Hirayama Disease: A Rare Case Report and Review.

Hirayama disease, or brachial monomelic amyotrophy, is not a common neurological disease characterized by unilateral or asymmetric bilateral lower motor weakness of distal upper limbs. The basic pathophysiology is compression of the dural sac and spinal cord during flexion of the neck. A case of a 21-year-old male presented with chief complaints of tremors in both hands (right more than left) with gradually progressive weakness of the right hand and forearm. Electromyography (EMG), nerve conduction velocity (NCV), and magnetic resonance imaging (MRI) neck in flexion showed focal atrophy of lower cervical myotomes and confirmed the diagnosis of monomelic amyotrophy.

Association of Fasting Proinsulin Levels with Glycemic Profile in Indian Type 2 Diabetes Patients.

Type 2 diabetes mellitus is a major cause of cardiovascular disease and mortality, with mortality rate 27% higher in the diabetes cohort. Hyperproinsulinemia is a sign of beta cell dysfunction that is augmented by the increased demands placed on beta cell by chronic hyperglycemia. Hyperproinsulinaemia is the result of secretion of immature proinsulin-rich granules from beta cells, as a response to an increased demand for insulin i.e. an insulin resistant state According to previous studies intact proinsulin was a stronger predictor for type 2 diabetes than specific insulin. We plan to confirm this finding in the Indian demographic. MATERIAL: An observational cross-sectional study was carried out in LHMC with 150 subjects having type 2 diabetes aged between 35-80 years. The subjects taking insulin or any diabetogenic drugs; with history of chronic respiratory, cardiac or metabolic illness other than diabetes were excluded from the study. laboratory examination was conducted after drawing 10ml of venous blood and patients consent. 3 ml of the blood was stored after centrifugation at -20c for assay of fasting proinsulin level. Fasting blood glucose, HbA1c and lipid profile were analyzed. Co-relation between said parameters was established using spearman test of correlation and Wilcoxin-Mann-Whitney U test was used to make comparisons. OBSERVATION: Assessment of glycemic profile of the study population revealed half the subjects having FBS >150mg/dl (n=78, p= 52.0%). Mean HbA1c was 8.40 ± 2.26% with 61 subjects having a well-controlled HbA1c of <7.5%, 35 with 7.5-9.0 % and 54 with HbA1c >9.0%. Association of pro insulin with FBS and HbA1c was positive and strongly significant (rho=0.26 & p value= 0.001, rho=0.23& p value= 0.005 respectively). The mean values of proinsulin in normoglycemic and hyperglycemic subjects were 12.1ug/ml and 17.4 ug/ml respectively. Association of proinsulin with triglyceride levels was found to be positive and significant (rho= 0.22, p value= 0.008). The mean value of pro insulin in subjects with hypertriglyceridemia was 17.9ug/ml as compared to 12.7ug/ml in normal subjects. CONCLUSION: The results of the study demonstrated significant positive association between fasting proinsulin levels and glycemic indicators (p value FBS =0.001, HbA1c= 0.005, triglyceride =0.008). Proinsulin however has multiple associations with the diabetes pathophysiology which need to be studied further. Other studies have also demonstrated proinsulin to be an independent cardiovascular risk factor by stimulating plasminogen activator inhibitor-1 secretion and blocking fibrinolysis. Hence, proinsulin needs to be evaluated for use as a early marker for diabetes progression.

Spot Urine Albumin Creatinine Ratio can be a Predictor of Cognitive Function in Type 2 Diabetes Mellitus.

INTRODUCTION: In diabetes mellitus (DM), the underlying pathophysiology of albuminuria and cognitive dysfunction is similar. So, we hypothesized that urinary albumin excretion (UAE) could be linked to cognitive dysfunction in type 2 diabetes mellitus. METHODS AND MATERIALS: It was a hospital-based observational study. Patient aged 40-60 years with type 2 DM were included in this study. Complete assessment with detailed history, physical examination, and necessary biochemical investigations including spot urine albumin creatinine ratio (uACR) was done. Cognitive status was determined in all the individuals with the application of Hindi translated version of the mini-mental status examination (MMSE) questionnaire. RESULTS: In 80 patients, the mean MMSE score was 25.37 ± 3.34. Cognitive dysfunction (score <26) was present in 45% of individuals. Spot uACR, estimated glomerular filtration rate (eGFR), glycated hemoglobin (HbA1c), presence of retinopathy and dyslipidemia were significantly different between the normal and subnormal scoring groups. On multivariate analysis spot uACR was found to be independently predicting odds of developing cognitive dysfunction (OR 1.01, CI 1.004-1.022; P = 0.001). The mean MMSE scores in normo-albuminuric (n = 15), moderately increased albuminuric (n = 48) and severely albuminuric (n = 17) patients were 28.00 ± 1.60, 25.54 ± 3.33 and 22.58 ± 2.31, respectively, which were significantly different among the three groups (P < 0.001). CONCLUSIONS: Spot uACR could be helpful in predicting cognitive decline in people with type 2 DM.

Catatonia - an unusual presenting clinical manifestation of systemic lupus erythematosus / Catatonía - manifestación clínica de presentación infrecuente del lupus eritematoso sistémico

A 24-year-old female presented with catatonia and symptoms suggestive of Depressive Disorder. She also gave history of undocumented low grade irregular fever. The patient was worked up to rule out any organic cause or psychiatric illness. However, further investigations revealed immunological profile diagnostic of Systemic Lupus Erythematosus (SLE) with CNS involvement (CNS lupus). The diagnosis of SLE in this patient presenting with catatonia was of practical importance because catatonia as one of the manifestations of SLE or as a standalone presenting symptom is extremely rare. Hence, clinicians should be aware of this rarity so that diagnosis of Neuropsychiatric SLE (NPSLE) or catatonia as a presenting feature of SLE is never missed (AU)

Mujer de 24 años que se presentó con catatonía y síntomas indicativos de trastorno depresivo. También presentó historia de febrícula discontinua no registrada. Se llevó a cabo evaluación diagnóstica para descartar cualquier causa orgánica o enfermedad psiquiátrica. Sin embargo, exploraciones complementarias posteriores revelaron un perfil inmunológico diagnóstico de lupus eritematoso sistémico (LES) con implicación del SNC (lupus del SNC). El diagnóstico de LES en esta paciente que se presenta con catatonía era de significado práctico ya que la catatonía, como una de las manifestaciones del LES o como un síntoma que se presenta de forma independiente, es extremadamente rara. Por ello, los médicos deben ser conscientes de esta rareza y no deben olvidar nunca el diagnóstico de LES neuropsiquiátrico (LESNP) o catatonía como rasgo presente en el LES (AU)

Catatonia - An unusual presenting clinical manifestation of systemic lupus erythematosus.

A 24-year-old female presented with catatonia and symptoms suggestive of Depressive Disorder. She also gave history of undocumented low grade irregular fever. The patient was worked up to rule out any organic cause or psychiatric illness. However, further investigations revealed immunological profile diagnostic of Systemic Lupus Erythematosus (SLE) with CNS involvement (CNS lupus). The diagnosis of SLE in this patient presenting with catatonia was of practical importance because catatonia as one of the manifestations of SLE or as a standalone presenting symptom is extremely rare. Hence, clinicians should be aware of this rarity so that diagnosis of Neuropsychiatric SLE (NPSLE) or catatonia as a presenting feature of SLE is never missed.

Herpes Zoster as the Presenting Manifestation of Systemic Lupus Erythematosus (SLE): A Rare Case Report.

Systemic Lupus Erythematosus (SLE) is a chronic autoimmune disease and is usually diagnosed with the SLICC criteria. Here we report a case of SLE presenting as Herpes Zoster (HZ). She had presented with painful vesicular eruptions from 8(th) thoracic nerve to 10(th) thoracic nerve segments and oliguria. There were no clinical manifestations suggestive of SLE. However, on further workup, haematological and immunologic laboratory profiles were suggestive of SLE. A diagnosis of lupus nephropathy was confirmed by renal biopsy and final diagnosis of SLE as the underlying systemic illness associated with HZ was established. We report this case because this patient had none of the manifestations of SLE, as a result of which this would have been an incomplete diagnosis.