Association of tumor necrosis factor-alpha and interferon gamma gene polymorphisms and their plasma levels in leprosy, HIV and other peripheral neuropathies.

OBJECTIVE: Mycobacterium leprae and Human Immunodeficiency Virus (HIV) are causative agents known to be involved in nerve damage in leprosy and HIV-peripheral neuropathy (HIV-PN) respectively. Among other peripheral neuropathies the most common is diabetic neuropathy, which is metabolically induced. The proinflammatory cytokines TNF-α and IFN-γ have been implicated in the pathogenesis of peripheral neuropathy. The association between the plasma levels of these cytokines and their single nucleotide polymorphisms (SNPs) were investigated in leprosy neuropathy (LN), HIV-PN and other peripheral neuropathies (OPN). METHODS: Eighty-eight individuals with LN (PB=36; MB=52), 39 with HIV-PN, 52 patients with OPN, 101 HIV positive individuals without neuropathy (HIV) and 113 healthy subjects (HS) were included in the study. Plasma cytokine levels were measured by sandwich ELISA and one way ANOVA was carried out among the groups. SNPs of TNF-α- 308 G/A, -238 G/A and IFN-γ +874 T/A were investigated by amplification refractory mutation system polymerase chain reaction (ARMS-PCR). Their frequencies were compared between groups by Pearson's chi squared test. RESULTS: Plasma TNF-α and IFN-γ was significantly increased in LN (p<0.05), HIV-PN (p<0.05) and OPN (p<0.05) as compared to HS. A significant association was found between IFN-γ +874 A/A genotype in LN (p<0.05; OR=7.9), HIV-PN (p<0.05; OR=8.9) and OPN (p<0.05; OR=8.9) as compared to HS. CONCLUSION: Elevated levels of plasma TNF-α and IFN-γ and the association of IFN-γ +874 A/A genotype SNP in LN, HIV-PN and OPN suggests a common involvement of these cytokines in susceptibility/pathogenesis of peripheral neuropathy.

Spectrum of congenital myopathies: a single centre experience.

BACKGROUND: Congenital myopathies (CMs) are rare and they are clinically and genetically heterogeneous. Muscle biopsy is characterized by structural abnormality that is diagnostic. There are few studies from India. MATERIALS AND METHODS: This is a retrospective study of 12 years. The demographic data, clinical features and laboratory data of patients diagnosed as CMs on muscle biopsy were retrieved from medical records. The slides were reviewed for morphological and structural abnormalities using the following stains hematoxylin and eosin, modified Gomori trichrome, masson trichrome, periodic acid schiff, adenosine triphosphatase preincubated at pH 9.4, 4.6 and 4.3, nicotinamide adenine dinucleotide tetrazolium reductase, succinic dehydrogenase and cytochrome c oxidase. Immunohistochemistry was performed with dystrophin, sarcoglycans and desmin wherever necessary. RESULTS: There were 50 patients with CMs: Centronuclear myopathy (23), myotubular myopathy (3) and central core disease (CCD) (8), nemaline myopathy (5), congenital fiber type proportion (10) and desmin related myopathy with arrythmogenic right ventricular cardiomyopathy (ARVD) (1). Of the 50 patients, 30 (60%) presented in the first decade of life. Proximal muscle weakness and hypotonia were the common presenting features. Type 1 atrophy and predominance were seen in most cases on muscle biopsy. CCD had one patient with high creatine phosphokinase levels, biopsy in one patient showed both rods and cores, in the other limb girdle muscular dystrophy like picture and one biopsy showed uniform type 1 fibers. There was one desmin related myopathy with ARVD, who had cardiac transplantation and both skeletal and cardiac muscle showed characteristic rimmed vacuoles and inclusions positive for desmin. CONCLUSION: CMs are rare and the diagnosis can only be established on muscle biopsy. Defining the specific CMs helps the clinician in counseling the patient and family.

Risk factors, clinical profile, and long-term outcome of 428 patients of cerebral sinus venous thrombosis: insights from Nizam's Institute Venous Stroke Registry, Hyderabad (India).

BACKGROUND: With the widespread use of neuroimaging and hematological workup, many of the previously held concepts about cerebral sinus venous thrombosis (CSVT) are changing. OBJECTIVE: The objective of this study was to investigate the risk factors, clinical profile, and outcome of the fully investigated cases of CSVT from a major university referral hospital in South India. MATERIALS AND METHODS: Consecutive patients of CSVT confirmed by definite neuroimaging criteria and fully investigated for prothrombotic states, between June 2002 and September 2010, were prospectively studied in the Venous Stroke Registry of Nizam's Institute of Medical Sciences, Hyderabad, South India. RESULTS: Of the 428 patients, 230 (53.7%) were men and the mean age was 31.3 years (range 8-65 years). Seizures were noted in 126 (29.4%) patients, stroke like presentation was found in 122 (28.5%) patients, and benign intracranial hypertension like presentation was found in 78 (18.2%) patients. Common risk factors were anemia in 79 (18.4%), hyperhomocysteinemia in 78 (18.2%), alcoholism in 67 (15.6%), oral contraceptive pill intake in 49 (11.4%), postpartum state in 42 (9.8%), anticardiolipin antibodies in 31 (7.2%), and protein S deficiency in 53 (12.3%) patients. Good outcome at 90 days (modified Rankin Scale £ 2) was observed in 273 (71.2%) of 383 patients available for follow-up. In-house mortality was noted in 33 (7.7%) and recurrence in 22 (5.1%) patients. CONCLUSIONS: Compared to the previous studies, prevalence of CSVT was higher in men. Anemia, hyperhomocysteinemia, alcoholism, oral contraceptive use, and postpartum state were the most common risk factors. Overall prognosis was good, but a small percentage of patients died or showed recurrence.

Antibodies to myelin P0 and ceramide perpetuate neuropathy in long standing treated leprosy patients.

Anti neural antibodies are known to play a role in the immunopathogenesis of nerve damage in leprosy and HIV/AIDS. Myelin Protein zero (P0) and ceramide are two nerve components which maintain the integrity of the peripheral nerve. The present study was undertaken to identify antibodies to myelin P0 and ceramide in the sera of treated leprosy patients, HIV positive individuals and healthy subjects using enzyme linked immunosorbant assay (ELISA). The results revealed that treated leprosy patients continue to have significantly elevated myelin P0 and ceramide antibody levels as compared to healthy subjects (P < 0.05). The elevated antibody response to myelin P0 and ceramide in leprosy patients indicate a low grade autoimmune activity that perpetuates nerve damage in treated leprosy. There was no significant difference in the myelin P0 and ceramide antibody levels between HIV positive and healthy subjects (P > 0.05) suggesting that these antibodies do not play a role in early HIV infection.

Predictors of major neurological improvement after intravenous thrombolysis in acute ischemic stroke: a hospital-based study from south India.

BACKGROUND: Despite the increasing use of recombinant tissue plasminogen activator (rt-PA) in acute ischemic stroke, uncertainty persists about the short- and long-term outcome of the thrombolysed patients. OBJECTIVE: To identify predictors of major neurological improvement at 24 h after intravenous rt-PA administration in patients of acute ischemic stroke and their relationship with outcome at 12 months. MATERIALS AND METHODS: We analyzed the data of the patients with acute ischemic stroke treated as per the National Institute of Neurological Disorders and Stroke (NINDS) criteria with intravenous rt-PA between January 2000 and June 2009 at a tertiary care center in south India. Major neurological improvement was defined by an 8-point improvement in National Institute of Health Stroke Scale (NIHSS) score or an NIHSS score of 0 or 1 at 24 h. Good outcome was defined as a 12-month modified Rankin Scale (mRS) of 0 to 1. RESULTS: Of the 72 patients with acute ischemic stroke treated with intravenous rt-PA, 23 (32%) patients had major neurological improvement at 24 h. Age <60 years (OR 1.9, 95% CI 1.7 to 3.2), admission glucose levels <8 mmol/L (OR 3.87, 95% CI 1.9 to 9.2) and mild to moderate baseline stroke severity (NIHSS median score 10+ 6) were associated with major neurological improvement after adjusting for co variables. Major neurological improvement at 24 h was an independent predictor of good outcome (mRS=1) at 12 months (OR 13.9, 95% CI 6.84 to 40.2). CONCLUSIONS: Age <60 years, glucose levels <8 mmol/L and mild to moderate stroke severity (NIHSS median score 10+/-6) was associated with major neurological improvement after intravenous rt-PA. Major neurological improvement at 24 h after the administration of intravenous thrombolysis independently predicted good outcome at 12 months.

The Effect of Dual Tasking and Deep Brain Stimulation Frequency Parameters on Gait in Advanced Parkinson's Disease.

OBJECTIVE: To study the effect of dual tasking and deep brain stimulation frequency parameters on gait in advanced Parkinson's disease. MATERIALS AND METHODS: This is an open label interventional study evaluating 40 post STN-DBS patients with gait disturbances. All patients were diagnosed as PD by a movement disorder specialist using the United Kingdom Parkinson's Disease Society Brain Bank (UKPDSBB) criteria. Patients underwent bilateral subthalamic deep brain stimulation by a qualified neurosurgeon. Patients were managed on a combination of dopamine replacement therapy as well as deep brain stimulation. Patients were assessed by stand walk sit (SWS) test for a 5 meter distance and FOG scoring during medication 'ON' state and device "ON" state, at four frequencies 180, 130, 90, 60 HZ and device "OFF" state. RESULTS: Out of 40 patients, 38 patients showed a significant improvement in gait at a single frequency (best response frequency) which is different for each patient. The mean FOG score showed significant improvement at all stimulation frequencies when compared to OFF stimulation (P < 0.05). The mean number of steps was 18.9 at best response frequency and 21.48 at 130 Hz (P < 0.0001). Number of freezing episodes also were significantly less with best frequency when compared to 130 Hz stimulation (0.28 and 0.65 respectively, (P < 0.0001). The mean FOG score was 6.45 at best frequency and 9.48 at 130 Hz (P < 0.0001). Mean Dual tasking score was 3.53 at best frequency and 5.15 at 130 Hz (P < 0.0002). CONCLUSION: Optimization of frequency setting for each patient can improve gait and that each patient may have a different optimal frequency.

The co-encapsulated antioxidant nanoparticles of ellagic acid and coenzyme Q10 ameliorates hyperlipidemia in high fat diet fed rats.

Obesity is the major cause of type 2 diabetes with hyperlipidemia as one of its complications and antioxidants were found to be beneficial in such disease conditions. The present investigation is geared towards reduction of the dose required/improve the bioavailability of the combination of antioxidants, ellagic acid and coenzyme Q10 by co-encapsulating them into nanoparticles and study the possible synergism in ameliorating hyperlipidemia in high fat diet fed rats. The co-encapsulated particles at 10% (w/w of polymer) loading of ellagic acid and coenzyme Q10 have particle size of 260 nm. Male Sprague-Dawley (SD) rats on feeding high fat diet for over 4 weeks developed hyperlipidemia. The hyperlipidemic rats on 2 weeks post treatment with antioxidant combination administered as oral suspension or nanoparticles found to ameliorate the hyperlipidemic conditions and nanoparticles were found to be equally/more effective at 3 times lower dose in sustaining cholesterol lowering effect for extended periods, lowering glucose and triglycerides and in improving endothelial functioning, indicating the ability of the nanoparticles in improving efficacy of the duo. The results promise the potential of nanoparticles in improving the efficacy of ellagic acid and coenzyme Q10 in treating high fat diet induced hyperlipidemia in rats.

Vasculitic Neuropathy: A Retrospective Analysis of Nerve Biopsies and Clinical Features from a Single Tertiary Care Center.

OBJECTIVE: Vasculitic neuropathy can be either restricted to the peripheral nerves or associated with systemic involvement of other organs. The objective of this study was to analyze the nerve biopsies reported as "vasculitic neuropathy" with clinical features. MATERIALS AND METHODS: All cases diagnosed with vasculitic neuropathy were retrospectively analyzed and categorized as systemic vasculitis and nonsystemic vasculitic neuropathy based on the clinical features. The histological features were further evaluated and classified according to the Peripheral Nerve Society Guidelines. RESULTS: Of the 126 cases, there were 65 nonsystemic vasculitis, 45 secondary systemic vasculitis, and 16 primary systemic vasculitis. Definite vasculitis was more common in the systemic vasculitis group. The epineurial vessels were predominantly involved with chronic axonal changes. CONCLUSION: The sensitivity of definite vasculitis on nerve biopsy was 54.76%. The sensitivity increases when the diagnostic criteria of definite and probable vasculitis were applied taking into account perivascular inflammation accompanied by vascular changes and axonopathy.

Molecular and Histopathological Characterization of Patients Presenting with the Duchenne Muscular Dystrophy Phenotype in a Tertiary Care Center in Southern India.

OBJECTIVE: To study the histopathological characteristics and mutation spectrum of patients presenting with the Duchenne muscular dystrophy (DMD) phenotype. METHODS: This was a descriptive study conducted over a period of 8 years. Multiplex ligation-dependent probe amplification (MLPA) was done in patients presenting with the DMD phenotype. If MLPA was negative, patients were offered muscle biopsy for histopathological studies and/or next generation sequencing (NGS) based multigene panel testing for muscular dystrophies. RESULTS: Of the 510 patients included, mutation in the DMD gene was detected by MLPA in 372 (72.9%), of whom 342 (67.1%) had exonic deletions and 30 (5.9%) had exonic duplications. Exons 45-55 were most commonly involved in large deletions and exons 1-10 were the commonest exons involved in duplications. In the MLPA-negative cohort, 27 proceeded for muscle biopsy. NGS was done in 14 patients, 10 of whom had pathogenic mutations in the DMD gene, 3 were non dystrophinopathies and no pathogenic variant could be identified in one patient. CONCLUSIONS: For patients presenting with the DMD phenotype, MLPA of the DMD gene has a high diagnostic rate of about 73%, and non-dystrophinopathies may constitute a small but significant proportion.

Chlamydia pneumoniae antibodies in various subtypes of ischemic stroke in Indian patients.

BACKGROUND: As infections occur more frequently in developing countries, we carried out this prospective case-control study, to establish the association, if any, between C. pneumoniae antibodies and ischemic stroke particularly in relation to its subtypes. DESIGN: Antibodies (IgG and IgA) to C. pneumoniae in serum were measured by microimmunofluorescence test in 200 consecutive ischemic stroke patients and 200 age and sex matched controls. RESULTS: Seventy two out of 200 ischemic stroke patients (36%) had positive C. pneumoniae antibodies (IgG or IgA), compared to 35 out of 200 controls (17.5%) (p<0.0001). IgG antibody was positive in 64/200 (32%) ischemic stroke patients, compared to 34/200(17%) controls (p<0.0001) and IgA was positive in 20/200(10%) ischemic stroke patients compared to 1/200(0.5%) controls (p<0.0001). Logistic regression analysis showed statistically significant association between C. pneumoniae antibody positivity and ischemic stroke, thereby establishing it as an independent risk factor. Prevalence of C. pneumoniae antibodies was significantly higher in all stroke subtypes (except the stroke of undetermined etiology) compared to controls. CONCLUSION: Significant and independent association was found between C. pneumoniae antibodies and ischemic stroke in this sample of south Indian population. The association was found in all ischemic stroke subtypes, except stroke of undetermined etiology.

Oral nanoparticulate atorvastatin calcium is more efficient and safe in comparison to Lipicure in treating hyperlipidemia.

Atorvastatin calcium (AC) is a second-generation 3-hydroxy-3-methylglutaryl-CoA reductase inhibitor approved for clinical use as a lipid lowering agent. AC, the world's best selling drug is associated with poor oral bioavailability and serious adverse effects like rhabdomyolysis on chronic administration. A biodegradable nanoparticulate approach was introduced here with a view to improving the efficacy and safety of AC. Poly lactide-co-glycolic acid (PLGA) nanoparticles containing atorvastatin calcium were prepared using two stabilizers i.e. didodecyl dimethyl ammonium bromide (DMAB) and Vitamin E tocopheryl polyethylene glycol 1000 succinate (Vit E-TPGS) using a co-solvent approach by emulsion-diffusion-evaporation method. AC loaded PLGA nanoparticles prepared using DMAB and Vit E-TPGS were found to be 120.0 +/- 4.2 nm and 140.0 +/- 1.5 nm (z-average) in size respectively. In vitro release studies at pH 7.4 revealed a zero order release profile for nanoparticles. Efficacy and safety parameters of the prepared nanoparticles against marketed formulation were evaluated in high fat diet fed (hyperlipidemic) rats. It was found that atorvastatin calcium nanoparticles were equally effective in comparison to Lipicure, at a 66%-reduced dose in treating the hyperlipidemia characterized by alterations in PTC, LDL-C, VLDL-C, HDL-C, PTG and PGL in the high fat diet fed rats. On the other hand, when evaluated for safety, nanoparticulate formulation showed no/negligible myotoxicity characterized by lower PC, BUN, CK, LDH and AST levels in comparison to the marketed formulation.

Major histocompatibility complex class I expression can be used as a diagnostic tool to differentiate idiopathic inflammatory myopathies from dystrophies.

AIM: Utility of major histocompatibility complex (MHC) Class I antigen immunostaining was studied to differentiate idiopathic inflammatory myopathies from dystrophies. MATERIALS AND METHODS: Forty muscle biopsies including seven dermatomyositis (DM), six polymyositis (PM), two sporadic inclusion body myositis (sIBM), 20 dystrophies (one Duchenne, three Becker's, four alpha, one gamma sarcoglycanopathy, nine limb girdle, one myotonic and one fascioscapulohumeral muscular dystrophy) and five controls were stained with antibody for MHC Class I antigen (Novocastra clone W6/32 HL 1:100 dilution). RESULTS: Polymyositis and sIBM showed MHC class I antigen positivity along sarcolemma of single and small groups of muscle fibers. The regenerating fibers in the perifascicular area in DM showed intense cytoplasmic positivity of MHC class I antigen. Muscle fibers in all dystrophies except regenerating fibers and control normal muscle were negative for MHC. Capillaries and lymphocytes were positive controls. There were no false positives in the study. CONCLUSION: MHC Class I immunostaining can be used as a complementary diagnostic tool for the diagnosis of idiopathic inflammatory myopathies.

Lipid storage myopathies with unusual clinical manifestations.

We describe the clinical presentation, course and pathologic findings found in three adult patients with lipid storage myopathy. Excessive lipid storage was found in Type 1 fibers of muscle. Clinical improvement on oral levo-carnitine therapy suggests the possibility of carnitine deficiency as the most likely etiology in two of the patients and one had mitochondrial myopathy confirmed on genetic analysis.

Necrotizing Autoimmune Myopathy: Clinicopathologic Study from a Single Tertiary Care Centre.

BACKGROUND: Idiopathic inflammatory myopathies (IIMs) are a group of chronic, autoimmune disorders which include a new entity, necrotizing autoimmune myopathy (NAM). NAM lacks inflammation and presents with markedly elevated creatinine phosphokinase (CPK) levels. It is associated with connective tissue diseases (CTDs), statin use, malignancies, and most cases are idiopathic. OBJECTIVES: The objectives of this study are to describe the clinicopathologic features in muscle biopsy-proven cases of NAM. To emphasize the role of laboratory parameters such as CPK levels and myositis profile in the diagnosis of NAM. MATERIALS AND METHODS: This is a retrospective study including 15 patients of NAM diagnosed on muscle biopsy over a period of 2 years. The slides of the biopsies were reviewed, and clinical data, electromyography findings, and CPK levels were obtained. Myositis profile was done. RESULTS: Necrotizing myopathy accounted for 13.63% (15 cases) of total inflammatory myopathies (110 cases) in the study. These were grouped into CTD-associated NAM, statin-associated NAM, paraneoplastic NAM and idiopathic NAM which was the common type. All cases presented with progressive proximal muscle weakness and had markedly elevated CPK levels. Anti-3-hydroxy-3-methyl-glutaryl-coenzyme A reductase and antisignal recognition particle antibodies were seen to be positive in six patients. Muscle biopsies showed predominant fiber necrosis with significant fiber degeneration and regeneration in the absence of inflammation. All patients received immunotherapy with significant improvement was seen in six patients with two mortalities. CONCLUSION: Necrotizing myopathy is a new addition to the spectrum of IIM. Clinicopathologic correlation is important for appropriate diagnosis. It is found to be refractory to corticosteroids monotherapy. The course of illness is not uniform, and in some patients, there can be rapid worsening with mortality.

Predictors of Intracerebral Hemorrhage in Acute Stroke Patients Receiving Intravenous Recombinant Tissue Plasminogen Activator.

BACKGROUND: Symptomatic Intracerebral hemorrhage (sICH) is a serious complication of recombinant tissue-plasminogen activator (rt-PA) therapy for acute ischemic stroke (AIS). OBJECTIVE: To estimate the prevalence and predictors of sICH in patients after receiving IV rt-PA for AIS. MATERIAL AND METHODS: Consecutive patients of AIS thrombolysed between January 2010 and June 2016 in a University hospital in Hyderabad (India) were studied prospectively for sICH and it's various variables compared with the control group without sICH to determine any sigificantant difference. RESULTS: Out of 113 patients , sICH was detected in 12 (10.61%) whose mean age(58±12.0 years) and gender ratio ( 2:1 ) was not statistically significant from controls. In s ICH group mean NIHSS score was 16.53± 5.81 vs 10.19± 5.06 in controls (p<0.001), gap between stroke onset and thrombolysis was 227.50±46.15 min vs 178.50± 69.20 min in controls(p=0.018). At presentation mean blood sugar was 208.75±90.97 mg/dl in sICH group vs 146.83±70.21 mg/dl in controls (p=0.002). Prior diabetes was in 7(53.30%) vs 23 (22.8%) in controls (p= 0.014)and hypertension in 11 (91.7%) vs (56(55.4%) in controls (p= 0.026) The mortality in sICH was 7 (58.30%)vs 4 (4.94%) in controls (p<.0.001). At 3 months mean mRS ofsICH patients was 5.57± 0.54 vs 2.17± 1.69 in controls (p<.05). CONCLUSION: High NIHSS score, increased stroke onset to thrombolysis time , high blood sugar at presentation ,prior diabetes and hypertension increase the chances of sICH. None of these contraindicate thrombolysing strokes but should caution the physician.

Decreased calcineurin activity in circulation of Duchenne muscular dystrophy.

OBJECTIVES: In an analysis of enzymes in easily accessible tissues like blood cells, serum can provide a valuable information and a simple tool for disease and carrier detection. In the study presented we have analyzed calcineurin activity in Duchenne muscular dystrophy (DMD) and carrier sera and lymphocytes for its diagnostic value and its status in DMD pathology. DESIGN AND METHODS: We have monitored calcineurin activity in sera and lymphocytes of DMD, in carriers and in controls using colorimetric method by following the p-nitrophenol released in the presence and absence of Trifluoperazine (TFP), an inhibitor of calcineurin. RESULTS: Results showed a significant decrease in serum and lymphocyte calcineurin activity in DMD (p<0.001) without alteration in carriers compared to normal. CONCLUSION: Further studies are required to understand possible alterations mediated by calcineurin with reference to DMD lymphocytes as any alteration in phosphorylation/dephosphorylation pathway can disturb the normal functioning of these cells. The decreased calcineurin activity observed in DMD serum compared with controls could be further examined for its diagnostic utility.

Sarcoglycanopathies: a clinico-pathological study.

BACKGROUND: Limb girdle muscular dystrophy (LGMD) is a heterogeneous group of disorders characterized by limb girdle weakness. There are no clear clinical features that distinguish various types of LGMD. MATERIALS AND METHODS: We studied 26 patients with chronic progressive weakness in limb girdle distribution without early facial involvement with muscle biopsies suggestive of dystrophy/myopathy and positive for dystrophin antibodies. Immunohistochemistry studies of muscle biopsies were done on all patients to classify different types of sarcoglycanopathies. RESULTS: The mean age of presentation was in the third decade. There were 14 male and 12 female patients. The common pattern of inheritance was autosomal recessive, seen in 53.8%. The more frequent type of LGMD was sarcoglycanopathy (SGP) (53.8%). Amongst the SGPs, alpha-SGP (26.9%) was the most common followed by beta-SGP (15.3%), gamma-SGP (3.8%) and delta-SGP (7.6%). Calf hypertrophy was noted in 53.5% of LGMD and 57.1% of SGPs, extensor digitorum brevis hypertrophy in 42% of LGMD and 35.7% of SGPs, winging of scapula in 39.2% of the LGMD group and 35.7% of the SGPs, valley sign in 28.5% of the LGMD group and 21.4% of the SGPs. Hip abductor sign was positive in 71.4% of LGMD and 64.2% of SGPs. Differential weakness of knee flexors was more common in SGP (57.1%). The mean creatine phosphokinase (CK) value was 2519IU/L and was elevated in 92.8% patients. Muscle biopsy showed a dystrophic pattern in 75% of LGMD and a myopathic pattern in the remaining. Symptomatic cardiac involvement was seen in one patient. ECG changes were seen in 44% of LGMD patients and 50% of the SGP. The common changes noted were T wave inversion in V1, V2 (16%), left ventricular hypertrophy LVH (12%) and right bundle branch block (RBBB) in 12% of the LGMD group. CONCLUSION: Sarcoglycanopathy is a more frequent form of LGMD whereas alpha type is the most common among the SGP. The four types of SGP do not differ in the pattern of muscle involvement. A relatively earlier onset, selective weakness of knee flexors and a very high CK may help differentiate SGP from other forms of LGMD. Immunohistochemistry is very useful in classifying the different types of LGMD prior to genetic analysis.

Predictors of mortality in rhinocerebral mycosis.

INTRODUCTION: Rhinocerebral mycosis is a rapidly progressive fatal opportunistic infection, predominantly affecting people in an immunocompromised state. Aggressive surgical therapy, with repeated debridement in combination with intravenous amphotericin B can lead to a high rate of cure. AIM: To determine the predictors of mortality in rhinocerebral mycosis. MATERIALS AND METHODS: The demographic data, clinical features, radiological (MRI/CT) findings, treatment details of patients with a diagnosis of rhinocerebral mycosis confirmed on histopathology were analyzed retrospectively. The outcome was assessed as alive and dead. Univariate analysis with odds ratio (OR) was employed in data analysis. Chi-square test was used for P value. RESULTS: There were 38 patients. The age range was 7-82 (mean 48.68) years; 30 (79%) were males. Craniofacial pain was the most common initial presenting symptom, noted in 29 (76.3%). Rhino-orbital involvement was noted in 24 (63.2%) and 12 (31.6%) had associated focal neurological deficits. Immunocompromised state was noted in 24 (63.2%). Eighteen (47.4%) patients died. The predictors for mortality: odds ratio (95% CI) were 2.45 (1.01-3.89) for elderly age, 5.67 (4.13-7.21) for intracranial extension, 2.6 (1.26-3.94) for immunocompromised state, 2.62 (1.25-3.99) for infection with zygomycosis and 2.33 (1.01-3.65) for anemia. CONCLUSION: Rhinocerebral mycosis is associated with high mortality in spite of aggressive therapy. Intracranial extension with focal neurological deficits is a major predictor of mortality in rhinocerebral mycosis.