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Primary Sjogren’s Syndrome (PSS) is an autoimmune exocrinopathy, with protean manifestations affecting multiple organ systems. Neurological manifestations are documented in about 20% of PSS cohorts in literature, with peripheral manifestations being commoner. Central nervous system manifestations of PSS (CNS-SS) encompass ischemic strokes, demyelinating lesions, aseptic meningitis, encephalitis, cerebellar ataxia, cognitive impairment and movement disorders. Ischemic stroke as presenting manifestation of PSS is extremely rare. We hereby describe a 50-year-old male, who presented for evaluation of 2 episodes of discrete focal neurological deficits over a duration of 6 weeks, with neuro-imaging findings revealing evidence of acute-subacute bihemispheric infarcts. Further evaluation revealed evidence of strongly positive anti phospholipid antibodies (aPL), indirect immunofluorescence antinuclear antibody (IIF-ANA), anti Sjögren’s syndrome-A (SS-A/Ro) and anti-Ribonuclear protein (RNP) antibodies, with histopathological evidence of periductal and periacinar lymphocytic infiltration as well as acinar atrophy and interstitial fibrosis of minor salivary glands on lip biopsy, consistent with a diagnosis of Sjögren’s syndrome, constituting a diagnosis of Antiphospholipid syndrome (APS) associated with PSS.

Background: Diagnostic errors in neurological diagnosis are a source of preventable harm. Software tools like Differential Diagnosis (DDx) apps in neurology that hold the potential to mitigate this harm are conspicuously lacking.Materials and Methods: A multicenter cross-sectional observational study was designed to compare the diagnostic accuracy of a Neurology DDx App (Neurology Dx) with neurology residents by using vascular neurology clinical vignettes. The study was conducted at 7 leading neurology institutes in India. Study participants comprised of 100 neurology residents from the participating institutes.Measurements: Detecting diagnostic accuracy of residents and App measured as a proportion of correctly identified high likely gold standard DDx was prespecified as the main outcome. Proportions of correctly identified first high likely, first 3 high likely, first 5 high likely, and combined moderate plus high likely gold standard differentials by residents and App were secondary outcomes.Results: 1,000 vignettes were attempted by residents. Frequency of gold standard, high likely differentials correctly identified by residents was 27% compared to 72% by App (absolute difference 45%, 95% CI 35.7-52.8). When high and moderate likely differentials were combined, residents scored 17% compared to 57% by App (absolute difference 40%, 95% CI 33.8-50.0). Residents correctly identified first high likely gold standard differential as their first high likely differential in 34% compared to 18% by App (absolute difference 16%, 95% CI 1.2-25.4).Conclusion: App with predefined knowledge base can complement clinical reasoning of neurology residents. Portability and functionality of such Apps may further strengthen this symbiosis between humans and algorithms (CTRI/2017/06/008838).

Guillain-Barre Syndrome (GBS) is an acute inflammatory polyradiculoneuropathy which can lead to rapid neuromuscular respiratory failure, with an estimated annual incidence of 1-2 per 100,000 person-years. Even though cranial nerve involvement is known to occur in GBS, radiological correlation on neuroimaging studies are less frequently reported in pediatric population. We hereby report the case of a 14-year-old boy with acute motor axonal neuropathy variant of GBS, who had extensive contrast enhancement of multiple cranial nerves on Magnetic Resonance Imaging brain, associated with clinicoradiological dissociation on presentation.

Varicella-zoster virus (VZV) infection is notorious for central nervous system involvement, the spectrum of which encompasses vasculopathic manifestations as well. Central nervous system VZV vasculopathy (CVV) most commonly manifests as ischemic strokes or TIA, even though other less common modes of presentation are also well documented in the literature. The pathophysiological mechanism is primarily attributed to active virus infection in the blood vessels secondary to decline in varicella-specific cell-mediated immunity. More than one-third of those with CVV do not have preceding skin lesions of zoster. Hence, a high index of clinical suspicion should be entertained in the appropriate clinical scenario. We hereby describe the case of a 40-year-old lady with systemic lupus erythematosus on long-term oral steroids who presented with acute ischemic stroke involving the right PCA territory, with asymptomatic chronic infarcts in the left MCA. Further evaluation revealed concentric vessel enhancement involving right PCA on high-resolution MR vessel wall imaging with the “Moya-Moya” phenomenon on the left supraclinoid ICA/MCA segment. CSF showed mononuclear pleocytosis with a hemorrhagic component, along with positive VZV DNA PCR. A diagnosis of “CNS VZV Vasculopathy sine herpete” was made and the patient was initiated on appropriate management.

Background Non-contrast CT (NCCT) brain imaging biomarkers of hematoma expansion in intracerebral hemorrhage (ICH) has gained relevance in recent times. Though intra-hematoma hypodensities (IHH) can predict hematoma expansion and outcome, it is postulated to be time-dependent. Aim To assess the differential prevalence of IHH in spontaneous ICH over time and assess its predictive valve in early hematoma expansion and functional outcome at 3 months. Material and methods Patients with ICH within 48 h of stroke onset were included. Baseline clinical and demographic data were collected. Baseline NCCT brain was analyzed for hematoma volume, characterization of IHH, with 24-hours follow-up NCCT hematoma volume calculated for identification of hematoma expansion. Poor functional outcome was defined as mRS ≥3. Results Around 92 subjects were included in the study. IHH was found in 40%. Prevalence of IHH was higher in those with baseline NCCT performed within 3 h of symptom onset compared to those beyond 3 h (71% vs 29%, P = 0.002). The hematoma expansion was more common in patients with IHH compared to those without (54% vs 29%; P = 0.02). Multivariate analysis revealed the presence of IHH (rather than pattern or number) to be strongly associated with poor functional outcome at 3 months (OR 3.86; 95% CI: 1.11-13.42, P = 0.03). Conclusion There is a decreasing prevalence of IHH as the time from symptom onset to NCCT increases. Nevertheless, its presence is significantly associated with hematoma expansion and predicted poor short-term functional outcomes in spontaneous ICH.

Highlights • When comparing stroke admissions from March 1st-May 10th in 2019 and 2020 at a single comprehensive stroke center in Middle East, there was a 41.9% increase in stroke admissions in 2020. A higher rate of large vessel occlusion (LVO) and significant delay in initiation of mechanical thrombectomy after hospital arrival was observed in 2020. • Among all COVID-19 admissions in 2020, 5.24% patients suffered stroke including 3.21% with ischemic and 2% with hemorrhagic stroke. • Patients with COVID-19 and ischemic stroke were significantly younger, predominantly male, had fewer vascular risk factors, had more severe clinical presentation, and higher rate of LVO ccompared to ischemic stroke patients without COVID-19 • For hemorrhagic stroke, COVID-19 patients did not differ from non-COVID-19 patients., Objective: To compare ischemic and hemorrhagic stroke patients with COVID-19 to non-COVID-19 controls, and to describe changes in stroke admission patterns during the pandemic. Methods:This is a single center, retrospective, observational study. All consecutive patients admitted with primary diagnosis of ischemic/ hemorrhagic stroke between March1st -May10th 2020 were included and compared with the same time period in 2019. Results: There was a 41.9% increase in stroke admissions in 2020 (148 vs 210,P = .001). When comparing all ischemic strokes, higher rate of large vessel occlusion (LVO) (18.3% vs 33.8%,P = .008) and significant delay in initiation of mechanical thrombectomy after hospital arrival (67.75 vs 104.30 minutes,P = .001) was observed in 2020. When comparing all hemorrhagic strokes, there were no differences between the two years. Among 591 COVID-19 admissions, 31 (5.24%) patients with stroke including 19 with ischemic (3.21%) and 12 with hemorrhagic stroke (2.03%) were identified. Patients with COVID-19 and ischemic stroke were significantly younger (58.74 vs 48.11 years,P = .002), predominantly male (68.18% vs 94.74%,P = .016), had lesser vascular risk factors, had more severe clinical presentation (NIHSS 7.01 vs 17.05,P

Cerebral microbleed (CMB) is a novel neuroimaging marker of cerebral small vessel disease.To determine the prevalence of CMB in the subtypes of acute ischemic minor stroke (AIS) and transient ischemic attack (TIA) and to identify the risk factors associated with location and number of CMB.Patients with AIS (National Institute of Health Stroke Scale of 5 or less) or TIA were included. CMB was characterized using the Microbleed Anatomical Rating Scale (MARS).Of the 488 subjects [mean age (standard deviation): 57.5 years (14.4 years), males (77.7%)] recruited, CMB was noted in 140 (28.7%). About 35% with CMB had a lacunar stroke etiology, whereas LAA and CE subtype constituted 33.6 and 10.7%, respectively (P = 0.000). Lacunar subtype was more likely to harbor multiple CMB (four or more) and CMB in all locations (lobar, deep or infratentorial). On multivariate analysis, systemic hypertension [P = 0.025; odds ratio (OR) 0.33 (95% confidence interval (CI) 0.129-0.874)], serum triglyceride (TG) levels below 150 mg/dL [P = 0.001; OR 3.70 (95% CI 1.698-8.072)], and presence of white matter hyperintensities on magnetic resonance imaging brain [P = 0.026; OR 2.18 (95% CI 1.096-4.337)] were associated with the presence of CMB. Those with serum TG levels of less than 150 mg/dL were more likely to harbor lobar (P = 0.002) or infratentorial CMB (P = 0.022), whereas those with serum creatinine levels of more than1.5 mg/dL have lobar CMB (P = 0.033).Our study showed a differential distribution of CMB in ischemic stroke subtypes and association of risk factors with the presence, number and location of CMB.

The mechanisms and phenotype of ischemic stroke associated with coronavirus disease 2019 (COVID-19) remain uncertain. A retrospective study was conducted in patients with COVID-19 presenting with ischemic stroke from March 1 to May 25, 2020, and cases with large-vessel occlusion were identified. To provide baseline institutional stroke data within and outside the COVID-19 pandemic, all consecutive ischemic stroke and TIA admissions (COVID and non-COVID) to the hospital during a 10-week period from March 1 to May 10, 2020, were collected and compared with data from the same time period in 2019. Among 20 patients with COVID-19 and acute ischemic stroke, 15 (75%) had large-vessel occlusion. These patients were young (mean age, 46.5 years), male (93%), without major burden of traditional cardiovascular risk factors, and had a severe stroke presentation. Large-vessel occlusions were observed in multiple vessels (40%), uncommonly affected vessels, and atypical locations with a large thrombus burden. Systemic thrombosis separate from large-vessel occlusion was not uncommon (26%). At short-term follow-up, stroke etiology remained undetermined in 46% of patients and functional outcome was poor. The above findings raise the possibility of stroke related to mechanisms induced by the COVID-19 infection itself, including a hypercoagulable state and/or endothelial damage. In addition, they document the severe presentation and poor outcomes of large-vessel occlusion in COVID-19 ischemic stroke.

High-resolution vessel wall imaging (HRVWI) by MRI is a novel noninvasive imaging tool which provides direct information regarding vessel wall pathologies. The utility of HRVWI in differentiating various intracranial vasculopathies among ischemic stroke is still evolving. Consecutive ischemic stroke/TIA patients within 2 weeks of symptom onset between January 2016 to December 2017, with symptomatic vessel stenosis of 50% or more/occlusion on baseline luminal imaging studies were recruited into the study. Stroke subtypes were classified as per TOAST classification initially on the basis of luminal imaging findings alone and subsequently after incorporation of HRVWI findings as well. Forty-nine subjects were recruited into the study. The median age of the population was 42 years (range 11 to 75) with 69% being males. Incorporation of HRVWI findings classified 38.8% subjects into intracranial atherosclerotic disease (ICAD), 32.6% as stroke of other determined aetiology (ODE) (inflammatory vasculopathy [IVas] being the major subgroup [81.2%]) and 28.6% into stroke of undetermined aetiology (UE). HRVWI enabled a diagnostic reclassification in an additional 47.3% among the baseline UE category as against luminal imaging findings alone. ICAD was likelier to have eccentric vessel wall thickening, eccentric vessel wall enhancement and T2 juxtaluminal hyperintensity with surrounding hypointensity (P

Context: The data on the role of Transcranial Doppler (TCD) in the management of acute primary intracerebral hemorrhage (ICH) is meager. Aims: To study TCD variables associated with hematoma expansion in acute primary ICH. Settings and Design: The study was carried out in the neurosciences department of Post Graduate Institute of Medical Education and Research (PGIMER), Chandigarh from July 2010 to September 2011 employing a prospective, double blinded non randomized study design. Materials and Methods: Acute ICH patients within 24 h of symptom onset were recruited. Baseline neuroimaging study (Computerized tomography, CT scan of brain) was performed to assess the pure hematoma volume by AXBXC/2 method. Baseline TCD parameters were obtained from both the middle cerebral arteries (MCAs; affected and unaffected hemisphere): Peak Systolic velocity, End Diastolic velocity, Mean Flow velocity, Resistance Index, and Pulsatility Index. Follow up (24 h) assessment of hematoma volume and TCD were carried out. Each of the TCD variables were compared in hematoma expansion (>33% increase in hematoma volume on the follow-up CT) and non-expansion group. Statistical analysis: On univariate analysis, the Student's t-test and contingency tables with the X2 test were used. A forward stepwise multivariate logistic regression analysis with hematoma expansion at 24 h as the dependent variable and ROC analysis was carried out, using SPSS software version 16 (Chicago, IL). P value < 0.05 was considered significant. Results: Twenty-five patients completed the study. Ten patients (40%) had hematoma expansion. Multivariate analysis revealed unaffected hemisphere MCA Pulsatility Index ratio [unaffected hemisphere MCA Follow up Pulsatility Index/baseline Pulsatility Index] of > 1.055 as the lone correlate of hematoma expansion (sensitivity of 90% and specificity of 60%). Conclusion: Frequent assessment with TCD could aid in prediction of hematoma expansion by measuring unaffected hemisphere Pulsatility Index ratios.

Background and Purpose— The Indo-US Collaborative Stroke Project was designed to characterize ischemic stroke across 5 high-volume academic tertiary hospitals in India. Methods— From January 2012 to August 2014, research coordinators and physician coinvestigators prospectively collected data on 2066 patients with ischemic stroke admitted Results— The mean age was 58.3±14.7 years, 67.2% men. The median admission National Institutes of Health Stroke Scale score was 10 (interquartile range, 5–15) and 24.5% had National Institutes of Health Stroke Scale ≥16. Hypertension (60.8%), diabetes mellitus (35.7%), and tobacco use (32.2%, including bidi/smokeless tobacco) were common risk factors. Only 4% had atrial fibrillation. All patients underwent computed tomography or magnetic resonance imaging; 81% had cerebrovascular imaging. Stroke etiologic subtypes were large artery (29.9%), cardiac (24.9%), small artery (14.2%), other definite (3.4%), and undetermined (27.6%, including 6.7% with incomplete evaluation). Intravenous or intra-arterial thrombolysis was administered in 13%. In-hospital mortality was 7.9%, and 48% achieved modified Rankin Scale score 0 to 2 at 90 days. On multivariate analysis, diabetes mellitus predicted poor 3-month outcome and younger age, lower admission National Institutes of Health Stroke Scale and small-artery etiology predicted excellent 3-month outcome. Conclusions— These comprehensive and novel clinical imaging data will prove useful in refining stroke guidelines and advancing stroke care in India.

This study aimed to assess the feasibility of professional based conventional speech language therapy (SLT) either alone (Group A/less intensive) or assisted by novel computer based local language software (Group B/more intensive) for rehabilitation in early post stroke aphasia.Comprehensive Stroke Care Center of a tertiary health care institute situated in South India, with the study design being prospective open randomised controlled trial with blinded endpoint evaluation.This study recruited 24 right handed first ever acute ischemic stroke patients above 15years of age affecting middle cerebral artery territory within 90days of stroke onset with baseline Western Aphasia Battery (WAB) Aphasia Quotient (AQ) score of93.8 between September 2013 and January 2016.The recruited subjects were block randomised into either Group A/less intensive or Group B/more intensive therapy arms, in order to receive 12 therapy sessions of conventional professional based SLT of 1h each in both groups, with an additional 12h of computer based language therapy in Group B over 4weeks on a thrice weekly basis, with a follow up WAB performed at four and twelve weeks after baseline assessment. The trial was registered with Clinical trials registry India [2016/08/0120121].All the statistical analysis was carried out with IBM SPSS Statistics for Windows version 21.20 subjects [14 (70%) Males; Mean age: 52.8years±SD12.04] completed the study (9 in the less intensive and 11 in the more intensive arm). The mean four weeks follow up AQ showed a significant improvement from the baseline in the total group (p value: 0.01). The rate of rise of AQ from the baseline to four weeks follow up (ΔAQ %) showed a significantly greater value for the less intensive treatment group as against the more intensive treatment group [155% (SD: 150; 95% CI: 34-275) versus 52% (SD: 42%; 95% CI: 24-80) respectively: p value: 0.053].Even though the more intensive treatment arm incorporating combined professional based SLT and computer software based training fared poorer than the less intensive therapy group, this study nevertheless reinforces the feasibility of SLT in augmenting recovery of early post stroke aphasia.

Pancake kidney is a rare renal fusion anomaly with increased risk of complications, such as stone disease and infections, due to altered urodynamics. Image-guided (ultrasonography and fluoroscopy) percutaneous nephrostomy (PCN) is performed to decompress an obstructed pancake kidney. However, routine image guidance may be unable to provide a suitable access in complex clinical scenarios. The approach for PCN in a low-lying fused kidney is difficult due to a limited safe posterior retroperitoneal paraspinal route, and anterior transperitoneal approach poses risks of urine leak and peritonitis. We report a case of an obstructed pancake kidney managed by CT-guided PCN through a transiliopsoas approach.

Multiple endocrine neoplasia type I (MEN-I) is an uncommon isease affecting 1 in 30,000 individuals worldwide [1]. Both spoadic and familial forms have been described. Sporadic forms resent with two of the three principal MEN-I related endocrine umours (pituitary, pancreatic and/or parathyroid) while the familal form consists of a MEN-I case with atleast one first degree elative showing one of the endocrine characterising tumours. Priary hyperparathyroidism (PHPT) is the most common and usually he presenting manifestation of MEN-I affecting more than 95% of ll patients.

Brucellosis remains an important public health problem especially in the underdeveloped countries as well as the Middle East. It may be a "master mimic" leading on to grave diagnostic dilemmas. Chronic neurobrucellosis is seldom associated with signs and symptoms of toxaemia due to multi organ dysfunction. We report the case of a 23-year-old man who presented with fever of 8 weeks associated with ascending weakness of lower limbs and sensorineural type of hearing loss of 6 weeks duration with prominent systemic findings in the form of hepatosplenomegaly and significant loss of weight. He responded well to treatment with combination antimicrobials with clinicoradiological resolution after 6 months of therapy. Even though a rare complication of brucellosis, neurobrucellosis causes significant morbidity if not promptly recognised and treated. Favourable outcomes can be achieved in neurobrucellosis with appropriate protracted polymicrobial antibiotic therapy as was illustrated in our patient.

Most intracranial vascular disorders like atherosclerosis, vasculitis, and reversible cerebral vasoconstriction syndrome (RCVS) share similar lumenographic findings in traditional imaging modalities like computed tomography (CT), magnetic resonance imaging (MRI) and digital subtraction angiographic studies. Hence, there is a need for an advanced imaging modality like vessel wall imaging (VWI) to confirm the diagnosis so that appropriate clinical management could be done. Now, currently vessel wall imaging could be done in a high resolution manner with three dimensional (3D) imaging sequences. The aim of this article is to deal with the protocol as well as the current imaging applications of the high resolution VWI.

Ulcerative colitis (UC) may have diverse extraintestinal manifestations. Nutritional deficiencies, medications or immune-mediated epiphenomena are considered to be pathogenic mechanisms involved. We describe a case of a 56-year-old woman who presented with rapidly progressive tingling paraesthesias in both lower limbs followed by sensory ataxia, ascending spastic quadriparesis, urgency and urge incontinence of 3 months duration. She had an episode of bloody diarrhoea 1 month later for which a colonoscopy was carried out with colonic biopsy features revealing a diagnosis of UC. In view of lack of alternative aetiology, the posterolateral column disease in our patient was attributed to an extraintestinal manifestation of UC. She improved dramatically with a course of intravenous steroids followed by tapering dose of oral steroids and oral mesalamine for her UC. Although rare, UC should be considered in the differential diagnosis of posterolateral column disease of the spinal cord in the appropriate clinical scenario.

An 18-year-old woman presented with headache, right focal seizures, and diminished vision in the left eye for 20 days. Fundus examination revealed bilateral papilledema with large premacular sub–internal limiting membrane and subhyaloid bleeding in the left eye (figure 1A). MRI brain showed hemorrhagic infarct in the left temporal lobe (figure 2, A and B). Magnetic resonance venography showed left transverse and sigmoid sinus thrombosis (figure 2C). A diagnosis of Terson syndrome1 (vitreous hemorrhage) was made. Procoagulant workup was negative and the patient improved with conservative management2 (figure 1B). Even though Terson syndrome has been reported with subarachnoid hemorrhage, its occurrence with cortical venous sinus thrombosis is rare.

A 45-year-old male presented with progressive dysarthria and dysphagia for 1 year. Examination revealed bilateral facial (Figure 1A) and neck flexor weakness, with depressed gag and tongue fasciculations. Fasciculations, wasting and brisk reflexes were noted in upper limbs. Facial reflexes were not brisk, and pseudobulbar affect was not present. Bell’s …

A 24-year-old lady, recently married, was brought to our hospital by her husband when he incidentally noticed a tuft of hair in her lower back which he considered as a bad omen after consulting a rural Indian faith healer. On physical examination, she had a tuft of hair in her lower back which was present since birth and progressively increasing in size (figure 1A). Rest of the physical examination was normal with no neurological deficits …

Marchiafava-Bignami disease (MBD) is a rare complication of chronic alcoholism. Its clinical diagnosis has considerably changed during recent times, with MRI of the brain paving way for in life diagnosis. We believe that physicians need to have a high index of suspicion, because acute onset MBD is not always fatal and complete recovery is possible, provided the diagnosis is made early and treated appropriately. We report a case of MBD who was diagnosed early in the disease course with subsequent clinical and radiological recovery on institution of appropriate treatment.

BACKGROUND: “Swirl sign” on an initial CT scan indicates ongoing hemorrhage in a hematoma and has been associated with increased mortality. OBJECTIVE: To assess CT swirl sign(CTSS) for predicting outcome in acute primary intracerebral hemorrhage. METHODS: Acute ICH patients within 24 hours of symptom onset were recruited. Baseline Non contrast and contrast enhanced CT scan and CT angiography of the brain was done. Follow up CT(FU CT) scan was done 24 hours from baseline.Hematoma volume(HV), CT spot and swirl sign were assessed. All patients were managed as per standard guidelines. Outcome assessment with modified rankin scale(mRS) was carried out at 1 month and 3 months RESULTS: 25 patients were recruited.Mean age was 52.44+11.35years,72% males, time from symptom onset ranged from 180-990 mins (mean-499 mins), GCS range 7-15 ,NIHSS range 6-33 (Mean 19.76), Mean systolic, diastolic and mean arterial pressures at baseline were 186.40 ,108.72 and 134.32 mm Hg. 10 (40%) had hematoma expansion (HE; > 33% increase in hematoma volume on FU CT). Mortality was 36% (9). Prevalence of CT spot sign was 4% . CTSS at baseline and follow-up was 24% (6)and 32%(8) respectively. 87.5% with delayed CTSS had a poor outcome (mRS>2). Delayed CTSS was associated with increased early mortality (1 month) (p = 0.005) and poor outcome at 3 months (p=0.03). CONCLUSION: Delayed CT Swirl sign (>24 hours) is a predictor of mortality and outcomes in acute primary supratentorial intracerebral hemorrhage. It may serve as a marker for aggressive BP lowering even in patients presenting after 6-8 hours

A 64-year-old woman, diabetic and hypertensive, presented with sudden onset painless diplopia and alteration of consciousness. She was drowsy with neurological examination revealing exotropia of both eyes in primary position with bilateral internuclear ophthalmoplegia on attempted horizontal gaze (Figure 1a). Pupils were normal bilaterally. Convergence, vertical saccades, pursuits and vestibular ocular reflexes were impaired. Cranial magnetic resonance imaging (MRI) showed an acute infarct in the midline of mesencephalic …

A 44-year-old woman had progressive burning parasthesias and sensory loss in both upper and lower limbs, followed by weakness of distal muscles of the hands and feet for four years. She had redness and swelling of both hands and feet, multiple painless ulcers over the dorsum of the fingers (Figure 1A ) and feet (Figure 1B), and thickened, non-tender, bilateral, ulnar, superficial cutaneous branch of radial and common peroneal nerves. She also had predominant small fiber sensory polyneuropathy in a glove and stocking pattern, intrinsic muscle weakness of the hands and feet, and intact deep tendon reflexes. Results of hematologic and biochemical tests were within references ranges. Tests results for vasculitis and viral markers were negative. A nerve conduction study showed asymmetrical predominantly axonal sensorimotor polyneuropathy (Figure 3 ) and absent waveforms on sympathetic skin response testing on both upper and lower limbs. A split skin smear was positive for acid-fast bacilli by Ziehl-Nielsen staining (Figure 2A, B). A skin biopsy from the leg showed dermal perivascular lymphocytic infiltrates, nerve twig inflammation, and positive staining for lepra bacilli. Figure 1. A, Multiple ulcers on the dorsum of the fingers (arrows) of the patient with associated swelling and redness. B, Active ulcers on the dorsum of the big toe and midfoot (arrows) and a healed scar on the upper dorsum. Figure 2. Ziehl-Nielsen staining of the split skin smear (A, B) revealing acid fast bacilli (white arrows). Figure 3. Nerve conduction parameters for the patient and normal reference values (N/A: Not Applicable). Glove and stocking sensory loss in lepromatous leprosy is caused by involvement of small dermal sensory and autonomic nerve fibers,1 whereas non-trophic ulcers are caused by cutaneous vasculitis and small fiber involvement by lepra bacilli.1,2 The patient was given a multi-bacillary regimen2 (dapsone, 100 mg/day; rifampicin, 600 mg/day; and clofazimine, 50 mg/day) and showed symptomatic healing of cutaneous ulcers and reduction of burning paraesthesias at two-months follow-up.

A 59-year-old cattle breeder hailing from rural north western India presented with insidious onset and gradually progressive hoarseness of voice and painless dysphagia for 5 months followed by ascending paraparesis and hearing loss for 2 months. He had significant loss of weight with anorexia of 5 months duration. Physical examination revealed pallor, bilateral 7th, 8th, 9th, 10th and left 12th cranial nerve palsies with sensorimotor deficits in radicular distribution (L3-S4) and neck stiffness. Hemogram showed elevated erythrocyte sedimentation rate of 42 mm in …

A 27-year-old man, recent visitor to the Middle East, presented with 6-week history of fever (up to 102°F) followed by altered behavior and left hemiparesis. CSF was acellular with raised protein (138 mg/dL). CSF bacterial culture was sterile; adenosine deaminase normal (3 U/L); cryptococcal antigen, Venereal Disease Research Laboratory test, and Japanese B serology were negative. HIV serology and vasculitic workup were unremarkable. Serum Brucella agglutination titer was 320 IU (immunoglobulin M fraction 280 IU). Cranial MRI showed nonenhancing bilateral white matter and basal ganglia hyperintensities on T2-weighted images (figure, A–C). The patient was treated with IV ceftriaxone (1 month) along with oral doxycycline and rifampicin (4 months). At 3 months, Brucella agglutination titer was

A 43-year-old smoker presented with painless binocular diplopia and numbness in the left frontotemporal region of 4 weeks duration. Physical examination revealed non-tender bony swelling in the left zygomaticotemporal region with posterior cervical lymphadenopathy and non-tender hepatomegaly (figure 1A, white arrow). Neurological examination revealed left superior oblique and lateral rectus palsy with sensory loss in the ophthalmic division of the left trigeminal nerve. A clinical diagnosis of left cavernous sinus syndrome was made. Gadolinium-enhanced MRI of …

Sir, We read with interest Thompson et al. ’s comments on our report of facial nuclear degeneration in bulbar onset amyotrophic lateral sclerosis.1 Thompson et al. contend …

A 33-year-old man with bipolar affective disorder presented with acute onset of rigidity, tremors, and confusion. He had been taking oral lithium (1,200 mg daily) for the past 2 months. Baseline blood, EEG, and CSF analyses were normal. Serum lithium level was 0.67 mEq/L (0.3–1.3). Gadolinium MRI brain showed multiple bilateral symmetric T2-weighted hyperintensities (figure, A–C). On suspicion of lithium-induced neurotoxicity, lithium was replaced with valproate. At 1-month follow-up, his extrapyramidal symptoms had resolved completely, with significant resolution noted on cranial MRI (figure, D–F). Paradoxical lithium neurotoxicity occurs at therapeutic or low serum levels due to lithium-induced toxic demyelination.1-3

A 26-year-old man with complex cyanotic congenital heart disease (Ostium secundum atrial septal defect with pulmonary and tricuspid atresia) presented with an acute onset of holocranial headache and vomiting. He had conjunctival plethora (figure 1A), central cyanosis and clubbing. Fundus showed no papilloedema. There were no focal neurological deficits. His haemoglobin and haematocrit were 17.5 g/dl and 58%, respectively. Chest roentenograph showed a ‘boot-shaped …

Chronic relapsing inflammatory optic neuropathy is a steroid responsive recurrent optic neuropathy. We describe a patient who presented with recurrent episodes of painful visual loss over a period of 19 years and atypical findings on cranial MRI.