PubMed Health⌕ Search

Biomedical subjects

M C Sharma

Publications and source records attributed to M C Sharma.

At least 37 records · Page 2Linked to original sources
PubMed2005

Hallervorden spatz disease: MR and pathological findings of a rare case.

We describe a child with pathologically proven Hallervorden Spatz disease. He presented with extrapyramidal symptoms and characteristic "eye-of-the-tiger" sign on magnetic resonance imaging. He was given the possible benefit if any of deep brain stimulation with no much improvement. Pathological examination of the brain showed iron deposition in bilateral globus pallidi, spongiform change and neuron axonal degeneration (spheroids).

Child↗
PubMed2004

Primary lumbosacral Wilms tumor associated with diastematomyelia and occult spinal dysraphism. A report of a rare case and a short review of literature.

BACKGROUND: The occurrence of an extrarenal Wilms tumor in the lumbosacral region is an extremely uncommon condition. CASE REPORT: We report a case of Wilms tumor in the lumbosacral region that was associated with diastematomyelia and occult spina bifida. An 18-month-old girl presented with a swelling over the lower back with a tuft of hair on it, which she had had since birth. Imaging of the spine revealed spina bifida, bony diastematomyelia, and tethered cord. Excision of the bony spur and detethering of the cord was done. After a year, she had recurrence of swelling at the same site, weakness of both lower limbs, and incontinence of bladder and bowel. Excision of the mass and bony spur and detethering of the spinal cord were done. Histopathological examination showed features of a Wilms tumor.

Female↗
PubMed2004

Fungal granuloma of the brain caused by Cladosporium bantianum--a case report and review of literature.

Involvement of the brain by neurotropic, dematiaceous fungi namely Cladosporium bantianum is extremely rare. The disease is very resistant to treatment and prone for frequent relapses despite treatment with amphotericin B and flucytosine, the drugs of choice for the infection. Surgery is often required for resection of the fungal granuloma. Isolation of the fungus from the tissue specimens and its culture, showing dark colored fungal colonies clinches the diagnosis. Animal inoculation studies can provide insights to the portal of entry of the organism. We hereby report a case of fungal granuloma of the brain due to C. bantianum, which responded favorably to intensive antifungal treatment alone, with relevant review of literature.

Adult↗
PubMed2004

Central core disease.

Central core disease is a congenital myopathy characterized by generalized hypotonia, muscle weakness and presence of central cores on muscle biopsy. It generally presents during infancy. It is familial with autosomal dominant inheritance [Chromosome 19q13.1; Gene Locus RyR1 (Ryanodine receptor gene)]. We report here two cases of central core disease in a 3-year-old male child and 8 year old female child.

Activities of Daily Living↗
PubMed2004

Leigh's syndrome.

A 15-month-old female child presented with sudden onset cough and hyperventilation along with evidence of metabolic acidosis. She had past history of recurrent vomiting, episodes of abnormal posturing, difficulty in deglutition and regression of milestones since 12 months of age. CT scan of the brain revealed hypodense lesions in bilateral basal ganglia and on MRI there were T2 hyperintensities in bilateral lentiform nuclei, caudate nuclei, thalamus, red nuclei and dentate nuclei. Biochemical examination revealed persistently elevated serum lactate levels with high lactate/pyruvate ratio. Resuscitative measures were of no avail and the child succumbed to the illness on the second day of admission. Neuropathological examination at autopsy demonstrated marked spongiosis, focal necrosis, endothelial proliferation, reactive astrogliosis and extensive demyelination involving bilateral basal ganglia, midbrain and spinal cord which were typical of Leigh's sub acute necrotizing encephalomyelopathy.

Autopsy↗
PubMed2004

Scalp malignant peripheral nerve sheath tumor (MPNST) with bony involvement and new bone formation: case report.

Malignant peripheral nerve sheath tumors (MPNSTs) are rare neoplasms, usually arising from somatic soft tissues or peripheral nerves. Primary MPNST of the scalp is extremely rare, with only a single case reported so far. Here, we describe an unusual case of scalp MPNST in a 50-year-old male. The tumor was associated with bony projection, intracranial extension and underlying bone destruction. The tumor was treated with complete surgical excision followed by adjuvant radiotherapy. Histologically, the tumor showed malignant spindle cells with focal S-100 positivity on immunohistochemistry and a diagnosis of MPNST was made. This case is being reported for its rarity and presence of associated bony projection, which to the best of our knowledge, has not been described before in soft tissue sarcomas.

Head and Neck Neoplasms↗
PubMed2004

Cerebellopointine angle medulloblastoma.

Cerebellopontine angle (CPA) medulloblastoma is a rare tumour. To date, only 19 cases have been reported over a period of 8 years, we have treated 14 cases of CPA medulloblastomas. This is first and the largest series of CPA medulloblastomas reported in the world literature.

Adolescent↗
PubMed2004

Primary plasma cell granuloma of petrous bone.

Plasma cell granulomas are rare intracranial lesions that can mimic a variety of intracranial tumours. As they are usually benign lesions, their identification assumes importance. We report a case of plasma cell granuloma in a 52-year-old man presenting with features of left sided V, VII and bilateral VIII nerve involvement who underwent subtotal excision of the lesion. The relevant literature is reviewed.

Bone Diseases↗
PubMed2004

Pituitary abscess associated with prolactinoma.

We present a 37-year-old female, who was admitted with a long standing history of menstrual abnormalities and galactorrhoea followed by progressive visual loss. X-ray of the skull showed an enlarged sella with destruction of the floor. CT scan of the head revealed a giant pituitary adenoma with sellar and parasellar extension. At surgery, through a transsphenoidal approach, solid tissue and cystic areas containing pus from which Escherichia coli was cultured were encountered. She had an uneventful postoperative recovery. At 2 years follow up, she was doing well.

Abscess↗
PubMed2004

Primary central nervous lymphoma presenting as bilateral cerebellopontine angle lesions: a rare case report.

The authors describe a rare case of primary central nervous system (CNS) lymphoma presenting as bilateral cerebellopontine angle lesions. Imaging showed bilateral cerebellopontine angle lesions and also a small mass in right lateral ventricle and anterior third ventricle region. The right cerebellopontine angle mass was surgically excised and radio- and chemotherapy given for the remaining lesions. This is the second case reported in the world literature. The case is discussed and the literature is reviewed.

Adult↗
PubMed2004

Effects of sarcoptic mange and its control with oil of Cedrus deodara, Pongamia glabra, Jatropha curcas and benzyl benzoate, both with and without ascorbic acid on growing sheep: epidemiology; assessment of clinical, haematological, cell-mediated humoral immune responses and pathology.

The aim of this study was to evaluate the therapeutic efficacy of commonly used acaricidal drugs in India and also to assess the effect of ascorbic acid (AA) as adjunct therapy in 72 growing sheep with sarcoptic mange, aged 5-6 months and weighing 20.4-31.7 kg. Eight replicates of nine animals were formed based on sex, and day 0 body weight. Another set of 12 healthy sheep (5-6 months old) constituted the healthy control group. Drugs were applied locally on the affected parts daily and recovery changes in skin lesions were observed at the time of every application. L-Ascorbic acid was administered intramuscularly. Skin scrapings were collected daily from each group and examined for the presence of mites. Blood samples from each group were collected and analysed for total erythrocytes, leucocytes, haematocrit, haemoglobin and lymphocytes on 0, 14th and 28th day post-treatment (PT). Cell-mediated (CMI) and humoral immune (HI) responses were assessed on 0 and 28 days PT. Within groups treated with the drug alone, recovery in oil of Jatropha curcas group was faster and lesions were free from mites after the eighth application. For each drug, the recovery was faster with AA as an adjunct therapy when compared with the group treated with the drug alone. Within all groups, recovery in oil of J. curcas plus ascorbic acid (OJC-AA) group was fastest and the lesions were mite-free after three applications. The overall mean values were higher for all haematological parameters in OJC-AA group. Recovery with respect to suppressed CMI and HI responses during infection was also faster in this group. Gross and microscopic pathological studies revealed a marked adverse effect of infection on skin, kidney, spleen and liver. The OJC-AA group indicated faster recovery. It is concluded from this study that oil of J. curcas was found more efficacious in controlling sarcoptic mange in sheep and AA might be beneficial as adjunct in case of sarcoptic mange to get quick recovery with lesser applications of main therapy. However, the pharmacology of AA with reference to health of skin needs to be investigated.

Animals↗
PubMed2004

Effects of sarcoptic mange and its control with oil of Cedrus deodara, Pongamia glabra, Jatropha curcas and benzyl benzoate, both with and without ascorbic acid on growing sheep: assessment of weight gain, liver function, nutrient digestibility, wool production and meat quality.

The aim of this study was to evaluate the therapeutic efficacy of commonly used acaricidal drugs in India and also to assess the effect of ascorbic acid as adjunct therapy in 72 growing sheep with sarcoptic mange, aged 5-6 months and weighing 20.4-31.7 kg. Eight replicates of nine animals were formed based on sex, and day 0 body weight. Drugs were applied locally on the affected parts daily and recovery changes in skin lesions were observed at the time of every application. L-ascorbic acid was administered intramuscularly. Skin scrapings were collected daily from each group and examined for the presence of mites. Body weights were measured every 10th day from day 0 to 60. Nutrient digestiblity was evaluated by studying digestibility coefficients for dry matter, crude protein, ether extract, crude fibre, nitrogen free extract, total carbohydrates and nutrient balance (nitrogen, calcium and phosphorus) for a 30-day period. The liver function was evaluated by bromosulphophthalein (BSP) dye retention time. The animals were shorn on day 60 post-treatment (PT). Meat quality assesment was carried out by killing sheep at 60 days PT and estimating pH, water-holding capacity (WHC), tenderness, muscle colour, rib eye area and fat thickness. The lambs treated with oil of Jatropha curcas ascorbic acid had significantly (P < 0.05) greater mean daily body weight gains (63.29 g) than the infected untreated control (41.10 g). This was also higher than the mean daily weight gain in other treated groups. Infected untreated sheep showed significantly (P < 0.01) reduced digestibility coefficients for dry matter, crude protein, crude fibre, ether extract and total carbohydrate, but no significant differences for nitrogen-free extract. Treated sheep had significantly higher positive nitrogen, calcium and phosphorus balances compared with infested untreated sheep. Oil of J. curcas plus ascorbic acid (OJC-AA) treated group was better over all other treated groups with respect to nutrient digestibility. The BSP test revealed significant (P < 0.05) increase in BSP retention time in sheep with sarcoptic mange. Post -treatment, the BSP retention time decreased in all treated groups and the decrease was maximum in OJC-AA treated group. The carcasses of sheep treated with OJC-AA had significantly (P < 0.01) higher water holding capacity, rib eye area and back fat thickness than the untreated infected control group. The muscle pH and tenderness values were significantly lower in OJC-AA treated group post-slaughter than infested untreated control group. Muscle colour of OJC-AA treated group was maximum bright red. The lambs treated with OJC-AA had significantly (P < 0.05) greater clean fleece weight and fleece yield than the untreated infected group. It is concluded that OJC was the better therapy for sarcoptic mange of sheep and ascorbic acid as adjunct therapy is advisable. OJC-AA therapy may be better from the point of view of improving two most important production parameters in sheep, that is, wool yield and meat production.

Animals↗
PubMed2004

Congenital fiber type disproportion: a rare type of congenital myopathy: a report of four cases.

Congenital fiber type disproportion is a rare type of congenital myopathy which presents as hypotonia, delayed motor milestones and dysmorphic facies. During the past 2 years we received 449 muscle biopsies, of which 4 cases were diagnosed as congenital fiber type disproportion (CFTD). In addition to CFTD, one case also had centronuclear features. Three of them were females and one was a male child. Although rare, it should be considered in the differential diagnosis of childhood muscle diseases. Histochemical staining is necessary for the diagnosis of this entity.

Adolescent↗
PubMed2004

Sarcoglycanopathies: a clinicopathological study of 13 cases [corrected].

BACKGROUND: Limb girdle muscular dystrophy (LGMD) is a phenotypic expression of a heterogeneous group of diseases and sarcoglycanopathy is one of the causes of LGMD. There is only one study on sarcoglycanopathies in the Indian literature. No data is available from northern India. MATERIALS AND METHODS: All cases of muscular dystrophies, which were diagnosed in our laboratory in the last six years, were reviewed. Immunohistochemistry for various sarcoglycan proteins was done. Clinical features and pathological findings of the cases that were diagnosed as sarcoglycanopathies were reviewed. RESULTS: In the last 6 (1/2) years (1998-June 2004), we received 1435 muscle biopsies, of which 498 cases were of muscular dystrophies, and 13 cases were of sarcoglycanopathies (8 of gamma, 3 of alpha, 1 of both alpha and gamma, and 1 with absence of all four sarcoglycans). Sarcoglycanopathies comprised 2.6% of all muscular dystrophies, 11.8% of LGMD and 0.90% of all muscle diseases diagnosed in our laboratory. The mean age of onset was 7.2 years and the M:F ratio was 1.1:1. Most of them presented with difficulty in getting up, climbing stairs, calf hypertrophy and markedly raised CPK levels. Histological features were like dystrophinopathies. CONCLUSION: Sarcoglycanopathies are a relatively rare cause of LGMD and should be confirmed by immunohistochemistry as it will facilitate counseling and also prognostification. Although rare, in patients with muscle weakness, calves hypertrophy and raised CPK levels this possibility should be considered and needs to be differentiated from dystrophinopathies.

Adolescent↗
PubMed2003

Clear cell odontogenic carcinoma: a diagnostic dilemma.

A 26-year-old man presented with a swelling in the right side of face and CT scan revealed a destructive tumor in the right maxilla. Tumor recurred within 5 years of its excision and histopathological examination revealed a clear cell odontogenic carcinoma. The rarity of this tumor, occurrence in maxilla and young age of the patient are some of the rare features which need documentation. The importance of its diagnosis and various differential diagnoses are discussed.

Adenocarcinoma, Clear Cell↗
PubMed2003

Biochemical characterization of muscle tissue of limb girdle muscular dystrophy: an 1H and 13C NMR study.

The metabolic differences between the muscle biopsies of patients with limb girdle muscular dystrophy (LGMD) and normal controls were characterized using high-resolution 1H and 13C NMR spectroscopy. In all, 44 metabolites were unambiguously assigned in the perchloric acid extracts of skeletal muscle tissue, using 2D double quantum filtered (DQF COSY), total correlation (TOCSY), and 1H/13C heteronuclear multiple quantum coherence (HMQC) spectroscopy. The concentrations of glycolytic substrate, glucose (p=0.03), gluconeogenic amino acids, glutamine (p=0.02) and alanine (p=0.009) together with glycolytic product, lactate (p=0.04), were found to be significantly lowered in LGMD patients as compared with controls. The reduction in the concentration of glucose may be attributed to the decrease in the concentration of gluconeogenic amino acids in the degenerated muscle. Reduction in the rate of anaerobic glycolysis and lowered substrate concentration appear to be the possible reasons for the decrease in the concentration of lactate. A significant reduction in the concentration of choline in LGMD patients was also observed compared with controls. Lower concentration of choline may be the result of decreased rate of membrane turnover in LGMD patients. The data presented here provide an insight into the potentials of in-vitro NMR spectroscopy in the study of muscle metabolism.

Amino Acids↗
PubMed2003

Skeletal muscle metabolism in Duchenne muscular dystrophy (DMD): an in-vitro proton NMR spectroscopy study.

The metabolic differences in the skeletal muscle of patients with Duchenne muscular dystrophy (DMD) and normal subjects (controls) were investigated using in-vitro high-resolution proton NMR spectroscopy. In all, 56 metabolites were unambiguously identified in the perchloric acid extract of muscle tissue using one- and two-dimensional NMR. The concentrations of glycolytic substrate glucose (Glc; p < 0.05), gluconeogenic amino acids such as glutamine (Gln; p < 0.05) and alanine (Ala; p < 0.05) and the glycolytic product lactate (Lac; p < 0.05) were statistically significantly lower in DMD patients as compared to controls. A significant reduction in the concentrations of total creatine (TCr; p < 0.05), glycerophosphoryl choline + phosphoryl choline + carnitine (GPC/PC/Car; p < 0.05), choline (Cho; p < 0.05) and acetate (Ace; p < 0.05) was also observed in these patients. Decrease in the level of glucose may be attributed to the reduction in the concentrations of gluconeogenic substrates or membrane abnormalities in degenerated muscle of DMD patients. Lower levels of choline containing compounds indicate membrane abnormalities. Decrease in the concentration of lactate in the muscle of DMD patients may be due to the reduction in anaerobic glycolytic activity or lower substrate concentration. The decrease in the concentration of acetate may reflect reduced transport of fatty acids into mitochondria due to decreased concentration of carnitine in DMD patients. Kreb's cycle intermediate alpha-ketoglutarate was observed only in the diseased muscle, which is suggestive of predominant oxidative metabolism for energy generation.

Adolescent↗