Clinical uncertainty in large vessel occlusion ischemic stroke: does automated perfusion imaging make a difference? An intra-rater and inter-rater agreement study.

BACKGROUND: Limited research exists regarding the impact of neuroimaging on endovascular thrombectomy (EVT) decisions for late-window cases of large vessel occlusion (LVO) stroke. OBJECTIVE: T0 assess whether perfusion CT imaging: (1) alters the proportion of recommendations for EVT, and (2) enhances the reliability of EVT decision-making compared with non-contrast CT and CT angiography. METHODS: We conducted a survey using 30 patients drawn from an institutional database of 3144 acute stroke cases. These were presented to 29 Canadian physicians with and without perfusion imaging. We used non-overlapping 95% confidence intervals and difference in agreement classification as criteria to suggest a difference between the Gwet AC1 statistics (κG). RESULTS: The percentage of EVT recommendations differed by 1.1% with or without perfusion imaging. Individual decisions changed in 21.4% of cases (11.3% against EVT and 10.1% in favor). Inter-rater agreement (κG) among the 29 raters was similar between non-perfusion and perfusion CT neuroimaging (κG=0.487; 95% CI 0.327 to 0.647 and κG=0.552; 95% CI 0.430 to 0.675). The 95% CIs overlapped with moderate agreement in both. Intra-rater agreement exhibited overlapping 95% CIs for all 28 raters. κG was either substantial or excellent (0.81-1) for 71.4% (20/28) of raters in both groups. CONCLUSIONS: Despite the minimal difference in overall EVT recommendations with either neuroimaging protocol one in five decisions changed with perfusion imaging. Regarding agreement we found that the use of automated CT perfusion images does not significantly impact the reliability of EVT decisions for patients with late-window LVO.

The Clinical Outcome in AChR-Positive Generalized Myasthenia Gravis: A Retrospective Observational Study.

Background: Myasthenia gravis (MG) is an autoimmune disease affecting the neuromuscular junction, and in over 80% of cases, antibodies are identified against the nicotinic type of acetylcholine receptor (AChR) on the muscle endplate. Despite the availability of various treatment options, patients with MG experience relapses and remission during the course of the disease. Aims and Objective: To understand the clinical profile, predictors of outcomes in acetyl choline receptor (AChR) antibody positive generalized MG. Methods: This is a retrospective, single-centre, observational study of 108 patients with AChR positive generalized MG. We collected data on clinical and demographical profiles, treatments received, and treatment responses from those who fulfilled inclusion criteria over a mean follow up period of 33.75 ±7.30 months. Clinical outcomes were studied in terms of the type of remission and crisis or disease exacerbations patients had, considering different variables and treatment received. Results: We found the commonest initial symptoms were ocular or oculo-bulbar, which progressed to generalized MG in the first year of disease onset. 36 (33.3%) patients experienced a crisis requiring mechanical ventilation within a mean period of 9.4 ±4.77 months from the disease onset. Multivariate regression analysis showed late-onset MG (age of onset between 50-70 years) and treatment with rituximab were better correlated with remission, (odd ratio of 4.7; 95 % CI ,1.12 -12.6; P value < 0.05 and odd ratio of 4.56; 95 % CI ,1.2 -10.04; P value < 0.05) respectively. While treatment with Mycofenolate Mofetile (MMF) was associated with a higher number of relapses (odd ratio of 1.8; 95 % CI ,0.08 -0.96; P value < 0.05). Treatment with Rituximab showed a higher rate of remission as compared to treatment refractory (TR) on conventional immunosuppressant therapy (IST). Out of 35(32 %) thymoma patients, 21 patients underwent thymectomy and these patients showed significantly greater rate of remission as compared both thymoma patients who denied thymectomy as a treatment option (N = 10 ;55.60 % vs N = 4; 23.50%). Conclusion: In this study of AChR antibody positive generalized MG patients, we found that nearly one-third of them experienced myasthenic crisis despite receiving the best medical care. Rituximab appeared to be effective in the treatment of refractory MG and those who failed thymectomy. Thymectomy was associated with better outcomes in patients, both with or without a thymoma.

Spontaneous obliteration of a spinal perimedullary fistula.

We herewith report a young patient who had an incidental spinal vascular malformation of the cervicomedullary junction discovered during a work-up for anosmia. Angiography demonstrated a perimedullary spinal arteriovenous fistula with supply from lateral spinal arteries arising from bilateral V3 level segmental arteries. It was decided to manage the patient conservatively with magnetic resonance imaging monitored biannually. On a recent follow-up magnetic resonance, nearly 10 years later, we noted a subtle change in caliber and imaging characteristics at the posterior margin of the cervical medullary junction. Repeat digital-subtraction angiography showed no evidence of early venous filling from the previously involved branches. Microcatheter exploration of the right lateral spinal artery confirmed spontaneous occlusion of the spinal perimedullary arteriovenous fistula, without any persistent shunting. Spontaneous resolution of a spinal vascular malformation is rare; this case demonstrates the dynamic nature of shunting vascular malformations and that spontaneous obliteration of arteriovenous shunts is possible.

Atypical Optic Neuritis: The Potential Red Flags.

Background: Many potential causes of optic nerve inflammation exist, including typical and atypical causes, which require different management strategies. Objective: The objective of this study is to identify red flags that help differentiate typical from atypical optic neuritis (ON). Materials and Methods: This prospective study included 66 patients (100 eyes) with immune-mediated ON from January 2016 to June 2019, carefully excluding the nonimmune causes. The clinico-radiological features, investigations, therapy, and outcome were analyzed. Results: We evaluated 33 cases each of typical and atypical ON. The typical group included 29 idiopathic ON and four associated with multiple sclerosis. Atypical ON included 19 neuromyelitis optica (NMO), seven MOG-associated ON (MOG-ON), and others due to Sjogren's syndrome, granulomatous polyangiitis, sarcoidosis, and IgG4 disease. Atypical ON occurred significantly and more frequently with extremes of ages (<10 or >70 years), bilateral simultaneous or severe vision loss with early disc pallor, multiple attacks, symptoms/neuro-imaging indicating non-MS disease e.g., long segment ON/myelitis, large confluent lesions, the involvement of optic tract, chiasma, area postrema or diencephalon, and (pachy) meningitis. Systemic involvement and poor outcomes despite steroids and second-line immunosuppression were observed more often in the atypical ON. Conclusions: The red flags indicating atypical ON are onset at extremes of age, multiple attacks, bilateral simultaneous or severe to very severe vision loss, early disc pallor, neurological symptoms, or imaging abnormalities suggesting non-MS disease, systemic involvement, and poor steroid responsiveness. The awareness might help the clinician promptly identify and escalate therapy to ensure a better outcome.

Validation of a New Graphic Facial Nerve Grading System: FAME Scale.

Background and Objective: Most of the existing qualitative facial nerve grading systems are very subjective while the quantitative grading systems are more complex, require longer data input time and specific software. There is a need for having a scoring system with graphic criteria to improve the subjectivity, reliability and convenience. We aimed to develop and validate such a reliable graphic scale for use in Bell's palsy. Methods: Face videos of patients with unilateral facial paralysis were recorded using smartphones and analyzed for six items including five voluntary facial movements apart from complications of facial palsy (synkinesis, hyperkinesis, and contracture). 15 videos were used for pilot study, 75 for the development of scale and 110 for its validation. Each video was rated on two separate occasions by 3 independent raters, a score of 0-4 was assigned to each item using the graphic scoring criteria, and a composite score was obtained (range 0-24). Five disease severity categories: normal (score 0), mild (score 1-6), moderate (score 7-12), severe (score: 13-18) and profound facial weakness (score: 19-24). Results: The proposed scale and its component items had high inter-rater and intra-rater reliability (Kappa >0.7). Good correlation (Pearson co-efficient >0.7) was seen among the voluntary movements. The proposed scale is a valid tool to score motor deficits and complications of facial palsy. Conclusions: The proposed scale is a valid and reliable graphic scale to describe facial motor dysfunction and its secondary defects.

Proximal Dominant Hereditary Motor and Sensory Neuropathy with TFG Mutation: First Case Report from India.

Hereditary motor and sensory neuropathy with proximal predominance (HMSN-P) is a rare degenerative disorder inherited in an autosomal dominant fashion. This disease was described first in Japanese descendants from Okinawa and Shiga prefectures in mainland Honshu in 1997. The disease is characterized by adult onset of proximal weakness and atrophy, muscle cramps, fasciculations, areflexia, high incidence of elevated creatine kinase, hyperlipidemia, and diabetes mellitus, resembling Kennedy disease, though the mode of inheritance is autosomal dominant, rather than X linked. We examined a 56-year-old male patient with clinical features suggestive of HMSN-P and positive family history in an autosomal dominant fashion. Clinical, electrophysiological, and genetic factors were also reviewed. The appearance of HMSN-P in India and elsewhere calls for clinicians in nonendemic regions to be familiar with this rare disorder, which has typically been geographically confined.

Myelin Oligodendrocyte Glycoprotein (MOG)-IgG Associated Demyelinating Disease: Our Experience with this Distinct Syndrome.

BACKGROUND: Discovery of serum myelin oligodendrocyte glycoprotein (MOG) antibody testing in demyelination segregated MOG-IgG disease from AQ-4-IgG positive NMOSD. AIMS: To study clinico-radiological manifestations, pattern of laboratory and electrophysiological investigations and response to treatment through follow up in MOG-IgG positive patients. METHOD: Retrospective data of MOG-IgG positive patients was collected. Demographics, clinical manifestations at onset and at follow up and relapses, anti AQ-4-IgG status, imaging and all investigations were performed, treatment of relapses and further immunomodulatory therapy were captured. RESULTS: In our 30 patients, F: M ratio was 2.75:1 and adult: child ratio 4:1. Relapses at presentation were optic neuritis {ON}(60%), longitudinally extensive transverse myelitis {LETM}(20%), acute disseminated encephalomyelitis {ADEM}(13.4%), simultaneous ON with myelitis (3.3%) and diencephalic Syndrome (3.3%). Salient MRI features were ADEM-like lesions, middle cerebellar peduncle fluffy infiltrates, thalamic and pontine lesions and longitudinally extensive ON {LEON} as well as non-LEON. Totally, 50% patients had a relapsing course. Plasma exchange and intravenous immunoglobulin worked in patients who showed a poor response to intravenous methylprednisolone. Prednisolone, Azathioprine, Mycophenolate and Rituximab were effective attack preventing agents. CONCLUSIONS: MOG-IgG related manifestations in our cohort were monophasic/recurrent/simultaneous ON, myelitis, recurrent ADEM, brainstem encephalitis and diencephalic Syndrome. MRI features suggestive of MOG-IgG disease were confluent ADEM-like lesions, middle cerebellar peduncle fluffy lesions, LETM, LEON and non-LEON. Where indicated, patients need to go on immunomodulation as it has a relapsing course and can accumulate significant disability. Because of its unique manifestations, it needs to be considered as a distinct entity. To the best of our knowledge, this is the largest series of MOG-IgG disease reported from India.

Myelin Oligodendrocyte Glycoprotein (MOG) Antibody-Associated CNS Demyelination: Clinical Spectrum and Comparison with Aquaporin-4 Antibody Positive Neuromyelitis Optica Spectrum Disorder.

BACKGROUND: The clinical phenotypes of myelin oligodendrocyte glycoprotein (MOG) antibody disease, its disease course, and treatment are poorly understood and much work needs to be done towards this. OBJECTIVE: To characterize the clinico-radiologic spectrum and treatment outcomes of MOG antibody disease and differentiate it from aquaporin-4 (AQP-4) antibody positive neuromyelitis optica spectrum disorders (NMO-SD). METHODS: A single-center, observational study from Western India during 2017-2019, of 48 patients with either MOG antibody positive (21 patients) or AQP-4 antibody positive (27 patients) central nervous system demyelination. RESULTS: MOG antibody group had median age 32.2 years, no gender bias, median disease duration 40 months, relapses in 9 patients (43%), and median 2.5 (1-16) episodes per patient. Onset phenotypes included isolated bilateral optic neuritis (ON) (43%), isolated unilateral ON (19%), acute brainstem syndrome (19%), simultaneous ON with myelitis (9%), isolated myelitis (5%), and acute disseminated encephalomyelitis optic neuritis (ADEM-ON) (5%). Characteristic neuroimaging abnormalities were anterior segment longitudinally extensive ON, upper brainstem, and thoracic cord involvement (both short and long segment lesions). Most patients (86%) responded well to steroids, only 3/21 required rescue immunotherapy. In total, 6 out of 46 eyes affected developed permanent visual disability, while one patient had motor disability. The features differentiating MOG from AQP-4 antibody group were: no female predilection, preferential optic nerve involvement, characteristic neuroimaging abnormalities, and favorable therapeutic response and outcome. CONCLUSIONS: MOG disease commonly presents as severe ON, myelitis, acute brainstem syndrome, ADEM or their combinations. Early identification, treatment, and maintenance immunosuppression are necessary. It can easily be differentiated from NMO-SD using clinico-radiological features and therapeutic response.

Bilateral hip pain in a young man? It may be worth considering juvenile-onset ankylosing spondylitis (JAS).

A 15-year-old boy with severe bilateral hip joint pain and restriction of mobility presented to the casualty ward. He had earlier been treated for tuberculosis of the hip, with no relief. Our work up revealed a case of severe juvenile-onset ankylosing spondylitis with predominant hip involvement and accompanying sacroiliitis.

Isolated Subclavian Artery Thrombosis.

We presume this case to highlight the importance of underestimated percutaneous angioplasty with stent in isolated subclavian artery thrombosis.