Browsing by Author "Sharma, M C"

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    Adhalin deficiency: an unusual cause of muscular dystrophy.
    (2001-11-05) Dua, T; Kalra, V; Sharma, M C; Kabra, M
    Childhood muscular dystrophies have a wide clinical spectrum, motor disability and are variably inherited. Although the phenotype may appear similar they may represent distinct genetic entities. Advances in immunohistochemistry, gene deletion and linkage studies have enabled precise characterization. We report a family with an early onset weakness and calf pseudo hypertrophy in 2 male sibs with an usually mild course. Deletion screening was negative for 24 exons of the DMD gene in both. Muscle immunohistochemistry revealed normal dystrophin I and II staining but complete absence for adhalin, a dystrophin associated glycoprotein. Classifying them as adhalinopathy. Severe childhood autosomal recessive muscular dystrophies (SCARMD) result from mutation in the sarcoglycan complex (59). Adhalinopathy is now used to describe SCARMD. The adhalinopathy described in our patients is the first report from India.
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    Assessment of proliferative potential of meningiomas using PCNA LI and AgNOR counts.
    (1998-07-07) Sudha, K; Karak, A K; Sharma, M C; Mathur, M; Sarkar, C
    Seventy-six cases of international meningiomas were studied using staining for Proliferating Cell Nuclear Antigen (PCNA) and silver nucleolar organizer regions (AgNORs) in order to find out any correlation of these parameters to the histological typing of the tumours and their biological behaviour. Histologically, 35 of the 76 cases were benign, 12 atypical and 19 malignant. Ten cases were recurrent. A male preponderance with a male: female ratio of 1.6:1 was noted. Five of 76 cases were in children below 15 years of age. Majority of the tumours were convexity meningiomas. Overall PCNA labeling index (LI) values ranged from 0.1% to 11.0%. Benign group had an LI of 0.9 +/- 1.42 whereas atypical, malignant and recurrent group had LIs of 4.06 +/- 2.33, 2.91 +/- 2.66 and 3.36 +/- 3.76 respectively. One way analysis of variance test showed a significant difference in the distribution of LI between benign versus atypical, malignant and recurrent group (P < 0.05). A highly significant difference was also observed between PCNA LI of recurrent benign group versus non-recurrent benign group (p < 0.01, wilcoxon Rank Sum Test). On further classifying the tumours based on LI values, it was observed that 30 of 41 (73%) cases of combined atypical, malignant and recurrent group (i.e. biologically more aggressive group) had LI of > 1%, whereas 26 of 35 cases (74%) belonging to the benign group had LI of < 1%. Overall AgNOR counts ranged from 1.27 to 3.11. No statistically significant difference was found in AgNOR counts amongst the different groups of meningiomas. There was no correlation between PCNA LI and AgNOR counts. It was thus concluded that PCNA LI but not AgNOR counts in the primary tumour could be of potential value for more accurate assessment of biologic behaviour of meningiomas in conjunction with the conventional A histological parameters.
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    Autopsy always teach and tell: neurodegeneration with brain iron accumulation: a case report.
    (2007-10-01) Gupta, Ruchika; Kumar, Ashok; Sharma, M C; Sarkar, Chitra; Goyal, Vinay; Bihari, Madhuri
    Neurodegeneration with brain iron accumulation (NBIA), or Hallervorden- Spatz disease, is an extremely rare autosomal recessive disorder with cysteine-iron complex accumulation in globus pallidus, seen histopathologically. Magnetic resonance imaging offers an opportunity for diagnosis; however, therapeutic options are still ineffective. We report a case of 13-year-old girl, symptomatic since the age of three years with dystonia, poor scholastic performance and speech disturbances. She was admitted with aspiration pneumonia, and died before she could be investigated. Examination of brain at autopsy revealed iron deposition in bilateral globus pallidi, confirmed by special stains and elemental dispersion analysis by spectrometry and a diagnosis of Hallervorden- Spatz disease or NBIA was made. This report highlights the importance of autopsy and scanning electron microscopic examination in unsuspected cases where cause of death is not known.
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    Biochemical investigations of streptomycin production by Streptomyces griseus.
    (1983-02-01) Singh, V P; Narang, J S; Sharma, M C; Singh, D V
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    Central core disease.
    (2004-11-02) Gulati, Sheffali; Salhotra, Amandeep; Sharma, M C; Sarkar, Chitra; Kalra, Veena
    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.
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    Cerebellar medulloblastoma in a 65 year old Indian male.
    (2000-06-06) Jaiswal, A K; Chandra, P S; Kamal, R; Sharma, M C; Mahapatra, A K
    A case of cerebellar medulloblastoma in a 65 year old male is reported. Cerebellar medulloblastoma is classically seen during childhood, and less than 25% of these tumours are found in adults below 40 years of age. Rarely, cases are reported above the age of 40 years. So far only three cases have been reported in patients aged above 64 years and none of these case reports are from India.
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    Chemotherapy induced refractory anaemia with ring sideroblasts in carcinoma breast.
    (1994-02-01) Dhot, P S; Sharma, M C; Kumar, L; Saxena, R
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    Clinical course and management of pancreatoblastoma in children.
    (1998-04-30) Kataria, R; Bhatnagar, V; Agarwala, S; Sharma, M C; Gupta, A K; Mitra, D K
    Pancreatoblastoma is a rare malignant tumour. Two children with this tumour were managed in the last 2 years. Both presented with progressively increasing abdominal mass. The diagnosis was established only after laparotomy. In the first child, an 8 year old girl, the mass was arising from the body of the pancreas and only incomplete resection was feasible. She received postoperative chemotherapy and went into remission for a few months before presenting with jaundice and abdominal pain due to recurrent, metastatic disease in the liver and porta hepatitis. Further therapy was refused by the patient because of anorexia and social problems. The second patient, a 5-year-old girl, underwent distal pancreatectomy for complete removal of a large mass arising from the tail of the pancreas. Chemotherapy was begun postoperatively but discontinued by the patient. However, she has remained disease free 1 year after diagnosis. Histologic, histochemical and ultrastructural features of the tumour are detailed and the management discussed.
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    A clinico-pathological study of 29 cases of gliosarcoma with special reference to two unique variants.
    (1997-09-05) Sarkar, C; Sharma, M C; Sudha, K; Gaikwad, S; Varma, A
    In the present series, the clinical and pathological features of 29 patients of gliosarcoma diagnosed over a 12 yr period (1984-1995) are reviewed. Gliosarcomas constituted 0.48 per cent of all intracranial tumours and 4.9 per cent of all cases of glioblastoma multiforme. Most patients (68.6%) with these tumours were above 40 yr of age. However, an interesting observation in the present series was that 10.3 per cent of patients (3/29) were below 14 yr of age, the youngest being 9 months. A male preponderance was noted and the temporal lobe was involved in 55 per cent patients. Histologically, in 25 of the 29 tumours, the sarcomatous component had the appearance of fibrosarcoma. Tumours from 4 patients were unique in that one showed rhabdomyoblastic differentiation in the mesenchymal areas as confirmed by immunohistochemical stains and electron microscopy (gliomyosarcoma). In three others, the neoplastic spindle cell component was closely associated with discrete areas of osteogenic sarcoma. Follow up in 12 patients (including the 4 patients with unique variants) revealed poor outcome similar to glioblastomas. All of them died within 1 month to 1.5 yr following surgery and postoperative radiotherapy. This study possibly represents the most comprehensive and largest series of gliosarcomas being reported from India.
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    A comparative study of classical vs. desmoplastic medulloblastomas.
    (2003-03-17) Pramanik, P; Sharma, M C; Mukhopadhyay, P; Singh, V P; Sarkar, C
    Classical and desmoplastic medulloblastomas (MBs) have been suspected to be biologically different, though comparative studies on markers of biological aggressiveness in these two variants are sparse in the literature. 87 classical and 43 desmoplastic variants of MB were studied with respect to clinical and histological characteristics, MIB-1 labeling index (MIB-1 LI), apoptotic index (AI), ratio of AI to MIB-1 LI, expression of p53 and Bcl-2 protein and 3-year progression-free survival. The only differences documented between the variants were with regard to age distribution and location. Thus, classical histology cases occurred predominantly in children and 80% were midline in location. In contrast, lateral location was seen more frequently with tumors of desmoplastic histology, which occurred in an almost equal distribution between children (56%) and adults (44%). No difference was noted between the variants with regard to proliferation index, apoptotic index, their ratio on or their molecular controls (p53 and Bcl-2). This was reflected in the clinical outcome wherein no significant difference was observed in the 3-year progression-free survival between the variants. It is concluded that the two histological variants of medulloblastoma are not different with regard to biological parameters of aggressiveness. The growth rate and clinical outcome in medulloblastomas have no correlation with the histological variant.
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    Congenital fiber type disproportion: a rare type of congenital myopathy: a report of four cases.
    (2004-06-23) Sharma, M C; Ralte, A M; Atri, S K; Gulati, S; Kalra, V; Sarkar, C
    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.
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    Congenital orbital teratoma: a report of two cases.
    (1997-03-01) Sharma, M C; Sarkar, C; Gaikwad, S; Mahapatra, A K; Bahadur, S
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    Dedifferentiated chondrosarcoma of the maxilla.
    (2007-01-14) Munshi, Anusheel; Atri, Surinder Kumar; Pandey, Kailash Chander; Sharma, M C
    A 20-year-old male reported with right-sided facial swelling, epistaxis and right sided proptosis of two months duration. Computerized tomography scan of the face revealed a mass in the right maxillary sinus. The patient underwent surgery and the postoperative histopathology was suggestive of dedifferentiated chondrosarcoma. The present report discusses this rare presentation and the issues in its management.
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    Demonstration of Leishmania parasites in skin lesions of Indian post kala-azar dermal leishmaniasis (PKDL) cases.
    (2000-03-29) Sharma, M C; Gupta, A K; Verma, N; Das, V N; Saran, R; Kar, S K
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    Desmin-related myopathy: report of a rare case.
    (2005-06-13) Sridhar, E; Sharma, M C; Sarkar, C; Singh, S; Das, T
    The Protein Surplus Myopathies (PSM) are characterized by accumulation of protein aggregates, identifiable ultrastructurally, resulting due to mutations of the encoding genes. Desmin-related myopathies (DRM) are a form of PSM characterized by mutations of the desmin gene resulting in the formation of protein aggregates comprising mutant protein desmin and disturbance of the regular desmin intermediate network in the muscle fibers. We describe a rare case of DRM in a 23-year-old man who presented with complaints of difficulty in climbing stairs and running since the age of 5 years. EMG studies revealed a myopathic pattern. Muscle biopsy showed the features of muscular dystrophy with bluish rimmed vacuoles and sarcoplasmic inclusions, which were immunoreactive to desmin. Ultrastructural examination showed sarcoplasmic bodies and granulofilamentous inclusions. Although rare, the possibility of DRM/desminopathy should be considered in the presence of bluish rimmed vacuoles on light microscopy and characteristic ultrastructural inclusions. To the best of our knowledge this is the first case of DRM/desminopathy reported from India.
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    A diagnostic dilemma: pulmonary blastoma.
    (1996-10-01) Sandhu, M; Sharma, M C; Kamble, R M
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    Diagnostic yield in computed tomography guided stereotactic biopsies.
    (1998-05-29) Sharma, M C; Singh, A; Verma, A; Gaikwad, S; Sarkar, C
    Fifty three patients underwent computerised tomography (CT) guided stereotactic biopsies from different CT defined zones of attenuation with the Leksell stereotactic apparatus from October 1993 through January 1995. Multiple lesions were seen in 16 cases and 3 of them had multiple rim enhancing lesions. Astrocytoma was the most common histological diagnosis and thalamus was the commonest site of these tumours. The overall positivity rate was 98.2%. Positive yield from the centre of the lesion, peripheral and perilesional areas was 92.1%, 54.7% and 17.6%, respectively. The definite pathological diagnosis was made in 81.1% of cases. Post-operative neurological worsening was seen in 6 patients, of which 2 recovered without any surgical treatment, in 1 patient ventriculo-peritoneal shunt was done post-biopsy whereas in another evacuation of hamatoma was done which relieved headache and vomiting while 2 patients (3.7%) died.
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    The effect of age and sex on incidence of kala-azar.
    (1990-12-01) Sharma, M C; Gupta, A K; Saran, R; Sinha, S P
    Of 89 persons suspected to be suffering from kala-azar, the parasite could be demonstrated in 54 of them by smear and culture examination of the bone marrow aspirates. In the pre-pubertal age group (4-12 years), incidence of kala-azar in both the sexes was practically the same (P greater than 0.05). However, in the reproductive age-group (males 13-60 years, females 12-45 years), there were significantly less cases in females than males (P less than 0.05). This protection could be due to the presence of female sex hormones. The incidence of kala azar in females was 83.3 per cent in pre-pubertal age group, while in the reproductive age group, it was 35.5 per cent. The difference is statistically highly significant (P less than 0.01).
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    Effects of elevated temperature and lower pH on infectivity of Leishmania donovani promastigotes.
    (1999-12-11) Gupta, A K; Narayan, S; Sharma, M C; Saran, R; Kar, S K