Rangasetty Srinivasa

Hashimoto encephalopathy: A study of the clinical profile, radiological and electrophysiological correlation in a Tertiary Care Center in South India

Background: Hashimoto encephalopathy (HE) is a poorly understood and often misdiagnosed entity with variable clinical spectrum. There are many uncertainties that still remain about this condition and the pathological significance of thyroid peroxidase (TPO) antibody. Objective: To characterize the clinical, laboratory and radiologic findings in patients with HE. Design: Retrospective analysis of clinical features and diagnostic test data. Main Outcome Measures: Clinical features, laboratory, radiologic, electroencephalography (EEG) findings associated with HE and therapeutic outcome. Results: Thirteen consecutive patients were identified as having HE. The median age at onset was 48.5 years (range, 19–62 years). There was a female preponderance (76.9%). Clinical manifestations were cognitive impairment and behavioral changes in 10 (76.9%), sleep disturbance in 9 (69.2%), seizures in 6 (46.1%), headache in 4 (30.8%), psychosis or paranoia in 5 (38.5%), transient symptoms in 6 (46.1%), myoclonus in 4 (30.8%), ataxia or gait disorder in 4 (30.8%). The most frequent laboratory abnormalities were increased TPO (n = 13) in all cases, increased thyroid stimulating hormone levels (n = 6), and increased erythrocyte sedimentation rate (n = 5). The cerebrospinal fluid protein level was elevated in 8 of 9 patients (88.8%). Magnetic resonance imaging abnormalities were present in 2 patients (15.4%). EEG changes were seen in 7 patients (53.8%). All but two patients showed significant therapeutic benefit with steroids. Conclusions: HE has a wide range of clinical, laboratory, and radiologic findings. All patients with an unexplained encephalopathy should be screened for this condition as treatment response is excellent. To the best of our knowledge, this is the largest single center clinical series of HE from the Indian subcontinent.

A study of clinical profile, radiological and electroencephalographic characteristics of suspected Creutzfeldt-Jakob disease in a tertiary care centre in South India

Introduction: Creutzfeldt-Jakob disease (CJD) is a progressive, fatal, neurodegenerative disease classified under transmissible spongiform encephalopathies (TSE) or prion diseases. It is characterized by long asymptomatic period followed by rapid clinical deterioration leading to the death within months. The disease is still under-reported in India. Objective: The aim of this study was to describe the clinical, radiological and electroencephalographic characteristics of eight cases of CJD encountered in MS Ramaiah Medical college and Hospital, Bangalore over the past 3 years (2010-2013). This was retrospective, observational, hospital-based study. Results: The mean age of patients was 66.6 years (range: 54-82) and there was female predominance (five patients). The main clinical manifestations were cognitive disturbance (8/8) and myoclonus (8/8), followed by behavioral disturbance (5/8), ataxia (5/8) and extra-pyramidal symptoms/signs (4/8). Time interval (mean) between onset of disease to death was 6.6 months (range: 3-14). Brain MRI abnormalities were noted in 6 patients: Fluid-attenuated inversion recovery hyperintensities with restriction on diffusion-weighted image/apparent diffusion coefficient (DWI/ADC) in caudate and putamen, and diffusion hyperintensities without restriction on ADC in parieto-occipital, frontal and temporal regions. Classical electroencephalogram (EEG) changes of periodic triphasic waves were seen in 87% of patients. The CSF 14-3-3 protein assay was positive in two patients (out of four). Seven cases were probable CJD and one was possible CJD. Conclusion: A strong clinical suspicion aided by characteristic brain MRI and EEG abnormalities is essential for timely diagnosis of this fatal disease.

Spontaneous subdural hematoma and antiplatelet therapy: Does efficacy of Ticagrelor come with added risk?

Antiplatelet therapy has established clinical benefit on cardiovascular outcome and has reduced the rates of re-infarction/in stent thrombosis following percutaneous coronary intervention in acute coronary syndromes. Major bleeding episodes can occur with antiplatelet therapy and intracranial hemorrhage (ICH) is one of the most feared complications resulting in significant morbidity and mortality. Identification of high risk groups and judicious use of antiplatelet therapy reduces the bleeding risk. Ticagrelor is a newer P2Y12 receptor antagonist with established clinical benefit. However, risks of having an ICH with these newer molecules cannot be ignored. Here, we report a case of spontaneous acute subdural hematoma developing in a patient on antiplatelet therapy with aspirin and ticagrelor. Early recognition, discontinuation of the medication and appropriate management resulted in resolution of hematoma and good clinical outcome. Authors have reviewed the antithrombotic drugs and their tendencies in causing intracranial bleeds from a neurophysicians perspective.

Utility of cerebrospinal fluid cortisol level in acute bacterial meningitis.

Background: Meningitis remains a serious clinical problem in developing as well as developed countries. Delay in diagnosis and treatment results in significant morbidity and mortality. The role and levels of intrathecal endogenous cortisol is not known. Objective: To study the cerebrospinal fluid (CSF) cortisol levels and to evaluate its role as a diagnostic and therapeutic marker in acute bacterial meningitis. Materials and Methods: Thirty patients with acute bacterial meningitis with no prior treatment were evaluated. Cortisol levels were compared with 20 patients with aseptic (viral) meningitis and 25 control subjects. Results: Mean CSF cortisol level was 13.85, 3.47, and 1.05 in bacterial meningitis, aseptic meningitis, and controls, respectively. Mean CSF cortisol level in bacterial meningitis was significantly higher as compared to controls (P < 0.001). There was significant difference in CSFcortisol levels in bacterial and aseptic meningitis (P < 0.001). Conclusions: Cortisol levels in CSF are highly elevated in patients with acute bacterial meningitis. This suggests that intrathecalcortisol may serve as a valuable, rapid, relatively inexpensive diagnostic marker in discriminatingbetween bacterial and aseptic meningitis. This helps in earlier institution of appropriate treatment and thereby decreasing morbidity and mortality.

Intravenous thrombolysis for acute ischemic stroke: Review of 97 patients

Background: Intravenous thrombolysis (IVT) has now become a standard treatment in eligible patients with acute ischemic stroke (AIS) who present within 4.5 h of symptom onset. Objective: To determine the usefulness of IVT and the subset of patients who will benefit from IVT in AIS within 4.5 h. Materials and Methods: Patients with AIS within 4.5 h of symptom onset who underwent IVT were studied prospectively. The study period was from October 2011 to October 2015. Results: A total of 97 patients were thrombolysed intravenously. The mean onset to needle time in all patients was 177.2 ± 62 min (range: 60–360). At 3 months follow-up, favorable outcome was seen in 65 patients (67.1%) and poor outcome including death in the remaining 32 patients (32.9%). Factors predicting favorable outcome was age <65 years (P = 0.02), the National Institute of Health Stroke Scale (NIHSS) <15 (P < 0.001), small vessel occlusion (P = 0.006), cardioembolism (P = 0.006), and random blood sugar (RBS) <250 mg/dl (P < 0.001). Factors predicting poor outcome was diabetes mellitus (P = 0.01), dyslipidemia (P = 0.01), NIHSS at admission >15 (P = 0.03), RBS >250 mg/dl (P = 0.01), Dense cerebral artery sign, age, glucose level on admission, onset-to-treatment time, NIHSS on admission score >5 (P = 0.03), and occlusion of large artery (P = 0.02). Conclusion: Milder baseline stroke severity, blood glucose <250 mg/dL, younger patients (<65 years), cardioembolic stroke, and small vessel occlusion benefit from recombinant tissue plasminogen activator.

Use of multiplex PCR based molecular diagnostics in diagnosis of suspected CNS infections in tertiary care setting—A retrospective study

OBJECTIVES CNS infections like meningitis and encephalitis pose enormous healthcare challenges due to mortality, sequelae and socioeconomic burden. In tertiary setting, clinical, microbiological, cytological and radiological investigations are not distinctive enough for diagnosing microbial etiology. Molecular diagnostics is filling this gap. We evaluated the clinical impact of a commercially available multiplex molecular diagnostic system - SES for diagnosing suspected CNS infections. PATIENTS AND METHODS This study was conducted in our tertiary level Neurology ICU. 110 patients admitted during Nov-2010 to April-2014 were included. CSF samples of patients clinically suspected of having CNS infections were subjected to routine investigation in our laboratory and SES test at XCyton Diagnostics. We studied the impact of SES in diagnosis of CNS infections and its efficacy in helping therapeutic management. RESULTS SES showed detection rate of 42.18% and clinical specificity of 100%. It had 10 times higher detection rate than conventional tests. Streptococcus pneumoniae and Mycobacterium tuberculosis were two top bacterial pathogens. VZV was most detected viral pathogen. SES results elicited changes in therapy in both positive and negative cases. We observed superior patient outcomes as measured by GCS scale. 75% and 82.14% of the patients positive and negative on SES respectively, recovered fully. CONCLUSION Detecting causative organism and ruling out infectious etiology remain the most critical aspect for management and prognosis of patients with suspected CNS infections. In this study, we observed higher detection rate of pathogens, target specific escalation and evidence based de-escalation of antimicrobials using SES. Institution of appropriate therapy helped reduce unnecessary use of antimicrobials.

Bilateral cerebral hemorrhage in herpes simplex encephalitis: Rare occurrence

Journal of Neurosciences in Rural Practice ¦ Volume 7 ¦ Supplement 1 ¦ December 2016 and leg pain caused by lumbar annular tears. Zhonghua Yi Xue Za Zhi 2010;90:3198‐202. 4. Hancock M, Maher C, Macaskill P, Latimer J, Kos W, Pik J. MRI findings are more common in selected patients with acute low back pain than controls? Eur Spine J 2012;21:240‐6. 5. Dongfeng R, Hou S, Wu W, Wang H, Shang W, Tang J, et al. The expression of tumor necrosis factor‐a and CD68 in high‐intensity zone of lumbar intervertebral disc on magnetic resonance image in the patients with low back pain. Spine (Phila Pa 1976) 2011;36:E429‐33. 6. Moon HJ, Kim JH, Lee HS, Chotai S, Kang JD, Suh JK, et al. Annulus fibrosus cells interact with neuron‐like cells to modulate production of growth factors and cytokines in symptomatic disc degeneration. Spine (Phila Pa 1976) 2012;37:2‐9. 7. Peng B, Hou S, Wu W, Zhang C, Yang Y. The pathogenesis and clinical significance of a high‐intensity zone (HIZ) of lumbar intervertebral disc on MR imaging in the patient with discogenic low back pain. Eur Spine J 2006;15:583‐7. Access this article online

Parainfectious ocular flutter and truncal ataxia in association with dengue fever

Ocular flutter is an eye movement disorder characterized by purely horizontal rapid saccadic oscillations lasting for a few minutes which stops spontaneously. Postinfectious ocular flutter and truncal ataxia are a rare entity. There are reported cases of opsoclonus myoclonus ataxia in association with dengue virus infection. However, there are no reported cases of parainfectious ocular flutter and truncal ataxia in association with dengue virus infection. Hereby, we report a child with dengue fever who had ocular flutter and truncal ataxia.

Acute generalized chorea as presenting manifestation of uremic encephalopathy

1 year on medical management. On examination, his tongue was dry, pulse rate of 102/min, blood pressure of 146/86 mmHg, and respiratory rate of 20 breaths/min. Neurologically, he was conscious and responding to verbal commands with mild slurred speech. Fundus examination was normal. There was generalized choreiform movement involving face and all limbs. Asterixis could not be made out due to choreiform movements of limbs. Tone in limbs was decreased with sluggish reflexes. Plantar response was withdrawal. There was no neck rigidity. Complete hemogram showed raised total leukocyte counts (16,000 cells per cumm), normal platelet count, and hemoglobin. Serum electrolytes, random blood glucose, serum ammonia, and liver and thyroid function test were normal. Blood urea nitrogen was 76.4 mg/dl, creatinine of 6.2 mg/dl, and glycosylated hemoglobin of 8.2%. Blood gas analysis showed pH: 7.314, bicarbonate: 17.6 mmol/L, and lactate: 2.1 mmol/L. Brain MRI showed hypointense on T1 and hyperintense lesions on T2 and fluid‐attenuated inversion recovery sequences in bilateral basal ganglia. Diffusion‐weighted imaging showed no restriction in bilateral putamen [Figure 1]. Electroencephalogram showed mild slowing of background rhythm. He was started on sodium valproate (1 g/day) but had no improvement. He Sir, Renal dysfunction results in a clinical metabolic condition known as uremia. It causes altered mental status due to involvement of cerebral cortex termed as uremic encephalopathy. Acute hyperkinetic or hypokinetic extrapyramidal disorder in patients with uremia is a very rare syndrome. It was first described by Wang et al. due to bilateral basal ganglia lesions in uremia.[1] Hereby, we report an elderly patient with diabetic nephropathy who presented with acute‐onset generalized chorea. Brain magnetic resonance imaging (MRI) showed bilateral basal ganglia lesions. Choreiform movements got ameliorated with hemodialysis.

Spinocerebellar ataxia type 7 sans retinal degeneration: A phenotypic variability

Autosomal dominant cerebellar ataxia (ADCA) was classified into Type I, Type II, and Type III, based on the clinical phenotypes by Harding. ADCA Type I presents with both cerebellar and noncerebellar signs and includes SCA1–SCA4, SCA8, SCA10, SCA12-SCA23, SCA25, SCA27, SCA28, and SCA32–SCA36. ADCA Type II consists of syndromes in association with pigmentary retinopathies and includes SCA7. ADCA Type III includes mostly pure cerebellar syndromes and includes SCA5, SCA6, SCA11, SCA26, SCA30, and SCA31.[2] Now, ADCA has been replaced by SCA. SCA7 belongs to ADCA Type II phenotype. SCA7 is caused by the expansion of CAG trinucleotide repeats in exon 3 of ATXN7 gene on the chromosome 3p12–21.1. The gene encodes for the protein ataxin-7. Ataxin-7 is an 892-amino acid nuclear protein of unknown function. The polyglutamine expansion in the N-terminal segment of ataxin-7 exerts a toxic effect on the cerebellar Purkinje cells. In the healthy individuals, CAG repeats range from 4 to 19. The repeat length of 28–33 is regarded as mutable normal alleles. Repeats of 34 and 35 are alleles with reduced penetrance. Repeats above 37 are the pathogenic full-penetrance alleles.[3] The prevalence of SCA7 has been reported to be the highest in South Africa and Scandinavian region. The age of onset can be early (<25 years) or late (>40 years) onset. The phenotypic abnormalities are dependent on the repeat lengths. Cerebellar ataxia is seen in all age onsets. Retinal degeneration precedes cerebellar ataxia with higher repeat length and in early onset and vice versa with lower repeat sizes and in late onset. This is due to early-onset toxicity in the retinal tissue than in cerebellum from higher length of a pathogenic polyglutamine stretch in ataxin-7 protein.