Hyperhomocysteinemia induced locked-in syndrome in a young adult due to folic acid deficiency.

'Locked-in syndrome (LIS)' is a neurological disorder, often missed initially and can have grave consequences. A rare case of LIS caused due to folic acid deficiency-induced hyperhomocysteinemia is being described here. A 16-year-old boy presented with complaints of sudden onset weakness of all the four limbs with loss of voice for one day. All the tendon reflexes were increased, bilateral planters were extensor and sensory system was intact. Patient was conscious and responded to verbal commands by ocular movements in vertical direction. Hence, a diagnosis of LIS was made. Magnetic resonance imaging of the head revealed an acute infarct in ventral pons. Serum homocysteine level was elevated (20.65 µmol/l) and folic acid level was severely low (1.7 nmol/ml). Cause of LIS was found to be hyperhomocysteinemia induced stroke in the pons, related to folic acid deficiency. The patient was managed with antiplatelet agents and folic acid supplementation and was discharged subsequently. Recognition of LIS is important as casual remarks at bedside can severely traumatize an already paralyzed but conscious and awake patient. Folic acid deficiency can lead to hyperhomocysteinemia, which can cause strokes and even LIS. Prevention of hyperhomocysteinemia may possibly prevent such neurological disasters.

Variations in electroencephalography with mobile phone usage in medical students.

BACKGROUND: Electromagnetic fields (EMFs) emitted by cellular telephones may cause neurological ill effects like cognitive dysfunction, emotional instability, and even brain tumors. Slowing of brain activity on electroencephalography (EEG) has been shown. However, these findings need further validation. AIMS: EEG changes and adverse effects experienced following cell-phone use were studied. SETTINGS AND DESIGN: The study was conducted in the Department of Neurology of a tertiary care university hospital in India on North Indian students of the University, from August 2017 to October 2017. MATERIALS AND METHODS: Twenty-one students underwent video-EEG recording before and after application of Samsung GT-56312 dual SIM smart phone in switched off, switched on, and switched on mode with conversation. STATISTICAL ANALYSIS USED: Average EEG frequencies and amplitudes were calculated for different brain regions. Chi-square tests and t-tests were used for comparison between variables. RESULTS: The mean age of 7 (33.3%) male and 14 (66.7%) female subjects was 20.76 ± 1.48 years. The average EEG frequencies following mobile phone application with conversation were higher and the amplitudes lower than the baseline values. Frequencies were greater on the right side. Slow waves were detected in the frontal region in 38.1%, in the parietal region in 33.3%, in the occipital and temporal region in 19.1%; and, generalized slow waves were seen in 9.5% students. During the experiment, 23.8% experienced headache, 19% experienced irritation, and 9.5% felt drowsy. Headache and loss of concentration (33.3%), sleep disturbances (28.6%), and fatigue (19%) were frequent in daily life. CONCLUSIONS: Experimental application of mobile phones may lead to some EEG changes and certain ill effects on the well-being. Hence, prolonged use of these gadgets warrants caution.

Prevalence of HIV Associated Neurocognitive Disorder using Modified Mini Mental State Examination and its Correlation with CD4 Counts and Anti-retroviral Therapy.

INTRODUCTION: HIV Associated Neurocognitive Disorder (HAND) is still prevalent even in the ART (Anti-Retroviral Therapy) era. It may have some association with CD4 counts and Anti-Retroviral Therapy. The prevalence of HAND in HIV-patients, was, therefore studied in the context of ART and CD4 counts. METHODS: Modified Mini Mental State Examination scores of 200 (65% males) HIV-positive patients and 200 controls were analyzed in the context of ART and CD4 counts. RESULTS: Maximum number of participants were educated between 8th-12th class (89.5%), aged between 25-50 years (81.5%) and a higher proportion of males had a CD4 count <500 (69.2%) (p=0.007). Using 3MS, 21% patients (mean 76.24±1.51) and none of the controls were found to be neurocognitively impaired. Mean scores of patients with CD4 counts<500(82.54±5.58) were lesser in comparison to those of patients with CD4 counts>500 (p<0.001). Those with an ART duration of <48 months had a lower score in comparison to those with an ART duration of >72 months (p=0.005).Most decrease from maximum value was seen in similarities (48.3 %), second recall (36.1 %), repetition (33.4 %), copying two pentagons (28.3 %), read and obey (24.0 %), mental reversal (22.7 %) and first recall (21.3%) parameters of Modified Mini Mental State Examination. CONCLUSIONS: HAND was less prevalent in the present study in comparison to past literature. CD4 counts and ART duration had an inverse association with the degree of cognitive impairment. The parameters of Modified Mini Mental State Examination showing maximum impairment may be compiled to form a short screening questionnaire.

Progressive supra-nuclear palsy: frequency of cardinal extrapyramidal features at first presentation.

OBJECTIVES: Cardinal extrapyramidal features of progressive supranuclear palsy (PSP) help in clinically differentiating this condition from Parkinson's disease and other Parkinsonian syndromes. However, not all extrapyramidal features may be initially present, thus posing a difficulty in early diagnosis. We studied their frequency at the time of first presentation. METHODS: Patients diagnosed clinically with PSP using the National Institute for Neurological Disorders and Society for PSP (NINDS/SPSP) criteria and seen between August 2010 and April 2013 were examined for the presence, 'presence with deviation' or absence of six extrapyramidal features: axial rigidity, symmetry, extended posture, backward falls, absence of tremors and lack of levodopa response. RESULTS: Twenty-eight patients (mean (SD) age 64.86 (9.72) years; 16 (57%) men) met the inclusion criteria. Of these, 14% had all six extrapyramidal features associated with PSP, 39% had five, 29% had four, 14% had three and 4% had two. The most frequent extrapyramidal sign was axial rigidity (68%). Axial plus peripheral rigidity was found in 18% of patients and peripheral rigidity alone in 14%. Extrapyramidal features were symmetrical in 29% and asymmetrical beyond 1 year in 29%. Body posture was extended in 46% and flexed in 21%. Backward falls were found in 50% and forward falls in 11%. Pill-rolling tremors were observed in 29%. Response to levodopa therapy was poor in 21% and good beyond 6 months in 39%. CONCLUSIONS: Only 14% of PSP patients present with all six cardinal extrapyramidal features. Also, deviations from standard descriptions are common in the initial stages of disease.

Nature of Parkinsonian features in multiple system atrophy.

Objectives: For this observational study, we evaluated the clinical profile of Parkinsonian features in multiple system atrophy (MSA), as there is no clarity about the specifics of these features in this disease compared to progressive supranuclear palsy (PSP) and Parkinson's disease (PD). Materials and Methods: Here, we selected 57 patients with MSA based on standard criteria and grouped them into two categories - Parkinsonian variant of MSA (MSA-P) and cerebellar variant of MSA (MSA-C). However, researchers did not distinguish among patients based on the nature of extrapyramidal syndrome or levodopa responsiveness. Then, we examined the patients at the time of their first visit to outpatient clinics or indoor wards and recorded and analyzed the specific extrapyramidal features or their variations. Results: The extrapyramidal features including levodopa responsiveness were highly variable among MSA-C as well as MSA-P patients. A subset of patients presented with features resembling PSP (symmetry [56.1%], axial rigidity [52.6%], backward falls [28.1%], and down-gaze restriction [17.5%]), while others presented with features resembling PD (asymmetry [43.9%], tremors [71.9%], and peripheral rigidity [40.4%]). After grouping patients based on predominant extrapyramidal features, 36.8% of patients had PD-like, 19.3% had PSP-like, and 43.9 % had mixed presentation. Moreover, 86% of patients had a perceptible levodopa response, including a sustained response for more than six months in 64% of patients. Conclusion: Extrapyramidal features in MSA patients may be PD-like, PSP-like, or mixed. Moreover, an initial presentation resembling PSP or PD may be deceptive and one must follow it up for MSA.

How well does peripheral blood neutrophil-to-lymphocyte ratio predict the severity and prognosis of hemorrhagic Stroke.

OBJECTIVE: We explored the blood neutrophil-to-lymphocyte ratio (NLR) as a prognostic marker and its relation with mortality and Modified Rankin Scale (mRS) score at discharge and at 3 months following ICH and also compared NLR with intracerebral hemorrhage (ICH) score, Sequential Organ Failure Assessment (SOFA) score and National Institutes of Health Stroke Scale (NIHSS) score. METHODS: The investigators calculated the NIHSS score, SOFA score, ICH score and NLR of 90 adult patients within 3 days of onset of stroke with evidence of hemorrhagic stroke in brain imaging and correlated it with in-hospital mortality, 3-month mortality and mRS at 3 months following stroke using regression analysis. RESULTS: Out of 90 individuals, there were 54 (60%) males and 36 (40%) females. The mRS score at 3 months significantly related to the admission NLR ratio >7 and SOFA score. Similarly, the in-hospital death and 3-month mortality was related to the admission NLR ratio >7 and ICH score. However, at a cut off value of NLR>3 for assessing the prognosis of the patients, we did not get significant results for mRS at 3 months following stroke and for in-hospital and 3-month mortality. CONCLUSION: A high NLR ratio >7 predicted worse outcomes in terms of mortality and morbidity at 3-months following haemorrhagic stroke. Hence, like ICH score, NLR can predict 3-month mortality following an acute haemorrhagic stroke and can also predict morbidity following 3 months of brain haemorrhage.

The Psychosocial Impact of COVID-19 on an Adult Indian Population.

BACKGROUND: Coronavirus disease (COVID-19) was a pandemic with many physical, psychological, and socioeconomic effects. COVID-19 caused a global increase in anxiety and depression because of its novelty, high infectivity, varied presentation, and unpredictable mortality. In the face of collapsing healthcare facilities, monetary setbacks, and loneliness because of lockdowns, people were anxious, and this was compounded by media sensationalism. We aimed to study the psychosocial impact of COVID-19 on the adult Indian population. METHODS: An online survey using SurveyMonkey was floated through WhatsApp messages in April 2020, using the 'chain-referral sampling' method. Responses from individuals >18 years were included, and questions included age, sex, occupation, demographics, and socioeconomic conditions. The prevalence of anxiety and depression was assessed using the Generalized Anxiety Disorder (GAD-7) and the Patient Health Questionnaire (PHQ-9) scales. Data was analyzed using IBM SPSS software, and predictors of anxiety and depression were assessed. RESULTS: A total of 2640 responses from individuals between 18 years and 81 years were analyzed, of which 39% were from females and 85% from those <50 years of age. There were students (15.6%), teachers (10.7%), healthcare workers (16.8%), homemakers (9%), and daily wage laborers (4.1%), among others. Nearly 80% lived in cities, 55% had salaried jobs, 37% were working from home, 22% were temporarily unemployed, 10% were feeling work stress, 11% had increased alcohol intake, and 7.5% saw an increase in domestic violence. The income of 50% was adversely affected. Nearly 50% of our respondents had some symptoms of anxiety, and 23% had significant anxiety (GAD ≥5). The presence of anxiety was significantly higher in females, younger adults, city dwellers, healthcare workers, unemployed people, individuals living away from home, those without fixed salaries, those with work stress, and in people whose incomes had been adversely affected by the pandemic. On logistic regression analysis, female sex, younger age, unemployment, lack of salaried jobs, work stress, being a healthcare worker, and media reports were independent predictors of anxiety. About 60% of our respondents had some symptoms of depression, with 26% having significant depression (PHQ-9 ≥5). The presence of depression was significantly higher in females, younger adults, city dwellers, unemployed people, individuals living away from home without fixed salaries, and people with work stress. On logistic regression analysis, younger age, female sex, unemployment, lack of salaried jobs, work stress, and media reports were independent predictors of depression. Among our respondents, 70% used the time during the lockdown to study, 77% caught up with their families, and 56% reconnected with hobbies. Nearly 88% of our respondents had adjusted to their changing circumstances, helped by their religious beliefs and faith, the support of family and friends, good government measures, and the assurance of healthcare. CONCLUSIONS:  Significant anxiety and depression were seen in 23% and 26% of respondents, respectively. Being a healthcare worker was an independent predictor of anxiety. Female sex, younger age, unemployment, work stress, and sensational media reports were independent predictors of both anxiety and depression.

Combined involvement of muscle, nerve, and myoneural junction following leptospira infection.

Leptospirosis is a zoonosis prevalent worldwide and is endemic in many parts of India. In early leptospiremic as well as late immune phase of the disease kidney, liver, heart, and lungs are commonly involved. Neurological manifestations are rare but may occur during immune phase in the form of aseptic meningitis, meningoencephalitis, seizures, myelitis, polyradiculoneuritis, and myalgia. In this report, we describe a rare case of leptospirosis with combined involvement of nerve, muscle, and myoneural junction in generalized fashion along with pulmonary, renal, and hepatic dysfunction.

Is International HIV Dementia Scale good enough to diagnose HIV-associated neurocognitive disorders?

Introduction: Human immunodeficiency virus (HIV)-associated neurocognitive disorders (HAND) comprise impairment of multiple cognitive domains and cause significant morbidity. International HIV Dementia Scale (IHDS) is a quite sensitive and specific method for screening for HAND, and Modified Mini-Mental State Examination (3MS), though nonspecific, contains more parameters for screening for neurocognition. Hence, we compared 3MS and IHDS as screening tools for HAND with an aim to find out which was a better screening tool for HAND. Methods: Using 3MS and IHDS, we assessed the cognitive status of 200 HIV-positive patients (65% males) and 84 controls, presenting to the Department of Medicine, King George's Medical University, Lucknow, India from September 2015 to September 2019. Results: According to 3MS, 42 (21%) HIV-positive patients were neurocognitively impaired (mean 76.24 ± 1.51), and 158 (79%) patients were not (mean 87.02 ± 4.16). As per IHDS, 185 (92.5%) HIV patients were neurocognitively impaired (mean 8.45 ± 0.88), and 15 (7.5%) patients were not (mean 11.13 ± 0.35). The mean 3MS score of controls was 87.56 ± 4.26, and the IHDS score was 9.73 ± 1.00. According to Patient Health Questionnaire-9 (PHQ-9), moderate depression occurred in only 3.5% of the patients, and the rest had only minimal or mild depression. In IHDS, psychomotor speed was the most affected parameter, whereas in 3MS, similarities were the most affected. Conclusion: IHDS may be over diagnosing neurocognitive impairment in HIV patients due to difficulty in understanding the test, especially psychomotor speed testing. 3MS may be more accurate for detecting neurocognitive impairment in HIV patients, and scale combining both these methods may be a still better choice.

Rabies encephalitis.

Rabies is an almost always fatal disease that physicians and patients dread due to its dismal prognosis and limited treatment options. Transmission of this disease occurs through the bite of dogs and wild animals (like jackal in our case). Other rare forms of transmission may be through inhalation in bat-infested caves and human-to-human transmission by infected corneal transplants, solid organ and tissue transplantation, and sometimes in laboratory settings. Its diagnosis is usually clinical in the absence of availability of special laboratory investigations at the point-of-care facility. Few people have described the role of imaging in diagnosis. We hereby report a patient with rabies encephalitis, having a history of jackal bite and classical MRI findings that we can use for early diagnosis in the absence of typical clinical features and specialised diagnostic testing.

PSP-FTD Complex: A Possible Variant of PSP.

INTRODUCTION: This study tried to find out type of lobar features found in patients with progressive supranuclear palsy (PSP) and whether they differ from those of frontotemporal dementia (FTD) as both of these are tauopathies. METHODS: We studied lobar functions of 45 patients with PSP. RESULTS: Five (11.1%) patients had no lobar feature; 11 (24.4%) had PSP-like features like apathy, frontal release signs, impaired motor Luria written sequences, and fist-edge-palm test; and 29 (64.4%) patients had FTD-like lobar features like disinhibition, poor naming, and word finding difficulty. Among features resembling FTD, behavioural variant type occurred in 31.1%, primary progressive aphasia type occurred in 58.6%, 3.4% patients had semantic dementia type features, and 6.9% were unclassified. CONCLUSIONS: Hence, patients with PSP with lobar features may fall in the middle of PSP-FTD spectrum with frontal lobe features typical of PSP (PSP-frontal like) and those with frontal lobe features resembling FTD (PSP-FTD complex) in between.

Autistic features in Unverricht-Lundborg disease.

We studied three patients with Unverricht-Lundborg disease for autistic features along with other clinical features associated with progressive myoclonus epilepsy. We diagnosed this disease based on noise and touch sensitive myoclonus, ataxia, cognitive decline, typical EEG features, normal MRI of the brain and applied Children's Global Assessment Scale and Childhood Autism Spectrum Test to these children. The CGAS score was 35 in two and 50 in one of them. CAST scores were above 15 in all of three of them. Autistic features may be an important clinical feature of this disease. History and physical examination for myoclonus should probably be taken in autistic children.

Long-Segment Myelitis, Meningoencephalitis, and Axonal Polyneuropathy in a Case of Scrub Typhus.

Scrub typhus, a mite-borne zoonotic disease, is endemic in several parts of India. It may cause multisystemic disease involving lungs, heart, spleen, liver, hematological system, and nervous system. Neurological involvement may include meningoencephalitis, cerebellitis, cranial nerve palsies, plexopathy, transverse myelitis, muscle dysfunction, neuroleptic malignant syndrome, parkinsonian syndrome, and Guillain-Barre syndrome. Here, we report a rare patient of scrub typhus, who developed meningoencephalitis followed by long-segment myelitis and axonal polyneuropathy, with hepatic, renal, hematological, and pulmonary involvement, following acute febrile illness with associated neurocysticercosis. He gained consciousness with a resolution of almost all of his complaints, with the exception of muscular power, which showed partial improvement following treatment with doxycycline, azithromycin, and steroids. What needs to be explored is whether the existence of neurological scrub typhus with neurocysticercosis is the coincidental price paid for living in the tropics or there is something more to it as in case of Japanese encephalitis and neurocysticercosis co-infection.

Relevance of non-specific MRI features in multiple system atrophy.

OBJECTIVES: Rarity of specific MRI features like 'hot-cross bun' sign and 'hyperintense putamen rim' reduce diagnostic utility of MRI in MSA. We therefore, studied some non-specific MRI features in addition to the specific ones, to find their diagnostic utility. PATIENTS AND METHODS: Clinical and MRI features of 53 indoor and outdoor patients with MSA were analyzed in the context of its Parkinsonian (MSA-P) and cerebellar (MSA-C) variants. RESULTS: Of 53 cases (mean age: 59.53±9.74years), 16 (30.2%) had MSA-C and 37 (69.8%) had MSA-P. Midbrain atrophy was found in 37 (69.8%) MSA patients (70.3% of MSA-P and 68.8% of MSA-C), cerebellar atrophy in 45 (84.9%) MSA patients (81.1% of MSA-P and 93.8% of MSA-C), 'hot-cross bun' sign in 13 (24.5%) MSA patients (27% of MSA-P and 18.8% of MSA-C), hyperintense putamen rim in 19 (35.8%) MSA patients (37.8% of MSA-P and 31.3% of MSA-C) and corpus callosal atrophy in 39 (73.6%) MSA patients (75.7% of MSA-P and 68.8% MSA-C). The midbrain atrophy was mainly lateral tegmental and resembled a positive 'Morning glory' sign in 16 (30.2%). CONCLUSION: "Hot cross bun" sign and "hyperintense putamen rim" sign were rarely seen in MSA. Combination of mid brain atrophy, corpus callosum atrophy and cerebellar atrophy was more commonly observed in both MSA-C and MSA-P and may be taken as of diagnostic significance.

N-hexane neuropathy with vertigo and cold allodynia in a silk screen printer: A case study.

N-hexane neuropathy is an occupational disease caused by exposure to n-hexane, which is used as a solvent in silk screen printing. Here, we describe a 35-year-old man, a silk screen printer by profession, who presented with dizziness, distal swelling of both lower limbs for 10 months and tingling and burning sensation in both feet for 9.5 months along with cold allodynia. The patient had normal results of a motor and sensory system examination, apart from an impaired temperature sense. Nerve conduction tests showed a conduction block in bilateral common peroneal nerves and absence of conduction in bilateral sural nerves. These symptoms resolved when further exposure to n-hexane was ceased but cold allodynia remained. Thus, cold allodynia and impaired temperature sense can be a manifestation of n-hexane neuropathy. Hence, abnormalities on nerve conduction studies can be detected in n-hexane neuropathy patients, even before clinical examination detects any such abnormalities. In the case of the patients presenting with sensory motor neuropathy, history of occupational exposure to n-hexane becomes important, as the sooner the disease is detected, the better the chances of recovery.

Autonomic predominant multiple system atrophy in the context of Parkinsonian and cerebellar variants.

OBJECTIVES: Autonomic dysfunction is often a late feature of multiple system atrophy (MSA). Based on early features, MSAp (Parkinsonian) and MSAc (cerebellar) variants are known. We studied the severity of autonomic dysfunction at first examination in MSA patient that may have relevance in its early diagnosis. METHODS: Clinical (including autonomic), radiological and lab features of 53 MSA patients were analyzed in the context of MSAp (16 patients) and MSAc (37 patients). RESULTS: Most frequent autonomic symptoms were erectile dysfunction in 30 out of 38 males (78.9%), bladder symptoms in 35 (66%), followed by blackouts/fainting attacks in 33 (62.3%). Autonomic abnormalities were detected in hand grip test (86.8%), deep breathing test (79.2%), cold pressor test (71.7%), Valsalva maneuver (75.5%), heart rate variability at 30:15 beats after standing (50.9%) and systolic BP on standing (41.5%). Six (16.2%) MSAp and 1 (6.3%) MSAc patients continued to have autonomic symptoms for several months (38.7±26.11, median 36 months) before the appearance of cerebellar (1 patient) or Parkinsonian (6 patients) features. CONCLUSION: Autonomic dysfunction may be the only presenting feature in some patients with MSA. Routine autonomic testing may help in the early diagnosis of MSA in patients presenting with Parkinsonian or cerebellar features.