Browsing by Author "Nair, Muralidharan"

Now showing 1 - 7 of 7
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    A 6-year-old girl with progressive ataxia
    (JOURNAL OF CLINICAL NEUROSCIENCE, 2012)
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    Evolution and long term outcome in patients presenting with large demyelinating lesions as their first clinical event
    (JOURNAL OF THE NEUROLOGICAL SCIENCES, 2010)
    Objectives: To describe the long term evolution and outcome of a homogeneous cohort of patients (n = 14) with large demyelinating lesions (LDLs) as the first clinical event from a group of idiopathic inflammatory demyelinating diseases of central nervous system.Methods: Detailed review of LDLs from December 2002-January 2007 was made. Patients had at least two magnetic resonance imaging (MRI) and minimum follow-up of 2 years. The disability was assessed using Estimated Expanded Disability Status Scale (EDSS) and Rappaport Disability Rating Scale (DRS) at onset and last follow-up.Results: Fourteen consecutive LDL patients (male = 7), with mean age 32.7 years and mean follow-up of 45.5 months were included. Motor deficits (79%) and cognitive symptoms (43%) marked the onset, none had optic neuritis. All except two responded to steroids. Follow-up MRI showed complete resolution in 43%, 57% showing marked reduction in size. On follow-up, 2 relapsed. Mean EDSS and DRS at presentation were 5.93 and 9.07 and at last follow-up were 1.75 and 2.25 (p<0.001).Conclusions: Our data suggests that, patients presenting with LDLs as their first clinical event behaves distinctly in their presentation, imaging characteristics, prognosis and long term outcome as compared to MS and ADEM. Albeit significant disability at the onset, these patients show an excellent response to treatment with good functional recovery in long term and rare relapses. (C) 2010 Elsevier B.V. All rights reserved.
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    Extraoccipital photoparoxysmal response in a case of focal encephalitis
    (EPILEPTIC DISORDERS, 2011)
    Photoparoxysmal response (PPR) is commonly associated with idiopathic generalised epilepsies. Most of the focal events induced by intermittent photic stimulation (IPS) are reported to be of occipital origin. Only six temporal lobe epilepsy patients have been reported in the literature with focal PPR at extraoccipital sites. We report a four-year-old girl with possible encephalitis who presented initially with epilepsia partialis continua of limbs on the right side. Interictally, she had left centro-parietal periodic lateralized epileptiform discharges (PLEDs). She responded to medical treatment and was free of seizures and motor and cognitive deficits at six months follow-up. Repeat EEG at follow-up showed left centro-parietal spikes accentuated by IPS. This is the first report of an extraoccipital, extratemporal focus showing PPR. The possible mechanism of PPR from this fronto-parietal epileptogenic focus is discussed.
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    Hypertrophic pachymeningitis in a patient with Takayasu arteritis: One more association
    (ANNALS OF INDIAN ACADEMY OF NEUROLOGY, 2012)
    Hypertrophic pachymeningitis (HP) is a rare chronic inflammatory disease of the dura mater, described in association with various infections, systemic vasculitides such as Wegeners granulomatosis and giant cell arteritis. However, HP in association with Takayasu arteritis (TA) has not been described. We report a young woman who presented with headache, seizures, and right third and fourth cranial neuropathy. Magnetic resonance imaging of the brain showed HP in bifrontal and right temporal region extending to cavernous sinus. She was also found to have systemic hypertension, stenosis of left subclavian, and left renal artery with narrowing of abdominal aorta, satisfying the diagnostic criteria for TA. A detailed evaluation for secondary causes of HP failed to reveal an alternative etiology. This report describes an unusual association of HP in a patient with TA, also emphasizing that seizures and cranial neuropathy may further expand the spectrum of neurological manifestations in patients with TA.
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    Late-onset Boucher-Neuhauser Syndrome (late BNS) associated with white-matter changes: a report of two cases and review of literature
    (JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY, 2011)
    Boucher-Neuhauser syndrome (BNS) is rare autosomal recessive disease, characterised by cerebellar ataxia, hypogonadotropic hypogonadism and chorio-retinal degeneration. The authors report a family (brother, 22 years and sister 24 years) with late-onset BNS (> 10 years). They had subnormal intelligence; the cerebellar ataxia was progressive over 2 years with early functional dependence. Puberty was attained in a brother with testosterone injections, while the girl had primary amenorrhoea. There were no associated visual complaints. They both had diffuse periventricular white-matter hyperintensities in cerebral cortex and diffuse cerebellar atrophy in the MRI.
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