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Neuropsychology plays an important role in optimizing epilepsy management, but there are no practice guidelines for low- and middle-income countries with emerging services. The National Epilepsy Surgery Support Activity Network (NESSAN), a national working group that supports and optimizes epilepsy surgery programs in India, explored the present status of pre- and post epilepsy surgery neuropsychological assessment practices across India as an initial step in developing national evidence-based test guidelines. An online survey was conducted using two questionnaires, one for neurologists and the second for neuropsychologists and speech-language pathologists working in epilepsy surgery centers. The questions to neurologists covered the setting, description, and nature of the neuropsychology services provided for adults and children as part of the epilepsy surgery program, and their perceptions about the importance and indications for neuropsychological assessment in epilepsy surgery. The questions to neuropsychologists inquired about their qualifications and training, pre- and postsurgical assessment procedures, and domain-specific cognitive tests and scales for mood, quality of life, and language evaluations. Forty-three centers responded, and neuropsychology services were available at three fourth of the centers, but a dedicated full-time neuropsychologist was available in only 16% of centers. Ninety percent of the neurologists considered neuropsychological evaluation an indispensable part of presurgical evaluation. However, only a few of them believed that the results influenced their decision about the extent of resection and ruling against surgery. The survey found considerable heterogeneity in terms of qualifications and training of neuropsychologists, evaluation procedures, test protocols, and normative data that will need to be harmonized to ensure uniform practice across the country. We believe that the results from this survey provide a road map for implementing measures to minimize existing inadequacies and harmonize neuropsychology services in India and as well as in other countries with emerging neuropsychology services. PLAIN LANGUAGE SUMMARY: Neuropsychological assessment is an integral part of the pre- and post epilepsy surgery evaluations. In India, the National Epilepsy Surgery Support Activity Network (NESSAN), a body of multidisciplinary professionals, conducted a survey to examine existing neuropsychology practices. The responses from 43 centers across the country revealed considerable heterogeneity in tests and few trained and qualified neuropsychologists. In low- and middle-income countries (LMIC) with emerging neuropsychology services, there is a need to have uniform test protocols, specialized training, and guidelines for optimizing neuropsychology services across all the epilepsy surgery centers.
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IgG4-related disease (IgG4-RD) is an immune-mediated multi-system disorder. The nervous system (IgG4-RND) is rarely affected. We describe a short case series. We performed an ambispective analysis of IgG4-RND patients admitted at our centre between January 2016 and December 2022. Eight patients (M: F-2:6) were included with a mean age at presentation of 40.63 ± 17.88 years and disease duration of 5.16 ± 4.08 years. The common diseased sites were pachymeninges (7), orbits (4), paranasal sinuses (3), frontal lobe (1), hypophysis (1), leptomeninges (1), and middle ear (1). Common symptoms were headache and cranial neuropathy. The common nerves involved were the optic nerve, followed by the third, fifth, sixth, and seventh. Cerebrospinal fluid showed lymphocytic pleocytosis. Histopathology showed lymphoplasmacytic infiltrate (8), fibrosis (5), >10 IgG4 + cells (7), and IgG4/IgG >40% (6). Six had a relapsing course. The rituximab-based treatment regimen showed a favourable response.
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Doença Relacionada a Imunoglobulina G4 , Humanos , Doença Relacionada a Imunoglobulina G4/diagnóstico , Dura-Máter , Lobo Frontal , Imunoglobulina G , ÍndiaRESUMO
The calcified stage of the neurocysticercosis (NCC) is the common cause of acquired epilepsy in low and middle income countries in people aged > 20 years. Approximately 30% of adult onset seizures and epilepsy are attributable to NCC. In India and some of the Latin American countries, epilepsy due to solitary calcified NCC is the common adult onset epilepsy. The current evidence suggests that the calcified cysticercus granuloma is probably the epileptogenic focus. The mechanisms involved in the epileptogenic process are not well understood; Focal-onset seizures with or without impaired awareness are the common seizure type. Focal-onset seizure can evolve to bilateral tonic-clonic seizure. Seizure outcome with anti-seizure medication, most often with monotherapy, is very good. The seizure disorders associated with various stages of NCC can be preventable.
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Background: Traditional diagnostic techniques such as clinical examination and electrodiagnosis are less sensitive in diagnosing ulnar neuropathy at the elbow (UNE). Ultrasonography (USG) is increasingly being used to diagnose UNE. However, clinical applicability is limited by the lack of uniformity in the previous studies. Therefore, we aimed to study in the Indian patients the diagnostic utility of the ulnar nerve cross-sectional area (CSA) and a novel parameter-entrapment index (EI) in UNE measured by USG and to find if both these parameters correlate with the electrodiagnostic severity. Methods: This retrospective casecontrol study included 28 patients (36 nerves) of UNE and 12 (24 nerves) age- and gender-matched healthy controls. Electrodiagnostic severity was graded using the Padua classification. USG was performed in both groups, and CSA was measured at the medial epicondyle (ME) and 5 cm proximally and distally. EI was calculated by multiplying the ratio of CSA above ME over CSA at ME by 100. Best cutoffs were derived by the receiver operating characteristic curve analysis. Results: UNE group had significantly higher CSA at all three locations and lower EI than the control group. CSA at ME ≥9.7 mm2 and EI ≤61.5 has sensitivity and specificity of 88.9%/87.5% and 72.2%/79.2%, respectively. There was no significant difference in CSA and EI between nonsevere and severe UNE groups. Conclusion: CSA at ME and EI have good sensitivity and specificity in diagnosing UNE. However, they cannot differentiate nonsevere from severe UNE.
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The MDS Video Challenge continues to be the one of most widely attended sessions at the International Congress. Although the primary focus of this event is the presentation of complex and challenging cases through videos, a number of cases over the years have also presented an unusual or important neuroimaging finding related to the case. We reviewed the previous Video Challenge cases and present here a selection of those cases which incorporated such imaging findings. We have compiled these "imaging pearls" into two anthologies. The first focuses on pearls where the underlying diagnosis was a genetic condition. This second anthology focuses on imaging pearls in cases where the underlying condition was acquired. For each case we present brief clinical details along with neuroimaging findings, the characteristic imaging findings of that disorder and, finally, the differential diagnosis for the imaging findings seen.
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"Guitar pick sign," also referred to as posterior globe tenting, is a radiological surrogate marker of tense orbit and profound vision loss. It is seen commonly in traumatic retrobulbar hemorrhage and carotico-cavernous fistula and less frequently in orbital cellulitis, subperiosteal abscess, and invasive fungal infections. We report a case series of Coronavirus disease-19-associated rhino-orbito-cerebral mucormycosis with guitar pick sign, of which none survived, and discuss the causative pathomechanisms, severity grade, and the clinical relevance of this unique radiological finding.
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COVID-19 , Mucormicose , Celulite Orbitária , COVID-19/complicações , Humanos , Mucormicose/complicações , Mucormicose/diagnóstico , Mucormicose/microbiologia , Órbita , Tomografia Computadorizada por Raios XRESUMO
AIM: As an initial step to develop guidelines for epilepsy monitoring units (EMUs) appropriate for developing countries, we inquired the existing practices in EMUs in India. METHODS: After checking for the content and face validity as well for clarity, we sent a 52-item online non-anonymized questionnaire to all the 52 EMUs in India. RESULTS: The questionnaire was completed by 51 of the 52 EMUs (98% response rate). The majority of the EMUs are located in major cities and 51% are located in non-governmental corporate hospitals. There are total of 122 prolonged video-EEG monitoring (PVEM) beds in India and 70% EMUs have ≤2 beds. Approximately two-thirds of the EMUs have defined protocols for pre-procedure consent and risk assessment, management of seizure clusters and status epilepticus, continuous observation of patients, and periictal testing. Only one-third of the EMUs have protocols for management of post-ictal psychosis, anti-suffocation pillows, and protected environment within bathrooms. The waiting period for PVEM is more (49.9 ± 101 vs. 4.9 ± 10.9 days; p = 0.04) and mean cost for 3-day PVEM is less (INR 8311 ± 9021 vs. 30,371 ± 17,563; p <0.0001) in public as compared to private hospitals. There was a negative correlation between cost of PVEM and the waiting period (r=-0.386; p = 0.01). Safety practices are similar in public and private hospitals. CONCLUSIONS: Although practices in EMUs in India vary widely, they are comparable to those in developed countries. India has severe shortage of EMUs and long waiting lists for affordable PVEM.
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Epilepsia , Estado Epiléptico , Eletroencefalografia , Epilepsia/diagnóstico , Epilepsia/epidemiologia , Epilepsia/terapia , Humanos , Monitorização Fisiológica , ConvulsõesRESUMO
OBJECTIVE: To determine treatment responses to various antiseizure medicines (ASMs) in patients with drug resistant juvenile myoclonic epilepsy (DRJME) METHODS: We reviewed records of all JME patients attending epilepsy clinics at 5 centers during a 5-year period. We used International Consensus Criteria to diagnose JME and International League Against Epilepsy Criteria to define drug resistance and sustained seizure freedom. We only used broad spectrum medicines which included valproate, lamotrigine, topiramate, levetiracetam, clobazam, phenobarbitone, clonazepam, and zonisamide. We considered an ASM successful if patient achieved seizure freedom within 3 months of attaining maintenance dose. RESULTS: We studied 116 patients (61 males) with DRJME. At terminal followup, 82 (70.7%) patients had achieved sustained seizure freedom with a mean followup of 3.2 ± 1.3 years after last dose change. In patients where valproate failed as first- or second-line ASM (n=70; 60.3%), 49(70%) became seizure-free. In this group, 33(67%) patients became seizure-free after addition of lamotrigine. Success rate of lamotrigine and valproate combination was 69% as compared to 9% with all other combinations (p = 0.001). In patients who were not exposed to valproate as initial therapy (n=46), 33 (71.7%) became seizure-free, 30 (91%) after adding valproate. At last follow-up, 75 (90%) seizure-free patients were receiving valproate including 45 (55%) patients with a combination of valproate and lamotrigine. Only one of 24 patients became seizure-free after failing valproate and lamotrigine combination. CONCLUSION: Seizure freedom can be achieved in two-thirds of patients with DRJME. A combination of valproate and lamotrigine is the most effective duotherapy.
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Epilepsia Mioclônica Juvenil , Preparações Farmacêuticas , Anticonvulsivantes/uso terapêutico , Feminino , Humanos , Masculino , Epilepsia Mioclônica Juvenil/tratamento farmacológico , Resultado do Tratamento , Ácido Valproico/uso terapêuticoRESUMO
Several case reports of COVID-19 in patients with myasthenia gravis (MG) have been documented. However, new-onset autoimmune MG following COVID-19 has been reported very rarely. We report one such case here. A 65-year-old man presented to us with dysphagia 6 weeks following mild COVID-19. He was evaluated and diagnosed as antiacetylcholine receptor antibody (AchR) positive, non-thymomatous, generalised MG. He subsequently developed myasthenic crisis and improved after treatment with intravenous immunoglobulin, prednisolone and pyridostigmine. Systematic literature review showed eight more similar cases. Analysis of all cases including the one reported here showed these features: mean age 55.8 years, male gender (5), time interval between COVID-19 and MG (5-56 days), generalised (5), bulbar and/or ocular symptoms (4), anti-AchR antibodies (7) and antimuscle-specific kinase antibodies (2). All have improved with immunotherapy. Although, many hypothesis are proposed to explain causal relationship between the two, it could as well be sheer coincidence.
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COVID-19 , Miastenia Gravis , Idoso , Humanos , Masculino , Pessoa de Meia-Idade , Miastenia Gravis/complicações , Miastenia Gravis/diagnóstico , Miastenia Gravis/tratamento farmacológico , Brometo de Piridostigmina/uso terapêutico , Receptores Colinérgicos , SARS-CoV-2RESUMO
Tumor-like mass lesion is a rare subtype of primary angiitis of the central nervous system (ML-PACNS). This report describes six patients of histologically verified ML-PACNS. The mean age was 44.5 years (range 25-68) and four were males. Presenting symptoms headache (5), focal neurologic deficits (5), and seizures (4). On magnetic resonance imaging (MRI) the lesion was unifocal in two and multifocal in four patients. Consistent radiological findings were mass lesions with heterointense internal morphology with areas of diffusion restriction (DWI), and variable post-contrast enhancement. Pathologically vasculitis was classified as: Granulomatous in one, necrotizing in two and lymphocytic in three. There were two deaths. In conclusion patients with ML-PACNS are likely to be younger and more likely to present with seizures. MRI lesions with hetrointense internal morphology with areas of DWI was a consistent finding and may be a clue for the diagnosis of ML-PACNS.
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Neoplasias , Vasculite do Sistema Nervoso Central , Adulto , Idoso , Biópsia , Sistema Nervoso Central , Feminino , Humanos , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Vasculite do Sistema Nervoso Central/diagnóstico por imagemRESUMO
This report describes a patient with thymomatous myasthenia gravis (MG) with aplastic anemia in pharmacological remission and COVID-19 who developed respiratory failure in the course of the disease and reviews the published literature on this topic. Analysis of the clinical characteristics of the eight patients with MG including our patient suggests two possible mechanisms for respiratory failure: myasthenic crisis (MC) or pulmonary complications of COVID-19. Patients with MC were young women in high-grade MGFA Class whereas patients with respiratory failure due to pulmonary complications of COVID-19 were elderly men in pharmacological remission or MGFA Class I. These observations suggest that COVID-19, like other infections, may precipitate MC in patients with severe grade MG before COVID-19. The only differentiating feature between the two types of failure was severity myasthenic weakness. This clinical distinction has management implications. These observations need to be validated in a larger sample.
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COVID-19 , Miastenia Gravis , Insuficiência Respiratória , Idoso , Feminino , Humanos , Masculino , Debilidade Muscular , Miastenia Gravis/complicações , Insuficiência Respiratória/etiologia , SARS-CoV-2Assuntos
Infarto Encefálico/diagnóstico por imagem , Encéfalo/diagnóstico por imagem , Paresia/diagnóstico por imagem , Infarto Encefálico/complicações , Infarto Encefálico/tratamento farmacológico , Fibrinolíticos/uso terapêutico , Humanos , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Paresia/etiologia , Ativador de Plasminogênio Tecidual/uso terapêutico , Resultado do TratamentoRESUMO
BACKGROUND: Recently, the International League Against Epilepsy (ILAE) has proposed new classification schemes for seizures and epilepsy. The applicability of these classification schemes has not been studied in resource-poor countries. AIM: To determine the incidences of epilepsies in a cohort of school children using the ILAE 2017 classification of epilepsies. MATERIALS AND METHODS: The study cohort included 7,408 school going children [age range 5-15 years]. They were followed for new-onset unprovoked seizure and seizure recurrence through first grade to fifth grade. The data collected during the first visit included clinical details including details of seizure semiology. All children had EEG and contrast CT scan. All these data were used to classify seizure type and epilepsy using ILAE classification schemes. The ILAE 2014 epilepsy definition was used to diagnose epilepsy. The average annual incidence rates (AAIR) and 95% confidence intervals (CI) were calculated for epilepsies by onset and by etiology. RESULTS: The AAIR of epilepsy was 57.85 (95% CI 39.0-82.6) per 100,000 population. The AAIRs per 100,000 for different epilepsies by mode of onset were focal epilepsies 25.07 (95% CI 13.35-42.87); generalized epilepsies 13.5 (95% CI 5.43-27.8); and unknown onset epilepsies 19.28 (95% CI 9.25-35.46). The AAIRs per 100,000 of epilepsies by etiology were focal genetic epilepsies 7.71 (95% CI 2.1-19.75); genetic generalized epilepsies 13.5 (95% CI 5.43-27.8); and epilepsies due to structural cause 17.36 (95% CI 9.25-35.46). The lesional epilepsies seen in this cohort were geographically specific to resource-poor countries. The AAIR of epilepsy due to calcific neurocysticercosis (cNCC) was 9.64 (95% CI 3.1-22.5) per 100,000. CONCLUSIONS: This study demonstrates that the ILAE 2017 classification of epilepsies can be applied in resource-poor countries with limited workup. Lesional epilepsies seen in this study is geographically specific to resource-poor countries.