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A 49-year-old woman with no relevant medical history presented to an external neurology clinic with progressive hand and foot paresthesia and gait instability for over 6 months. Initially, she reported tingling and shooting electrical-type sensations involving her feet. Within several weeks of the onset of paresthesia, she noted a burning pain sensation involving the left hand. Sensory symptoms continued to worsen and she felt pressure-like sensation around the neck and scalp region. This was shortly followed by right hand numbness and loss of dexterity. Over the next month, she developed progressive gait imbalance and transitioned to a cane for ambulation.

ObjectiveTo determine clinical manifestations, immunotherapy responsiveness and outcomes of glutamic acid decarboxylase-65 (GAD65) neurological autoimmunity.MethodsWe 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).ResultsOn 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.InterpretationHigh-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.

This study aimed to evaluate the effectiveness and safety of transvenous lead extraction (TLE) in patients with arrhythmogenic right ventricular cardiomyopathy (ARVC).

This study sought to assess the incidence and outcomes of life-threatening complications from atrial fibrillation ablations in a high volume center.

We thank Dr. Cao and colleagues for raising several interesting points regarding our article.1We agree that recurrent optic neuritis (ON) cannot be definitively ruled out. However, patients' examinations at the time of scans did not reveal clinical evidence of ON. Long-term visual acuity was similar between patients with and without interattack enhancement, and the impact of acute therapeutics on asymptomatic lesions in neuromyelitis optica spectrum disorder is unknown.1Therefore, we do not feel that acute intervention is entirely justified.

Morbidity and mortality for acute pulmonary embolism (PE) remain high. Therapies such as catheter-directed thrombolysis may improve outcomes, but these are generally reserved for higher-risk patients. Imaging may help guide the use of the newer therapies, but current guidelines focus more on clinical factors. Our goal was to create a risk model that incorporated quantitative echocardiographic and computed tomography (CT) measures of right ventricular (RV) size and function, thrombus burden, and serum biomarkers of cardiac overload or injury.