Non-resolving liver abscess with Echinococcus cross-reactivity in a non-endemic region.

A 40-year-old woman presented with high fever with chills and rigors. Imaging studies revealed multiple liver abscesses with hepatosplenomegaly and gall-stones. Ultrasound-guided aspirate revealed pus that was negative on Gram and acid-fast staining and for amebic trophozoites. ELISA for echinococcus was strongly positive, but she did not respond to albendazole therapy. At surgery, Fasciola hepatica was detected and she responded well to bithinol postoperative.

Gangliogliomas: A report of five cases.

Gangliogliomas are rare tumors of the Central Nervous System. Five gangliogliomas were diagnosed out of 1560 brain tumours surgically resected out in a period of 5 years accounting for 0. 32%. We have tried to discuss in detail the pathological features of these tumours and have mentioned the clinical and radiological features associated with them. All the slides, tissue blocks and pathology reports of the surgical specimens of gangliglioma were reviewed and the clinical and radiological data reviewed. The ages of the patients ranged from 7-65 years with 4 males and 1 female. The tumors were located in the lateral ventricle (a rare site), temporal, parietal and the frontal lobes with duration of seizures varying from 1-9 years. The tumors were diagnosed by the presence of a dual population of neoplastic ganglionic and glial components. The glial components consisted of pilocytic astrocytes (l case), fibrillary astrocytes (2 cases), oligodendrocytes (1 case) and anaplastic astrocytes and oligodendrocytes (1 case). There was one-grade I GG, three-Grade II GGs and one-grade III GG. Astrocytes were the commonest glial component of GGs, either pilocytic or fibrillary. Oligodendrocytes as the glial component of GGs was seen in 2 cases one of which was anaplastic and this is a rare finding.

Neurological mitochondrial cytopathies.

The mitochondrial cytopathies are genetically and phenotypically heterogeneous group of disorders caused by structural and functional abnormalities in mitochondria. To the best of our knowledge, there are very few studies published from India till date. Selected and confirmed fourteen cases of neurological mitochondrial cytopathies with different clinical syndromes admitted between 1997 and 2000 are being reported. There were 8 male and 6 female patients. The mean age was 24.42+/-11.18 years (range 4-40 years). Twelve patients could be categorized into well-defined syndromes, while two belonged to undefined group. In the defined syndrome categories, three patients had MELAS (mitochondrial encephalopathy, lactic acidosis and stroke like episodes), three had MERRF (myoclonic epilepsy and ragged red fibre myopathy), three cases had KSS (Kearns-Sayre Syndrome) and three were diagnosed to be suffering from mitochondrial myopathy. In the uncategorized group, one case presented with paroxysmal kinesogenic dystonia and the other manifested with generalized chorea alone. Serum lactic acid level was significantly increased in all the patients (fasting 28.96+/-4.59 mg%, post exercise 41.02+/-4.93 mg%). Muscle biopsy was done in all cases. Succinic dehydrogenase staining of muscle tissue showed subsarcolemmal accumulation of mitochondria in 12 cases. Mitochondrial DNA study could be performed in one case only and it did not reveal any mutation at nucleotides 3243 and 8344. MRI brain showed multiple infarcts in MELAS, hyperintensities in putaminal areas in chorea and bilateral cerebellar atrophy in MERRF.

Glossopharyngeal schwannoma : a case report and review of literature.

We report a rare case of glossopharyngeal schwannoma whose clinical presentation and the radiological work up suggested an acoustic schwannoma. The diagnosis was made at surgery, once attachment to ninth cranial nerve was seen. The clinical presentation, radiological features and surgical findings of the glossopharyngeal schwannoma are presented along with the review of literature.

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.

Disappearing cystic cerebellar medulloblastoma: the ghost tumour.

A case of medulloblastoma, which disappeared following steroid administration and a single sitting of radiotherapy (8Gy) is presented. At surgical exploration tumour was not found, multiple biopsies were negative. Post operative CT scan too did not show the tumour. Five months later, tumour reappeared in left cerebellar lobe in cystic and solid form. Patient was reoperated and tumour was decompressed. The biopsy showed medulloblastoma. To our knowledge, medulloblastomas are not known to be completely responsive to either of these modalities.

An oligo astrocytoma with widespread calcification along axonal fibres.

A rare case of a young man with a diffusely calcified oligo astrocytoma in right parieto occipital region, which spread along the axonal fibres into right temporal lobe and to the left parietal lobe is presented. Computed tomography (CT) and magnetic resonance imaging (MRI) demonstrated the extent of the lesion. The mass was partially resected.

Hypoglycaemic neuropathy: a case report.

A 53 years old male, a known case of ankylosing spondylitis having recurrent attacks of hypoglycaemia, developed symmetrical distal sensorimotor neuropathy. The neuropathy was axonal with secondary demyelination. Evidence of vasculopathy was also noted on histopathology of the nerve. Serum C-peptide level was low, a feature reported with autoimmune hypoglycaemia with antireceptor antibodies. The patient showed spontaneous recovery.

Alexander's disease: a case report of a biopsy proven case.

A case of infantile onset Alexander's disease in a two and a half year old male child is presented, who had progressively increasing macrencephaly since birth. A frontal lobe biopsy revealed collections of Rosenthal fibres in the subpial and perivascular areas with diffuse dysmyelination and presence of reactive astrocytes. The Rosenthal fibres were immunoreactive for glial fibrillary acidic protein and ubiquitin. Electron microscopic examination showed the Rosenthal fibres as intra-astrocytic and extracellular granular osmiphilic collections.

Nucleolar organiser regions in different colonic epithelia.

The argyrophilic technique (AgNOR) was applied to paraffin sections of 10 acute self-limited colitis, 15 ulcerative colitis (UC), 5 ulcerative colitis with indefinite dysplastic change, 10 adenomatous polyps, 20 colorectal adenocarcinomas and 10 normal colorectal mucosa. The mean number of nucleolar organiser regions (NORs) per nucleus ranged between 1.62-2.00 (95% CI 1.77-1.93) for normal colon, 2.47-3.80 (95% CI 2.71-3.21) for acute colitis, 1.66-2.75 (95% CI 2.13-2.44) for UC, 3.60-4.00 (95% CI 3.67-3.94) for UC with indefinite dysplasia, 3.00-4.04 (95% CI 3.41-3.81) for adenomatous polyps and 3.59-6.70 (95% CI 4.04-4.72) for colorectal adenocarcinoma. The differences observed were statistically significant. There was a significant difference of AgNOR counts between adenomatous polyp and UC with indefinite dysplasia in comparison to those observed in regenerative epithelium of acute colitis and UC without dysplasia. Hence the technique may be used as an adjunct to routine histology for delineating dysplastic changes in colonic epithelium.

Intestinal metaplasia--its association with gastric cancer.

Endoscopic gastric biopsies from 230 patients and post gastrectomy specimens of 18 patients were evaluated for the presence of intestinal metaplasia (IM) and its association with the gastric lesions. There were a total of 78 malignant and 170 benign lesions. IM was present in 53% of patients with gastric carcinoma (GC) and in 10.6% with benign lesions of the stomach. The IM in association with GC was of type 1 (Small Intestinal) in 58.8%; type II (mixed gastric and small intestinal) in 11.8% and type III (Colonic) in 29.4%. In patients with benign lesions the IM was predominantly of type I (94.5%) except in 1 patient who had type III metaplasia. Our findings indicate that type III IM was significantly more frequent in patient with GC than with benign lesions (29.4% Vs 5.5%; p < .005). Therefore we conclude that patients with type III IM should be kept on surveillance for GC.

Endomyocardial biopsy--its evaluation in cardiac disorders.

Endomyocardial biopsies (EMBs) of 47 patients with various cardiac disorders were analysed. Clinically 26 patients were diagnosed as having dilated cardiomyopathy (DCM), 9 myocarditis, six restrictive heart disease and six as Duchenne muscular dystrophy. EMB showed normal histological picture in six cases of DCM and findings consistent with DCM in rest of the twenty cases. None of these biopsies showed evidence of myocarditis. Diagnosis of myocarditis could be confirmed on EMB only in three out of nine clinically diagnosed cases. Cases with restrictive heart disease showed normal biopsy in two, infarction in one, findings consistent with endomyocardial fibrosis in one and amyloidosis in two cases. EMB on six cases of Duchenne muscular dystrophy showed nonspecific histological changes. To conclude, in this series, cases of DCM did not show evidence of underlying myocarditis in endomyocardial biopsies.

Thymic histology in myasthenia gravis.

Histological features of resected thymuses of 18 patients with myasthenia gravis were evaluated. Thymoma were seen in 16.6% of the patients. In the non-thymomatous group, thymic hyperplasia with follicle formation was seen in 33.3%, involuted thymus with occasional lymphoid follicle formation in 11.1%, thymitis with B cell infiltration in 16.6%, involuted thymus in 11.1% and normal thymus in 11.1% of patients. Immuno-histological staining for B and T lymphocytes delineated the group labelled as thymitis with B cell infiltration. Mast cell and eosinophils were frequently seen in thymuses with thymic hyperplasia with follicle formation and thymitis with B cell infiltration. Thymic hyperplasia with follicle formation was more frequent in young patients and these patients had better prognosis.

Solitary rectal ulcer syndrome.

Solitary rectal ulcer syndrome (SRUS) clinically presents with rectal bleed. Twenty three cases of SRUS are described highlighting that it is a distinct clinicopathological entity which can be diagnosed in the presence of typical clinical features, sigmoidoscopic findings and histopathological appearance of mucosal biopsy.

Histological and immunological analysis of gastric and intestinal lymphomas.

Immunophenotyping of 28 gastrointestinal non-Hodgkin's lymphomas was done using specific antibodies. Twenty six of these were successfully classified using this technique.

An unusual case of plasma cell granuloma involving lung and brain.

A young male aged 20 years had resection of the upper lobe of right lung for plasma cell granuloma. Five years later he developed three lesions in the brain, one of which was resected. Brain lesion showed similar histopathological picture. A unique case of plasma cell granuloma involving more than one organ is reported.

Surgical management of cardiac myxomas.

Cardiac myxomas are rare cardiac lesions, though they are the commonest tumours of the heart. Seventeen cases of cardiac myxomas have been operated during the last one decade. Exertional dyspnoea, palpitation and chest pain were the main presenting symptoms. Echocardiographic assessment was the only definitive diagnostic investigation required prior to surgery. Early surgical excision was planned in all the cases. Irrespective of the exposure techniques, removal of the tumour with wide excision of its base was practised. There was one early death due to low cardiac output in a patient brought in a shock like state. Follow up study has revealed 14 patients in NYHA class I and two patients are having class II symptoms. Periodic echocardiographic follow up study has not revealed any recurrence till date. It is concluded that an early diagnosis and surgery gives excellent long term results in these cases.