Browsing by Author "Nair, MD"

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    A novel association of ocular neuromyotonia with brainstem demyelination: Two case reports
    (MULTIPLE SCLEROSIS JOURNAL, 2014) Menon, D; Sreedharan, SE; Gupta, M; Nair, MD
    Ocular neuromyotonia (ONM) is a rare disorder of ocular mal-alignment in which painless, transient spontaneous or gaze-induced abnormal deviation of the eye manifests as episodic diplopia. With only a few cases reported in the literature, ONM mostly follows months to years after cranial irradiation for sellar or suprasellar lesions. Here we present two patients with this rare ocular condition, secondary to brainstem demyelination, the association of which is hitherto unreported in the literature. Both patients were 15-year-old girls who presented to us with episodic forced-eye deviation with diplopia. Examination during these attacks revealed ONM involving the superior rectus and medial rectus in the first and second patient, respectively. There was clinical evidence of intrinsic brainstem involvement with downbeat nystagmus and skew deviation in one patient without any other cerebellar or long tract signs. MRI showed evidence of demyelination involving the brainstem in both, with CSF showing positive immunological markers and with positive aquaporin-4 antibody in one patient. Both patients responded remarkably to immunomodulatory therapy and are asymptomatic at follow-up. That ONM can occur with brainstem demyelination has not been reported in the literature. This association may help in explaining the pathophysiology of ONM as secondary to segmental demyelination.
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    Axillary fold and scapular hump in spinal accessory nerve injury
    (NEUROLOGY INDIA, 2012) Jagtap, SA; Soni, H; Nair, MD
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    Bilateral perisylvian infarct: a rare cause and a rare occurrence
    (SINGAPORE MEDICAL JOURNAL, 2011) Singh, A; Kate, MP; Nair, MD; Kesavadas, C; Kapilamoorthy, TR
    Foix-Chavany-Marie opercular syndrome is a severe form of pseudobulbar palsy occurring due to bilateral anterior opercular lesions. We report a case of a 51-year-old man with sudden onset of inability to speak and dysphagia, and a history of synovial sarcoma of the right hand. Detailed language evaluation was normal. The patient had right upper motor neuron facial paresis and absent gag reflex bilaterally. Magnetic resonance (MR) imaging revealed acute and subacute infarcts involving the bilateral insular cortex. Two-dimensional echocardiography and cardiac MR imaging showed a mobile mass in the left atrium attached to the interatrial septum, which was likely a myxoma. Chest radiograph and computed tomography imaging of the chest revealed multiple cannonball shadows that were suggestive of secondaries in the lung. The probable cause of the cerebral lesions was the mass lesion in the heart or metastatic lesions from the synovial sarcoma. The cardiac surgeon and surgical oncologist recommended palliative care.
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    Carotid artery stenting: Results and long-term follow-up
    (NEUROLOGY INDIA, 2006)
    Background and Purpose: The role of carotid artery stenting (CAS) as an alternative to carotid endarterectomy in the treatment of for symptomatic carotid artery stenosis is investigated. Materials and Methods: Forty-seven patients underwent CAS over 10-year period. Forty-nine vessels were treated. Stenosis quantification was done using North American symptomatic carotid endarterectomy trial method. The mean follow-up period by clinical and Duplex examination ranged is 5.6 years. Results: The technical success rate was 100%. There were four deaths (8.1%) and two (4.1%) minor strokes within thirty days of procedure. There was no major strokes. All patients with minor stroke achieved complete recovery at 1-month follow up. Two deaths occurred probably due to hyperperfusion syndrome (HS) and two due to cardiac arrest. Conclusion: CAS is an effective treatment modality of symptomatic carotid artery disease but should be carefully done in high-risk groups having severe medical ailments and those having severe bilateral stenosis of the carotid arteries.
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    Circulating tumour necrosis factor alpha & soluble TNF receptors in patients with Guillain-Barre syndrome
    (INDIAN JOURNAL OF MEDICAL RESEARCH, 2003)
    Background & objectives: Tumour necrosis factor-alpha (TNF-alpha) is regarded as one of the immune factors that can induce demyelination of peripheral nerves in patients with Guillian-Barre syndrome (GBS). This present study was undertaken to find out the role of TNF-alpha and soluble TNF receptors in the pathogenesis of GBS; and to study the effect of intravenous immunoglobulin (ivlg) therapy on the serum TNF-a and soluble TNF receptors in patients with GBS.Methods: Thirty six patients with GBS in progressive stages of motor weakness were included in this study. The serum TNF-alpha and soluble TNF receptors (TNF-RI, TNF-RII) were measured in the serum samples of these patients before and after ivIg therapy by a sandwich ELISA.Results: Of the 36 patients with GBS, 26 (72.2%) showed elevated serum TNF-alpha levels prior to ivIg therapy. Following a complete course of ivIg therapy there was a progressive decrease in the serum TNF-alpha concentrations in these 26 patients. On the other hand, the soluble TNF receptors, particularly TNF-RII showed an increase in the serum of GBS patients following ivIg therapy.Interpretation & conclusion: The results indicate that ivIg reduces the serum TNF-alpha. concentrations in the GBS patients having elevated levels prior to ivlg therapy. Elevated serum levels of soluble TNF receptors following ivIg therapy may play a protective role by inhibiting the demyelinating effect of TNF-alpha in the peripheral nerves of patients with GBS.
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    Congenital myasthenic syndromes: Natural history and long-term prognosis
    (ANNALS OF INDIAN ACADEMY OF NEUROLOGY, 2013) Jagtap, SA; Abraham, K; Sarada, C; Nair, MD
    Introduction: Congenital myasthenia syndrome (CMS) is a rare, heterogeneous group of genetically determined, disorder of neuromuscular transmission. They have a varied presentation and progression and very few studies have addressed the natural history. Aim of the present study is to describe the clinical profile and natural history of patients with CMS. Materials and Methods: Study includes patients with CMS who attended comprehensive-neuromuscular-clinic (CNMC) during the period January, 2000-2008 with a minimum follow-up of 2 years, with inclusion criteria: (1) Onset in infancy or childhood with fluctuating ocular, bulbar, respiratory or limb muscle weakness (2) Acetylcholine receptor antibody negative (3) normal computed tomography (CT) thymus (4) Abnormal repetitive nerve stimulation (RNS) testing (5) Exclusion of other autoimmune disorders. Results: Out of 314 patients with myasthenia who attended the CNMC during study period, 15 (4.8%) were with CMS (8 boys, 7 girls). Patients were divided as infantile and childhood onset. The mean age of onset and diagnosis in infantile and childhood onset groups were 5.5 months/3.1 years and 3.6 years/6.5 years respectively. Eleven patients had ptosis and 4 had generalized presentation. Most common site of decremental response was over facial nerve in 12 (75%) patients. All patients showed good response to treatment with acetyl cholinesterase inhibitor with stable course on follow-up without exacerbations. Mean dose for neostigmine was 28 mg/day and for pyridostigmine was 153 mg/day. Conclusion: Ptosis is most common symptom at onset in CMS, emphasing importance of RNS of the facial nerve, in the absence of molecular diagnosis of CMS. Our CMS cohort had relatively stable course without intermittent exacerbations with fair response to acetyl cholinesterase inhibitor.
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    Intractable nausea and vomiting as presenting manifestation of neuromyelitis optica
    (ANNALS OF INDIAN ACADEMY OF NEUROLOGY, 2013) Jagtap, SA; Sarathchandran, P; Kambale, HJ; Nair, MD; Sarada, C
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    Intravenous immunoglobulin reduces serum tumor necrosis factor alpha in patients with Guillain-Barre Syndrome
    (NEUROLOGY INDIA, 2003)
    Background: Tumor necrosis factor a TNF-alpha has a possible role in the pathogenesis of the Guillain-Barre'syndrome (GBS). Alms: To study the effect of intravenous immunoglobulin (IVIg) on serum TNF-alpha concentrations in patients with GBS. Material and Methods: The effect of IVIg on TNF-alpha was evaluated in 36 patients with GBS. Serum TNF-alpha concentration was measured by enzyme-linked immunosorbent assay (ELISA). The sera of 22 (61%) patients with GBS showed elevated concentrations of TNFalpha (35-182 pg/ml) and these sera were individually incubated in vitro with IVIg (0.25mg/ml) at 370 degreesC for 24 hours. Results: The serum TNF-alpha concentrations in the 22 GBS patients with elevated levels showed a steady decline (60.34-19.78 pg/ml) following incubation with IVIg. These 22 patients also received IVIg therapy, and serum TNF-alpha concentrations showed a significant decline (65.5-9.75 pg/ml) at the end of the therapy. At the time of discharge from the hospital, there was a positive correlation between neurological recovery and decline in TNF-alpha concentrations in these 22 GBS patients. Conclusions: The results of this study indicate that elevated levels of TNF-alpha occur in a proportion of patients with GBS and in these patients elevated serum TNF-alpha levels decline with IVIg therapy.
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    Limbic encephalitis: Clinical spectrum and long-term outcome from a developing country perspective
    (ANNALS OF INDIAN ACADEMY OF NEUROLOGY, 2014) Jagtap, SA; Das, GK; Kambale, HJ; Radhakrishnan, A; Nair, MD
    Introduction: Limbic encephalitis (LE) is characterized by rapidly progressive short-term memory loss, psychiatric symptoms and seizures. We describe the clinical spectrum, underlying etiology and long-term follow-up of patients with LE from India. Materials and Methods: This prospective study included patients during the period of January 2009 and December 2011 with the clinical features consistent with LE with one or more of the following: (1) Magnetic resonance imaging (MRI) evidence of temporal lobe involvement; (2) cerebrospinal fluid inflammatory abnormalities, or (3) detection of antineuronal antibodies. Patients with metastasis, infection, metabolic and nutritional deficits, stroke, were excluded. Results: There were 16 patients (9 females), mean age of presentation was 36.6 years (range 15-69 years). The mean duration of symptoms before presentation was 11 months (range 5 days-2 years). The most common symptom at presentation was short-term memory impairment in 7 patients followed by seizures in 5 and behavioral changes in three. Nine patients had seizures, 11 had change in behavior, language involvement in eight, cerebellar features in 3 and autonomic dysfunction in two. Four patients had associated malignancy, 3 of four presented with neurological symptoms and on investigations found to be have malignancy. Antineuronal antibody testing was done in 6 of 12 non paraneoplastic and two paraneoplastic patients, one positive for N-methyl-D-aspartate and one for anti-Hu antibody. MRI brain showed typical fluid attenuated inversion recovery or T2 bilateral temporal lobe hyperintensities in 50% of patients. At a mean follow-up of 21 months (3-36 months), 10 patients improved, 4 patients remained same and two patients expired. Conclusion: Early recognition of LE is important based upon clinical, MRI data in the absence of antineuronal surface antibody screen in developing nations. Early institution of immunotherapy will help in improvement in outcome of these patients in long-term.
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    Morbidity predictors in ischemic stroke. (vol 51, pg 49,2003)
    (NEUROLOGY INDIA, 2003) Panicker, JN; Thomas, M; Pavithran, K; Nair, MD; Sarma, PS
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    Multipoint incremental motor unit number estimation versus amyotrophic lateral sclerosis functional rating scale and the medical research council sum score as an outcome measure in amyotrophic lateral
    (Annals of Indian Academy of Neurology, 2014-12) Jagtap, SA; Kuruvilla, A; Govind, P; Nair, MD; Sarada, C; Varma, RP
    INTRODUCTION: Monitoring the disease progression in amyotrophic lateral sclerosis (ALS) is a challenge due to different rates of progression between patients. Besides clinical methods to monitor disease progression, such as the ALS functional rating scale (ALSFRS) and the medical research council (MRC) sum score, quantitative methods like motor unit number estimation (MUNE) are of interest. OBJECTIVE: The objective of the present study is to evaluate the rate of progression in ALS using multipoint incremental MUNE and to compare MUNE, ALSFRS and MRC sum score at baseline and at 6 months for progression of the disease. MATERIALS AND METHODS: Multipoint incremental MUNE using median nerve, ALS-FRS and MRC sum score was carried out in 29 ALS patients at baseline and then at 6 months. RESULTS: Of the 29 ALS patients studied, the mean MUNE at baseline was 21.80 (standard deviation [SD]: 19.46, range 4-73), 15.9 in the spinal onset group (SD: 14.60) and 30.16 (SD: 22.89) in the bulbar onset group. Spinal onset patients had 74.02% of baseline MUNE value while bulbar onset patients had only 24.74% baseline value MUNE at 6 months follow-up (Unpaired t-test, P = 0.001). ALSFRS and MRC sum score showed statistically significant decline (P < 0.001) at 6 months follow-up. MUNE had the highest sensitivity for progression of the disease when compared to the ALS FRS and MRC sum score. CONCLUSION: Multipoint incremental MUNE is a valuable tool for outcome measure in ALS and other diseases characterized by motor unit loss. The rate of decline of multipoint incremental MUNE is more sensitive than that of MRC sum score and ALSFRS-R, when expressed as the percentage change from baseline
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    Multipoint incremental motor unit number estimation versus amyotrophic lateral sclerosis functional rating scale and the medical research council sum score as an outcome measure in amyotrophic lateral sclerosis
    (ANNALS OF INDIAN ACADEMY OF NEUROLOGY, 2014) Jagtap, SA; Kuruvilla, A; Govind, P; Nair, MD; Sarada, C; Varma, RP
    Introduction: Monitoring the disease progression in amyotrophic lateral sclerosis (ALS) is a challenge due to different rates of progression between patients. Besides clinical methods to monitor disease progression, such as the ALS functional rating scale (ALSFRS) and the medical research council (MRC) sum score, quantitative methods like motor unit number estimation (MUNE) are of interest. Objective: The objective of the present study is to evaluate the rate of progression in ALS using multipoint incremental MUNE and to compare MUNE, ALSFRS and MRC sum score at baseline and at 6 months for progression of the disease. Materials and Methods: Multipoint incremental MUNE using median nerve, ALS-FRS and MRC sum score was carried out in 29 ALS patients at baseline and then at 6 months. Results: Of the 29 ALS patients studied, the mean MUNE at baseline was 21.80 (standard deviation [SD]: 19.46, range 4-73), 15.9 in the spinal onset group (SD: 14.60) and 30.16 (SD: 22.89) in the bulbar onset group. Spinal onset patients had 74.02% of baseline MUNE value while bulbar onset patients had only 24.74% baseline value MUNE at 6 months follow-up (Unpaired t-test, P = 0.001). ALSFRS and MRC sum score showed statistically significant decline (P < 0.001) at 6 months follow-up. MUNE had the highest sensitivity for progression of the disease when compared to the ALS FRS and MRC sum score. Conclusion: Multipoint incremental MUNE is a valuable tool for outcome measure in ALS and other diseases characterized by motor unit loss. The rate of decline of multipoint incremental MUNE is more sensitive than that of MRC sum score and ALSFRS-R, when expressed as the percentage change from baseline.
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    Predicting long-term morbidity in Indian patients with ische mic stroke - Reply
    (NEUROLOGY INDIA, 2003) Panicker, JN; Thomas, M; Pavithran, K; Nair, MD; Sarma, PS
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    Response to thymectomy in South Indian patients with myasthenia gravis
    (ACTA NEUROLOGICA SCANDINAVICA, 1996)
    Aims - The rate of remission among patients with myasthenia gravis (MG) following thymectomy and the predictors of the outcome have revealed vast variation in studies from different geographic regions raising suspicion about the influence of ethnic factors. Material & methods - We retrospectively evaluated the outcome of 71 South Indian MG patients who were thymectomized between 1987 through 1993 and analyzed the relationship between clinical and histopathological features and postthymectomy outcome. Results - The clinical severity of the disease did not differ between the 29 patients with and 42 patients without a thymoma. Seventynine percent of our patients responded favourably to thymectomy; without additional immunosupression therapy, 52% achieved a near-complete remission. An younger age and milder disease correlated with a good outcome. Patients with thymoma responded as favourably as those without a thymoma. Conclusions - The postthymectomy response of South Indian MG patients in general did not differ from that of Western and Oriental patients.
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    Satoyoshi syndrome
    (NEUROLOGY INDIA, 2004)
    Satoyoshi syndrome (Komuragaeri disease) is a rare disorder of presumed autoimmune etiology, characterized by painful muscle spasms, alopecia, diarrhea, endocrinopathy with amenorrhoea and secondary skeletal abnormalities. Most of the previous reports are of the Japanese people. We report the first case from India.
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    Satoyoshi syndrome: Comments - Reply
    (NEUROLOGY INDIA, 2004) Ashalatha, R; Kishore, A; Sarada, C; Nair, MD
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    Serum tumor necrosis factor-alpha in Guillain-Barre syndrome and its relation to plasma exchange
    (NEUROLOGIST, 2002)
    BACKGROUND- To correlate the serum tumor necrosis factor-alpha (TNFalpha) concentrations before, during and following plasma exchange in patients with Guillain-Barre syndrome (GBS). In this prospective study, 21 GBS patients were selected. Patients in clinical stages III to V were subjected to plasma exchange. The control group included equal numbers of age-matched patients with other neurological diseases and healthy voluntary blood donors. A sandwich ELISA method was applied to estimate serum TNFalpha concentrations in test and control groups.REVIEW SUMMARY-Twelve GBS patients had elevated serum TNFalpha levels that ranged between 74 and 182 pg/mL. All 12 GBS patients showed a steady decrease in the TNFalpha concentration following plasma exchange and also showed a positive correlation with neurological recovery.CONCLUSIONS- We conclude that serum TNFalpha concentrations are elevated in 57.1 % of GBS patients and TNFalpha level decreases following plasma exchange.
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    Serum tumour necrosis factor-alpha and soluble tumour necrosis factor receptors levels in patients with Guillain-Barre syndrome
    (ACTA NEUROLOGICA SCANDINAVICA, 2004)
    Objectives - To estimate the serum concentrations of Tumour Necrosis Factor alpha (TNF-alpha) and soluble TNF receptors (s TNF-RI and TNF-RII) in patients with Guillain-Barre syndrome (GBS), before and after treatment. Material and methods - The serum TNF-alpha and the soluble TNF receptors concentrations were measured by a sandwich enzyme-linked immunosorbent assay (ELISA) in 47 patients with GBS before and after the treatment - IVI therapy (n = 26); Plasma Exchange (n = 21). Results- At the time of admission, the serum TNF-alpha concentrations were elevated (32.5-182.5 pg/ml) in 41/47 GBS patients (87.2%). Following the treatment (IVIG or PE), there was a significant decrease in the serum TNF-alpha concentrations (8.5-58.5 pg/ml) in these 41 GBS patients. The soluble TNF receptors, particularly sTNF-RII concentrations were significantly increased in GBS patients treated by IVIG therapy. Conclusions - The results of this indicated that (a) Elevated serum concentrations of TNF-alpha showed a positive correlation with the disease severity in patients with GBS. (b) The decrease in the serum TNF-alpha and increase in the serum soluble TNF receptors, particularly sTNF-RII showed a positive correlation with the neurological recovery in GBB patients following treatment.
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    Significance of serum antibody to GD1b ganglioside in patients with Guillain-Barre syndrome
    (INDIAN JOURNAL OF MEDICAL RESEARCH, 2001)
    Background & objectives: The precise etiological factors in Guillain-Barre syndrome (GBS) are still unknown. However, humoral and cellular immune factors may have a role in the pathogenesis of GBS. The present study was undertaken to evaluate the clinical significance of circulating serum IgG antibody to GD1b ganglioside in patients with GBS.Methods: Serial samples of serum were collected from 18 patients with GBS undergoing plasma exchange (PE) during their hospital stay. Serum IgG antibody titers to GD1b, before, during as well as following PE were measured by an indirect enzyme-linked immunosorbent assay (ELISA).Results: In 10 of 18 patients with GBS the antibody to GD1b was present in high titers (1:640-1:5120) prior to PE and the antibody titers in these 10 patients decreased following PE. At the time of completion of the study, the anti GD1b antibody titers declined in relation to clinical recovery in 7 of 10 patients with GBS.Interpretation & conclusion: The findings of the present study show that antibody to GD1b gangliosides may be one of the immunological factors in the pathogenesis of GBS land PE decreases the anti GD1b antibody titers in these patients.
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    Subacute sclerosing panencephalitis: A clinical appraisal
    (ANNALS OF INDIAN ACADEMY OF NEUROLOGY, 2013) Jagtap, SA; Nair, MD; Kambale, HJ
    Introduction: Subacute sclerosing panencephalitis (SSPE) is a rare chronic, progressive encephalitis affecting primarily children and young adults, caused by a persistent infection of immune resistant measles virus. The aim of the present study is to describe the clinical profile and natural history of patients with SSPE. Methods: We collected data of patients with SSPE during 2004-2010 who fulfilled Dyken's criteria. We analyzed demographical, clinical, electrophysiological, and imaging features. Results: Study included 34 patients, 26 (76.5%) males with age of onset from 3 to 31 years. Twenty one patients were below 15 years of age formed childhood SSPE and 13 above 15 years of age constituted adult onset group. 85.3% had low-socioeconomic status. Eleven received measles vaccination and seven were unvaccinated. 59.9% patients had measles history. Most common presenting symptom was scholastic backwardness (52.5%) followed by seizures (23.5%). Three patients each had cortical blindness, macular degeneration, decreased visual acuity, and optic atrophy. Electroencephalographic (EEG) showed long interval periodic complexes and cerebrospinal fluid anti-measles antibody was positive in all. Magnetic resonance imaging was done in 70.5% with was abnormal in 52.5%. Mean incubation period of SSPE after measles was 9.6 years. The follow-up duration was 1-10 years, (average of 2 years). Only one patient died from available data of follow-up, 9 were stable and 10 deteriorated in the form of progression of staging. Conclusion: SSPE is common in low-socioeconomic status. The profile of adult onset did not differ from childhood onset SSPE, except for a longer interval between measles infection and presence of the ophthalmic symptom as presenting feature in adult onset group.