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.

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.

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.

Does Metabolic Syndrome Determine Severity and Disability of Chronic Low Backache?

INTRODUCTION: Obesity may be associated with more severe and disabling low backache (LBA) due to alteration in biomechanics, but there are no such studies from developing countries. AIMS: We report the frequency of metabolic syndrome (MS) in chronic LBA (CLBA) and its association with severity and disability of CLBA. SUBJECTS AND METHODS: Consecutive patients with CLBA attending to the neurology service from October 2015 to February 2016 were included in the study. Clinical and demographic parameters were recorded. Routine biochemical test was done. The severity of pain was assessed by a 0-10 Numeric Rating Scale (NRS) and disability by Oswestry Disability Index (ODI) version 2. Comparison of variables was done by Chi-square or independent t-test and correlation by Karl Pearson or Spearman's rank correlation test. RESULTS: Seventy-none (39.3%) patients had MS as per the International Diabetic Federation (IDF) criteria and 68 (33.8%) as per the National Cholesterol Education Program Adult Treatment Panel III criteria. Abdominal obesity was the most common (171 [85.1%]) feature of MS. The patients with MS had longer duration of sitting work and did less frequently exercise. The NRS score (6.95 ± 1.06 vs. 6.65 ± 0.95; P = 0.04) and ODI score (54.91 ± 8.42 vs. 51.89 ± 8.54; P = 0.01) were higher in CLBA patients with MS compared to those without MS. CONCLUSION: About 40% patients with CLBA have metabolic syndrome, and they have more severe pain and disability.

Neuronal Dysexcitability May Be a Biomarker of Migraine: A Visual Evoked Potential Study.

We report neuronal sensitization and impaired habituation in migraine using pattern reversal visual evoked potential (PRVEP) and correlate these with clinical characteristics of migraine. Sixty-five migraineurs and 30 healthy controls were included. A detailed clinical examination was done and migraine characteristics, including migraine trigger, photophobia, phonophobia, and allodynia were noted. Consecutive 5 blocks of PRVEP were recorded averaging 100 epochs at 3 Hz stimuli. The amplitude of N75 and P100 were measured. Amplitude of first block was considered for sensitization, and impaired habituation was considered if any subsequent block was not suppressed. Migraineurs had sensitization of P100 (14.01 ± 6.02 vs 11.60 ± 5.17 µV; P = .049), but not of N75 (42.22 ± 4.79 vs 11.08 ± 4.56 µV; P = .27) compared with the controls. Impaired habituation of N75 was more marked and persisted up to fourth block of VEP recoding. Impaired habituation of P100 was significant only in the third block. Baseline N75 and P100 amplitudes were higher in females, and N75 was also higher in those with longer duration of illness. These phenomena did not correlate with visual triggers. Sensitization and impaired habituation were marked during headache. Based on the PRVEP findings, it may be concluded that migraineurs have sensitization of P100 and impaired habituation of N75 especially during headache. These phenomena may be useful for therapeutic monitoring.

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.

Anterior spinal artery aneurysm in aortic stenosis of different etiology: Report of three cases.

Isolated aneurysms of spinal arteries are rare. Spinal artery aneurysms are commonly found in association with spinal cord arteriovenous malformation and coarctation of aorta and rarely with aortic arch interruption and Klippel-Trenaunay syndrome. Spinal angiograms are the gold standard for diagnosing these spinal artery aneurysms but with the advances in computed tomography technology these aneurysms can also be very well demonstrated in computed tomography angiograms. We describe three cases of anterior spinal artery aneurysm, those are flow related aneurysms, associated with coarctation of aorta and with Takayasu arteritis.

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.

Long-term Prednisolone in Post-stroke Complex Regional Pain Syndrome.

BACKGROUND: There is no study on the long-term use of prednisolone in post-stroke complex regional pain syndrome-1 (CRPS1). OBJECTIVE: To evaluate the efficacy and safety of long-term low dose prednisolone in post-stroke CRPS-I. STUDY DESIGN: Open-labeled randomized controlled trial. SETTING: Tertiary care teaching institute. METHODS: Seventy-seven out of 396 (19.4%) patients with stroke had CRPS-1 and 58 met the inclusion criteria. Their clinical details and CRPS, Visual Analogue Scale (VAS), modified Rankin Scale (mRS), and Barthel Index (BI) scores were noted. The patients were prescribed 40 mg prednisolone for 2 weeks followed by tapering in the next 2 weeks. Patients who responded were randomly assigned prednisolone 10 mg daily (group I) or no prednisolone (group II). They were followed up for the first and second month of randomization and their CRPS, VAS, mRS, and BI scores were noted. The primary outcome was improvement in CRPS score and secondary outcomes were VAS, mRS, BI scores, and severe adverse events (SAE). RESULTS: Fifty-six of fifty-eight (96.5%) patients responded to the initial high dose prednisolone and 26 each were assigned group I and group II treatment. Group I patients had further improvement in CRPS score. Fifty percent of patients in group II had deterioration at one month and needed reinstitution of prednisolone; following which 77% of them improved in the next month. The improvement in CRPS score paralleled the VAS score but not mRS and BI scores in the first and second months in group I compared to group II. There was no SAE necessitating withdrawal of prednisolone. LIMITATION: The design of the study is not double blind. CONCLUSION: In post-stroke CRPS-I, continuation of low dose prednisolone for 2 months is safe and effective.Key words: Shoulder hand syndrome, CRPS, corticosteroid, prednisolone, stroke, Visual Analogue Scale.