Cognitive behavioural impairment with irreversible sensorineural deafness as a complication of West Nile encephalitis.

Despite common clinical features to suggest encephalitis, different viral encephalitides are known to have some specific clinical features, which if present, may suggest infection by a particular virus. West Nile viral (WNV) encephalitis has not been described with any specific diagnostic feature so far. In this context, we describe three patients of West Nile encephalitis (WNE) who had behavioural and cognitive impairment with acute irreversible bilaterally symmetrical sensorineural deafness. Clinical profiles of these cases suggest that the patients who present with prominent behavioural and cognitive changes and have in addition features of bilateral sensorineural deafness may be considered as the possible case of WNE.

New daily persistent headache: An evolving entity.

New daily persistent headache (NDPH) is characterized by an abrupt onset of headache that becomes a daily entity, is unremitting and continuous from the onset, and lasts for more than 3 months. Dr Walter Vanast first described NDPH in the year 1986. Originally, it was proposed as a chronic daily headache but it was placed under "other primary headaches" in the International Classification of Headache Disorder Second Edition (ICHD 2nd edition). However, with evolving literature and better understanding of its clinical characteristics, it was classified as a "chronic daily headache" in the ICHD 3rd edition beta. There are still many knowledge-gaps regarding the underlying cause, pathophysiology, natural history and treatment of NDPH. This review tries to revisit the entity and discusses the current status of understanding regarding NDPH.

IMPETUS Stroke: Assessment of hospital infrastructure and workflow for implementation of uniform stroke care pathway in India.

BACKGROUND: India accounts for 13.3% of global disability-adjusted life years (DALYs) lost due to stroke with a relatively younger age of onset compared to the Western population. In India's public healthcare system, many stroke patients seek care at tertiary-level government-funded medical colleges where an optimal level of stroke care is expected. However, there are no studies from India that have assessed the quality of stroke care, including infrastructure, imaging facilities, or the availability of stroke care units in medical colleges. AIM: This study aimed to understand the existing protocols and management of acute stroke care across 22 medical colleges in India, as part of the baseline assessment of the ongoing IMPETUS stroke study. METHODS: A semi-structured quantitative pre-tested questionnaire, developed based on review of literature and expert discussion, was mailed to 22 participating sites of the IMPETUS stroke study. The questionnaire assessed comprehensively all components of stroke care, including human resources, emergency system, in-hospital care, and secondary prevention. A descriptive analysis of their status was undertaken. RESULTS: In the emergency services, limited stroke helpline numbers, 3/22 (14%); prenotification system, 5/22 (23%); and stroke-trained physicians were available, 6/22 (27%). One-third of hospitals did not have on-call neurologists. Although non-contrast computed tomography (NCCT) was always available, 39% of hospitals were not doing computed tomography (CT) angiography and 13/22 (59%) were not doing magnetic resonance imaging (MRI) after routine working hours. Intravenous thrombolysis was being done in 20/22 (91%) hospitals, but 36% of hospitals did not provide it free of cost. Endovascular therapy was available only in 6/22 (27%) hospitals. The study highlighted the scarcity of multidisciplinary stroke teams, 8/22 (36%), and stroke units, 7/22 (32%). Lifesaving surgeries like hematoma evacuation, 11/22 (50%), and decompressive craniectomy, 9/22 (41%), were performed in limited numbers. The availability of occupational therapists, speech therapists, and cognitive rehabilitation was minimal. CONCLUSION: This study highlighted the current status of acute stroke management in publicly funded tertiary care hospitals. Lack of prenotification, limited number of stroke-trained physicians and neurosurgeons, relatively lesser provision of free thrombolytic agents, limited stroke units, and lack of rehabilitation services are areas needing urgent attention by policymakers and creation of sustainable education models for uniform stroke care by medical professionals across the country.

Normotensive state during acute phase of hypertensive intracerebral hemorrhage.

Objectives: Hypertensive hemorrhage is a leading cause of intracerebral haemorrhage (ICH), although some of these patients may not present with high blood pressure (BP) at the time of ICH. Materials and Methods: This retrospective study included patients with history of hypertension presenting with ICH. Patients with systolic BP recording of more than 140 mmHg were included in hypertension group (group I). Patients whose BP rose to hypertension range after fluid correction were included in group II and patients with BP <140 mmHg on consecutive 1-week BP recordings were included in group III. Clinical features including volume of ICH of all the three groups were noted. Outcome in the form of mortality was analyzed. Chi-square test was used for categorical variables and independent t-test for continuous variables. P < 0.05 was considered significant. Results: Ninety-two ICH patients with history of hypertension were included in the study. Of them, 20 patients (22%) presented with BP <140 mmHg systolic at the time of ICH. After fluid correction, it rose to hypertensive range in 9 (10%) but remained normal in 11 patients (12%) during consecutive recordings for 1-week post-admission. On comparing normotensive and hypertensive groups, significant difference was seen in survival and volume of ICH. Conclusion: There is a subset of hypertensive patients who may present with normal BP recording during acute ICH. The BP rises subsequently with the correction of hypovolemia in some. The volume of hemorrhage in normotensives is relatively small but whether this translates into better prognosis needs further studies.

A New Surface Technique for Phrenic Nerve Conduction Study.

Objective: To report a new patient friendly and convenient technique for phrenic nerve conduction with alternative sites of stimulation and recording. Methods: Phrenic nerve conduction was performed in forty volunteers and ten patients of peripheral neuropathy. Active recording electrode was placed in tenth intercostal space 2.5 cm away from para-spinal muscles (mid-scapular line), reference electrode in eighth intercostal space just medial to subcostal margin with ground between stimulating and recording electrode. Stimulation was done at the level of crico-thyroid space near or under the posterior margin of sternocleidomastoid muscle. This new method was compared with existing ones. Analysis: Data was analysed using SPSS 23 version. Correlation between height, weight, body mass index, age, and chest expansion was done using bi-variate correlation. Mean latency and amplitude of the study method were compared with other methods using MANNOVA test. Results: Total of forty subjects were studied. Thirty-seven were male subjects. Mean age was 28.03 ± 9.63 years, height 168.0 ± 9.60 cm and chest expansion 3.53 ± 0.64 cm. Right sided phrenic nerve mean latency was 5.99 ± 0.629 ms and amplitude 1.088 ± 0.178 mV. Left sided phrenic nerve conductions showed mean latency of 6.02 ± 1.82 ms, amplitude of 1.092 ± 0.2912 mV. These standard deviations were smaller than what were observed with other methods suggesting increased consistency of our results. There was no correlation between phrenic nerve conduction with age, height, gender or chest expansion. Conclusion: This study method gave a better as well as consistent morphology, higher amplitude and required lower amount of current strength. It was superior to previously reported methods in consistency of normative data.

Study Protocol: IMPETUS: Implementing a Uniform Stroke Care Pathway in Medical Colleges of India: IMPETUS Stroke.

Introduction: In India, a national program for stroke (national programme for the control of cardiovascular diseases, diabetes, cancer, and stroke) and stroke management guidelines exist. Its successful implementation would need an organized system of stroke care in practice. However, many challenges exist including lack of awareness, prehospital notification systems, stroke ready hospitals, infrastructural weaknesses, and rehabilitation. We present here a protocol to investigate the feasibility and fidelity of implementing a uniform stroke care pathway in medical colleges of India. Methods and Analysis: This is a multicentric, prospective, multiphase, mixed-method, quasi-experimental implementation study intended to examine the changes in a select set of stroke care-related indicators over time within the sites exposed to the same implementation strategy. We shall conduct process evaluation of the implementation process as well as evaluate the effect of the implementation strategy using the interrupted time series design. During implementation phase, education and training about standard stroke care pathway will be provided to all stakeholders of implementing sites. Patient-level outcomes in the form of modified Rankin Scale score will be collected for all consecutive patients throughout the study. Process evaluation outcomes will be collected and reported in the form of various stroke care indicators. We will report level and trend changes in various indicators during the three study phases. Discussion: Acute stroke requires timely detection, management, and secondary prevention. Implementation of the uniform stroke care pathway is a unique opportunity to promote the requirements of homogenous stroke care in medical colleges of India.

Respiratory Assessment of Myasthenia Gravis Patients Using Repetitive Nerve Stimulation of Phrenic and Intercostal Nerves.

BACKGROUND: We evaluated decremental response from phrenic and intercostal nerves using slow repetitive nerve stimulation test (RNST) to look for its diagnostic significance in sero-negative predominantly bulbar myasthenia gravis (MG) with normal peripheral or cranial nerve RNST. METHODS: RNST from phrenic and intercostal nerves was performed along with standard RNST from abductor digiti minimi (ADM), trapezius, nasalis and orbicularis oculi muscles in 10 normal individuals (group I), 10 patients with neurological disorders other than MG (group II) and 10 patients with MG (group III). We evaluated the presence of positive response in first two groups (group I and II) and absence of negative response in group III. Spirometry was also performed in MG patients. RESULTS: Mean baseline decrement in I/C RNST in three groups was -2.06±1.33 %, -2.5±2.18% and -27.1±17.9 % respectively. One minute post exercise decrement in I/C RNST in three groups was -2.9±1.36%,-2.9±1.36% and -32.9±17.9% respectively. RNST of phrenic nerve showed mean baseline decrement of -2.1±2.3%, -3.2±2.6 % and -18.3±30.3% in three groups respectively. One minute post exercise decrement percentage were -2.2±1.18% in group I, -4.8±2.18% in group II and -29.2±19.2% in group III. RNST of peripheral nerves were negative in two patients who were bulbar sero-negative MG, however, significant decrement was seen in intercostal and phrenic nerve RNST. CONCLUSION: Intercostal and phrenic nerve RNST are a better test for assessing respiratory involvement specially in patients presenting with bulbar symptoms and having negative RNST of peripheral nerves.

Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) in limited cutaneous sclerosis: a rare disease combination.

A 26-year-old patient of limited cutaneous sclerosis presented to us with insidious-onset posterior fossa symptoms of headache, vomiting, vertigo and gait imbalance, progressing over a period of 3 weeks. A diagnosis of chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids was made by combining the clinical features with radiological evidence showing punctate infiltration of the pons, brainstem and cerebellum. Relevant differentials in the form of neurosarcoid, infections, central nervous system (CNS) lymphoma and Neuro-Behcet's disease were ruled out by history and investigations. The patient responded dramatically to steroid therapy, and had no neurological deficits after 18 months of follow-up. This case highlights the rare association of a not-so-common immunological disease with a rare neurological disease.

Intravenous Steroid Days and Predictors of Early Oral Steroid Administration in Tuberculous Meningitis: A Retrospective Study.

Intravenous (IV) dexamethasone is recommended for 14 days in stage 1 and 28 days in stage 2/3 tuberculous meningitis (TBM). We used a different steroid protocol. We shifted TBM patients to oral steroids after 48 hours of sustained improvement on IV steroids (oral group). Patients who worsened after shifting to oral steroids were reinitiated on IV steroids. Once they showed a consistent improvement for 48 hours, the IV steroids were overlapped with oral steroids for 7-10 days to taper off IV steroids (overlap group). We compared total IV steroid days in our patients with the recommended treatment and identified predictors that favored the oral group. This was a retrospective study. We included 98 patients with TBM (66 in the overlap group and 32 in the oral group) from January 2013 to July 2018. The median IV steroid days were 9 days (interquartile range of 4-12; 2-3.5 days in the oral group and 10-11.5 days in the overlap group). The mortality rate was 6.1%. The logistic regression model showed that TBM patients with basal exudate, tuberculoma, and modified Rankin scale (mRS) < 3 had a higher probability for going to the oral group. We conclude that total IV steroid days can be reduced in TBM patients by our method of steroid use. Presence of basal exudates and tuberculoma may favor early shifting from IV to oral steroid, whereas higher mRS may require a relatively longer course of IV steroid.

Clinical characteristics of migraine in patients with calcified neurocysticercosis.

BACKGROUND: Patients with neurocysticercosis (NCC) may have an increased frequency of migraine. We studied the characteristics of migraine attacks in patients with calcified NCC on brain imaging. METHODS: Of 350 migraine patients who visited our outpatient department from January 2016 through June 2018, 166 had undergone brain imaging. Seventy-two patients with migraines had calcified NCC. The migraine attacks of the patients with calcification (MiC) were compared with those of 94 patients without calcification (MiNC). RESULTS: Side-locked headaches were seen in 48.6% of the MiC patients. Aura preceding the migraine attack was more common in the MiC group than in the MiNC group (22.22% vs 7.4%; p=0.001). The MiC group had fewer headache episodes per month (5 vs 6; p<0.0001) with fewer common associated features (62.5% vs 84.1%; p=0.006). They also required fewer drugs for secondary prophylaxis (2 vs 3; p=0.002). CONCLUSIONS: Some of the clinical characteristics of migraine attacks are sufficiently different in patients with and without NCC. This may suggest some influence of NCC in the pathophysiology of migraine to account for alterations in the clinical manifestations of the disease. Whether this difference could be inferred as NCC having a causative role in migraine needs further research.

Why do neurologists miss catatonia in neurology emergency? A case series and brief literature review.

Catatonia is a well-described clinical syndrome characterized by features that range from mutism, negativism and stupor to agitation, mannerisms and stereotype. Causes of catatonia may range from organic brain disorders to psychiatric conditions. Despite a characteristic syndrome, catatonia is grossly under diagnosed. The reason for missed diagnosis of catatonia in neurology setting is not clear. Poor awareness is an unlikely cause because catatonia is taught among conditions with deregulated consciousness like vegetative state, locked-in state and akinetic mutism. We determined the proportion of catatonia patients correctly identified by neurology residents in neurology emergency. We also looked at the alternate diagnosis they received to identify catatonia mimics. Twelve patients (age 22-55 years, 7 females) of catatonia were discharged from a single unit of neurology department from 2007 to 2017. In the emergency department, neurology residents diagnosed none of the patients as catatonia. They offered diagnosis of extrapyramidal syndrome in 7, meningitis in 2, and conversion reaction, acute psychosis/encephalopathy and non-convulsive status epilepticus in one each. Their final diagnosis at discharge was catatonia due to general medical condition in 6 (progressive supranuclear palsy in 2, post-status epilepticus, uremic encephalopathy, glioblastoma multiforme and tuberculous meningitis in one each), catatonia due to major depression in 4, schizophrenia and idiopathic catatonia in one each. Extrapyramidal syndrome appeared as common mimic of catatonia. The literature reviewed also revealed the majority of organic catatonia secondary to causes that are usually associated with extrapyramidal features. Therefore, we suggest that neurologists should consider catatonia in patients presenting with extrapyramidal syndromes.

Benign Acute Childhood Myositis: A Benign Disease that Mimics More Severe Neuromuscular Disorder.

CONTEXT: Proximal lower limb weakness presenting acutely with or without preceding fever is a strong mimic of Guillain-Barré syndrome (GBS). Benign acute childhood myositis (BACM) forms an important differential diagnosis in such cases. AIM: To characterize the clinical and laboratory findings of patients with BACM for better understanding of the disease. SETTINGS AND DESIGN: This prospective longitudinal study was conducted in a tertiary care hospital of northern India. MATERIALS AND METHODS: Thirty-two patients presenting in the outpatient or emergency clinic of the hospital with severe myalgia that exacerbated with straight leg raising test and fever from July 2016 to July 2017 were included in the study. STATISTICAL ANALYSIS: All the continuous data were expressed as number and percentage or mean ± standard deviation/median. Non-parametric continuous data between groups were analyzed by Friedman's test. RESULTS: The mean age of the patients was 14.3 (±8.7) years and they presented after a nonspecific febrile illness in most of the cases (53.1%). The symptoms resolved after a mean of 5.7 (±1.6) days. Myalgia was present in 21 (65%) cases, whereas proximal weakness was the prominent finding in 14 (43%) cases. Electrolyte abnormality (hypokalemia) was present in four (12.5%) cases. In all patients, the muscle enzymes (creatine phosphokinase, serum glutamic oxaloacetic transaminase, serum glutamic pyruvic transaminase, and lactate dehydrogenase) were elevated at presentation, and electromyography showed myopathic pattern. A significant recovery took place in the next 5-7 days. CONCLUSION: BACM should be actively looked for in cases of painful acute proximal limb weakness in the adolescents.

Acute intractable vomiting: Do I belong somewhere else?

Patients suffering from acute intractable vomiting are usually treated in the Gastroenterology department. The causes of acute intractable vomiting range from acute pancreatitis and acute intestinal obstruction to cardiac causes like acute myocardial infarction and neurological causes like posterior circulation stroke. However, most of the underlying causes of acute intractable vomiting also produce other telltale signs/symptoms. Rarely, isolated acute intractable vomiting may be the initial symptom of a recurrent neurological syndrome of neuromyelitis optica spectrum disorder (NMOSD). Not only can it be promptly treated if diagnosed correctly, but also a timely diagnosis may help in prevention of recurrent neurological deficits, which can sometimes be life threatening. We present three cases of NMOSD that presented with intractable vomiting and were treated in a Gastroenterology facility prior to their diagnosis.