neurology (E.S., A.M., S.J.P., E.P.F., A.A.R., D.M.N., N.L.Z.), Mayo Clinic, Rochester.

Background And Purpose: The diagnosis of spontaneous spinal cord infarction (SCI) is limited by the lack of diagnostic biomarkers and MRI features that often overlap with those of other myelopathies, especially acute myelitis. We investigated whether the ratio between serum neurofilament light chain levels and MRI T2-lesion area (neurofilament light chain/area ratio-NAR) differentiates SCI from acute myelitis of similar severity.

Methods: We retrospectively identified Mayo Clinic patients (January 1, 2000-December 31, 2019) with (1) SCI, (2) AQP4 (aquaporin 4)-IgG or MOG (myelin oligodendrocyte glycoprotein)-IgG-associated myelitis at disease clinical presentation, or (3) idiopathic transverse myelitis from a previously identified population-based cohort of patients seronegative for AQP4-IgG and MOG-IgG. Serum neurofilament light chain levels (pg/mL) were assessed at the Verona University (SIMOA, Quanterix) in a blinded fashion on available stored samples obtained ≤3 months from myelopathy presentation. For each patient, the largest spinal cord lesion area (mm) was manually outlined by 2 independent raters on sagittal T2-weighted MRI images, and the mean value was used to determine NAR (pg/[mL·mm]).

Results: Forty-eight patients were included SCI, 20 (definite, 11; probable, 6; possible, 3); acute myelitis, 28 (AQP4-IgG-associated, 17; MOG-IgG-associated, 5; idiopathic transverse myelitis, 6). The median expanded disability status scale score (range) at myelopathy nadir were 7.75 (2-8.5) and 5.5 (2-8), respectively. Serum neurofilament light chain levels (median [range] pg/mL) in patients with SCI (188 [14.3-2793.4]) were significantly higher compared with patients with AQP4-IgG-associated myelitis (37 [0.8-6942.9]), MOG-IgG-associated myelitis (45.8 [4-283.8]), and idiopathic transverse myelitis (15.6 [0.9-217.8]); =0.01. NAR showed the highest accuracy for identification of SCI versus acute myelitis with values ≥0.35 pg/(mL·mm) yielding 86% specificity and 95% sensitivity (area under the curve=0.93). The positive and negative likelihood ratios were 6.67 and 0.06, respectively. NAR remained independently associated with SCI after adjusting for age, gender, immunotherapy before sampling, and days from myelopathy symptoms onset to sampling (=0.0007).

Conclusions: NAR is a novel and promising clinical biomarker for differentiation of SCI from acute myelitis.
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http://dx.doi.org/10.1161/STROKEAHA.120.031482DOI Listing
January 2021

Brainstem and cerebellar involvement in MOG-IgG-associated disorder versus aquaporin-4-IgG and MS.

J Neurol Neurosurg Psychiatry 2020 Dec 28. Epub 2020 Dec 28.

Neurology, Mayo Clinic, Rochester, Minnesota, USA

Objective: To determine the frequency and characteristics of brainstem or cerebellar involvement in myelin-oligodendrocyte-glycoprotein-antibody-associated-disorder (MOGAD) versus aquaporin-4-IgG-seropositive-neuromyelitis optica spectrum disorder (AQP4-IgG-NMOSD) and multiple sclerosis (MS).

Methods: In this observational study, we retrospectively identified 185 Mayo Clinic MOGAD patients with: (1) characteristic MOGAD phenotype, (2) MOG-IgG seropositivity by live cell-based assay and (3) MRI lesion(s) of brainstem, cerebellum or both. We compared the symptomatic attacks to AQP4-IgG-NMOSD (n=30) and MS (n=30).

Results: Brainstem or cerebellar involvement occurred in 62/185 (34%) MOGAD patients of which 39/62 (63%) were symptomatic. Ataxia (45%) and diplopia (26%) were common manifestations. The median age in years (range) in MOGAD of 24 (2-65) was younger than MS at 36 (16-65; p=0.046) and AQP4-IgG-NMOSD at 45 (6-72; p=0.006). Isolated attacks involving the brainstem, cerebellum or both were less frequent in MOGAD (9/39 (23%)) than MS (22/30 (73%); p<0.001) but not significantly different from AQP4-IgG-NMOSD (14/30 (47%); p=0.07). Diffuse middle cerebellar peduncle MRI-lesions favoured MOGAD (17/37 (46%)) over MS (3/30 (10%); p=0.001) and AQP4-IgG-NMOSD (3/30 (10%); p=0.001). Diffuse medulla, pons or midbrain MRI lesions occasionally occurred in MOGAD and AQP4-IgG-NMOSD but never in MS. Cerebrospinal fluid (CSF) oligoclonal bands were rare in MOGAD (5/30 (17%)) and AQP4-IgG-NMOSD (2/22 (9%); p=0.68) but common in MS (18/22 (82%); p<0.001). Disability at nadir or recovery did not differ between the groups.

Conclusion: Involvement of the brainstem, cerebellum or both is common in MOGAD but usually occurs as a component of a multifocal central nervous system attack rather than in isolation. We identified clinical, CSF and MRI attributes that can help discriminate MOGAD from AQP4-IgG-NMOSD and MS.
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http://dx.doi.org/10.1136/jnnp-2020-325121DOI Listing
December 2020

In NPH, setting valve opening pressure close to lumbar puncture opening pressure decreases overdrainage.

Neurol Neurochir Pol 2020 13;54(6):531-537. Epub 2020 Oct 13.

Mayo Clinic, 4500 san pablo rd, 32224 Jacksonville, United States.

Background: The management of normal pressure hydrocephalus (NPH) can be difficult, partly because there are frequent treatment complications such as overdrainage which, when serious, may require surgical intervention. We previously reported a correlation between the difference of lumbar puncture opening pressure minus the valve opening pressure setting (LPOP-VOP) (which we refer to as the delta) and increased rates of overdrainage. This led to a modification in our practice, whereby we now set the VOP equal to, or close to, the LPOP, resulting in lower deltas.

Objective: In this new study, our aim was to compare the rate of overdrainage in our patients with higher and lower deltas and assess the significance of setting the VOP equal, or close, to the patient's LPOP.

Methods: 1. We reproduced the association between delta and overdrainage. 2. We compared the incidence of overdrainage in those whose VOP was set close to LPOP (low delta) versus those with VOP setting distant from the LPOP (higher delta). 3. We compared symptom improvement in those with a low versus higher delta.

Results: We confirmed the relation between high delta and an increased rate of overdrainage, lower rates of overdrainage in those whose VOP was set close to the LPOP (Delta Adjusted Practice), and better improvement of symptoms when the VOP was set closer to the LPOP.

Conclusion: We propose that the initial VOP should be set as close as possible to the patient's LPOP to decrease overdrainage without compromising symptom improvement.
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http://dx.doi.org/10.5603/PJNNS.a2020.0077DOI Listing
January 2021

Striatal Encephalitis: Potential Inflammatory Vasculopathy in Systemic Lupus Erythematosus.

Can J Neurol Sci 2020 Sep 11:1-2. Epub 2020 Sep 11.

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

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http://dx.doi.org/10.1017/cjn.2020.198DOI Listing
September 2020

Sea Fan Frond Neovascularization: A Unique Finding in a Patient With Susac Syndrome.

J Neuroophthalmol 2020 Aug 19. Epub 2020 Aug 19.

Departments of Ophthalmology (SAM, MH, TWO, MTB, JJC); and Neurology (NLZ, FA, MTB, JJC), Mayo Clinic College of Medicine, Rochester, Minnesota.

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http://dx.doi.org/10.1097/WNO.0000000000001037DOI Listing
August 2020

Longitudinally Extensive Spinal Cord Lesion in Erdheim-Chester Disease.

JAMA Neurol 2020 Jul 27. Epub 2020 Jul 27.

Department of Neurology, Mayo Clinic, Rochester, Minnesota.

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http://dx.doi.org/10.1001/jamaneurol.2020.2460DOI Listing
July 2020

Use of diffusion-weighted imaging to distinguish seizure-related change from limbic encephalitis.

J Neurol 2020 Nov 24;267(11):3337-3342. Epub 2020 Jun 24.

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

Objective: To determine whether diffusion-weighted imaging (DWI) can help differentiate peri-ictal signal abnormality from limbic encephalitis (LE) among patients with medial temporal lobe T2-hyperintensity.

Methods: We retrospectively identified patients with peri-ictal medial temporal lobe T2-hyperintensity using a Mayo Clinic database, and reviewed their DWI to look for unique diffusion restriction patterns. We then identified patients with medial temporal lobe T2-hyperintensity and LE, and reviewed their DWI to see if these patterns were ever present. Presence of diffusion restriction patterns was confirmed by a blinded neuro-radiologist.

Results: We identified 10 patients without LE who had peri-ictal unilateral medial temporal lobe T2-hyperintensity, ipsilateral to focal seizure onset. Nine of 10 (90%) had at least one of two diffusion restriction patterns potentially unique to seizure activity; four had gyriform hippocampal diffusion restriction ("Pattern 1"), three had diffuse hippocampal diffusion restriction that spared the most medial temporal lobe structures ("Pattern 2"), and two had both diffusion restriction patterns. The median patient age was 62 years (range 2-76 years) and 3/9 (33%) were female. In comparison, among patients with medial temporal lobe T2-hyperintensity and LE, only 5/57 (9%) had one of the diffusion restriction patterns ("Pattern 2") identified (P < 0.0001); all five had seizures reported.

Conclusions: In patients with medial temporal lobe T2-hyperintensity and one of the diffusion restriction patterns described herein, the signal abnormality may be a peri-ictal phenomenon rather than indicative of LE and should prompt investigation for seizure. Even in patients with LE, these patterns should raise concern for seizure.
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http://dx.doi.org/10.1007/s00415-020-10007-1DOI Listing
November 2020

Frequency and characteristics of MRI-negative myelitis associated with MOG autoantibodies.

Mult Scler 2021 02 27;27(2):303-308. Epub 2020 Feb 27.

Department of Neurology, Mayo Clinic, Rochester, MN, USA/Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN, USA.

Background: Myelitis accompanied by a negative spinal cord MRI may lead to diagnostic uncertainty.

Objective And Methods: We retrospectively investigated the frequency of negative spinal cord MRI (performed <6 weeks from onset) in Mayo Clinic patients with myelin oligodendrocyte glycoprotein (MOG)-IgG-associated myelitis (2000-2019).

Results: The initial spinal cord MRI was negative in 7/73 (10%) patients, despite severe acute disability (median EDSS, 7 (range, 4.5-8)); myelitis symptoms/signs were frequent (paraparesis, neurogenic bladder, sensory level, Lhermitte's phenomenon). Myelitis lesions became overt at follow-up MRI in three patients.

Conclusions: A negative spinal cord MRI should not dissuade from MOG-IgG testing in patients with acute/subacute myelitis.
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http://dx.doi.org/10.1177/1352458520907900DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7500857PMC
February 2021

Clinical Reasoning: A 70-year-old man with rapid stepwise paraparesis and sensory loss.

Neurology 2020 02 28;94(6):e651-e655. Epub 2020 Jan 28.

From the Department of Neurology, Mayo Clinic, Rochester, MN.

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http://dx.doi.org/10.1212/WNL.0000000000008925DOI Listing
February 2020

MOG-IgG myelitis coexisting with systemic lupus erythematosus in the post-partum setting.

Mult Scler 2020 07 17;26(8):997-1000. Epub 2019 Oct 17.

Department of Neurology, College of Medicine, Mayo Clinic, Rochester, MN, USA.

Background: Longitudinally extensive transverse myelitis (LETM) accompanying systemic lupus erythematosus (SLE) is often due to coexisting aquaporin-4-IgG seropositive neuromyelitis optica spectrum disorder but has not been associated with myelin oligodendrocyte glycoprotein-IgG (MOG-IgG).

Objective And Methods: Case report at an academic medical center.

Results: A 32-year-old woman developed severe transverse myelitis (paraplegia) shortly after SLE onset in the post-partum period. Magnetic resonance imaging (MRI) revealed an LETM, cerebrospinal fluid showed marked inflammation, and testing for infections was negative. Serum live-cell-based assay for MOG-IgG was positive but aquaporin-4-IgG was negative.

Conclusion: In patients with SLE and LETM, MOG-IgG testing should be considered, in addition to AQP4-IgG.
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http://dx.doi.org/10.1177/1352458519872895DOI Listing
July 2020

Spinal cord infarction in a patient with multiple sclerosis.

Mult Scler Relat Disord 2019 Nov 7;36:101435. Epub 2019 Oct 7.

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

We describe a 49 year old woman with relapsing-remitting multiple sclerosis (MS) with a suspected severe recurrent attack of myelitis that was ultimately diagnosed as a spinal cord infarction (SCI). This case of SCI in a patient with an established diagnosis of MS highlights the clinical, laboratory, and radiographic characteristics that help distinguish SCI from inflammatory myelitis due to MS.
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http://dx.doi.org/10.1016/j.msard.2019.101435DOI Listing
November 2019

Aquaporin-4 and MOG autoantibody discovery in idiopathic transverse myelitis epidemiology.

Neurology 2019 07 24;93(4):e414-e420. Epub 2019 Jun 24.

From the Departments of Neurology (E.S., E.S., S.J.P., B.G.W., B.M.K., N.L.Z., A.S.L.-C., E.P.F.), Health Sciences Research (J.P.W.), and Laboratory Medicine and Pathology (S.J.P., J.J., E.P.F.), Mayo Clinic College of Medicine, Rochester, MN.

Objective: Diagnostic criteria from 2002 classify transverse myelitis (TM) as idiopathic or disease associated but predate the discovery of aquaporin-4 (AQP4)-immunoglobulin G (IgG) and myelin oligodendrocyte glycoprotein (MOG)-IgG, which associate with TM. Prior incidence estimates of idiopathic TM (ITM) range from 1 to 6.2 per 1 million. We sought to determine whether the population-based incidence and prevalence of ITM were reduced by testing patients with ITM for AQP4/MOG-IgG and reclassifying seropositive cases as having disease-associated TM.

Methods: For this observational study, we retrospectively identified all cases of incident (January 1, 2003-December 31, 2016) and prevalent (December 31, 2016) ITM in Olmsted County (85% white) by using the Rochester Epidemiology Project medical records linkage system. ITM was defined by the 2002 Transverse MyelitisConsortium Working Group diagnostic criteria. Available sera were tested for AQP4-IgG and MOG-IgG.

Results: Twenty-four patients (incident 22, prevalent 17) initially met 2002 ITM criteria (longitudinally extensive TM [LETM] 6). Sera were tested for AQP4-IgG in 22 of 24 (92%) and MOG-IgG in 21 of 24 (88%). Three seropositive cases (AQP4-IgG 2, MOG-IgG 1) were identified and reclassified as having disease-associated TM, accounting for 14% of total incident and 12% of total prevalent cases. AQP4-IgG and MOG-IgG seropositive cases represented 50% (3 of 6) of idiopathic LETM. After reclassification of seropositive patients, the final ITM incidence was 8.6 per 1,000,000 and prevalence was 7.9 per 100,000. Three cases of ITM (14%) subsequently fulfilled multiple sclerosis criteria within the study period.

Conclusions: The availability of AQP4-IgG and MOG-IgG modestly reduced ITM incidence and prevalence, which remained higher than previously reported in this predominantly white population. Incorporation of these biomarkers into future revisions of TM diagnostic criteria should be considered.
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http://dx.doi.org/10.1212/WNL.0000000000007828DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7508328PMC
July 2019

Acute truncal ataxia in a healthy adult with varicella zoster virus cerebellitis: A case report and literature review.

J Neurol Sci 2019 05 2;400:186-187. Epub 2019 Apr 2.

Department of Neurology, Mayo Clinic, Rochester, MN 55905, USA.

Acute cerebellitis is a well recognized complication of varicella zoster virus (VZV) infection in children. It has been described in adults in the setting of virus reactivation with a preceding herpes zoster rash, but it is exceedingly rare in adults who are not elderly or immunocompromised, particularly in the absence of a rash. To our knowledge, there has been only one reported case of acute cerebellitis in an immunocompetent adult less than age 65 with virological confirmation of acute VZV infection. We describe a 59-year-old immunocompetent man who presented with acute truncal ataxia without rash and was diagnosed with VZV cerebellitis, supported by anti-VZV IgM and anti-VZV IgG antibodies in the serum and a positive VZV polymerase chain reaction in cerebrospinal fluid. He had robust improvement with intravenous acyclovir treatment and was free of neurologic disability at two month follow-up. This case highlights the importance of virological evaluation in patients with acute ataxia, even in the absence of typical features of infection.
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http://dx.doi.org/10.1016/j.jns.2019.04.001DOI Listing
May 2019

Clinical, Radiologic, and Prognostic Features of Myelitis Associated With Myelin Oligodendrocyte Glycoprotein Autoantibody.

JAMA Neurol 2019 03;76(3):301-309

Department of Neurology, Mayo Clinic College of Medicine, Rochester, Minnesota.

Importance: Recognizing the characteristics of myelin oligodendrocyte glycoprotein autoantibody (MOG-IgG) myelitis is essential for early accurate diagnosis and treatment.

Objective: To evaluate the clinical, radiologic, and prognostic features of MOG-IgG myelitis and compare with myelitis with aquaporin-4-IgG (AQP4-IgG) and multiple sclerosis (MS).

Design, Setting, And Participants: We retrospectively identified 199 MOG-IgG-positive Mayo Clinic patients from January 1, 2000, through December 31, 2017, through our neuroimmunology laboratory. Fifty-four patients met inclusion criteria of (1) clinical myelitis; (2) MOG-IgG positivity; and (3) medical records available. We excluded 145 patients without documented myelitis. Myelitis of AQP4-IgG (n = 46) and MS (n = 26) were used for comparison.

Main Outcomes And Measures: Outcome variables included modified Rankin score and need for gait aid. A neuroradiologist analyzed spine magnetic resonance imaging of patients with MOG-IgG and control patients blinded to diagnosis.

Results: Of 54 included patients with MOG-IgG myelitis, the median age was 25 years (range, 3-73 years) and 24 were women (44%). Isolated transverse myelitis was the initial manifestation in 29 patients (54%), and 10 (19%) were initially diagnosed as having viral/postviral acute flaccid myelitis. Cerebrospinal fluid-elevated oligoclonal bands occurred in 1 of 38 (3%). At final follow-up (median, 24 months; range, 2-120 months), 32 patients (59%) had developed 1 or more relapses of optic neuritis (n = 31); transverse myelitis (n = 7); or acute disseminated encephalomyelitis (n = 1). Clinical features favoring MOG-IgG myelitis vs AQP4-IgG or MS myelitis included prodromal symptoms and concurrent acute disseminated encephalomyelitis. Magnetic resonance imaging features favoring MOG-IgG over AQP4-IgG or MS myelitis were T2-signal abnormality confined to gray matter (sagittal line and axial H sign) and lack of enhancement. Longitudinally extensive T2 lesions were of similar frequency in MOG-IgG and AQP4-IgG myelitis (37 of 47 [79%] vs 28 of 34 [82%]; P = .52) but not found in MS. Multiple spinal cord lesions and conus involvement were more frequent with MOG-IgG than AQP4-IgG but not different from MS. Wheelchair dependence at myelitis nadir occurred in one-third of patients with MOG-IgG and AQP4-IgG but never with MS, although patients with MOG-IgG myelitis recovered better than those with AQP4-IgG.

Conclusions And Relevance: Myelitis is an early manifestation of MOG-IgG-related disease and may have a clinical phenotype of acute flaccid myelitis. We identified a variety of clinical and magnetic resonance imaging features that may help clinicians identify those at risk in whom MOG-IgG should be tested.
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http://dx.doi.org/10.1001/jamaneurol.2018.4053DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6440233PMC
March 2019

Characteristics of Spontaneous Spinal Cord Infarction and Proposed Diagnostic Criteria.

JAMA Neurol 2019 01;76(1):56-63

Department of Neurology, Mayo Clinic, Rochester, Minnesota.

Importance: Spinal cord infarction (SCI) is often disabling, and the diagnosis can be challenging without an inciting event (eg, aortic surgery). Patients with a spontaneous SCI are often misdiagnosed as having transverse myelitis. Diagnostic criteria for SCI are lacking, hindering clinical care and research.

Objective: To describe the characteristics of spontaneous SCI and propose diagnostic criteria.

Design, Setting, And Participants: An institution-based search tool was used to identify patients evaluated at Mayo Clinic, Rochester, Minnesota, from January 1997 to December 2017 with a spontaneous SCI. Patients provided written consent to use their records for research. Participants were 18 years and older with a diagnosis of spontaneous SCI (n = 133), and controls were selected from a database of alternative myelopathy etiologies for validation of the proposed diagnostic criteria (n = 280).

Main Outcomes And Measures: A descriptive analysis of SCI was performed and used to propose diagnostic criteria, and the criteria were validated.

Results: Of 133 included patients with a spontaneous SCI, the median (interquartile range) age at presentation was 60 (52-69) years, and 101 (76%) had vascular risk factors. Rapid onset of severe deficits reaching nadir within 12 hours was typical (102 [77%]); some had a stuttering decline (31 [23%]). Sensory loss occurred in 126 patients (95%), selectively affecting pain/temperature in 49 (39%). Initial magnetic resonance imaging (MRI) spine results were normal in 30 patients (24%). Characteristic MRI T2-hyperintense patterns included owl eyes (82 [65%]) and pencil-like hyperintensity (50 [40%]); gadolinium enhancement (37 of 96 [39%]) was often linear and located in the anterior gray matter. Confirmatory MRI findings included diffusion-weighted imaging/apparent diffusion coefficient restriction (19 of 29 [67%]), adjacent dissection/occlusion (16 of 82 [20%]), and vertebral body infarction (11 [9%]). Cerebrospinal fluid showed mild inflammation in 7 of 89 patients (8%). Diagnostic criteria was proposed for definite, probable, and possible SCI of periprocedural and spontaneous onset. In the validation cohort (n = 280), 9 patients (3%) met criteria for possible SCI, and none met criteria for probable SCI.

Conclusions And Relevance: This large series of spontaneous SCIs provides clinical, laboratory, and MRI clues to SCI diagnosis. The diagnostic criteria proposed here will aid clinicians in making the correct diagnosis and ideally improve future care for patients with SCI. The validation of these criteria supports their utility in the evaluation of acute myelopathy.
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http://dx.doi.org/10.1001/jamaneurol.2018.2734DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6440254PMC
January 2019

At the Intersection of Patient Experience Data, Outcomes Research, and Practice: Analysis of HCAHPS Scores in Neurology Patients.

Mayo Clin Proc Innov Qual Outcomes 2018 Jun 24;2(2):137-147. Epub 2018 May 24.

Robert D. and Patricia E. Kern Center for the Science of Health Care Delivery, Mayo Clinic, Rochester, MN.

Objective: To assess variation in patient-reported experience in inpatient neurology patients.

Patients And Methods: We retrospectively identified 1045 patients 18 years and older admitted to a neurology service and discharged from January 1, 2013, through September 30, 2016, who completed Hospital Consumer Assessment of Healthcare Providers and Systems (HCAHPS) surveys. Multivariable logistic regression evaluated the associations of patient factors with HCAHPS measures. Key driver analysis identified associations between HCAHPS measures and the Global score (combination of 0-10 hospital rating and likelihood to recommend). Multivariable logistic regression compared HCAHPS scores between neurology patients and those admitted to a neurosurgery (n=2190) or internal medicine (n=3401) service during the same period.

Results: Among patients admitted to a neurology service, overall (summary) scores did not vary significantly by diagnosis after adjustment for age, education, and overall health, but patients with neurologic diagnoses other than stroke, epilepsy, and neurodegenerative disease were more likely to report lower Pain Management scores compared with patients with cancer. Key driver analysis showed Care Transition scores as drivers of the Global score. After adjustment, general internal medicine service patients were more likely to report low Summary scores and neurosurgery service patients were significantly less likely to report low Summary scores compared with neurology service patients.

Conclusion: Efforts to improve how neurology patients experience their care should be aimed at targeting patients' perceptions of pain management, and improving care transitions is an important first-priority target for improvement. This analysis may help other institutions improve hospital rating, value-based payments, and patient-centered outcomes.
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http://dx.doi.org/10.1016/j.mayocpiqo.2018.03.007DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6124338PMC
June 2018

Unique Gadolinium Enhancement Pattern in Spinal Dural Arteriovenous Fistulas.

JAMA Neurol 2018 12;75(12):1542-1545

Department of Neurology, Mayo Clinic, Rochester, Minnesota.

Importance: Spinal dural arteriovenous fistula (sDAVF) is often misdiagnosed as an inflammatory or a neoplastic myelopathy, often because of intraparenchymal gadolinium enhancement on magnetic resonance imaging (MRI); proper early diagnosis is important because deficits are reversible and a delay in treatment is associated with permanent morbidity. Tortuous flow voids on MRI are not universally present; thus, recognition of a unique gadolinium enhancement pattern may also aid in the early recognition and treatment of sDAVF.

Objective: To describe a unique pattern of spinal cord gadolinium enhancement on MRI in sDAVF.

Design, Setting, And Participants: This retrospective evaluation included pretreatment MRIs from 80 patients referred to the Mayo Clinic, Rochester, Minnesota, from January 1, 1997, through December 31, 2017, with a confirmed diagnosis of sDAVF and a control group of 144 patients with alternative confirmed myelopathy diagnoses. All participants underwent a neurologic evaluation at the Mayo Clinic.

Main Outcomes And Measures: Evidence of at least 1 focal geographic nonenhancing area within a long segment of intense holocord gadolinium enhancement (termed the missing-piece sign) on MRI.

Results: Of 51 patients with an sDAVF and a pretreatment MRI with gadolinium enhancement, 44 (86%) had intraparenchymal contrast enhancement, and 19 of these patients (43%) displayed the characteristic missing-piece sign. Of these 19 patients, symptom onset occurred at a median age of 67 years (range, 27-80 years); 15 patients were men. Progressive myelopathy features affecting the lower extremities occurred during a median of 33 months (range, 1-84 months). Eleven patients (58%) received an alternative diagnosis before confirmation of sDAVF. Tortuous flow voids were present on T2-weighted MRI in 13 of 19 patients. More than 1 digital subtraction angiogram was required for 5 patients to confirm the diagnosis. The missing-piece sign was not seen in any patients from the control group.

Conclusions And Relevance: This unique gadolinium enhancement pattern in sDAVF was not found in a large control group of patients with other myelopathy. Identifying the missing-piece sign on MRI could potentially result in earlier time to angiography with improved outcomes for patients with an sDAVF.
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http://dx.doi.org/10.1001/jamaneurol.2018.2605DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6583194PMC
December 2018

Autoimmune and Paraneoplastic Myelopathies.

Semin Neurol 2018 Jun 16;38(3):278-289. Epub 2018 Jul 16.

Department of Neurology, Mayo Clinic, Rochester, Minnesota.

Prompt recognition of an inflammatory myelopathy is critical, as a specific diagnosis and management plan allows for optimal patient outcomes. Many treatment options are now available for autoimmune and paraneoplastic myelopathies, but specific management strategies and expected prognosis vary widely depending on the underlying etiology. An understanding of the relevant clinical details, imaging findings, and other diagnostic information that can help achieve a specific myelopathy diagnosis and treatment plan is essential for all neurologists, given the variety of contexts in which myelopathies are encountered. We provide an outline of the diagnostic evaluation and treatment of various inflammatory myelopathies seen in autoimmune and paraneoplastic diseases, including multiple sclerosis, aquaporin-4 immunoglobulin G (IgG) seropositive neuromyelitis optica spectrum disorder, sarcoidosis, myelin oligodendrocyte glycoprotein IgG associated disease, and other rare inflammatory myelopathies; we also highlight common mimickers of inflammatory myelopathies.
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http://dx.doi.org/10.1055/s-0038-1660856DOI Listing
June 2018

Clinical Reasoning: A 30-year-old man with headache and sleep disturbance.

Neurology 2018 04;90(17):e1535-e1540

From the Department of Neurology, Mayo Clinic, Rochester, MN.

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http://dx.doi.org/10.1212/WNL.0000000000005356DOI Listing
April 2018

Spinal cord infarction: Clinical and imaging insights from the periprocedural setting.

J Neurol Sci 2018 05 17;388:162-167. Epub 2018 Mar 17.

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

Objective: Describe the range of procedures associated with spinal cord infarction (SCI) as a complication of a medical/surgical procedure and define clinical and imaging characteristics that could be applied to help diagnose spontaneous SCI, where the diagnosis is often less secure.

Methods: We used an institution-based search tool to identify patients evaluated at Mayo Clinic, Rochester, MN from 1997 to 2016 with a periprocedural SCI. We performed a descriptive analysis of clinical features, MRI and other laboratory findings, and outcome.

Results: Seventy-five patients were identified with SCI related to an invasive or non-invasive surgery including: aortic aneurysm repair (49%); other aortic surgery (15%); and a variety of other procedures (e.g., cardiac surgery, spinal decompression, epidural injection, angiography, nerve block, embolization, other vascular surgery, thoracic surgery) (36%). Deficits were severe (66% para/quadriplegia) and maximal at first post-procedural evaluation in 61 patients (81%). Impaired dorsal column function was common on initial examination. Imaging features included classic findings of owl eyes or anterior pencil sign on MRI (70%), but several other T2-hyperintensity patterns were also seen. Gadolinium enhancement of the SCI and/or cauda equina was also common when assessed. Six patients (10%) had an initial normal MRI despite a severe deficit.

Conclusions: Procedures associated with SCI are many, and this complication does not exclusively occur following aortic surgery. The clinical and radiologic findings that we describe with periprocedural SCI may be used in future studies to help distinguish spontaneous SCI from alternate causes of acute myelopathy.
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http://dx.doi.org/10.1016/j.jns.2018.03.029DOI Listing
May 2018

Evaluation of idiopathic transverse myelitis revealing specific myelopathy diagnoses.

Neurology 2018 01 15;90(2):e96-e102. Epub 2017 Dec 15.

From the Department of Neurology, Mayo Clinic, Rochester, MN.

Objective: To evaluate specific myelopathy diagnoses made in patients with suspected idiopathic transverse myelitis (ITM).

Methods: A total of 226 patients 18 years and older were referred to Mayo Clinic Neurology for suspected ITM from December 1, 2010, to December 31, 2015. Electronic medical records were reviewed for detailed clinical presentation and course, laboratory and electrophysiologic investigations, and neuroimaging to determine the etiology. Current diagnostic criteria for ITM and alternative myelopathy diagnoses were applied. All cases where any discrepancy was suspected from the final reported clinical diagnosis were reviewed by each author and a consensus final diagnosis was made.

Results: The diagnostic criteria for ITM were met in 41 of 226 patients (18.1%). In 158 patients (69.9%), an alternative specific myelopathy diagnosis was made: multiple sclerosis or clinically isolated syndrome, 75; vascular myelopathy, 41; neurosarcoidosis, 12; neuromyelitis optica spectrum disorder, 12; myelin oligodendrocyte glycoprotein myelopathy, 5; neoplastic, 4; compressive, 3; nutritional, 3; infectious, 2; and other, 2. A myelopathy was not confirmed in 27 patients. Time from symptom onset to final clinical diagnosis in patients without ITM was a median of 9 months (range 0-288). Fifty-five patients (24%) required treatment changes according to their final clinical diagnosis.

Conclusions: The majority of patients with suspected ITM have an alternative specific myelopathy diagnosis. A presumptive diagnosis of ITM can lead to premature diagnostic conclusions affecting patient treatment.
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http://dx.doi.org/10.1212/WNL.0000000000004796DOI Listing
January 2018

Breast cancer-related paraneoplastic neurologic disease.

Breast Cancer Res Treat 2018 02 7;167(3):771-778. Epub 2017 Nov 7.

Department of Surgery, Mayo Clinic, 200 First Street Southwest, Rochester, MN, 55905, USA.

Purpose: Paraneoplastic neurologic disease (PND) is an aberrant immune-mediated response against the nervous system triggered by malignancy. Given the rarity, a paucity of data describing breast cancer-related PND (BC-PND) exists; we sought to further examine this specific patient population.

Methods: We retrospectively identified patients at our institution from 1997 to 2016 with BC-PND. Retrospective review with a descriptive analysis determined factors associated with PND and BC, which were compared to national breast cancer median of age (61 years) and average stage at diagnosis (60% local disease).

Results: BC-PND was diagnosed in 56 female patients at a median age of 52.8 years. Only 20% of invasive cancer patients had local disease. The majority of patients were hormone receptor positive and Her2 negative. Neurological symptoms presented prior to BC diagnosis in 57.1% of patients. Of all patients, 30 (53.6%) had autoantibodies detected: Purkinje Cell Cytoplasmic Autoantibody Type-1 (PCA-1[anti-Yo]), n = 10; amphiphysin-IgG, n = 9; Anti-Neuronal Nuclear Autoantibody Type-2 (ANNA-2[anti-Ri]), n = 5; and others, n = 6. The most common neurologic findings were cerebellar ataxia, myelopathy, and myopathy. Immunotherapy benefit was found to be robust (21.6%), mild to moderate (52.9%), absent (17.6%), or indeterminate (7.8%).

Conclusions: PND symptoms often presented prior to BC diagnosis, with the BC biologic subtype characteristics typical of the general BC population. BC diagnoses were often made at younger ages than that of the general BC population and with later-stage disease. Roughly 75% of patients benefited from immunotherapy. These data provide helpful information to providers treating this population of patients.
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http://dx.doi.org/10.1007/s10549-017-4566-0DOI Listing
February 2018

CLIPPERS.

Curr Neurol Neurosci Rep 2017 Sep;17(9):65

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

Purpose Of Review: Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is a recently described treatable, inflammatory, brainstem predominant encephalomyelitis. The diagnosis of CLIPPERS is challenging without a specific biomarker, and thus it is important to consider if both the clinical and radiographic features are consistent with the diagnosis, or rather a disease mimicker.

Recent Findings: Many patients with CLIPPERS-like lesions have been described in the literature with follow-up revealing a range of alternative diagnoses, such as malignancies, vasculitis, and other specific inflammatory diseases. As a result, some have proposed that CLIPPERS might represent a pre-malignancy state or simply an initial clinical syndrome of a variety of possible etiologies. We describe the typical clinical, radiographic, and pathological features of CLIPPERS and emphasize consideration for alternative diagnoses when findings are not classic. A recommended diagnostic evaluation and initial treatment plan is provided.
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http://dx.doi.org/10.1007/s11910-017-0773-7DOI Listing
September 2017