Poster Session : PS 0128 ; Cardiology : Case Report - An Unusual Association of Mitral Stenosis with Diffuse Systemic Sclerosis

( Girish Ramteke ),( Sanjay Dubey ),( Neeraj Dayani ),( Mridulata Prajapati ),( Paridhi Shivde ) 대한내과학회 2014 대한내과학회 추계학술대회 Vol.2014 No.1

Introduction: Disorders of collagen tissue are caused by humoral and cellular mediated immune responses mediated by cytokines like TGF ß, TNF a etc. The various disease manifestations include mixed connective tissue diseases, rheumatoid arthritis, sjogren`s syndrome, systemic sclerosis etc. One of the associations is that of cardiac valvesstenotic lesion-though extremely rare. We report one such rare case. Case presentation: 60 year old female presented with a history of tightness of skin, contractures of fingers, inability to open mouth and pitting scars over the tip of digits. There was no history of breathlessness. There is a history of rheumatic heart disease-mitral stenosis-open mitral valvulotomy 7 years ago. On evaluation she had features suggestive of Mauskopf`s facies, microstomia and other features of systemic sclerosis and the ACR criteria suggested systemic sclerosis. Modified Rodnan skin thickness score was 43 out of 51. Her Antiscl-70 antibody was positive and skin biopsy showed dermal sclerosis. HRCT chest showed- septal thickening with ground glass appearance. Though the patient was asymptomatic her echo revealed chordal shortening, cuspal retraction which was an additional feature compared to earlier echo 7 years ago. This association of valvular involvement in systemic sclerosis is an extremely rare association with only 5 cases reported in literature. Conclusion: Association of valve- stenotic has been very rarely associated with collagen disease like systemic sclerosis; though other collagenous tissues of the body are involved cardiac valves are generally spared. With only 5 cases reported in the literature we report one such case of this rare association where in an initially affected and treated mitral valve due to rheumatic heart disease has been now involved due to systemic sclerosis.

Poster Session : PS 0609 ; Pulmonology ; Reversible Cerebellar Ataxia Following Prolonged Treatment with Metronidazole

( Vikas Asati ),( Girish Bhageshwar Ramteke ),( Ved Prakesh Pandey ),( Ajay Deep Bhatnagar ),( Atul Shende ),( Karuna Mujalda ) 대한내과학회 2014 대한내과학회 추계학술대회 Vol.2014 No.1

Metronidazole, a 5-nitroimidazole is widely used for treatment of anaerobic bacterial, protozoal infections, adverse reactions include nausea, dry-mouth, vomiting, diarrhoea. Neurologic toxicity is rare. We report a rare case of ataxia & cerebellar lesions following therapy with metronidazole, which resolved rapidly after discontinuation of drug. 50- year old male presented with fever, right-hypochondriac pain, diagnosed as multiple liver abscesses, managed initially by drainage-of abscess, intravenous ciprofioxacin, metronidazole for 7 days. He improved, was discharged on oral metronidazole800mg thrice-a-day for 7days. He did not return for followup, but continued medication for next 20 days when he presented with slurring of speech, in-coordination, progressive unsteadiness of gait, subsequent inability to walk. He was alert, oriented, speech dysarthric, horizontal nystagmus 2-3 beats on lateralgaze; no vertical nystagmus, heelto- shin, fi nger-to-nose tests abnormal, power normal. Tone decreased. Refi exes equal. Stance wide-based, felt unsteady, unable to walk without support, sensory normal. MRI Brain-abnormal T2 as well as fiAIR hyperintensities in Dentate nuclei, deep cerebellar parenchyma, splenium of corpus callosum. Serum Metronidazole levels were normal. After discontinuation of metronidazole, patient started regaining ambulatory function was able to walk with-support in 4 days, without-support in 10days. Follow-up MRI at 8 weeks was normal with resolution of hyperintensities of dentatenuclei, cerebellum. Discussion: Cerebellar toxicity is rare adverse-event in patients treated with metronidazole. While proposed mechanism was high cumulative dose in most patients (25-1080 grams), our patient inspite of taking about 75 grams had normal serum Metronidazole levels. Exact mechanism by which Metronidazole causes reversible cerebellar ataxia and Dentate nuclei changes is unclear. Cerebellar toxicity should be considered in any patient who presents with ataxia-dysarthria and is receiving prolonged therapy with metronidazole. MRI should be performed for defi nitive diagnosis and metronidazole withdrawn. Further studies are needed to defi ne the pathogenesis of this unusual event.

Poster Session : PS 0314 ; Hematology : Acute Psychosis: A Presentation of Cyanocobalamin Defi ciency Megaloblastic Anemia

( Anurag Bahekar ),( Girish Bhageshwar Ramteke ),( Sanjay Dubey ),( Amith R ),( Ankit Meshram ),( Amit Agarwal ) 대한내과학회 2014 대한내과학회 추계학술대회 Vol.2014 No.1

Mental disturbances due to vitamin B12 deficiency have been reported previously as chief or sole symptoms, occasionally even preceding decrease of cobalamin in serum. Frank psychosis related to vitamin B12 defi ciency is considered very rare and has received little attention. We report a 28 yrs young female presenting with acute psychosis due to cyanocobalamine deficiency which reversed with treatment. Presentation was irrelevant talking, picking of clothes, inability to recognize people for 3 days. No fever, headache, vomiting, weakness, head trauma, drug intake, stressor or addiction. No past major illness, psychiatric illness or memory loss. Pt. had weaknes easy fatigability since 2 years was on vegetarian diet. Bladder bowel habits were regular. Hb 2. 9 gm/dl, thrombocytopenia 1 lakh/cumm, normal leucocytes, MCV-142. 7 fi , smear showed dimorphic picture, pencil cells, tear drop cells, anisopoikilocytosis, hypersegmented neutrophils. Diagnosis of megaloblastic anemia was made. CSF, CT head, electrolytes were normal. B12 level 77. 12 pmol/l (N200-800 pmol/l). Endoscopy- atrophic gastritis. Histology-atrophic gastriris. Antiparietal cell antibody-negative. Patient recovered fully within 3 days therapy with Inj. cyanacobalamin 1000 microgm i. m. with no need of antipsychotics at discharge. Biermer`s disease, also called acquired pernicious anemia, disorder of vitamin B12 absorption characterized by megaloblastic anemia, gastrointestinal symptoms, can lead to neurological abnormalities. Frequent in elderly, less in adults(15%), extremely rare inchildren, adolescents. It is asymptomatic for years, non-specifi c symptoms of anemia, atrophic gastritis. Possible mechanism for psychotic behaviour suggested by Hutto, reported cobalamin and folate participate in synthesis of monoamine neurotransmitters in brain by increasing BH4(tetrahydrobiopterin) synthesis. Treatment:Oral administration of high-dose vitamin B(12) (1-2 mg daily) as effective as intramuscular administration in correcting defi ciency. We recommend consideration be given to B-12 defi ciency as an etiological possibility in some cases of depression, organic psychosis, certain childhood-disorders and dementia.

Poster Session : PS 0151 ; Neurology : Arnold Chiari with Hemiparesis, Hemisensory Loss with Lower Cranial Nerve Palsy

( Vikas Asatl ),( Girish Bhageshwar Ramteke ),( Vinatak Jatale ),( Vep Prakash Pandey ) 대한내과학회 2014 대한내과학회 추계학술대회 Vol.2014 No.1

The Chiari I malformation (CMI) is caudal displacement of cerebellar tonsils into cervical spinal-canal. CMI is defi ned by tonsillar herniation more than 5 mm below foramen-magnum, may be congenital or acquired. We are presenting case of Arnold chiari with hemiparesis, hemisensory loss with lower cranial nerve palsy. 50 year male, complaints of tingling, numbness left LL, UL since 7 months, Weakness left LL, UL 6 months, drycough, diffi culty in swallowing, regurgitation of food, headache 3 months. Decubitus -supine position left leg extended laterally rotated, higher functions normal. Trigeminal- there is loss of all sensation modalities on left side of face, Gag refl ex is normal with normal palatal movement, Left side shoulder cannot be raised, power of tongue is slightly decreased on left, resting nystagmus with fast component toward right side. On attempting to see right side nystagmus increases while on seeing to left decreases. Heel-knee, fi nger-nose-test on right side is normal, on left side unable to do due to motor weakness. MRI Brain parenchyma normal. Inferior cerebellar tonsillar herniation in dorsal subarachnoid space extending about 6-7 mm below the posterior arch of atlas-Chiari Malformation type I, Mild indentation is seen on the dorsal surface of upper cervical cord. Minimal inferior displacement of 4th ventricle, no hydrocephalus, no syrinx. Discussion: Chiari malformations consist of varying degree of ectopia of cerebellum. Incidence in MRI is between 0.56% and 0.77%.Type I: Elongation of tonsils and medial parts of the inferior lobes of cerebellum into cone-shaped projections, which accompany medulla oblongata into spinal canal. Type II Displacement of parts of inferior vermis, pons, medulla-oblongata together with elongation of fourth ventricle (most cases with spina bifi da). Type III The entire cerebellum herniates into the cervical canal. Type IV is with Cerebellar hypoplasia.

Poster Session:PS 0193 ; Endocrinology : Carbimazole Induced Cholestatic Jaundice in Toxic Multinodular Goitre

( Amith R ),( Girish Ramteke ),( Dharmendra Jhavar ),( Lalan Pratap Singh ),( Anu Daber ),( Manoj Gupta ),( Nd Punjabi ) 대한내과학회 2014 대한내과학회 추계학술대회 Vol.2014 No.1

Antithyroid drugs are used to treat toxic multinodular goitre(TNG). Carbimazole is usually the drug of choice except in pregnancy where propylthiouracil is used. It is well tolerated and common side effects include allergy, upper GI upset, rarely agranulocytosis. Hepatitis is rare but serious complication.We report a 55yr female with TNG, who developed cholestatic hepatitis after carbimazole therapy for 2 months. She recovered completely following withdrawal of the drug.Toxic multinodular goitre (Plummer`s disease) is a 2nd most common cause for hyperthyroidism after Grave`s disease, 15-30% cases, (>50 years) and women. Unlike Grave`s disease which is autoimmune and antithyroids are started universally, TNG is not known to recur after therapy. So surgery or radioiodine are treatment of choice. Antithyroids are used for symptomatic relief in patients waiting surgery. Side effects are mild, include allergic reactions, upper GI intolerance, agranulocytosis, vasculitis-like reaction particularly propylthiouracil. Hepatotoxicity is rarebut serious side-effect with both carbimazole and propylthiouracil (PTU). Histology with PTU shows toxic hepatitis &necrosis and it is cholestatic hepatitis with carbimazole. Our case demonstrates carbimazole induced cholestatic hepatitis in patient with TNG. Clinical,biochemical fi nding with relevant review of literature is presented. Patient was put on propranolol, prednisolone in the interim for her thyrotoxicosis and exophthalmosis. Liver function tests improved signifi cantly following stoppage of carbimazole. The high degree of alkaline phosphatase could also be due to effect of hyperthyroidism on bone resorption. She is currently euthyroid. In summary, jaundice as a complication of thionamide treatment of hyperthyroidism is rare, this complication cannot be predicted by deranged liver enzymes at presentation, but typically occurs within three months of therapy, it can be fatal, particularly when there are additional hepatotoxic factors, and the drug must be withdrawn immediately and alternative therapy, such as radioiodine must be considered in appropriate patients.

Slide Session : OS-END-15 ; Endocrinology : A Study of Endothelial Dysfunction by Flow Mediated Dilatation of Brachial Artery in Euglycemic & Hyperglycemic Individuals

( Sarath Menon ),( Girish Ramteke ),( Dharmendra Jhavar ),( Manoj Gupta ) 대한내과학회 2014 대한내과학회 추계학술대회 Vol.2014 No.1

Background: Endothelial dysfunction is regarded as an early marker for atherosclerosis & a precursor for future cardiovascular events. Objectives: Study was done to find out endothelial dysfunction (ED) in euglycemic & hyperglycemics. finding any endothelial dysfunction in high risk euglycemic & pre-diabetic individuals and compared degree of ED with different types of hyperglycemia, glycemic parameters & common cardio-vascular (CV) risk factors. MATERIAL & Methods: A cross-sectional, prospective study was done in subjects divided into two groups. First group included hyperglycemic individuals (80) Pre-diabetes( 20), Type1DM(20), Type2 DM (34), clinically labeled MODY(06) and second group included 40 euglycemic individuals. Body mass index, fasting lipid profile, FBS, PPBS, RBS, HbA1c were obtained. Endothelial dependent flow mediated dilatation was assessed with 7.5MHz high resolution ultrasound of brachial artery. A p-value of <0.05 was considered to be statistically significant. Results: Flow mediated dilatation was impaired with increase in age, BMI, lipid profile (p=0.04,0.02,0.02 respectively). FMD was seen inversely related to all glycemic parameters like FBS(r=.680,p=<0.001),PPBS(r=-.660,p=<0.001),RBS(r=.680,p=<0.001), HbA1c(r=-.820,p=<0.001).Flow mediated dilatation was impaired in Pre-diabetes, Type 1 DM,Type2 DM, Clinical MODY, but did not show significant difference between each other (9.03+0.73% vs 8.21+1.18% vs 6.95+2.14% vs 9.4+0.45%,p=0.20) but had significant difference when compared to euglycemics (14.01+3.06%,p=<0.001).FMD was impaired even in high-risk euglycemics(p=0.02) Conclusion: Results showed impaired endothelial function in hyperglycemics and endothelial dysfunction was seen even in Pre-diabetes and in high risk euglycemic individuals, thus showing endothelial damage in these early stages. Endothelial function declined with increase in the severity of glycemic parameters and common CV risk factors. Our study suggested FMD should be considered as a surrogate marker for future cardiovascular events.

Poster Session : PS 0152 ; Neurology : Subacute Sclerosing Pan Encephalitis(SSPE): A Rare Presentation

( Amith Ram ),( Bhageshwar Girish Ramteke ),( Anjali Matani ),( Apoorva Paurank ),( Archana Verma ),( Anurag Bahekar ) 대한내과학회 2014 대한내과학회 추계학술대회 Vol.2014 No.1

Subacute Sclerosing Pan-Encephalitis is a prototype neurodegenerative disease of childhood characterised by onset of neurological symptoms between 5 and 15 years of age with a progressive downhill course and death within 2-4 years. Incidence of adult onset SSPE has been reported between 1-1.75% and 2.6%. To-date less than 140 cases of adult onset SSPE have been published, the majority being case reports. A 24 year old male presented with progressive neurological syndrome characterised by very frequent myoclonic jerks associated with brief dystonic posturing of upper limbs for 8 months. No history of seizures, cognitive decline or personality changes, no history-of measles in childhood but immunized. Routine blood investigations normal. EEG-periodic complexes, CSF- mild increase in proteins, normal sugar, cell count. Measles antibody titre elevated, serum as well as CSF levels, CSF/serum antibody quotient 2.62 which was strongly suggestive of SSPE. Other antibody titres were unremarkable. MRI-bilateral & near symmetrical T2 hyperintense signal in posterior putamen, slightly larger on right, restriction of diffusion & are hypo on T1. No cortical or white matter changes seen. Our patient is not classical case of SSPE. Atypical for age, late-onset, qualifi es as adult-onset of SSPE (defi ned onset after 18 years), well preserved cognition. He did not have seizures, spasticity or visual complaints which are common. Imaging fi ndings are also atypical as common fi ndings early in disease are white matter changes and cortical atrophy mainly in the posterior region of brain and the hyperintense signals seen in late course of the disease are seen early in this patient. EEG Stereotyped periodic complexes with normal background and is strongly suggestive SSPE. MRI reveals T2 hyperintense lesions in bilateral putaminal region slightly larger on right side. Treatment: No curative-therapy is available, few disease-modifying agents have been tried which may delay progression.