Pellets in the Eye: Cerebral Aneurysm and Encephalomyeloneuropathy as Presenting Syndromes.

Lead poisoning following retained gunshot pellets resulting in recurrent episodes of encephalomyeloneuropathy is rare and association of intracranial aneurysm with lead poisoning is interesting. The case report describes about a 58-year ayurvedic doctor who was hospitalized for recurrent abdominal pain and limb weakness appearing spontaneously and improving in a few days to weeks. He had 20 such attacks in last 30 years starting at 25 years after the gunshot wound that resulted in retention of 2 lead pellets in his skull. Cranial imaging demonstrated left posterior communicating aneurysm. His initial blood lead level was 206.10 µg/dl, and following chelation with oral d-penicillamine, the lead level declined and was asymptomatic. After 4-year follow-up, he developed subarachnoid hemorrhage, which was managed with intravascular coiling of the ruptured aneurysm. The gunshot pellets are generally not removed, especially, if they are in inaccessible location. Our patient highlights that such cases should be closely monitored.

Effect of high rate rTMS on somatosensory evoked potential in migraine.

Background Sensitization and impaired habituation of cortical neurons have been reported in migraineurs. Repetitive transcranial magnetic stimulation (rTMS) may change these phenomena and be the basis of therapeutic response. We report the effect of 10 Hz rTMS on sensitization and habituation of median somatosensory evoked potential (SEP) in migraineurs, and correlate these changes with clinical response. Methods Migraineurs having four or more episodes of headache per month were included and their clinical details were noted. Three sessions of 10 Hz rTMS, 600 pulses in 412.4 seconds were delivered on the left frontal cortex corresponding to the hot spot of right abductor digiti minimi, on alternate days. Median SEP was done before and 30 minutes after the third rTMS session. Sensitization (block I N20 amplitude) and impaired habituation (if N20 amplitude of block 2 or 3 were not suppressed compared to block I) were noted. The reduction in frequency and severity of headache in the next month were noted and correlated with SEP changes. Results Ninety-four migraineurs were included; 56 received true rTMS and 38 sham stimulation. Following stimulation, reduction in N20 amplitude of block 1 correlated with a reduction in frequency and severity of headache at one month. The impaired habituation significantly improved in the true rTMS group compared to sham stimulation, and correlated with a reduction in the severity of headache but not with frequency. Conclusion In migraineurs, 10 Hz rTMS improves habituation and may be the biological basis of headache relief.

Spectrum and Predictors of Refractory Status Epilepticus in a Developing Country.

OBJECTIVE: Refractory status epilepticus (RSE) can influence the outcome of status epilepticus (SE). In the present study, we report the aetiology and predictors of outcomes of RSE in a developing country. METHODS: This is a prospective hospital-based study of SE patients (continuous seizures for five minutes or more). Those who had SE persisting after two antiepileptic drugs were defined as having RSE. We present the demographic information, duration, and type of SE, and we note its severity using the status epilepticus severity score (STESS), its aetiology, comorbidities and imaging findings. The outcome of RSE was defined as cessation of seizures and the condition upon discharge, as assessed by the modified Rankin Scale. RESULTS: A total of 35 (42.5%) of our 81 patients had RSE. The median duration of SE before starting treatment was 2 hours (range=0.008-160 h). The most common causes of RSE were stroke in 5 (14.3%), central nervous system (CNS) infections in 12 (34.3%) and metabolic encephalopathies in 13 (37.1%) patients. Some 21 (60%) patients had comorbidities, and the STESS was favourable in 7 (20%) patients. A total of 14 (20%) patients died, but death was directly related to SE in only one of these. Some 10 patients had super-refractory status epilepticus, which was due to CNS infection in 5 (50%) and metabolic encephalopathy in 3 (30%). On multivariate analysis, an unfavourable STESS (p=0.05) and duration of SE before treatment (p=0.01) predicted RSE. Metabolic aetiology (p=0.05), mechanical ventilation (p60 years (p=0.003) were predictors of poor outcomes. CONCLUSIONS: RSE was common (42.5%) among patients with SE in a tertiary care center in India. It was associated with high mortality and poor outcomes. Age above 60 years and metabolic aetiology were found to be predictors of poor outcomes.

Status epilepticus in scrub typhus.

Scrub typhus is an emerging infection, and there is little information about status epilepticus (SE) in scrub typhus. We report the clinical spectrum and outcome of SE in scrub typhus. In a 3-year prospective hospital-based observational study, all scrub typhus patients with SE were included. Scrub typhus was diagnosed by immunochromatography assay. SE was defined if convulsions lasted longer than 5 min. The patients' demographic, clinical, computed tomography (CT), magnetic resonance imaging (MRI), and electroencephalography (EEG) findings were noted. Response to antiepileptic drugs (AEDs) and outcome at 1 month and 1 year were recorded. Between 2012 and 2014, there were 66 patients with scrub typhus admitted with central nervous system (CNS) involvement, 10 (15.2%) of whom had SE (generalized convulsions in 5, secondary generalized in one). The median age of the patients was 34 (range 18-71) years and seven were female. The duration of SE ranged between 10 min and 48 h. SE responded to one AED in five patients, two AEDs in three patients, and more than two AEDs in two patients. Cranial MRI findings were normal. All patients recovered completely with doxycycline by 1 month and AED was withdrawn by 8 months in all. Although 15% patients with scrub typhus may have SE, they have good outcome.

Is palinopsia in migraineurs an enhanced physiological phenomenon?

Background We report the occurrence of palinopsia in patients with migraine and its correlation with migraine characteristics, triggers and allodynia. Methods This study included 153 consecutive patients with migraine and recorded their clinical details, including allodynia and migraine triggers and characteristics. Palinopsia was evaluated in migraineurs and 101 controls by using a questionnaire and a novel method. Results According to the questionnaire assessment, 9.8% migraineurs had palinopsia. According to the novel method, 57.5% of migraineurs and 12% of controls had palinopsia. Migraineurs most frequently had palinopsia to red color (51.6%), followed by yellow (49.7%), blue (47.7%), green (46.4%) and the least to white (30.7%). A similar pattern with a lesser frequency was noted in controls. The duration of palinopsia was longer in migraineurs than in controls (32.68 ± 20.24 vs. 5.92 ± 4.55 seconds; p < 0.001). Migraineurs with palinopsia differed from those without in terms of noise as a migraine trigger ( p < 0.001) and allodynia as a migraine-associated phenomenon ( p = 0.03). In multivariable analysis, predictors of palinopsia were the frequency ( p = 0.003) and severity ( p = 0.04) of headache and the presence of headache during examination ( p = 0.0001). Conclusion Migraineurs had a pattern of palinopsia to different colors that was similar to the controls, but the palinopsia of migraineurs was more frequent and of longer duration, especially during headaches.

Systemic Inflammatory Response Syndrome Predicts Severity of Stroke and Outcome.

BACKGROUND: This study was undertaken to evaluate the frequency of systemic inflammatory response syndrome (SIRS) at admission and its correlation with clinical and radiological severity of stroke and outcome. METHODS: Two hundred consecutive stroke patients within 48 hours of ictus were prospectively included, and their clinical details including Glasgow Coma Scale (GCS), National Institutes of Health Stroke Scale (NIHSS), and feature of raised intracranial tension were noted. Computed tomography/magnetic resonance imaging finding included stroke type, location, size, midline shift, herniation, and intraventricular hemorrhage score. SIRS was noted on days 1, 2, 7, and 15. Death and outcome at 3 months were based on modified Rankin Scale (mRS) score. RESULTS: Seventy-five (37.5%) had infraction and 125(62.5%) intracranial hemorrhage (ICH). SIRS was present in 120 (60%) patients: all the features in 56 (28%), 3 in 48 (24%), and 2 in 16 (8%). The presence of SIRS decreased with time: on the second day in 57%, seventh day in 43%, and 15th day in 21% of patients. Admission SIRS correlated with the GCS score (P < .001), NIHSS score (P < .001), volume of ICH (P < .001), infarction size (P < .001), hypernatremia (P = .001), and respiratory paralysis (P < .001). Thirty-one (15.5%) patients died, and 30 (97%) of them had SIRS. At 3 months, 110 (55%) patients had poor outcome (mRS >2) and of them 90 (82%) had SIRS (P < .001). On multivariate regression analysis, the number of SIRS criteria (P = .16) was not significantly related to 3-month outcome and death but independently related to NIHSS score at admission (odds ratio [OR] = 1.39; 95% confidence interval [CI] = 1.22-1.56; P < .001), GCS score (OR = 1.32; 95% CI = 1.01-1.71; P = .04), and duration of hospitalization (OR = 1.07; 95% CI = 1.01-1.15; P = .03). CONCLUSIONS: SIRS at presentation is a useful marker for clinicoradiological severity of stroke but not an independent marker of death and disability.

Clinical, nerve conduction and nerve biopsy study in vitamin B12 deficiency neurological syndrome with a short-term follow-up.

OBJECTIVE: The frequency and type of neuropathy in vitamin B12 deficiency neurological syndrome (VBDNS) is controversial. This study reports the frequency and type of nerve dysfunction in VBDNS using nerve conduction and sural nerve biopsy and its response to treatment. METHOD: Sixty-six patients with VBDNS diagnosed on the basis of low serum vitamin B12 level and/or megaloblastic bone marrow were subjected to clinical evaluation, hemoglobin, mean corpuscular volume, thyroid function test, HIV serology, and vasculitic profile. Peroneal motor and sural sensory nerve conduction studies were done. Sural nerve biopsy was done in six patients. The patients were treated with cyanocobalamin injection and followed up clinically and with nerve conduction study at 3 and 6 months. RESULTS: The median age of the patients was 46 (12-80) years and 11 patients were females. The duration of symptoms was 1-96 (median 7) months. Clinical features of neuropathy were present in 46 (69.7%) patients and nerve conduction was abnormal in 36 (54.5%) patients. On nerve conduction study, 8 (22.2%) patients had axonal, 4 (11.1%) had demyelinating, and 24 (66.7%) had mixed features. Nerve biopsy revealed acute axonal degeneration in early stage and chronic axonopathy with demyelination in the late stages of disease. The nerve conduction parameters improved at 6 months along with clinical recovery. CONCLUSION: Nearly 70% patients with VBDNS had evidence of neuropathy which is mainly axonal with some demyelinating features.

Paralytic rabies: MRI findings and review of literature.

Paralytic rabies closely simulates Guillain-Barre syndrome or ascending myelitis often causing clinical dilemma. Two such patients were managed in our hospital whose magnetic resonance imaging (MRI) revealed characteristic findings revealing T2 hyper intensity in central spinal cord and in posterior brainstem and hypothalamus. These MRI findings are helpful in the diagnosis of rabies in appropriate setting. We also review the literature on MRI changes in paralytic rabies.

Outcome of Guillain-Barré syndrome with bulbar palsy.

BACKGROUND: Elective intubation is advocated in Guillain-Barré syndrome (GBS) with bulbar palsy to prevent aspiration pneumonia and lung collapse. We evaluate the outcome of GBS patients with bulbar palsy, and also compare the risks and benefits of intubation and MV in them. METHODS: 187 GBS patients with bulbar palsy from a cohort of 547 GBS registry were analyzed. Detailed clinical records and peak disability on a 0-6 GBS Disability Scale (GBSDS) were noted. The patients were intubated if arterial blood gas (ABG) analysis revealed hypoxia, hypercarbia or acidosis. The patients with normal ABG parameters were fed by nasogastric tube, and nursed in lateral position. Occurrence of pneumonia, in-hospital death and outcomes at 6-months were classified as complete (GBSDS <2), partial (GBSDS 2-3) and poor (GBSDS >3). RESULTS: 76/187(40.6%) patients required MV, and they had a shorter duration of illness (p = 0.007), higher peak disability (p < 0.001), autonomic dysfunction (p < 0.001) and more frequently received IVIg (p = 0.02). Pneumonia (63% vs 10.8%; p < 0.001) and in-hospital deaths (7.9% vs 1.8%; p = 0.06) were more frequent in MV group compared to nasogastric fed group. At 6-months,104 (55.6%) patients recovered completely. On multivariate analysis, the independent predictors of poor outcome were peak disability [Adjusted Odds Ratio (AOR) 9.84, 95% Confidence Interval (CI) 3.15-30.74, p < 0.0001], day of hospitalization from disease onset (AOR 1.09, 95% Cl 1.01-1.01; p=0.009) and requirement of MV (AOR 0.10; 95% 0.02-0.50; p = 0.005). CONCLUSION: GBS patients with bulbar palsy may be managed by nasogastric feeding and nursing in lateral position without increasing the risk of pneumonia. Mechanical ventilation based on ABG does not worsen outcomes of GBS with bulbar palsy.

Evaluation of Apo A IV and Haptoglobin as Potential CSF Markers in Patients with Guillain-Barre´ Syndrome: A Cross-Sectional Study.

BACKGROUND: Brain- and blood-derived protein analysis in the cerebro-spinal fluid (CSF) in various studies performed abroad found that some proteins and their isoforms were altered significantly in Guillain-Barre´ syndrome (GBS) patients in comparison to controls. However, data are lacking in India with respect to the blood- or brain-derived proteins in patients of GBS. OBJECTIVE: This study aimed to identify the role of apolipoprotein A IV (Apo A IV) and haptoglobin as potential protein markers in CSF of patients with GBS in our population. MATERIALS AND METHODS: The study comprised 28 participants where 12 confirmed cases of GBS and 16 control subjects admitted for non-infectious neurological disorders were recruited after obtaining approval from the Institutional Ethics Committee. CSF glucose, protein, and adenosine deaminase were analyzed using an autoanalyzer. The concentrations of Apo A IV and haptoglobin were estimated with enzyme-linked immuno-sorbent assay (ELISA) kits. RESULTS: The CSF protein concentrations of cases were higher as compared to controls. The concentrations of haptoglobin and Apo A IV were higher in the confirmed cases of GBS as compared to the control subjects, and this difference was found to be significant. The receiver operating characteristic curve analysis for haptoglobin revealed that the area under the curve (AUC) was 0.867 (95% CI: 0.732-1.001), with a sensitivity of 83.8% and a specificity of 63.3%. The AUC for Apo A IV was 0.883 (95% CI: 0.758-1.009), with a sensitivity of 91.7% and a specificity of 73.3%. CONCLUSIONS: Haptoglobin along with Apo A IV can emerge as a potential biochemical marker in CSF for the diagnosis of GBS.

Is ß endorphin related to migraine headache and its relief?

BACKGROUND: Low ß endorphin level in serum and cerebrospinal fluid (CSF) has been reported in migraine. The basis of pain relief in migraine by repetitive transcranial magnetic stimulation (rTMS) may be related to ß endorphin (BE), which has not been evaluated. It is proposed to measure plasma ß endorphin level in migraine patients and the change in ß endorphin level following rTMS, and to correlate these changes with migraine relief. METHODS: Twenty-five patients with migraine diagnosed as per International Headache Society criteria and 20 gender- and age-matched controls were included. Their clinical characteristics including duration of migraine, its frequency, severity and functional disability, triggers, allodynia and number of analgesic used were noted. Plasma ß endorphin level was estimated before and after the third rTMS session. rTMS was delivered on the hot spot of right abductor digiti minimi on alternate days for 3 days and each session consisted of 600 pulses at 10 Hz. The clinical response was noted weekly for 1 month and correlated with ß endorphin level. RESULTS: The median age of the patients was 35 (20-50) years and 19 were females. Eight patients had episodic and 17 chronic migraine. ß endorphin level was significantly lower in migraine (4.35 ± 2.29 ng/ml) compared to controls (6.68 ± 2.93 ng/ml). ß endorphin level was lower in chronic compared to episodic migraine (3.74 ± 2.20 versus 5.65 ± 2.02 ng/ml). Following rTMS, the headache frequency, severity, functional disability and analgesic intake significantly reduced on the seventh day of rTMS and remained significant until the fourth week compared to the baseline. The clinical improvement was associated with increase in ß endorphin level (4.35 ± 2.29 versus 6.58 ± 3.33 ng/ml). CONCLUSION: It can be concluded from this study that the basal plasma ß endorphin level was low in migraine patients, especially in chronic migraine. The improvement in migraine after rTMS was associated with increase in ß endorphin level.

Metabolic syndrome and insulin resistance in migraine.

Metabolic syndrome is associated with migraine but there is no study comparing the characteristics of migraine with and without metabolic syndrome from Southeast Asia. This study was therefore undertaken to compare the clinical characteristics of migraine in patients with and without metabolic syndrome and insulin resistance. 135 consecutive patients with migraine diagnosed on the basis of International Headache Society criteria were subjected to clinical evaluation as per fixed protocol. Headache severity, frequency and functional disability were recorded. Metabolic syndrome was diagnosed as per National Cholesterol Education Programme: Adult Treatment Panel III and International Diabetic Federation criteria. Insulin resistance was calculated by homeostases model assessment. Their age ranged between 14 and 61 years and 108 were females. Metabolic syndrome was present in 31.9% patients and only 13 were obese. Insulin resistance was present in 11.1%. Metabolic syndrome was correlated with age, gender, number of triggers, years of headache and duration of migraine attacks. Insulin resistance correlated with duration of migraine attacks. From this study, it can be concluded that metabolic syndrome was present in 31.9% of the migraineurs which was mainly in elderly who had longer duration of headache and multiple triggers.

Impact of the COVID-19 Pandemic on the Frequency, Clinical Spectrum and Outcomes of Pediatric Guillain-Barré Syndrome in India: A Multicentric Ambispective Cohort Study.

Objective: To study impact of COVID-19 pandemic on frequency, clinical/electrophysiological profile and treatment outcomes in pediatric Guillain-Barré syndrome (GBS). Background: GBS is the most frequent cause of pediatric acute flaccid paralysis. The effect of the COVID-19 pandemic on pediatric GBS is unclear in the literature. Methods: We conducted an ambispective, multicentric, cohort study involving 12 of 27 centres in GBS Consortium, during two periods: pre-COVID-19 (March-August 2019) and during COVID-19 (March-August 2020). Children ≤12 years who satisfied National Institute of Neurological Diseases and Stroke criteria for GBS/variants were enrolled. Details pertaining to clinical/laboratory parameters, treatment and outcomes (modified Rankin Scale (mRS) at discharge, GBS Disability score at discharge and 3 months) were analysed. Results: We enrolled 33 children in 2019 and 10 in 2020. Children in 2020 were older (median 10.4 [interquartile range 6.75-11.25] years versus 5 (2.5-8.4) years; P = 0.022) and had more sensory symptoms (50% versus 18.2%; P = 0.043). The 2020 group had relatively favourable mRS at discharge (median 1 (1-3.5) versus 3 (2-4); P = 0.042) and GBS disability score at 3 months (median 0 (0-0.75) versus 2 (0-3); P = 0.009) compared to 2019. Multivariate analysis revealed bowel involvement (P = 0.000) and ventilatory support (P = 0.001) as independent predictors of disability. No child in 2020 had preceding/concurrent SARS-CoV2 infection. Conclusions: The COVID-19 pandemic led to a marked decline in pediatric GBS presenting to hospitals. Antecedent illnesses, clinical and electrophysiological profile of GBS remained largely unchanged from the pre-pandemic era.

Pure Neuritic Leprosy with Bilateral Foot Drop and Central Nervous Involvement: A Clinical, Electrophysiological, and MR Correlation.

BACKGROUND: Central nervous system (CNS) involvement in leprosy is sparsely documented. Neurophysiological tests and magnetic resonance imaging (MRI) helps in demonstrating CNS involvement in the patient of pure neuritic leprosy. OBJECTIVES: To demonstrate CNS involvement in pure neuritic leprosy. METHODS: Detailed clinical presentation and skin lesions were evaluated. Sural nerve biopsy, MRI diffusion tensor imaging of spinal cord and optic nerve were performed. Visual evoked potential and tibial somatosensory evoked potential were done. Their clinical, electrophysiological, and MRI were done at follow-up visits. RESULTS: We report three patients of pure neuritic leprosy with bilateral foot drop as the initial presentation. MRI T2W sequence of cervico dorsal cord showed dorsal column hyperintensity in two patients. Diffusion-weighted MR revealed decrease fractional anisotropy and an increase in the apparent diffusion coefficient. Similar findings were also noted in the optic nerves. The patients were managed with multidrug therapy multibacillary regimen and steroid in tapering dose. At follow-up, they showed clinical improvement in vision and power of ankle dorsiflexor. CONCLUSIONS: Patients of pure neuritic leprosy may manifest with bilateral foot drop with the involvement of posterior column and cranial nerves.

A Study of Diffusion Tensor Imaging in Hirayama Disease.

BACKGROUND: Hirayama disease (HD) is a motor neuron disease and occasionally is associated with lower limb hyper-reflexia. Corticospinal tract dysfunction can be evaluated by diffusion tensor imaging (DTI), but there is paucity of study in HD. OBJECTIVE: We report corticospinal tract functions using DTI in the patients with HD and correlate with clinical findings. MATERIALS AND METHODS: The patients with HD diagnosed on the basis of clinical and electromyography findings were included. Their age, duration of illness, side of initial involvement, and progression were noted. Presence of lower limb hyper-reflexia, and cervical spine magnetic resonance imaging (MRI) findings were noted. Cranial MRI was done and DTI findings at internal capsule, cerebral peduncle, pons, and pyramid were noted. RESULTS: In total, 10 patients with HD and 5 matched controls were evaluated. The apparent diffusion coefficient (7.03 ± 0.27 vs 6.83 ± 0.36), fractional anisotropy (0.79 ± 0.04 vs 0.82 ± 0.05), axial diffusivity (5.08 ± 0.08 vs 5.04 ± 0.07), and radial diffusivity (3.79 ± 0.05 vs 3.76 ± 0.05) between HD patients and controls were not different in internal capsule. These values were also not significantly different in cerebral peduncle, pons, and pyramid. These values were also not significantly different between the severe and less severely affected sides. The fractional anisotropy did not correlate with lower limb hyper-reflexia (P = 1.00) and spinal cord atrophy (P = 0.60). CONCLUSION: DTI study in HD patients did not reveal corticospinal tract involvement in brain.

Association of venous varix and developmental venous anomaly: report of a case and review of literature.

Cerebral developmental venous anomalies (DVAs) are the most frequently encountered cerebral vascular malformation. Most are asymptomatic and incidentally detected. Here we present a case of DVA associated with venous varix presented with chronic headache. A 50-year-old woman presented with right hemicranial headache since 6 months. There was no neurological deficit. MRI showed a well-defined oval T2 hyperintense, T1 isointense extra-axial lesion in the right parietal region showing intense homogeneous enhancement. Prominent vascular flow void was extending from the lesion up to the deep parietal white matter. Subtle thin linear areas of blooming noted in the parietal white matter converging towards the vascular flow void. The venous sac is in communication with the cortical vein draining to the superior sagittal sinus. These MRI findings favoured a diagnosis of DVA in the right parietal lobe with prominent draining vein forming a cortical venous varix. The patient was managed conservatively with symptomatic treatment for headache.