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Retinal hypoplasia and degeneration result in vision loss in Friedreich ataxia

Rodden, Layne N; McIntyre, Kellie; Keita, Medina; Wells, Mckenzie; Park, Courtney; Profeta, Victoria; Waldman, Amy; Rummey, Christian; Balcer, Laura J; Lynch, David R
OBJECTIVE:Friedreich ataxia (FRDA) is an inherited condition caused by a GAA triplet repeat (GAA-TR) expansion in the FXN gene. Clinical features of FRDA include ataxia, cardiomyopathy, and in some, vision loss. In this study, we characterize features of vision loss in a large cohort of adults and children with FRDA. METHODS:Using optical coherence tomography (OCT), we measured peripapillary retinal nerve fiber layer (RNFL) thickness in 198 people with FRDA, and 77 controls. Sloan letter charts were used to determine visual acuity. RNFL thickness and visual acuity were compared to measures of disease severity obtained from the Friedreich Ataxia Clinical Outcomes Measures Study (FACOMS). RESULTS:The majority of patients, including children, had pathologically thin RNFLs (mean = 73 ± 13 μm in FRDA; 98 ± 9 μm in controls) and low-contrast vision deficits early in the disease course. Variability in RNFL thickness in FRDA (range: 36 to 107 μm) was best predicted by disease burden (GAA-TR length X disease duration). Significant deficits in high-contrast visual acuity were apparent in patients with an RNFL thickness of ≤68 μm. RNFL thickness decreased at a rate of -1.2 ± 1.4 μm/year and reached 68 μm at a disease burden of approximately 12,000 GAA years, equivalent to disease duration of 17 years for participants with 700 GAAs. INTERPRETATION/CONCLUSIONS:These data suggest that both hypoplasia and subsequent degeneration of the RNFL may be responsible for the optic nerve dysfunction in FRDA and support the development of a vision-directed treatment for selected patients early in the disease to prevent RNFL loss from reaching the critical threshold.
PMID: 37334854
ISSN: 2328-9503
CID: 5542542

Corrigendum to "Longitudinal stability of inter-eye differences in optical coherence tomography measures for identifying unilateral optic nerve lesions in multiple sclerosis" [Journal of the Neurological Sciences 449C (2023) Start page-End page/JOTNS D-23-00048R2]

Patil, Sachi A; Joseph, Binu; Tagliani, Paula; Sastre-Garriga, Jaume; Montalban, Xavier; Vidal-Jordana, Angela; Galetta, Steven L; Balcer, Laura J; Kenney, Rachel C
PMID: 37468371
ISSN: 1878-5883
CID: 5535862

Longitudinal stability of inter-eye differences in optical coherence tomography measures for identifying unilateral optic nerve lesions in multiple sclerosis

Patil, Sachi A; Joseph, Binu; Tagliani, Paula; Sastre-Garriga, Jaume; Montalban, Xavier; Vidal-Jordana, Angela; Galetta, Steven L; Balcer, Laura J; Kenney, Rachel C
INTRODUCTION:Optical coherence tomography (OCT)-derived peripapillary retinal nerve fiber layer (pRNFL) and ganglion cell+inner plexiform layer (GCIPL) thickness inter-eye differences (IEDs) are robust measurements for identifying clinical history acute ON in people with MS (PwMS). This study investigated the utility and durability of these measures as longitudinal markers to identify optic nerve lesions. METHODS:Prospective, multi-center international study of PwMS (with/without clinical history of ON) and healthy controls. Data from two sites in the International MS Visual System Consortium (IMSVISUAL) were analyzed. Mixed-effects models were used to compare inter-eye differences based on MS and acute ON history. RESULTS:Average age of those with MS (n = 210) was 39.1 ± 10.8 and 190 (91%) were relapsing-remitting. Fifty-nine (28.1%) had a history of acute unilateral ON, while 9/210 (4.3%) had >1 IB episode. Median follow-up between OCT scans was 9 months. By mixed-effects modeling, IEDs were stable between first and last visits within groups for GCIPL for controls (p = 0.18), all PwMS (p = 0.74), PwMs without ON (p = 0.22), and PwMS with ON (p = 0.48). For pRNFL, IEDs were within controls (p = 0.10), all PwMS (p = 0.53), PwMS without ON history (p = 0.98), and PwMS with history of ON (p = 0.81). CONCLUSION:We demonstrated longitudinal stability of pRNFL and GCIPL IEDs as markers for optic nerve lesions in PwMS, thus reinforcing the role for OCT in demonstrating optic nerve lesions.
PMID: 37167654
ISSN: 1878-5883
CID: 5503382

Case Report: Papilledema Secondary to Cerebral Venous Sinus Thrombosis after Severe COVID-19 Infection

Smith, Levi J; Khattar-Sullivan, Alanna; Devore, Eric; Blace, Nancy
SIGNIFICANCE/CONCLUSIONS:This case highlights ocular side effects of a rare, potentially life-threatening complication from coronavirus disease (COVID-19). Papilledema can occur due to increased intracranial pressure caused by cerebral venous sinus thrombosis, the incidence of which may be more likely in patients with a history of COVID-19 due to an induced hypercoagulable state. PURPOSE/OBJECTIVE:This case report presents a case of papilledema secondary to cerebral venous sinus thrombosis in a patient with a recent history of severe coronavirus disease (COVID-19). CASE REPORT/METHODS:A 29-year-old male hospitalized with a complicated course of coronavirus disease (COVID-19) was referred to the ophthalmology department for episodic blurry vision of both eyes and intermittent binocular diplopia. Clinical examination revealed diffuse bilateral optic disc edema. Magnetic resonance venography of the brain during his admission revealed subtotal occlusion of the right transverse sinus by thrombosis. At the time of diagnosis, the patient was already taking systemic anti-coagulation therapy for treatment of a recent pulmonary embolism also thought to be induced by COVID-19. After additional treatment with acetazolamide there was improvement in his optic nerve edema. CONCLUSIONS:Cerebral venous sinus thrombosis, a serious and potentially life-threatening condition, can occur as a rare complication of COVID-19. In such cases, patients may develop increased intracranial pressure, papilledema, and subsequent vison loss. Magnetic resonance venography should be ordered in patients with suspected papilledema to help rule out the presence of cerebral venous sinus thrombosis.
PMID: 36856578
ISSN: 1538-9235
CID: 5430842

White matter hyperintensities in former American football players

Alosco, Michael L; Tripodis, Yorghos; Baucom, Zachary H; Adler, Charles H; Balcer, Laura J; Bernick, Charles; Mariani, Megan L; Au, Rhoda; Banks, Sarah J; Barr, William B; Wethe, Jennifer V; Cantu, Robert C; Coleman, Michael J; Dodick, David W; McClean, Michael D; McKee, Ann C; Mez, Jesse; Palmisano, Joseph N; Martin, Brett; Hartlage, Kaitlin; Lin, Alexander P; Koerte, Inga K; Cummings, Jeffrey L; Reiman, Eric M; Stern, Robert A; Shenton, Martha E; Bouix, Sylvain
INTRODUCTION/BACKGROUND:The presentation, risk factors, and etiologies of white matter hyperintensities (WMH) in people exposed to repetitive head impacts are unknown. We examined the burden and distribution of WMH, and their association with years of play, age of first exposure, and clinical function in former American football players. METHODS:A total of 149 former football players and 53 asymptomatic unexposed participants (all men, 45-74 years) completed fluid-attenuated inversion recovery magnetic resonance imaging, neuropsychological testing, and self-report neuropsychiatric measures. Lesion Segmentation Toolbox estimated WMH. Analyses were performed in the total sample and stratified by age 60. RESULTS:In older but not younger participants, former football players had greater total, frontal, temporal, and parietal log-WMH compared to asymptomatic unexposed men. In older but not younger former football players, greater log-WMH was associated with younger age of first exposure to football and worse executive function. DISCUSSION/CONCLUSIONS:In older former football players, WMH may have unique presentations, risk factors, and etiologies. HIGHLIGHTS/CONCLUSIONS:Older but not younger former football players had greater total, frontal, temporal, and parietal lobe white matter hyperintensities (WMH) compared to same-age asymptomatic unexposed men. Younger age of first exposure to football was associated with greater WMH in older but not younger former American football players. In former football players, greater WMH was associated with worse executive function and verbal memory.
PMID: 35996231
ISSN: 1552-5279
CID: 5331552

Teaching Video NeuroImage: Alternating Skew Deviation as a Manifestation of Anti-GAD65-Associated Cerebellitis

Bell, Carter; Drummond, Patrick S; Grossman, Scott N
PMID: 36539301
ISSN: 1526-632x
CID: 5462092

Optical coherence tomography angiography measurements in multiple sclerosis: a systematic review and meta-analysis

Mohammadi, Soheil; Gouravani, Mahdi; Salehi, Mohammad Amin; Arevalo, J Fernando; Galetta, Steven L; Harandi, Hamid; Frohman, Elliot M; Frohman, Teresa C; Saidha, Shiv; Sattarnezhad, Neda; Paul, Friedemann
BACKGROUND AND OBJECTIVES/OBJECTIVE:Recent literature on multiple sclerosis (MS) demonstrates the growing implementation of optical coherence tomography-angiography (OCT-A) to discover potential qualitative and quantitative changes in the retina and optic nerve. In this review, we analyze OCT-A studies in patients with MS and examine its utility as a surrogate or precursor to changes in central nervous system tissue. METHODS:PubMed and EMBASE were systematically searched to identify articles that applied OCT-A to evaluate the retinal microvasculature measurements in patients with MS. Quantitative data synthesis was performed on all measurements which were evaluated in at least two unique studies with the same OCT-A devices, software, and study population compared to controls. A fixed-effects or random-effects model was applied for the meta-analysis based on the heterogeneity level. RESULTS:The study selection process yielded the inclusion of 18 studies with a total of 1552 evaluated eyes in 673 MS-associated optic neuritis (MSON) eyes, 741 MS without optic neuritis (MSNON eyes), and 138 eyes without specification for the presence of optic neuritis (ON) in addition to 1107 healthy control (HC) eyes. Results indicated that MS cases had significantly decreased whole image superficial capillary plexus (SCP) vessel density when compared to healthy control subjects in the analyses conducted on Optovue and Topcon studies (both P < 0.0001). Likewise, the whole image vessel densities of deep capillary plexus (DCP) and radial peripapillary capillary (RPC) were significantly lower in MS cases compared to HC (all P < 0.05). Regarding optic disc area quadrants, MSON eyes had significantly decreased mean RPC vessel density compared to MSNON eyes in all quadrants except for the inferior (all P < 0.05). Results of the analysis of studies that used prototype Axsun machine revealed that MSON and MSNON eyes both had significantly lower ONH flow index compared to HC (both P < 0.0001). CONCLUSIONS:This systematic review and meta-analysis of the studies reporting OCT-A measurements of people with MS confirmed the tendency of MS eyes to exhibit reduced vessel density in the macular and optic disc areas, mainly in SCP, DCP, and RPC vessel densities.
PMID: 36973708
ISSN: 1742-2094
CID: 5463142

Neurologists' Evaluations of Experience and Effectiveness of Teleneurology Encounters

Thawani, Sujata P; Minen, Mia T; Stainman, Rebecca S; Friedman, Steven; Bhatt, Jaydeep M; Foo, Farng-Yang A; Torres, Daniel M; Weinberg, Harold J; Kim, Nina H; Levitan, Valeriya; Cardiel, Myrna I; Zakin, Elina; Conway, Jenna M; Kurzweil, Arielle M; Hasanaj, Lisena; Galetta, Steven L; Balcer, Laura J; Busis, Neil A
PMID: 35834603
ISSN: 1556-3669
CID: 5266202

Correction to: Improvements in Cognitive Processing Speed, Disability, and Patient‑Reported Outcomes in Patients with Early Relapsing‑Remitting Multiple Sclerosis Treated with Natalizumab: Results of a 4‑year, Real‑World, Open‑Label Study

Perumal, Jai; Balabanov, Roumen; Su, Ray; Chang, Roger; Balcer, Laura J; Galetta, Steven L; Avila, Robin L; Rutledge, Danette; Fox, Robert J
BACKGROUND:STRIVE was a prospective, 4-year, multicenter, observational, open-label, single-arm study of natalizumab treatment in anti-JC virus antibody-negative patients with early relapsing-remitting multiple sclerosis (RRMS). OBJECTIVE:Study objectives examined the effects of natalizumab on cognitive processing speed, confirmed disability improvement (CDI), and patient-reported outcomes (PROs). METHODS:Clinical and PRO secondary endpoints were assessed annually over 4 years in STRIVE. The Symbol Digit Modalities Test (SDMT) was used as a measure of cognitive processing speed. PROs were assessed using the Multiple Sclerosis Impact Score (MSIS-29) and the Work Productivity and Activity Impairment Questionnaire (WPAI). RESULTS:At all four annual assessments, the proportion of patients in the intent-to-treat (ITT) population (N = 222) who exhibited clinically meaningful improvement in their SDMT score from baseline (i.e., change ≥ 4 points) ranged from 41.9 to 54.0%. The cumulative probability of CDI at 4 years in patients in the ITT population with a baseline Expanded Disability Status Scale score ≥ 2 (N = 133) was 43.9%. Statistically significant reductions in the mean change from screening in the MSIS-29 physical and psychological scores, indicating improved quality of life, were observed over all 4 years (P ≤ 0.0012 for all). A statistically significant decrease from screening in the impact of MS on regular activities, signifying an improvement in this WPAI measure, was also observed over all 4 years of the study. CONCLUSION/CONCLUSIONS:These results further extend our knowledge of the effectiveness, specifically regarding improvements in cognitive processing speed, disability and PROs, of long-term natalizumab treatment in early RRMS patients. CLINICALTRIALS/RESULTS:GOV: NCT01485003 (5 December 2011).
PMID: 36780107
ISSN: 1179-1934
CID: 5427042

Neurology faculty comfort and experience with communication skills

Zhang, Cen; Kurzweil, Arielle; Pleninger, Perrin; Nelson, Aaron; Gurin, Lindsey; Zabar, Sondra; Galetta, Steven L; Balcer, Laura J; Lewis, Ariane
BACKGROUND:Neurology faculty care for complex patients, teach, and work within multidisciplinary teams. It is imperative for faculty to have strong communication skills. METHODS:We surveyed NYU neurology teaching faculty to determine levels of comfort and experience over the past year with providing negative feedback to a trainee; debriefing after an adverse clinical outcome; and assisting a struggling colleague. We examined the relationship between levels of comfort and experience with 1) faculty self-identified sex and 2) number of years since completion of medical training. RESULTS:The survey was completed by 36/83 teaching neurology faculty (43 %); 17 (47 %) respondents were female and 21 (58 %) were ≤10 years post-training. The proportions of faculty who reported feeling uncomfortable were 44 % (16/36) for assisting a struggling colleague, 28 % (10/36) for providing negative feedback, and 19 % (7/36) for debriefing an adverse outcome. Proportions of faculty who reported they had no experience were 75 % (27/36) for assisting a struggling colleague, 39 % (14/36) for debriefing an adverse clinical event, and 17 % (6/36) for providing negative feedback. Female respondents and faculty who were ≤10 years post-training were more likely to report feeling uncomfortable with assisting a struggling colleague and to have had no experience doing so in the past year. On multivariate analyses accounting for sex and experience, sex remained independently associated with feeling uncomfortable with assisting a struggling colleague (OR = 12.2, 95 % CI: 2.1-69.6, p = 0.005). CONCLUSION/CONCLUSIONS:Faculty development may be needed to improve comfort and experience with challenging communication-based interactions. Female faculty and faculty early in their careers may benefit most.
PMID: 36642032
ISSN: 1532-2653
CID: 5433622