Publications by authors named "Sanjith Aaron"

49 Publications

Dissociative pseudo-ulnar sensory loss in a patient with a focal haemorrhage secondary to cerebral venous thrombosis - A case report.

Clin Neurol Neurosurg 2021 Nov 11;211:107025. Epub 2021 Nov 11.

DM Neurology, Department of Neurological Sciences, Christian Medical College, Vellore, India.

Introduction: Small cortical strokes can mimic weakness of peripheral nerve lesions. However, isolated sensory deficits involving the fingers due to cortical lesions are rare.

Methods: We present a case of a 46 year old man with cerebral venous thrombosis, and a haemorrhage restricted to the postcentral gyrus, who reported numbness in an ulnar neuropathy-like distribution. Testing of somatosensory (SSEP) and pain-related evoked potential (PREP) was done, and the lesion location was mapped to the template brain.

Results: The patient had impaired touch and pain but preserved proprioception. He had a normal SSEP response but a prolonged PREP. The lesion was mapped to Broadmann areas 1 and 3b of the postcentral gyrus.

Discussion: Sensory cortical representation is such that, the ulnar fingers are medial, and the radial ones are lateral. Also, modality-specific organization is noted with tactile sensation being mapped to areas 1 and 3b, and proprioceptive sensation to area 3a and 2. Thus focal lesions involving the post central gyrus can have selective sensory loss over some fingers and can have selective impairment of some modalities.

Conclusions: We highlight the rare finding of an ulnar-like sensory loss in a patient with cerebral venous thrombosis and the dissociate nature of the sensory loss in isolated cortical lesions.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.clineuro.2021.107025DOI Listing
November 2021

Patent foramen ovale closure in India; Feasibility, challenges and mid-term outcomes.

Indian Heart J 2021 Sep-Oct;73(5):656-659. Epub 2021 Sep 6.

Department of Biostatistics, Christian Medical College and Hospital, Vellore, 632004, India.

Patent foramen ovale closure (PFO) is an underutilized therapy, and our study explored the challenges and feasibility of PFO closure in the Indian setting. Eighty patients with Embolic Stroke of Undetermined Source (ESUS) were screened by transcranial Doppler (TCD) for PFO. Twenty-nine patients underwent successful closure. High-risk features of a long tunnel, inter-atrial septal aneurysm, and large defect were present in 31%, 28%, and 59%. Transcranial Doppler had a sensitivity and specificity of 78% and 53% (p = 0.02) to detect PFO. Anticoagulation was withdrawn in 85% of patients post closure. Two patients had residual shunts at follow-up of 19 (9,34) months.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.ihj.2021.09.001DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8514405PMC
November 2021

Role of Pain-Related Evoked Potential in the Diagnosis of Meralgia Paresthetica.

Ann Indian Acad Neurol 2021 May-Jun;24(3):379-382. Epub 2020 Sep 2.

Department of Neurological Sciences, Christian Medical College, Vellore, Tamil Nadu, India.

Introduction: Entrapment of the lateral femoral cutaneous nerve (LFCN) of thigh results in meralgia paresthetica (MP). Standard electrophysiological tests for MP are technically demanding and unreliable. We aimed to study the role of pain-related evoked potentials (PREP) in the diagnosis of MP.

Methods: Patients with MP and normal volunteers were included. PREP was recorded by stimulating the skin over the lateral thigh 20 cm below the anterior-superior iliac spine and recording from the cortex at Cz.

Results: A total of 28 subjects and 56 LFCNs were studied. 36 nerves had MP and 20 were normal. The mean PREP latency was 118 (8) ms among normal controls and 164 (10.8) ms in MP. The optimal cut-off point for the diagnosis of MP was 134 ms. Area under receiver operator characteristic curve was 0.97; sensitivity was 91.7% and specificity was 100%.

Conclusion: PREP is reliable and easy to use electrophysiological test in establishing the diagnosis of MP.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.4103/aian.AIAN_441_20DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8370174PMC
September 2020

Bone Mineral Density and Body Composition in Males with Motor Neuron Disease: A Study from Teaching Hospital in Southern Part of India.

Ann Indian Acad Neurol 2021 Mar-Apr;24(2):211-216. Epub 2020 Aug 28.

Department of Endocrinology, Christian Medical College, Vellore, Tamil Nadu, India.

Background: Osteoporosis and sarcopenia are important aspects of motor neuron disease (MND). Individuals with amyotrophic lateral sclerosis (MND-ALS) have an increased risk of falls and fractures. Currently, the standard of care does not involve a routine assessment of bone mineral density (BMD) and body composition in these patients. We aimed to assess BMD, bone mineral parameters and body composition in men with MND and compared them with healthy controls.

Methods: Consecutive males between 50 and 80 years of age diagnosed as MND-ALS by revised El Escorial criteria and able to walk unassisted attending Neurology outpatient clinic were recruited into the study. Age, gender and body mass index (BMI) matched healthy controls were recruited from the local community. BMD and body composition were assessed by dual-energy x-ray absorptiometry (DXA). Bone mineral parameters and bone turnover markers (BTMs) were also assessed in them.

Results: A total of 30 subjects with MND-ALS and 33 controls were recruited. The mean age (years) was 59.2 in cases and 61.2 in controls. The mean BMD (g/cm) between the two groups was similar; however, BTMs were significantly higher in the MND group ( < 0.05). Subjects with MND-ALS had significantly lower mean appendicular lean mass (ALM) (19.9 versus 22.4 kg; = 0.007) and ALM corrected for BMI than the healthy control group (0.858 versus 0.934 kg/kg/m; = 0.034). Sarcopenic obesity (Percentage fat mass >27% + ALM/BMI <0.786 kg/kg/m) was more prevalent in MND-ALS compared to controls (44.5% versus 16.7%; = 0.03).

Conclusion: Although BMD was not significantly different between subjects with MND-ALS and healthy controls, BTMs were significantly higher in the MND group indicating a high bone turnover state. Sarcopenia and sarcopenic obesity were also more in MND-ALS group than controls. Routine assessment for bone health parameters and body composition indices may be included in management of the patients with MND.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.4103/aian.AIAN_293_20DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8232495PMC
August 2020

Diagnostic utility of flow cytometry based coated-platelets assay as a biomarker to predict thrombotic or hemorrhagic phenotype in acute stroke.

Cytometry B Clin Cytom 2021 Jun 21. Epub 2021 Jun 21.

Department of Transfusion Medicine and Immunohematology, Christian Medical College, Vellore, Tamil Nadu, India.

Background: Coated-platelets are sub-population of platelets "coated" with highly procoagulant proteins and phosphatidylserine that sustains thrombin generation. They are produced upon dual agonist stimulation by collagen and thrombin. This study was conducted to assess if there was any difference in the levels of coated-platelets in patients with primary intracranial hemorrhage (PICH) and ischemic stroke due to large artery atherosclerosis (LAA) as compared to healthy controls, and to see if coated-platelet levels had any influence on the hemorrhagic transformation (HT) of ischemic stroke.

Methods: Coated-platelet levels were determined by flow cytometry using fluorescently tagged Annexin V antibody to identify phosphatidylserine exposed on the surface of platelets activated by dual agonists (convulxin and thrombin) in cross-sectional cohort of 75 patients with stroke and 34 controls.

Results: Patients with PICH (n = 35) had significantly lower coated-platelets than the controls (adjusted mean ± SE, 21.0 ± 1.9% vs. 36.1 ± 1.7%, p < 0.001), while patients with LAA (n = 30) had significantly higher coated-platelets than controls (adjusted mean ± SE, 51.9 ± 1.5% vs. 36.1 ± 1.7%, p < 0.001). Patients with subsequent HT of ischemic stroke (n = 10) had significantly lower coated-platelet levels at admission compared to those without HT (adjusted mean ± SE, 18.1 ± 2.6% vs. 51.9 ± 1.5%, p < 0.001).

Conclusions: Coated-platelet levels are significantly different in patients with hemorrhagic and ischemic stroke as compared with controls. Lower levels of coated-platelets measured by flow cytometry may be earliest predictor of subsequent HT in patients with ischemic stroke even before the radiological changes suggestive of HT are visualized.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1002/cyto.b.22026DOI Listing
June 2021

Clinical Spectrum, Therapeutic Outcomes and Prognostic predictors in Paraneoplastic Neurological Syndromes - Experiences from a Tertiary Care Center in India.

Ann Indian Acad Neurol 2021 Jan-Feb;24(1):32-39. Epub 2021 Feb 9.

Department of Neurological Sciences, Christian Medical College, Vellore, Tamil Nadu, India.

Background: Paraneoplastic Neurological Syndromes (PNSs) are a heterogeneous group of immune-mediated disorders that often precede tumor diagnosis. There are few systematic studies on the spectrum and follow-up of PNSs.

Objective: To analyze the clinical spectrum, associated tumors, antibody profile, outcomes, and prognostic predictors in a cohort of PNSs admitted in a tertiary care center.

Methods: This retrospective study included 97 patients (2008-2019). PNSs were further classified as "classical," "nonclassical," "definite," and "possible." Clinical profile, diagnostic strategies, therapeutic options, and predictors of outcomes were identified.

Results: The median age was 54 years (range 17-81). Thirty-nine (40.2%) had classical PNS, and 58 (59.8%) had nonclassical PNS, 74 (76.3%) had "Definite" PNS while 23 (23.7%) had "Possible" PNS. Cerebellar degeneration, peripheral neuropathy, and encephalopathy were the three most common neurological syndromes. Tumors were diagnosed in 66 (68%) patients; Lung cancer was the most common primary tumor. Antibodies were positive in 52 (53.6%). Anti-Yo antibody and anti-Ma2 antibody were the most common antibodies. The majority (57.7%) received immunotherapy in addition to definitive treatment for the tumor. A good outcome was seen in 53 (54.6%). Factors associated with good outcome were: early diagnosis, mRS <3 at presentation, absence of metastatic disease, and adjuvant immunotherapy.

Conclusion: A high index of clinical suspicion is essential for early diagnosis and prompt management of PNS, especially the nonclassical syndromes. Multimodality diagnostic imaging techniques and antibody profiling play a crucial role in the diagnosis. A favorable prognosis can be expected with the judicious use of immunotherapy and definitive treatment of malignancy.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.4103/aian.AIAN_975_20DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8061505PMC
February 2021

Utility of Serial Nerve Conduction Studies in the Electrodiagnosis of Guillain-Barre Syndrome.

Neurol India 2021 Mar-Apr;69(2):369-375

The Brunei Neuroscience Stroke and Rehabilitation Centre, India.

Background: Guillain-Barre syndrome can be electrophysiologically classified into demyelinating and axonal subtypes and nerve conduction studies remain the mainstay in electrodiagnosis. Accurate electrodiagnosis has both therapeutic and prognostic significance and different criteria sets have been proposed for classification.

Objectives: To electrophysiologically classify GBS patients into AIDP and axonal subtypes according to various published criteria (Cornblath, 1990: Hadden, 1998, Rajabally, 2015), investigate if serial NCS changes the classification, and to identify additional parameters which may support the electrodiagnosis.

Materials And Methods: In a retrospective study, we included all patients aged 15 to 80 years, admitted with a diagnosis of GBS between August 2015 and July 2017, who had at least two serial NCS. The various published criteria were applied to the two serial NCS and subtype classification along with diagnostic shifts on serial NCS were ascertained.

Results: At the first test, the established criteria gave a yield of 45.2% to 71% for AIDP, while 29% to 54.8% of patients were classified as axonal GBS. In the second study, there was a change in electrodiagnosis, ranging from 9.6% to 16.1%. The resolution of reversible conduction failure and misclassification of subtypes were the major reason for diagnostic shifts. Sural sparing pattern, facial nerve dysfunction, abnormal blink reflex, and phrenic nerve dysfunction were more common in AIDP.

Conclusions: Serial nerve conduction studies allow an accurate electrodiagnosis of GBS subtypes, which has both therapeutic and prognostic implications. Also, the use of additional parameters such as blink reflex facial and phrenic nerve conduction may supplement routine NCS.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.4103/0028-3886.314529DOI Listing
June 2021

Paradoxical Emboli to Artery of Percehron in Hereditary Haemorrhagic Telangiectasia.

Neurol India 2021 Jan-Feb;69(1):198-200

Department of Neurological Sciences, Neurology Unit, Christian Medical College and Hospital, Vellore, Tamil Nadu, India.

View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.4103/0028-3886.310068DOI Listing
June 2021

Acute Ischemic Stroke in Term Pregnancy Treated with Recombinant Tissue Plasminogen Activator.

Case Rep Neurol 2020 Sep-Dec;12(Suppl 1):4-8. Epub 2020 Dec 14.

Department of Neurology, Christian Medical College, Ludhiana, India.

Recombinant tissue plasminogen activator (rtPA) may be considered in acute ischemic strokes (AIS) occurring in pregnancy. This recommendation is based on case reports which had demonstrated the efficiency and safety of rtPA in early pregnancy. However, most of the pregnancy-related strokes occur in the peripartum period. There is a paucity of literature on the management of AIS in this clinically relevant period of pregnancy; with only 2 case reports describing the use of IV rt-PA in the last trimester. Here, we report a case of AIS in a full-term pregnancy treated successfully with intravenous rt-PA. The patient went on to have an uncomplicated vaginal delivery after 72 h post thrombolysis. To the best of our knowledge, this is only the third such case in the English literature.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1159/000506763DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7802499PMC
December 2020

Network Localization of Central Hypoventilation Syndrome in Lateral Medullary Infarction.

J Neuroimaging 2020 11 17;30(6):875-881. Epub 2020 Aug 17.

Department of Neurological Sciences, Christian Medical College, Vellore, India.

Background And Purpose: The brainstem plays a key role in the control of respiration. Strokes involving the lateral medulla can rarely produce a central hypoventilation syndrome (CHS) characterized by loss of automatic respiration called Ondine's curse. In this study, we investigated the neuroanatomical correlates of CHS in patients with lateral medullary infarction (LMI).

Methods: Cases of CHS following LMI were identified from searching our medical records and literature. Voxel-based lesion-symptom mapping and lesion network-symptom-mapping (LNSM) analysis was performed to identify the regions connected to the lesion sites based on normative functional connectome data.

Results: Sixteen patients with CHS and 32 controls were included. The ventro-lateral region of the rostral medulla showed a significant association with CHS. LNSM analysis showed connections of this region to the rostral ventro-lateral medulla and caudal pons.

Conclusions: In patients with LMI, disruption of the respiratory control network, at the level of ventro-lateral region of the rostral medulla, could result in CHS.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1111/jon.12765DOI Listing
November 2020

Monoclonal Gammopathies of 'Neurological Significance': Paraproteinemic Neuropathies.

Can J Neurol Sci 2021 09 5;48(5):616-625. Epub 2021 Jan 5.

The Brunei Neuroscience Stroke and Rehabilitation Centre, Brunei Darussalam.

Objectives: To study the clinical profile and outcomes of patients with paraproteinemic neuropathy (PPN) and to explore the utility of nerve conduction studies (NCSs) to differentiate between the demyelinating subtypes.

Methods: We did a retrospective analysis of patients diagnosed with PPN between January 2010 and December 2019 in an inpatient setting. The study population consisted of patients above 16 years of age presenting with clinical features suggestive of chronic peripheral neuropathy and on evaluation was found to have PPN.

Results: A total of 74 patients were identified. The patients were predominantly in the 6th decade, and the majority were males. The subtypes of PPN were monoclonal gammopathy of undetermined significance (MGUS) (45.9%), POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal plasma cell disorder, and skin changes) (24.3%), solitary plasmacytoma (17.6%), multiple myeloma (8.1%), and AL amyloidosis (4.1%). There are specific features on NCS which can help in identifying POEMS syndrome and IgM MGUS. The majority of patients with PPN tend to stabilize or improve with treatment; however, many have a severe residual disability. New terminology and classification of these entities as 'monoclonal gammopathies of neurological significance' can aid in early diagnosis and the development of effective treatment, to prevent residual disability.

Conclusion: PPN has a heterogeneous spectrum of clinical, biochemical, and electrophysiological features. NCS can help distinguish POEMS syndrome and IgM MGUS from other demyelinating subtypes.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1017/cjn.2020.278DOI Listing
September 2021

Clinical Spectrum and Outcome of Neurosarcoidosis: A Retrospective Cohort Study from a Teaching Hospital in India.

Ann Indian Acad Neurol 2020 Jul-Aug;23(4):528-535. Epub 2020 Jun 29.

Neurology Unit, Department of Neurological Sciences, Christian Medical College, Vellore, Tamil Nadu, India.

Context: Neurosarcoidosis (NS) is a chronic disease with a diverse clinical spectrum, therapeutic response, and outcome. There is scarce literature from our country regarding the same.

Aims: The aim of this study was to evaluate the clinical spectrum, therapeutic responses, and outcomes of NS in an Indian cohort.

Settings And Design: In a cross-sectional study, we included all patients with NS treated at a quaternary care teaching hospital in India from January 2007 to October 2019.

Subjects And Methods: Patients older than 18 years of age fulfilling the diagnostic criteria for NS from the Neurosarcoidosis Consortium Consensus Group were included in the study. The therapeutic response and the degree of disability at last follow-up were assessed.

Results: We identified 48 patients, among them 3 were categorized as having definite NS, 30 probable NS, and 15 possible NS. Cranial neuropathy was the most common presentation (47.9%), followed by myelopathy (25%). Systemic involvement was identified in 95.83% and mediastinal lymph nodes were the most common site. Clinical improvement was seen in 65.8% and disease stabilized in 28.9%, while 5.26% deteriorated. Fifty percent recovered without any residual disability, while 26.3% had minor and 23.7% had major residual sequelae.

Conclusions: NS is a diverse illness, with a heterogeneous spectrum of clinical presentation, treatment response, and outcome. Cranial neuropathy is the most common presenting feature and has a good prognosis while myelopathy has an unfavorable prognosis. Meningeal and brain parenchymal disease is difficult to diagnose accurately unless systemic involvement is present. The diagnosis of NS should be clinically suspected in the appropriate clinical setting, the presence of systemic involvement should be investigated, and histologic confirmation should be attempted.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.4103/aian.AIAN_638_19DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7657274PMC
June 2020

Cerebral Venous Thrombosis, Seasonal Trends, and Climatic Influence: A Region-SpecificStudy.

Ann Indian Acad Neurol 2020 Jul-Aug;23(4):522-527. Epub 2019 Jul 11.

Department of Neurology, Christian Medical College Hospital, Vellore, Tamil Nadu, India.

Background And Purpose: Studies looking at seasonal variation on cerebral venous thrombosis (CVT) are few with conflicting conclusions. In this region-specific study, we looked for climatic influence and seasonal trends on the incidence of CVT.

Methods: Imaging proven adult CVT cases treated over a period of 18 years from a specific geographical location with similar seasons and climatic conditions were studied. Metrological parameters prepared using 30 years of data was used. Quantum geographical information system (QGIS software) and SPSS v 22 were used for patient plotting and analysis.

Results: Total of 970 cases were studied. The incidence was significantly higher in summer 411 (42.3%) compared with autumn 317 (32.7%) and winter 242 (25.05); = 0.038. This trend was consistent across all the 18 years in time series analysis. Mean age was 33.5 years (range 18-88 years). A significant majority 673 (69.4%) were below 40 years of age; = 0.012. Females constituted 394 (40.6%) of cases. Postpartum CVT cases constituted 237 (30%). Interaction analysis showed younger age (<40 years) were more vulnerable for CVT in summer; = 0.009. There was no seasonal influence on postpartum CVT. Apart for a weak positive correlation between rain fall ( = 0.18, < 0.01); humidity and cloud cover was not influencing the incidence.

Conclusions: Higher ambient temperatures were consistently associated with higher incidence of CVT. This is the largest region-specific study on CVT in the world. These results may be applicable to other regions with similar climatic conditions.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.4103/aian.AIAN_409_19DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7657288PMC
July 2019

Isolated Infarction of the Medullary Pyramid - A Rare Pure Motor Stroke.

J Stroke Cerebrovasc Dis 2020 Oct 29;29(10):105163. Epub 2020 Jul 29.

Radiology Department, Ministry of Health of Oman, Khoula Hospital, Muscat, Oman, Phone:+968 98440199. Electronic address:

Acute pure motor hemiparesis can occur due to a vascular insult along the cortico spinal pathway. Rarely such a stroke can occur as a result of involvement of the pyramids located on the ventral aspect of the medulla. Neurological localization can be difficult in such strokes and moreover they can also pose imaging challenges since they are missed on the CT scans. So far only 4 such cases have been reported in literature. Here we present two cases of pure motor strokes caused by infarction of the medullary pyramids. We have described the anatomical correlates of the symptoms and signs and also highlight the importance of MRI in picking up these rare strokes.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.jstrokecerebrovasdis.2020.105163DOI Listing
October 2020

Prothrombin G20210A polymorphism in patients with venous and cryptogenic arterial strokes among ethnic groups in south and north India.

Natl Med J India 2019 Jul-Aug;32(4):213-215

Department of General Medicine, Christian Medical College, Vellore 632004, Tamil Nadu, India.

Background: Prothrombin (PT) G20210A is one of the genetic polymorphisms associated with thrombophilia. Studies show a low prevalence for this polymorphism in Asian populations with only one subject reported from India. We studied the prevalence and association of this polymorphism in patients with arterial and venous strokes and their matched controls in south and north India.

Methods: We recruited patients with cerebral venous thrombosis (mean age 37.2 years) and cryptogenic ischaemic stroke (mean age 36.7 years), and age- and sex-matched controls (mean age 37.6 years) from south and north India. Genotyping was carried out using polymerase chain reaction followed by restriction fragment length polymorphism, and the prevalence of the variants among the patients and controls was compared.

Results: The heterozygous allele of the polymorphism was detected in both groups with significantly higher prevalence among north Indians (5/154; 3.2%) compared with south Indians (4/516; 0.8%; p = 0.026). Thrombosis as a manifestation of this polymorphism was more among north Indians with 4/82 (4.9%) of patients with ischaemic stroke and cerebral venous thrombosis having this polymorphism compared with south Indian patients 1/72 (1.4%), p = 0.003.

Conclusion: PT G20210A is prevalent in India, especially among those from north India. Its role in predisposition to thrombosis needs to be studied further along with other known risk factors.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.4103/0970-258X.291290DOI Listing
August 2020

Clinical Features of Four West Nile Virus Cases and Its Molecular Characterization from a South Indian Tertiary Care Hospital.

Case Rep Infect Dis 2020 16;2020:1315041. Epub 2020 Jul 16.

Department of Clinical Virology, Christian Medical College, Vellore, Tamil Nadu, India.

West Nile virus (WNV) is currently a significant reemerging virus of the 21st century. It belongs to the family Flaviviridae and genus . Although it is primarily transmitted by the spp of mosquitoes, other routes of transmission are also well defined. Of eight lineages described, Lineage 1a has been reported from many parts of South India and is known to cause neuroinvasive illness. Many tests and serological techniques have been described to diagnose WNV infection such as complement fixation, neutralization, heamagglutination inhibition, ELISA, and PCR for molecular confirmation. The latter far outweighs the limitations inherent in the other tests. WNV infection is being reported from Vellore for the first time after 1968. This paper aims to describe four cases of WNV infection causing central nervous system manifestations with its molecular characterization. West Nile virus infection was diagnosed with the available molecular techniques such as PCR and sequencing, which emphasizes the need for considering West Nile virus in the differential diagnosis of acute meningoencephalitis and the wider availability of molecular diagnostic tests.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1155/2020/1315041DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7381993PMC
July 2020

Tolosa-Hunt Syndrome: Long-Term Outcome and Role of Steroid-Sparing Agents.

Ann Indian Acad Neurol 2020 Mar-Apr;23(2):201-205. Epub 2020 Feb 25.

Department of Neurological Sciences, Neurology Unit, Christian Medical College and Hospital, Vellore, Tamil Nadu, India.

Background: Tolosa-Hunt Syndrome (THS) is one of the causes of cavernous sinus syndrome causing painful ophthalmoplegia. Literature on long-term outcome of this rare condition is scarce.

Aims And Objectives: The aim is to study the recurrence and role of steroid-sparing agents in THS.

Methodology: All cases of THS treated at a tertiary-level teaching hospital during a 10-year period were studied. Clinical and radiological profile, response to treatment and recurrences were noted.

Results: A total of 44 cases were studied. The mean age was 49.5 years, Males constituted 23/44 (52%). The first symptom was pain in 90%. Ptosis with ophthalmoplegia was the most common deficit 29/44 (66%). Lesions confined to cavernous sinus 27/44 (61%) was the most frequent magnetic resonance imaging finding. All patients received steroids as the initial treatment and 15/44 (34%) received steroid-sparing agents. Follow-up ranged from 6 to 120 months (Mean 39 months). Two patients had alternative diagnosis of leptomeningeal malignancy and hypertrophic pachymeningitis on follow-up. Recurrences occurred in 18/37 (48.6%). Time for recurrence varied from 8 months to 7 years. (Mean 18 months). No clinical or radiological predictors for recurrence were identified. Patients who received steroid-sparing agents had a significantly lower recurrence 3/15 (20%) versus 14/26 (53.8%)P < 0.034.

Conclusions: Around 50% of patients with THS can have recurrence. Steroid-sparing agents appear to prevent recurrence. A prospective multicenter randomized controlled trial may help to evaluate the risk and benefits of steroid-sparing therapy and to identify any possible predictors for recurrence.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.4103/aian.AIAN_368_18DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7061504PMC
February 2020

Dynamic imaging of the craniovertebral junction is mandatory in patients with posterior circulation strokes.

Eur Spine J 2020 05 19;29(5):1078-1086. Epub 2020 Feb 19.

Department of Neurological Sciences, Christian Medical College, Vellore, India.

Introduction: The course of the vertebral artery after exiting from the C1 foramen transversarium and prior to entering the dura lends itself to compression in C1-2 instability. However, atlantoaxial dislocation presenting with vertebrobasilar insufficiency and posterior circulation stroke (PCS) is rare.

Methods: In this retrospective study, we identified 96 patients with PCS who had complete radiological data. Ten (10.4%) patients had craniovertebral junction (CVJ) anomalies, of which six underwent surgery and four were managed conservatively. The clinical and functional outcomes were measured in the two groups.

Results: Left-sided strokes were seen in 7/10 patients, the majority of whom had left dominant vertebral arteries. The mean age at presentation in those with CVJ anomalies was 27.2 ± 12.8 years that was significantly lower than those without CVJ anomalies, 52.2 ± 14.5 years (p ≤ 0.001). The etiologies of PCS in those < 50 years were CVJ anomalies (30%), atherosclerosis (30%) and vasculitis (27%); however, the overwhelming majority of strokes in the > 50 year age group was atherosclerosis (91%). Postoperatively, there were no recurrent strokes in the operated patients, who also obtained significant clinical improvement on the modified Rankin Scale, Nurick Scale and modified McCormick Scale as compared to those who did not undergo surgery.

Conclusion: Early diagnosis and surgical treatment of CVJ instability prevent recurrent strokes and improve outcomes in patients with PCS. Physicians and spine surgeons need to be sensitized regarding CVJ anomalies as a cause of PCS enabling early diagnosis with dynamic imaging particularly in the younger age group. These slides can be retrieved under Electronic Supplementary Material.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1007/s00586-020-06337-3DOI Listing
May 2020

Comorbidities and Long-Term Outcomes in a Cohort with Myasthenic Crisis: Experiences from a Tertiary Care Center.

Ann Indian Acad Neurol 2019 Oct-Dec;22(4):464-471. Epub 2019 Oct 25.

Department of Neurological Sciences, Christian Medical College, Vellore, Tamil Nadu, India.

Introduction: There is scarce literature regarding the clinical course, comorbidities and long-term outcomes after myasthenic crisis (MC). The natural history of myasthenia gravis (MG) in this subset remains uncertain.

Methods: The study included a cohort admitted with MC (2007-2017) in a tertiary care hospital. The comorbidities, outcomes after discharge, and prognostic factors were analyzed.

Results: Sixty-two patients (89 episodes of MC) were included. Demographic data was comparable between the early- (<50 years) and late-onset (≥50 years) groups. Comorbidities included stress cardiomyopathy (14.5%), arrhythmias (6.4%), neuropathy (17.7%), pancytopenia (12.9%), encephalopathy (11.2%), neuromyotonia (4.8%), myelopathy (3.2%), and myositis (3.2%). Pulmonary embolism ( < 0.008), dysautonomia ( < 0.002), sepsis ( < 0.008), neuropathy ( < 0.002), and phrenic dysfunction ( < 0.016) were associated with prolonged ventilation. Majority of the patients (42, 67.7%) had a favorable outcome (disease status) as defined by remission/minimal manifestations at the time of last follow-up (median 36 months, IQR 15-66). Persistent bulbar weakness ( < 0.001), neuropsychiatric illness ( < 0.001), and comorbidities ( < 0.017) were associated with refractory MG. Eighteen patients (29%) had recurrent crisis. Eleven patients succumbed in the cohort. The main predictors of mortality were tumor progression ( < 0.001) and cardiac illness ( < 0.004).

Discussion: A comprehensive treatment approach in MC will translate to good short- and long-term outcomes. The main cornerstones of therapy will include (1) Identification of refractory MG with the implementation of phenotype-based therapy; (2) Addressing comorbidities including cardiac autonomic neuropathy, bulbar weakness, phrenic dysfunction; and (3) Meticulous tumor surveillance.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.4103/aian.AIAN_197_19DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6839309PMC
October 2019

Neural Correlates of Urinary Retention in Lateral Medullary Infarction.

Int Neurourol J 2019 Sep 30;23(3):205-210. Epub 2019 Sep 30.

Department of Neurological Sciences, Christian Medical College, Vellore, India.

Purpose: The brainstem plays an important role in the control of micturition, and brainstem strokes are known to present with micturition dysfunction. Micturition dysfunction in cases of lateral medullary infarction (LMI) is uncommon, but often manifests as urinary retention. In this study, we investigated the neuro-anatomical correlates of urinary retention in patients with LMI.

Methods: This was a hospital-based retrospective study conducted in the neurology unit of a quaternary-level teaching hospital. Inpatient records from January 2008 to May 2018 were searched using a computerized database. Cases of isolated LMI were identified and those with micturition dysfunction were reviewed. MRI brain images of all patients were viewed, and individual lesions were mapped onto the Montreal Neurological Institute (MNI) space manually using MRIcron. Nonparametric mapping toolbox software was used for voxel-based lesion-symptom analysis. The Liebermeister test was used for statistical analysis, and the resultant statistical map was displayed on the MNI template using MRIcron.

Results: During the study period, 31 patients with isolated LMI were identified. Their mean age was 48 years and 28 (90%) were male. Six of these patients (19%) developed micturition dysfunction. All 6 patients had urinary retention and 1 patient each had urge incontinence and overflow incontinence. In patients with LMI, the lateral tegmentum of the medulla showed a significant association with urinary retention.

Conclusion: In patients with isolated LMI, we postulate that disruption of the descending pathway from the pontine micturition centre to the sacral spinal cord at the level of the lateral tegmentum results in urinary retention.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.5213/inj.1836256.128DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6790816PMC
September 2019

Ayurvedic Treatment of Acute Ischemic Stroke: A Prospective Observational Study.

Glob Adv Health Med 2019 30;8:2164956119849396. Epub 2019 May 30.

Institute for Stem Cell Science and Regenerative Medicine, Bengaluru, India.

Background: The western medical arsenal for treating stroke is rather limited, and the only treatments shown to improve outcomes are not accessible to most in the third world. Even in the developed world, many patients present too late to receive thrombolysis or thrombectomy. Stroke patients in India commonly use Ayurvedic therapies, but there are no published data regarding the efficacy or safety of these therapies, the latter being of particular concern in acute ischemic stroke (AIS).

Objective: To obtain preliminary data on the safety and efficacy of stand-alone whole-system Ayurvedic treatment in AIS.

Methods: We present here an observational study prospectively comparing outcomes in 2 cohorts of AIS patients treated with whole-system classical Ayurveda (n = 13) or conservative (nonthrombolytic, noninterventional) western biomedicine (n = 20).

Results: Pooled analysis of outcomes did not show statistically significant differences in mortality (15.38% vs 15%,  = 1.00), nonfatal adverse event rates (15.38% vs 30%,  = .4), or functional disability measures. A paired analysis performed using a matching algorithm to reduce baseline disparities between the cohorts also showed no statistically significant differences in outcomes.

Conclusions: The safety profiles of classical Ayurveda and conservative western biomedicine in AIS are similar. This is the first ever report of stand-alone Ayurvedic therapy in AIS. Our results support the conduct of a randomized controlled trial to study the efficacy of Ayurvedic treatment of AIS.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1177/2164956119849396DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6545648PMC
May 2019

Timed Vibration Sense and Joint Position Sense Testing in the Diagnosis of Distal Sensory Polyneuropathy.

J Neurosci Rural Pract 2019 Apr-Jun;10(2):273-277

Department of Neurological Sciences, Christian Medical College, Vellore, Tamil Nadu, India.

Introduction: Distal sensory polyneuropathy (DSP) is one of the most common neurological disorders. Although several studies have studied the role of the neurological examination in DSP, there are only limited studies on the utility of timed vibration sense (VBS) and joint position sense (JPS) testing in the diagnosis of DSP.

Objectives: The objective is to study the utility of timed VBS testing and JPS testing at the great toe in clinical detection of DSP.

Methods: This study was prospectively conducted in the neurology department of a tertiary care hospital in India. Patients with DSP referred to the electrophysiology laboratory from August 2017 to December 2017 were screened. Patients with symptomatic DSP which was confirmed by electrophysiological studies were taken as cases and normal participants with no symptoms or electrophysiological findings suggestive of DSP served as controls.

Results: We studied 127 patients and 194 controls. The mean age of the patients was 48.7 (14.5) years in the patient group and 39.7 (14.5) years in the control group. The male: female ratio was 77/50 in the patient group and 112/82 in the control group. Abnormal clinical examination was found in 95% of the patients with DSP. The most common abnormal examination components were impaired ankle reflexes (70%), vibration (85%), and JPS (39.6%) sensation. Using the receiver operating characteristic curve for the diagnosis of DSP, a vibratory response lasting <8 s at the great toe had a sensitivity of 85% and specificity of 42.8%. For JPS testing at the great toe, obtaining two or more incorrect responses had a sensitivity of 33% and specificity of 87.6%.

Conclusion: VBS testing was more sensitive and JPS testing was more specific in making a clinical diagnosis of DSP. For timed VBS, duration of >8 s at the great toe was a useful test to rule out DSP, and for JPS testing at the great toe, obtaining two or more incorrect responses was a useful test in ruling in the diagnosis of DSP.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.4103/jnrp.jnrp_241_18DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6454947PMC
April 2019

Clinical profile of primary progressive aphasias in a tertiary care centre from India.

Int J Speech Lang Pathol 2019 12 14;21(6):547-552. Epub 2019 Jan 14.

Department of neurological sciences, Christian medical college, Vellore, India.

Progressive language dysfunction due to a selective neurodegeneration of the language networks is called primary progressive aphasia (PPA). However, demographic data on PPA is limited. In this study from India, we determined the prevalence and clinical profile of patients presenting with PPA and its subtypes. Patients who were admitted to the neurosciences department during the period between January 2012 and December 2016 were screened, and patients who presented with slowly progressive aphasia for at least 2 years without other significant cognitive or behavioural symptoms and preservation of daily living activities were included. Patients had to fulfil the international consensus group criteria for PPA. All patients were evaluated with the mini-mental status examination (MMSE) and Strub and Black battery for neuropsychological testing. The language was tested using the progressive aphasia language scale (PALS). During the study period from January 2012 to December 2016, 23 patients fulfilled the international consensus criteria for PPA. Of these, 16 (69.6%) patients were diagnosed with PPA-G, 6 (26%) patients had PPA-S and 1 (4.4%) patient had PPA-L.: PPA is not an uncommon entity in India and the most common subtype in this study was PPA-G.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1080/17549507.2018.1545870DOI Listing
December 2019

Accuracy and reliability of Babinski sign versus finger and foot tapping in the diagnosis of corticospinal tract lesions.

Neurol India 2018 Sep-Oct;66(5):1377-1380

Department of Neurology, Christian Medical College, Vellore, Tamil Nadu, India.

Introduction: The Babinski sign is one of the most important clinical signs for detecting corticospinal tract (CST) lesions. However, due to variations in testing and interpretation, it has been associated with low interobserver agreement rates. In this study, the diagnostic value of finger and foot tapping in detecting CST lesions was compared to that of the Babinski sign.

Materials And Methods: Three groups of participants were recruited: Group 1 - individuals having CST lesions diagnosed on the basis of clinical examination as well as neuroimaging; group 2 - individuals having a non-CST neurological illness; group 3 - normal individuals who were relatives of the patients recruited. The sensitivity and specificity of finger tapping, foot tapping, and Babinski sign were calculated.

Results: 375 patients, 125 in each group, were included. The overall sensitivity for Babinski sign was 49.6% and specificity was 85.8%. The overall sensitivity for finger and foot tapping was 79.5% and specificity was 88.4%. The interobserver agreement between the medical students and the neurologist was greater for finger and foot tapping (Kappa = 0.83) when compared to Babinski sign (Kappa = 0.45).

Conclusion: Finger and foot tapping is a valid and reliable test in the clinical diagnosis of corticospinal lesions. The reliability and validity of Babinski sign is variable and thus its ability to diagnose the manifestations of corticospinal lesions is less when compared to the finger and foot tapping test.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.4103/0028-3886.241370DOI Listing
September 2019

The benefit of EXtending oral antiCOAgulation treatment (EXCOA) after acute cerebral vein thrombosis (CVT): EXCOA-CVT cluster randomized trial protocol.

Int J Stroke 2018 10 17;13(7):771-774. Epub 2018 May 17.

1 Department of Neurosciences and Mental Health, Neurology, Hospital de Santa Maria-CHLN, Lisbon, Portugal.

Rationale After a cerebral vein thrombosis, there is an increased risk of further venous thromboembolic events. The optimal duration of anticoagulation after cerebral vein thrombosis is unknown. Aim To compare efficacy and safety of a policy of short- (3-6 months) versus long-term (12 months) anticoagulation (any type venous thromboembolic events) after cerebral vein thrombosis for the prevention of venous thromboembolic events. Sample size estimates A sample of 1428 patients (749 per arm) allows detecting a reduction from 10 to 5% in the risk of venous thromboembolic event recurrence with 80% power at 5% significance, with 3% dropout rate. Methods and design An international multicenter, prospective cluster-randomized trial with equal allocation between both interventions (ISRCTN25644448). Each cluster is a participating center, which accepted to be randomly allocated to one of the anticoagulation policies. Eligible patients are adults with radiologically confirmed cerebral vein thrombosis within 30 days, and stable to initiate post-acute anticoagulation. Patients judged by the investigator to be an absolute indication for permanent anticoagulation are excluded. Follow-up is at 6, 12 and 24 months. Study outcomes Primary efficacy outcome is any symptomatic and confirmed fatal/nonfatal venous thromboembolic event (recurrent-cerebral vein thrombosis or non-cerebral venous thromboembolic event). Primary safety outcomes include bleeding events during treatment periods and death from any cause. Discussion This study responds to a knowledge gap in the post-acute management of cerebral vein thrombosis patients by comparing short- versus long-term anticoagulation for the prevention of venous thromboembolic event recurrence.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1177/1747493018778137DOI Listing
October 2018

Role of Noninvasive Imaging of Cerebral Arterial System in Ischemic Stroke: Comparison of Transcranial Color-coded Doppler Sonography with Magnetic Resonance Angiography.

J Clin Imaging Sci 2018 20;8:19. Epub 2018 Apr 20.

Department of Neurology, Christian Medical College, Vellore, Tamil Nadu, India.

Aim: To determine the accuracy of transcranial color-coded Doppler sonography (TCCS) in the evaluation of cerebral arterial system in patients with ischemic stroke attending a tertiary care hospital in South India.

Objectives: (1) To describe the topographical distribution of atherosclerotic lesions in the cerebral circulation in patients presenting with ischemic stroke from the Indian subcontinent and (2) to determine the accuracy of TCCS for detection and quantification of intracranial stenoses in various segments of the intracerebral arterial system in comparison with magnetic resonance angiography (MRA).

Materials And Methods: The demographic profile and risk factors of consecutive patients who presented to neurology outpatient department with cerebral ischemia and scheduled for MRA were determined. These patients had undergone neck Doppler, TCCS, and MRA. The agreement between the MRA and TCCS was assessed using kappa statistics. The sensitivity, specificity, and positive and negative predictive values of TCCS as compared to MRA were calculated.

Results: Ninety patients were included in the final analysis. Intracranial atherosclerosis was found in 35.6% of cases. The agreement between TCCS and MRA in detecting lesions for the different arterial segments in the intracranial circulation was 0.83 for anterior cerebral artery (ACA), 0.66 for M1 segment of middle cerebral artery (MCA), 0.45 for M2 segment of MCA, 0.86 for terminal internal carotid artery (TICA), 0.46 for posterior cerebral artery (PCA), and 0.81 for vertebral artery (VA). The sensitivity for the detection of hemodynamically significant arterial lesions in different vascular segments was 100%, 70%, 33.3%, 90.9%, 33.3%, and 72.7% for ACA, M1, M2, TICA, PCA, and VA, respectively.

Conclusion: Intracranial atherosclerosis was found to be the predominant distribution of cerebral atherosclerosis. TCCS is a safe method for evaluation of proximal basal cerebral arteries in the intracranial circulation with relatively better sensitivity in the anterior circulation.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.4103/jcis.JCIS_13_18DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5939038PMC
April 2018

The Spectrum of Autonomic Dysfunction in Myasthenic Crisis.

Ann Indian Acad Neurol 2018 Jan-Mar;21(1):42-48

Department of Neurology, Christian Medical College, Vellore, Tamil Nadu, India.

Background: Autoimmune autonomic dysfunction is described in Myasthenia Gravis. In myasthenic crisis, the spectrum of autonomic dysfunction is hitherto uncharacterized.

Objective: The objective of this study is to describe the spectrum of autonomic dysfunction in myasthenic crises using the composite autonomic symptom scale 31 (COMPASS 31) autonomic symptom questionnaire and power spectral analysis of heart rate variability (HRV), which is a simple way of estimating general autonomic dysfunction.

Methods: Adult patients with myasthenic crisis from January 1, 2014 to March 15, 2015, were prospectively included in this study. The COMPASS 31 questionnaire for symptoms of autonomic dysfunction and power spectral analysis of HRV were assessed. These were compared with the patient's demographic and clinical parameters and with previous literature. IRB approval was obtained.

Results: Sixteen patients were included (M:F 3:1). 15/16 patents (93%) had autonomic dysfunction on COMPASS 31 questionnaire. The domains of involvement were gastrointestinal (80%), orthostatic (67.7%), pupillomotor (67.7%); sudomotor (33.3%), and vasomotor (13.3%). Parasympathetic dysfunction predominance was suggested by the symptom profile. HRV analysis showed a low frequency (LF) spectral shift suggesting slowed parasympathetic responsiveness (LF normalized unit (nu): high frequency [HF] nu mean 8.35, standard deviation ± 5.4, 95% confidence interval 2.2-12.5), which significantly exceeded the mean LF nu: HF nu ratios of the majority of previously reported noncrises myasthenic populations.

Conclusions: Myasthenic crisis has autonomic dysfunction involving multiple organ systems. Increased latency of parasympathetic reflexes is suggested. A comprehensive management protocol addressing different autonomic domains is required for holistic patient care.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.4103/aian.AIAN_270_17DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5909145PMC
May 2018

Spectrum of Visual Impairment in Cerebral Venous Thrombosis: Importance of Tailoring Therapies Based on Pathophysiology.

Ann Indian Acad Neurol 2017 Jul-Sep;20(3):294-301

Department of Neurological Sciences, Christian Medical College and Hospital, Vellore, Tamil Nadu, India.

Visual impairment can complicate cerebral venous thrombosis (CVT). Here, we describe the various pathophysiological mechanisms and treatments available. A retrospective chart review of all patients treated for CVT in a large quaternary teaching hospital was done, and cases with visual impairment due to CVT were identified. The various mechanisms causing visual impairment in CVT were (1) raised intracranial pressure (ICP) caused by venous thrombosis without venous infarcts resulting in a benign intracranial hypertension-like presentation of CVT, (2) venous infarcts involving the occipital cortex, (3) raised ICP following the development of a secondary dural arteriovenous (AV) fistula, and (4) arterial occipital infarcts due to posterior cerebral artery compression secondary to herniation in large venous infarcts. Apart from using systemic anticoagulants to attempt recanalization and drugs with carbonic anhydrase inhibitor activity to reduce the ICPs, treatment modalities employed to save vision were (1) recanalization by local thrombolysis, stenting, or mechanical devices; (2) cerebrospinal fluid diversion procedures such as theco-periotoneal shunting; (3) optic nerve sheath fenestration; and (4) specific treatment for conditions such as dural AV fistula occurring as a late complication. CVT can cause visual impairment through different pathophysiological mechanisms. Depending on the mechanism, treatment strategies need to be tailored. Furthermore, very close monitoring is needed both in the acute and in the follow-up period, as new pathophysiological mechanisms can arise, compromising the vision. This may require a different treatment approach. Literature on this aspect of CVT is lacking.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.4103/aian.AIAN_11_17DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5586128PMC
September 2017

Clinical Spectrum, Therapeutic Outcomes, and Prognostic Predictors in Sjogren's Syndrome-associated Neuropathy.

Ann Indian Acad Neurol 2017 Jul-Sep;20(3):278-283

Department of Neurological Sciences, Christian Medical College, Vellore, Tamil Nadu, India.

Objectives: There are limited data regarding long-term follow-up and therapeutic outcomes in Sjogren's syndrome (SS)-associated peripheral neuropathy. In this study, we aim to study the clinical, electrophysiological spectrum and therapeutic responses among the different subtypes of SS-associated neuropathy. The predictors of suboptimal treatment response will be identified.

Methods: The study included a retrospective cohort of patients with SS-associated neuropathy between January 2012 and November 2015. Baseline clinical, laboratory, electrophysiological data and details of treatment were noted. Therapeutic outcomes were assessed at follow-up and compared among the different subtypes. Prognostic predictors were determined using logistic regression analysis.

Results: Fifty-four patients were included in the study. Sensory ataxic neuropathy (17, including 9 with sensory ganglionopathy) and radiculoneuropathy (11) were the main subtypes. Notable atypical presentations included acute neuropathies, pure motor neuropathies, and hypertrophic neuropathy. Concomitant autoimmune disorders were present in 24 (44.4%) patients. Most presentations were subacute-chronic (51, 94.4%). Minor salivary gland biopsy had a higher yield compared to serological markers (81.5 vs. 44.4%). Sensory ataxic neuropathy was associated with greater severity and autonomic dysfunction. Improvement was noted in 33 (61%) patients. Cranial neuropathy and radiculoneuropathy subtypes were associated with the best treatment responses. Chronicity, orthostatic hypotension, baseline severity, and marked axonopathy (nerve biopsy) were predictive of a suboptimal therapeutic response.

Conclusions: The study highlights the heterogeneous spectrum, atypical presentations, and differential therapeutic responses. SS-associated neuropathy remains underdiagnosed. Early diagnosis and prompt initiation of immunotherapy before worsening axonal degeneration is paramount. SS-associated neuropathy need not necessarily be associated with a poor prognosis.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.4103/aian.AIAN_116_17DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5586125PMC
September 2017
-->