To chronicle the history of medicine and neurology in India with a focus on its establishment and evolution.
The history of neurology in India is divided into two periods: ancient and modern. The ancient period dates back to the mid-second millennium Before Christ (B.C.) during the creation of the Ayurvedic Indian system of Medicine, which detailed descriptions of neurological disorders called Vata Vyadhi. The early 20th century witnessed the birth of modern Indian medicine with the onset of formal physician training at the nation's first allopathic medical colleges located in Madras (1835), Calcutta (1835) and Mumbai (1848). Prior to India's independence from Britain in 1947, only 25 medical schools existed in the entire country. Today, there are over 355. In 1951, physicians across the field of neurology and neurosurgery united to create the Neurological Society of India (NSI). Four decades later in 1991, neurologists branched out to establish a separate organization called the Indian Academy of Neurology (IAN).
Information was gathered through literature review using PubMed, MD Consult, OVID, primary texts and research at various academic institutions in India.
Neurological disorders were first described in ancient India under Ayurveda. The transition to modern medicine occurred more recently through formal training at medical schools beginning in the 1930's. Early pioneers and founders of the NSI (1951) include Dr. Jacob Chandy, Dr. B Ramamurthi, Dr. S. T. Narasimhan and Dr. Baldev Singh. Later, Dr. J. S. Chopra, a prominent neurologist and visionary, recognized the need for primary centers of collaboration and subsequently established the IAN (1991). The future of Neurology in India is growing rapidly. Currently, there are 1100 practicing neurologists and more than 150 post-graduate trainees who join the ranks every year. As the number of neurologists rises across India, there is an increase in the amount of basic, clinical and epidemiological research being conducted across the country every day.
The history of neurology in India roots back to its rich culture and tradition. Over time, there has been great structural and organizational evolution and the future of neurology in India appears to be bright. However, the number of neurologists and research in neurology needs to experience a significant growth in the future to ensure the best patient care.
Ayurveda; dementia; Epilepsy; history; stroke
In the context of inadequacy of neurology workforce in India, it is important to understand factors that post-graduate medical students consider for and against choosing neurology as their career option. Understanding these factors will help in planning strategies to encourage students to pursue a career in neurology. At present, there is a paucity of studies addressing this issue in India.
Aims and Objectives:
(1) To analyze factors, which post-graduate students consider for and against choosing neurology as a career specialty. (2) To access the level and quality of neurology exposure in the current MBBS and MD curricula.
Materials and Methods:
Statewide questionnaire based study was conducted in the state of Maharashtra for students eligible to take DM neurology entrance examination (MD Medicine and MD Pediatrics).
In this survey, 243 students were enrolled. Factors bringing students to neurology were - intellectual challenge and logical reasoning (72%), inspired by role model teachers (63%), better quality-of-life (51%) and scope for independent practice without expensive infrastructure (48%). Factors preventing students from taking neurology were - perception that most neurological diseases are degenerative (78%), neurology is mainly an academic specialty (40%), neurophobia (43%) and lack of procedures (57%). Inadequate exposure and resultant lack of self-confidence were common (31%, 70-80%). 84% of the students felt the need for a short term certification course in neurology after MD.
To attract more students to neurology, “role model” teachers of neurology could interact and teach students extensively. Neurologists’ efforts to shed their diagnostician's image and to shift their focus to therapeutics will help change the image of neurology. Out-patient neurology clinics should be incorporated early in the student's career. Procedures attract students; hence, they should be made conversant with procedures and interventions. Increasing the level of neurological exposure in our current MBBS and MD curriculum is necessary. A case could be made for consideration of short certification course in neurology for physicians.
Career; neurology; postgraduate; students
Clinicians during their training period and practice are often called upon to conduct studies to explore the association between certain exposures and disease states or interventions and outcomes. More often they need to interpret the results of research data published in the medical literature. Case-control studies are one of the most frequently used study designs for these purposes. This paper explains basic features of case control studies, rationality behind applying case control design with appropriate examples and limitations of this design. Analysis of sensitivity and specificity along with template to calculate various ratios are explained with user friendly tables and calculations in this article. The interpretation of some of the laboratory results requires sound knowledge of the various risk ratios and positive or negative predictive values for correct identification for unbiased analysis. A major advantage of case-control study is that they are small and retrospective and so they are economical than cohort studies and randomized controlled trials.
Analysis; case-control study; design
Rising stroke and higher mortality among Indian population needs focused attention for prevention and early management of stroke. In India, very few studies have been carried out to determine the causes of deficiencies in knowledge, attitude and practice (KAP) of stroke among Indians. Study on KAP is essential to improve the awareness about stroke, early diagnosis and institution of appropriate management. In this article, we have reviewed the existing literature on this issue and tried to compare it with those of developed countries and suggested the measures we need to adopt in India to improve awareness and knowledge base.
Awareness; risk factors; prevention strategies; stroke; India
Recent histological studies of thrombi retrieved from patients with an acute ischemic stroke using the endovascular thrombectomy devices and correlation with early vessel computed tomography (CT) and magnetic resonance imaging (MRI) characteristics have given relevant insights into the pathophysiology of thrombotic lesions and may facilitate the development of improved reperfusion treatment approaches. We present a review of recent studies on the histopathologic analysis of thrombi, studies of MRI, and CT imaging correlation with thrombus histology, and detailed structural analysis of thromboemboli retrieved by thrombectomy devices during an acute ischemic stroke.
Clot composition; histopathology; stroke; thrombus
Small dense (sd) low-density lipoprotein (LDL), tumor necrosis factor (TNF) alpha (α), and nitric oxide (NO) have recently emerged as important stroke risk factors. The aim of the study was to investigate the effects of increased levels of small LDL particle size, TNF-α and NO on the developed ischemic stroke and increased carotid artery intima-media thickness (CIMT).
Materials and Methods:
A total of 29 women and 25 men (a total of 54 ischemic stroke patients) and a similar age group of 50 controls (29 females and 21 males) were included in the study. CIMT, C-reactive protein (CRP), TNF-α, NO, and lipid subfraction test of the two groups were measured.
The mean LDL particle size was smaller in patients with stroke than in the controls (26.8 ± 0.31 nm vs. 27.0 ± 0.31 nm, P = 0.003). sd-LDL, TNF-α, NO, CRP, right CIMT, and left CIMT were higher in patients with stroke than in the controls (respectively; 8.2 ± 7.8 mg/dL vs. 3.3 ± 3.5 mg/dL, P < 0.001;75.6 ± 25.0 pg/mL vs. 65.4 ± 9.1 pg/mL, P = 0.009;76.4 ± 53.3 mmol/L vs. 41.5 ± 27.0 mmol/L, P < 0.001;1.9 ± 2.6 mm vs. 0.4 ± 0.3 mm P < 0.001;0.97 ± 0.38 mm vs. 0.83 ± 0.15 mm, P = 0.007;1.04 ± 0.44 mm vs. 0.87 ± 0.19 mm, P = 0.010).
These results show that sd-LDL is independently associated with the incidence of stroke and may be a risk factor in the development of stroke. In addition, TNF-α, NO, right CIMT, and left CIMT may be a risk factor in the development of ischemic stroke.
Carotid artery intima-media thickness; ischemic stroke; lipid subfraction; small dense-low-density lipoprotein; tumor necrosis factor-alpha
The use of biomarkers to predict stroke prognosis is gaining particular attention nowadays. Neuron specific enolase (NSE), which is a dimeric isoenzyme of the glycolytic enzyme enolase and is found mainly in the neurons is one such biomarker.
This study was carried out on patients of acute ischemic stroke with the aims to determine the correlation between NSE levels on the day of admission with infarct volume, stroke severity, and functional neurological outcome on day 30.
Materials and Methods:
Seventy five patients of acute ischemic stroke admitted in the Department of Medicine were included in the study. Levels of NSE were determined on day 1 using the human NSE ELISA kit (Alpha Diagnostic International Texas 78244, USA). Volume of infarct was measured by computed tomography (CT) scan using the preinstalled software Syngo (version A40A) of Siemen's medical solutions (Forchheim, Germany). Stroke severity at admission was assessed using Glasgow coma scale (GCS) and functional neurological outcome was assessed using modified Rankin scale (mRS) on day 30.
Statistical Analysis Used:
Statistical analysis was performed using the SPSS software for windows version 15.0 (SPSS).
A positive correlation was found between concentration of NSE on day 1 and infarct volume determined by CT scan (r = 0.955, P < 0.001). A strong negative correlation was found between GCS at presentation and concentration of NSE on day 1 (r = −0.806, P < 0.001). There was a positive correlation between NSE levels at day 1 and functional neurological outcome assessed by mRS at day 30 (r = 0.744, P < 0.001).
Serum levels of NSE in first few days of ischemic stroke can serve as a useful marker to predict stroke severity and early functional outcome. However, larger studies with serial estimation of NSE are needed to establish these observations more firmly.
Glasgow coma scale; infarct volume; ischemic stroke; modified Rankin scale; neuron specific enolase
Life-threatening, space occupying, infarction develops in 10-15% of patients after middle cerebral artery infarction (MCAI). Though decompressive craniectomy (DC) is now standard of care in patients with non-dominant stroke, its role in dominant MCAI (DMCAI) is largely undefined. This may reflect the ethical dilemma of saving life of a patient who may then remain hemiplegic and dysphasic. This study specifically addresses this issue.
Materials and Methods:
This retrospective analysis studied patients with DMCAI undergoing DC. Patient records, operation notes, radiology, and out-patient files were scrutinized to collate data. Glasgow outcome scale (GOS), Barthel index (BI) and improvement in language and motor function were evaluated to determine functional outcome.
Eighteen patients between 22 years and 72 years of age were included. 6 week, 3 month, 6 month and overall survival rates were 66.6% (12/18), 64% (11/17), 62.5% (10/16) and 62.5% (10/16) respectively. Amongst ten surviving patients with long-term follow-up, 60% showed improvement in GOS, 70% achieved BI score >60 while 30% achieved full functional independence. In this group, motor power and language function improved in 9 and 8 patients respectively. At last follow-up, 8 of 10 surviving patients were ambulatory with (3/8) or without (5/8) support. Age <50 years corresponded with better functional outcome amongst survivors (P value –0.0068).
Language and motor outcomes after DC in patients with DMCAI are not as dismal as commonly perceived. Perhaps young patients (<50 years) with DMCAI should be treated with the same aggressiveness that non-DMCAI is currently dealt with.
Craniectomy; dominant; middle cerebral artery; outcome; stroke
Data is scarce on prevalence of extracranial carotid atherosclerosis (ECA) in strokes <50 years and its association with lifestyle factors.
Study role of (a) ECA in non-cardio-embolic anterior circulation young strokes, and (b) smoking and alcohol in ECA.
Materials and Methods:
Cardiovascular risk factors and evidence of ECA on carotid doppler ultrasound (CDUS) was evaluated in an one-year preliminary cross-sectional study of consecutive strokes between 20 years and 49 years. Females were excluded.
There were 46 male strokes (mean age 38.26 yrs), 17.39% had hypertension, 2.23% had coronary artery disease; none was diabetic. Tobacco users and alcohol consumers were 24/46 (52.17%) cases each. ECA was found in 14/46 (30.44%) cases. Seven of these 14 (50%) i.e., 7/46 cases (15.21%) had carotid occlusion, four had <50%, three had >70% stenosis. ‘Smoking and smokeless tobacco use’ was found in 71.42% (10/14) symptomatic carotid lesions compared to 43.75% (14/32) strokes without carotid lesions. Prevalence odds ratio for tobacco use and ECA was 3.21 (95% CI: 0.83-12.44) while that of alcohol and ECA was 1.33 (95% CI: 0.38-4.72).
Prevalence of ECA in strokes <50 years was high due to lifestyle factors which predispose to atherosclerosis at younger age.
Alcohol; extracranial carotid atherosclerosis; lifestyle factors; smoking; young stroke
Complete long segment carotid occlusion presents a treatment challenge. These patients cannot be managed adequately by endarterectomy or stenting. Despite best medical management, many continue to develop recurrent strokes. In this select group of patients, there may be role for flow augmentation techniques like superficial temporal-middle cerebral artery bypass. We report a patient who was thus successfully treated and remains asymptomatic. The relevant literature is reviewed.
Extra cranial-intracranial bypass; recurrent stroke; superficial temporal - middle cerebral artery bypass; total carotid occlusion
Hemorrhagic type of moyamoya disease (MMD) is extremely rare in children. Ischemia following hemorrhage is very rare in MMD. There are only 11 reports of mixed-type of MMD, with the patient having both hemorrhage and ischemia in the same hemisphere at the time of presentation, or at different time periods. The ischemia is usually secondary to a precipitating cause. However, there are no reports of a child presenting with both ischemia and hemorrhage in different hemispheres. We present a previously unreported phenomenon of MMD, presenting as hemorrhage and ischemia in opposite hemispheres and review the relevant literature.
Hemorrhage; ischemia; mixed type; moyamoya disease; pediatric
The aim of this study is to characterize the clinical profile of patients with alcohol related seizures (ARS) and to identify the prevalence of idiopathic generalized epilepsy (IGE) in the same.
Materials and Methods:
100 consecutive male patients presenting to a tertiary care center in South India with new onset ARS were analyzed with alcohol use disorders identification test (AUDIT) score. All underwent 19 channel digital scalp electroencephalography (EEG) and at least computed tomography (CT) scan.
A total of 27 patients (27%) who had cortical atrophy on CT had a mean duration of alcohol intake of 23.62 years compared with 14.55 years in patients with no cortical atrophy (P < 0.001). Twenty-two patients (22%) had clustering in the current episode of whom 18 had cortical atrophy. Nearly, 88% patients had generalized tonic clonic seizures while 12% who had partial seizures underwent magnetic resonance imaging (MRI), which identified frontal focal cortical dysplasia in one. Mean lifetime duration of alcohol intake in patients presenting with seizures within 6 hours (6H-gp) of intake of alcohol was significantly lower (P = 0.029). One patient in the 6H-gp with no withdrawal symptoms had EEG evidence for IGE and had a lower AUDIT score compared with the rest.
CT evidence of cortical atrophy is related to the duration of alcohol intake and portends an increased risk for clustering. Partial seizures can be a presenting feature of ARS and those patients may benefit from MRI to identify underlying symptomatic localization related epilepsy (8.3% of partial seizures). IGE is more likely in patients presenting with ARS within first 6 hours especially if they do not have alcohol withdrawal symptoms and scalp EEG is helpful to identify this small subgroup (~1%) who may require long-term anti-epileptic medication.
Alcohol use disorders identification test; anti-epileptic drugs; convulsions; electroencephalography; ethanol; magnetic resonance imaging
In addition to changes in seizure frequency, pregnant women with epilepsy (WWE) are at increased risk of complications during pregnancy or delivery. In the absence of a nationwide WWE registry, hospital-based studies may provide important information regarding current management and outcomes in these patients.
The aims of this study were to determine changes in seizure frequency, and pregnancy and birth outcomes among pregnant WWE.
Materials and Methods:
We conducted a retrospective review of medical records of pregnant patients with epilepsy, who obtained medical care (from 2006 to 2011) at one of the general hospitals in the North-Eastern State of Malaysia. Data were collected for seizure frequency before and during the pregnancy, concurrent medications, pregnancy complications, and neonatal outcomes.
We reviewed records of 25 patients with a total of 33 different pregnancies. All patients were treated with antiepileptic medications during their pregnancies, with 42% monotherapy and 58% polytherapy. Seizure frequency decreased in 5 (15.2%), increased in 18 (54.5%) and unchanged in 10 (30.3%) cases of pregnancies. Pregnancy complications were anemia, gestational diabetes mellitus, gestational hypertension, intrauterine growth retardation, premature rupture of membrane, and vaginal bleeding. Preterm deliveries were recorded in 11 (33.3%) infants.
In our setting, many patients were being on polytherapy during their pregnancies. This underscores the need for planned pregnancies so that antiepileptic medications can be optimized prior to pregnancy.
Epilepsy; outcomes; pregnancy; women
Knowledge about epilepsy and its management is not satisfactory among school students in developing countries. The present study was planned to ascertain the knowledge, attitude and practices (KAP) of students regarding first-aid management of epilepsy seizures in school setting.
Materials and Methods:
A total of 177 students of government schools of Chandigarh, a city of northern India, were taken. They were administered with a pre-tested semi-structured questionnaire (for knowledge and attitude assessment) and an observational checklist after role play (for practice assessment) on first-aid management of epilepsy. A scoring system was devised to quantify the knowledge and practices of students.
Seventy-one percent of them had either heard or read about epilepsy. Half of the students believed epilepsy as a hindrance to education. Ayurvedic treatment was preferred by more than half of the students; however, many believed that visit to religious places and exorcism as ways to cure epilepsy. Nearly 74% of students would call a doctor as first-aid measure for seizure in a person with epilepsy.
We concluded that the knowledge about various aspects of epilepsy was average among school students in Chandigarh. However, there was no significant difference in knowledge, attitude and practice between students who lived in urban, urban slum and rural areas. It is recommended that first-aid management of seizures in epilepsy should be a part of school curriculum.
Beliefs; epilepsy; practices; role play; school health
One-third of women with epilepsy (WWE) may experience infertility (failure to conceive after 12 months of regular unprotected intercourse). We aimed to compare the hormone profile of WWE and infertility (WWE-I) with that of WWE who had conceived earlier (WWE-F).
Materials and Methods:
In the Kerala Registry of Epilepsy and Pregnancy, we compared the clinical and hormone profile of 50 WWE-I and 40 age-matched WWE-F. Subjects were examined and blood samples were drawn in follicular phase (1-14 days) for 21 WWE-I and 18 WWE-F, in luteal phase (15-30 days) for 23 WWE-I and 15 WWE-F and beyond 30 days for 6 WWE-I and WWE-F who had irregular cycles.
The two groups were comparable regarding physical, epilepsy syndrome, duration of epilepsy, body mass index, and serum cholesterol levels. Menstrual periods were irregular for 6 WWE-I and 5 WWE-F. The WWE-I group (compared to the WWE-F group) had significantly (P < 0.01) higher levels of dehydroepiandrostenedione (2.0 ± 1.7 ug/mL vs. 1.0 ± 0.7 ug/mL) and luteinizing hormone-LH (26.4 ± 37.3 mIU/mL vs. 9.9 ± 14.5 mIU/mL) and lower levels of progesterone (5.2 ± 9.2 ng/mL vs. 10.4 ± 13.4 ng/mL). There was no significant difference in the levels of FT3, FT4, thyroid stimulating hormone, prolactin, follicle-stimulating hormone (FSH), progesterone, testosterone, or androstenedione levels. The WWE-I had 8.5 times higher risk (95% confidence interval 1.2-59.9) of abnormal LH/FSH ratio. WWE who were on antiepileptic drugs (AEDs) (compared to WWE who were not on AEDs) had higher risk of elevated LH/FSH ratio.
The hormone profile of WWE-I is significantly different from that of WWE-F. These variations need to be interpreted with caution as a causal relationship to epilepsy or use of antiepileptic drugs need to be established through further studies.
Antiepileptic drug; infertility; pregnancy; reproductive endocrine
Our present observational study attempted to evaluate the clinical profiles, diagnosis, treatment and follow-up results of 51 pediatric neurocysticercosis patients over a mean duration of five years (from January 2006 to December 2010).
Materials and Methods:
Diagnosis was mainly based on clinical features, computed tomography (CT)/magnetic resonance imaging scan and exclusion of other causes. Patients with active, transitional cysts and seizure were treated with albendazole for 28 days, steroids and anticonvulsants.
A total of 38 patients completed this study. Mean age of the presentation was 8.47 ± 3.19 years 52.6% of the patients were female. Overall patients presented with generalized seizure in 55.3%, focal in 31.6%, headache ± vomiting in 63.2%, focal neurodeficit in 10.5% and combination of symptoms in 60.5% cases. Contrast CT brain showed a solitary lesion in 27 (71.1%) and multiple in the rest. At presentation lesions were transitional in 58.2%, inactive in 20% and mixed in 14.6%. After a mean of 2 years, seizure persisted in 9 (23.7%) and headache in 8 (21.1%) of whom six had normal electroencephalography (EEG) while one each showed focal slowing, generalized slowing and epileptiform discharges. During the follow-up, CT scan brain 44.7% lesions calcified, 31.6% disappeared, 10.5% regressed and the rest persisted.
Solitary ring enhancing lesions (transitional stage) involving the parietal lobe was the commonest CT picture at presentation. Generalized tonic-clonic seizure was the most common type of seizure. Number of lesions, persistence of lesion, number of seizures, EEG abnormality at presentation were not found to be prognostically significant (P > 0.05).
Brain; epilepsy; lesion; neurocysticercosis; parenchyma; solitary
Benign occipital seizure syndromes are benign childhood epilepsy syndromes and are mainly of two types, Panayiotopoulos syndrome, an autonomic epilepsy and idiopathic childhood occipital epilepsy of Gastaut (ICOE-G) including the idiopathic photosensitive occipital lobe epilepsy. Although both these types are categorized as occipital seizures, they are distinct in presentation and management. They can also be tricky to diagnose as visual symptoms may not always be the presenting feature and it is also not very easy to elicit visual hallucinations during history taking. These seizures have a good response to treatment; however, there could be atypical evolution and refractoriness to treatment especially with ICOE-G. We describe three children who presented with visual and non-visual symptoms and the electroencephalography (EEG) in all the three cases showed occipital paroxysms. We have emphasized the clues in the clinical history and EEG leading to the diagnosis of these distinct epilepsy syndromes. We have also discussed the natural course of these epilepsy syndromes with some atypical evolution, which clinicians need to be aware of during treatment of these children.
Gastaut; occipital seizures; panayiotopoulos syndrome
A fugue state is defined as an altered state of consciousness with varying degrees of motor activity and amnesia for the event. It may last for hours to days and may be psychogenic or organic in nature. Epileptic fugue states can be encountered in patients with absence or complex partial nonconvulsive status epilepticus or may occur as a postictal phenomenon in patients with generalized seizures. “absence status epilepticus” (AS) is rare and seen in only 2.6% of the cases with “childhood absence epilepsy” (CAE). The diagnosis of AS can be elusive, but sudden onset and termination of the fugue state, classical electroencephalogram (EEG) features, and response to a therapeutic trial of benzodiazepines helps in confirming the diagnosis and differentiating it from nonepileptic fugue states.
We report a childhood onset case, with a 10 years history of recurrent episodes of prolonged fugue state lasting for up to 24 h, as the sole manifestation of epileptic seizures. The EEG features were suggestive of an AS, but there was no history of typical absences, myoclonus, or generalized tonic clonic seizures. This unusual and rare case cannot be categorized into one of the defined epilepsy syndromes like CAE but belongs to a recently identified syndrome of idiopathic generalized epilepsy known as “Absence status epilepsy” in which AS is the sole or the predominant seizure type.
Absence status epilepticus; fugue state; idiopathic generalized epilepsy
Opercular myoclonic-anarthric status epilepticus (OMASE) is an uncommon disorder of diverse etiology. This condition is characterized by fluctuating cortical dysarthria associated with epileptic myoclonus involving glossopharyngeal musculature bilaterally. We report two cases of OMASE of vascular etiology in adults. In both patients, ictally clonic expression was consistent with epilepsia partialis continua and bilateral, symmetrical involvement of soft palate in one patient and tongue, lips, chin and inferior jaw in both patients due to bilateral projections of the inferior corticonuclear pathways. The inferior rolandic area of dominant and high frontal region in non-dominant hemispheres were involved by an epileptogenic lesion of vascular etiology, which was confirmed by magnetic resonance imaging of brain and single photon emission computerized tomography. Carotid Doppler study showed thrombosis of internal carotid artery in both patients, suggestive of an embolic origin. Early recognition of OMASE is important for early management of carotid occlusive disease.
Epilepsia partialis continua; glossopharyngeal musculature; opercular myoclonic status epilepticus-anarthria; stroke
We discuss the clinical and imaging perspective in a case of a 78-year-old male who developed slurring of speech and ataxia acute in onset for the last 3 days. During his hospital stay, he developed multiple episodes of focal seizures without secondary generalization involving the angle of mouth on the right side. The patient had ataxia and positive cerebellar signs. In the past, the patient was treated for amoebic liver abscess and had undergone percutaneous aspiration of abscess. The patient was prescribed oral metronidazole and was discharged. This time, the patient underwent magnetic resonance imaging examination, which revealed lesion highly suggestive of metronidazole-induced encephalopathy. The offending drug was discontinued immediately after which the patient improved clinically. A follow-up scan was performed after 12 days and showed complete resolution of lesions.
Diffusion-weighted imaging; fluid-attenuated inversion-recovery; metronidazole-induced encephalopathy; magnetic resonance imaging
In resource-poor settings, the management of neuromyelitis optica (NMO) and NMO spectrum (NMOS) disorders is limited because of delayed diagnosis and financial constraints.
To device a cost-effective strategy for the management of NMO and related disorders in India.
Materials and Methods:
A cost-effective and disease-specific protocol was used for evaluating the course and treatment outcome of 70 consecutive patients.
Forty-five patients (65%) had a relapse from the onset and included NMO (n = 20), recurrent transverse myelitis (RTM; n = 10), and recurrent optic neuritis (ROPN; n = 15). In 38 (84.4%) patients presenting after multiple attacks, the diagnosis was made clinically. Only 7 patients with a relapsing course were seen at the onset and included ROPN (n = 5), NMO (n = 1), and RTM (n = 1). They had a second attack after a median interval of 1 ± 0.9 years, which was captured through our dedicated review process. Twenty-five patients had isolated longitudinally extensive transverse myelitis (LETM), of which 20 (80%) remained ambulant at follow-up of 3 ± 1.9 years. Twelve patients (17%) with median expanded disability status scale (EDSS) of 8.5 at entry had a fatal outcome. Serum NMO-IgG testing was done in selected patients, and it was positive in 7 of 18 patients (39%). Irrespective of the NMO-IgG status, the treatment compliant patients (44.4%) showed significant improvement in EDSS (P ≤ 0.001).
Early clinical diagnosis and treatment compliance were important for good outcome. Isolated LETM was most likely a post-infectious demyelinating disorder in our set-up. NMO and NMOS disorders contributed to 14.9% (45/303) of all demyelinating disorders in our registry.
Demyelinating disease registry; immunosuppression; India; neuromyelitis optica; neuromyleitis optica spectrum disorders
To review clinical characteristics and response to immunomodulation therapy in autoimmune encephalitis presenting with status epilepticus (SE), epilepsy, and cognitive decline.
Observational, prospective case series.
All India Institute of Medical Sciences, New Delhi, India.
Materials and Methods:
Prospective analysis of 15 patients, who presented with SE, epilepsy, cognitive decline, and other neurological symptoms with positive autoantibodies. Demographic and clinical characteristics were recorded. Brain magnetic resonance imaging (MRI), cerebrospinal-fluid analysis (CSF), and tumor screening were done periodically. Treatment received and responses (categorized as per patients and treating doctor's information) were noted.
There were 15 (males = 10) patients of autoimmune encephalitis. The mean age of presentation was 24 years (range: 2-64 years). The most common onset was subacute (64%) and four (29%) patients presented as SE. Predominant clinical presentations were seizures (100%) almost of every semiology. CSF was done in 10 patients; it was normal in 60%. Brain MRI was done in all patients, in six (40%) it was normal, six (40%) showed T2W and FLAIR hyperintensities in bilateral limbic areas. Antibodies found were the N-methyl-D-aspartate receptor antibody in seven (50%), voltage-gated potassium channel antibody in five (36%), two of antiglutamic acid decarboxylase, and one patient with double stranded DNA (dsDNA) antibodies. None showed evidence of malignancy. Patients received immunotherapy, either steroids, intravenous immunoglobulin, or both. Follow-up showed significant improvement in majority of cases, neither further seizures nor relapse in nine (67%) cases. One death occurred, due to delayed presentation.
Uncommon but potentially reversible causes of SE, epilepsy, and cognitive decline may be immune-related and high index of suspicion will prevent missing the diagnosis.
Autoimmune encephalitis; cognitive decline; drug refractory epilepsy; seizures; status epilepticus
Cognitive impairments in multiple sclerosis (MS) are now well recognized worldwide, but unfortunately this domain has been less explored in India due to many undermining factors. The aim of this study was to evaluate cognitive impairments in Indian MS patients with visual or upper limb motor problems with the help of short version of Montreal cognitive assessment test (MoCA).
Subjects and Methods:
Thirty MS patients and 50 matched controls were recruited for the 12 points MoCA task. Receiver operating characteristic curve (ROC) analysis was performed to determine optimal sensitivity and specificity of the 12 points MoCA in differentiating cognitively impaired patients and controls.
The mean 12 points MoCA scores of the controls and MS patients were 11.56 ± 0.67 and 8.06 ± 1.99, respectively. In our study, the optimal cut-off value for 12 points MoCA to be able to differentiate patients with cognitive impairments from controls is 10/12. Accordingly, 73.3% patients fell below the cut off value. Both the groups did not have significant statistical differences with regard to age and educational years.
The 12 points, short version of MoCA, is a useful brief screening tool for quick and early detection of mild cognitive impairments in subjects with MS. It can be administered to patients having visual and motor problems. It is of potential use by primary care physicians, nurses, and other allied health professionals who need a quick screening test. No formal training for administration is required. Financial and time constraints should not limit the use of the proposed instrument.
Cognitive impairments; Indian; montreal cognitive assessment test; multiple sclerosis
Two women in their 60's are presented to us with sudden falls of acute onset. Prolonged observation revealed a gradually evolving syndrome of paroxysmal right sided faciobrachial dystonic (FBD) posturing lasting seconds. Both patients went on to develop hyponatremia, following which the episodes worsened and appeared on both sides. In both cases, prolonged electroencephalography monitoring and magnetic resonance imaging brain were normal and the response to conventional anticonvulsants was poor. One patient improved spontaneously over 6 months. The 2nd patient developed an amnestic syndrome and was started on intravenous methylprednisolone with which her movement disorder abated. Her amnestic syndrome improved and she was discharged on oral steroids. Both patients tested positive for leucine-rich glioma inactivated 1 (LGi1) antibodies. We present the first case reports of FBD episodes and drop attacks owing to LGi1 encephalitis from India and review the relevant literature pertinent to the subject.
Anti-leucine-rich glioma inactivated 1 antibodies; drop attacks; faciobrachial dystonic seizures; leucine-rich glioma inactivated 1 encephalitis; paroxysmal non-kinesogenic dystonia