Cellulolytic Activity of Thermophilic Bacilli Isolated from Tattapani Hot Spring Sediment in North West Himalayas.

Eight thermophilic bacterial strains were isolated from Tattapani Hot spring and screened for various hydrolytic enzymes including cellulases. The isolated bacterial strains were identified as Geobacillus thermodenitrificans IP_WH1(KP842609), Bacillus licheniformis IP_WH2(KP842610), B. aerius IP_WH3(KP842611), B. licheniformis IP_WH4(KP842612), B. licheniformis IP_60Y(KP842613), G. thermodenitrificans IP_60A1(KP842614), Geobacillus sp. IP_60A2(KP842615) and Geobacillus sp. IP_80TP(KP842616) after 16S ribotying. Out of the eight isolates Geobacillus sp. IP_80TP grew best at 80 °C whereas rest of the isolates showed optimal growth at 60 °C. G. thermodenitrificans IP_WH1 produced a thermotolerant cellulase with maximum activity at 60 °C.

Hypersexual features in Huntington's disease.

We report the case of a 30-year-old woman with a rare presentation of early adulthood Huntington's disease (HD) with hypersexuality. It is not known if sexual dysfunction in HD patients is due to a specific brain lesion or adverse psychosocial factors associated with HD. Although there are no evidence-based treatment guidelines for hypersexuality in HD, our patient exhibited significant improvement with olanzapine and haloperidol.

Cardiovascular autonomic neuropathy in chronic kidney diseases.

INTRODUCTION: This study was conducted to explore cardiovascular autonomic neuropathy and its pattern in chronic kidney disease patients. METHODS: Autonomic function using five standard tests was examined in 20 diabetic patients with CKD, 20 age and sex matched diabetic patients without CKD and 20 age and sex matched controls. Analysis of difference between the autonomic function was done in the three groups using Chi-square test or Fischer's test. RESULTS: Total 20 (100%) diabetic CKD patients were found to have autonomic neuropathy. Of these, 2 (10%) patients had early parasympathetic damage, 8 (40%) patients had definite parasympathetic damage, and 10 (50%) patients had combined damage. Heart rate response to standing was statistically significant (p = 0.014) among diabetic CKD patients when compared with diabetic patients without CKD. Combined form of autonomic dysfunction was more frequent in advanced stages of diabetic CKD. Three (42.85%) patients in stage 3 CKD, 4 (66.66%) patients in stage 4 CKD and 5 (71.42%) patients in stage 5 CKD, had combined autonomic failure. CONCLUSIONS: Autonomic neuropathy is common in native Nepalese diabetic CKD patients. Heart rate response to standing is significantly abnormal in diabetic CKD patients in comparison with diabetes mellitus patient without CKD. Severity of autonomic dysfunction increases with severity of CKD..

An interesting case report of Morvan's syndrome from the Indian subcontinent.

The French physician Augustine Marie Morvan first used the term 'la choriotae fibrillare' to describe a syndrome characterized by peripheral nerve hyperexcitability, dysautonomia, insomnia and fluctuating delirium. There are no published reports of the condition from the Indian subcontinent. We report the first such case from the region. Our patient, a 24-year-old male, presented with easy fatigability and stiffness in both the calves for 18 months; continuous twitching of muscles of all four limbs and jaw for two months; hyperhydrosis, palpitations, urinary symptoms, burning dysesthesia in hands, insomnia and abnormal sleep behavior for about a month. Patient had bilateral hyper-reflexia with extensor plantar on the right and equivocal response on the left. Electromyography revealed continuous muscle fiber activity. Thyroid function test, electroencephalography, computerized tomography scan (head) and routine cerebrospinal fluid analysis were normal. The patient showed marked clinical and electrophysiological improvement on prednisolone along with symptomatic therapy over the next two months.

IgA monoclonal gammopathy of undetermined significance with peripheral neuropathy.

Monoclonal gammopathy of undetermined significance (MGUS) and peripheral neuropathy may be causally linked. In most cases, the M-protein is of IgG or IgM type. Peripheral neuropathy associated with IgA MGUS is uncommon, and there are limited reports. Here, we report a case of a 55-year-old male who was diagnosed to have symmetrical sensorimotor peripheral neuropathy associated with IgA MGUS with deposits of IgA-monoclonal protein in the myelin sheath.

Autonomic neuropathy in patients with hepatic cirrhosis.

BACKGROUND: Autonomic neuropathy has been reported in patients with alcoholic liver disease but information on its occurrence in patients with non-alcoholic liver disease is contradictory. AIM: To assess autonomic functions in patients with alcoholic and non-alcoholic liver disease. STUDY DESIGN: Autonomic function using five standard tests was examined in 20 cirrhotics (10 alcoholics and 10 non-alcoholics) and 20 age and sex matched controls. The extent of autonomic dysfunction was determined in the patients and a comparison between the characteristics of patients with and without autonomic neuropathy was made. RESULTS: Sixteen (80%) of the cirrhotic subjects were found to have evidence of autonomic neuropathy. Of these, three (15%) patients had early parasympathetic damage, five (25%) had definite parasympathetic damage, and eight (40%) had combined (that is, both parasympathetic and sympathetic) damage. Nine (90%) of the alcoholics and seven (70%) of the non-alcoholics had autonomic dysfunction. Only one patient belonging to the alcoholic group had clinical evidence of peripheral neuropathy. Moreover, there was no significant association between subjective symptoms of autonomic neuropathy and objective evidence of autonomic damage as assessed by autonomic function tests. Autonomic dysfunction was significantly more frequent in advanced liver disease compared with early liver damage. Nine (75%) out of 12 cirrhotic subjects belonging to Child class B and six (85.7%) of the seven patients belonging to Child class C had autonomic neuropathy. CONCLUSION: This study shows that autonomic neuropathy is common in cirrhotic subjects, that it is found with comparable frequency in alcoholics and non-alcoholics, and that it increases in severity with increase in extent of liver damage, suggesting that liver damage contributes to the neurological deficit.

Paradoxical response in patients with CNS tuberculosis.

AIM: To report paradoxical response, i.e. recurrence of appearance of fresh symptoms, physical and radiological signs in a patient who had previously shown improvement with appropriate anti-tubercular medicines. MATERIAL AND METHODS: Ten out of 58 patients of CNS tuberculosis, diagnosed on basis of clinical, laboratory and radiological data that initially showed clinical response to therapy only to deteriorate later were included in the study. RESULTS: Out of ten, three were males and seven were female with age range 13 to 28 years. The duration of time between initiation of therapy and worsening of patient was from one to seven months. Nine out of ten patients developed fresh intracranial tuberculoma while one case otherwise showing improvement developed expansion of tuberculoma and other one of empyema developed tuberculoma while on therapy. All these cases responded to addition of second line therapy or increase in dose of drugs previously prescribed and introduction or increased dose of steroid. CONCLUSIONS: Clinical judgement, regular follow up, guarded reassurance of patient is required to detect parodoxial response in CNS tuberculosis.

Creutzfeldt-Jakob disease : report of 10 cases from North India.

Creutzfeldt-Jakob disease (CJD) is increasingly being reported over the last three decades as a result of heightened awareness of the disease. Various studies have reported annual incidence of 0.5-1.5 cases of CJD per million of general population. In India, the disease is still under reported. Over the period spanning from 1968-1997, National Institute of Mental Health and Neurosciences (NIMHANS), Bangalore recorded 69 cases of CJD from different parts of India in the CJD registry. This paper describes the clinical experience with cases of CJD managed at the Department of Neurology, G.B. Pant Hospital, New Delhi from 1990-1998. In this series, the mean age of the patients was 53.80 (+/- 7.32) years and there were 5 females and 5 males. Myoclonus was present in all the cases and abnormal behaviour with or without other features was the presenting complaint in 7 of the 10 patients, while one patient of CJD had cerebellar ataxia as the presenting feature. One patient with occipital variant of CJD presented with acute onset cortical blindness and myoclonic jerks. One of the patients had acute psychosis precipitated by emotional stress at the onset. Extrapyramidal features were noted in 7 of the 10 patients before death. The mean duration of symptoms from the onset of disease to death was 6.6 (+/- 6.11) months. Classical EEG changes were observed in all the patients, except in one possible case of occipital variant of CJD, where we did not have access to EEG record. Brain biopsy could be undertaken in 3 patients, and in 2 patients the features of subacute spongiform encephalopathy (SSE) were noted.

Metabolism of oestradiol-17 beta and oestrone in the human uterus.

A study of the metabolism of oestradiol in the human endometrium and myometrium of the proliferative and secretory phases of the cycle showed that the conversion of oestradiol to oestrone by endometrium in the proliferative phase was higher than that in the secretory phase. The decreased metabolic activity of the secretory phase endometrium was attributed to the influence of progesterone on the endometrium. The metabolic conversion of oestradiol to oestrone was enhanced when pyridine nucleotides were added to the system. The conversion of oestradiol to oestrone was maximum in the cytoplasmic and nuclear fractions of the endometrium. Furthermore, the conversion of oestradiol was low in all the subcellular fractions of the myometrium as compared with the endometrial subcellular fractions. The presence of co-factors increased the metabolic conversion of oestradiol to oestrone in the subcellular fractions of the endometrium. The presence of 17 beta-hydroxysteroid oxidoreductase was indicated in all the subcellular fractions. A correlation was found between the amount of oestradiol and oestrone bound to the receptors in the uterus and the rate of metabolism of oestradiol in the uterus. The physiological significance of metabolism of oestradiol and the hormone action are discussed.