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1.
Indian J Pathol Microbiol ; 62(2): 296-299, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-30971560

RESUMO

We report the case of a 5-year-old male child presenting with seizures for 4 months. Magnetic resonance imaging (MRI) revealed a cortical-based solid cystic lesion in the right parietal lobe. Histopathological examination showed a tumour comprised of spindled glial fibrillary acid protein (GFAP) positive neoplastic cells interspersed with bizarre pleomorphic cells showing nuclear pseudoinclusions and intermingled dysplastic ganglion cells variably immunopositive for synaptophysin, chromogranin, Neu-N and immunonegative for neuron filament protein (NFP). This report highlights the occurrence of the rare composite pleomorphic xanthoastrocytoma-ganglioglioma and the vagaries of immunohistochemical analysis in highlighting neuronal differentiation in such a case setting. In addition, to the best of our knowledge this is the youngest patient till date to present with this entity.


Assuntos
Neoplasias Encefálicas/diagnóstico , Encéfalo/patologia , Ganglioglioma/diagnóstico , Neurônios/patologia , Biomarcadores/análise , Encéfalo/diagnóstico por imagem , Neoplasias Encefálicas/patologia , Pré-Escolar , Cromogranina A/análise , Ganglioglioma/patologia , Humanos , Imuno-Histoquímica , Imagem por Ressonância Magnética , Masculino , Convulsões/etiologia , Sinaptofisina/análise
2.
Neurology ; 92(6): e587-e593, 2019 Feb 05.
Artigo em Inglês | MEDLINE | ID: mdl-30635494

RESUMO

OBJECTIVE: To determine the molecular etiology of disease in 4 individuals from 2 unrelated families who presented with proximal muscle weakness and features suggestive of mitochondrial disease. METHODS: Clinical information and neuroimaging were reviewed. Genome sequencing was performed on affected individuals and biological parents. RESULTS: All affected individuals presented with muscle weakness and difficulty walking. In one family, both children had neonatal respiratory distress while the other family had 2 children with episodic deteriorations. In each family, muscle biopsy demonstrated ragged red fibers. MRI was suggestive of a mitochondrial leukoencephalopathy, with extensive deep cerebral white matter T2 hyperintense signal and selective involvement of the middle blade of the corpus callosum. Through genome sequencing, homozygous GFPT1 missense variants were identified in the affected individuals of each family. The variants detected (p.Arg14Leu and p.Thr151Lys) are absent from population databases and predicted to be damaging by in silico prediction tools. Following the genetic diagnosis, nerve conduction studies were performed and demonstrated a decremental response to repetitive nerve stimulation, confirming the diagnosis of myasthenia. Treatment with pyridostigmine was started in one family with favorable response. CONCLUSIONS: GFPT1 encodes a widely expressed protein that controls the flux of glucose into the hexosamine-biosynthesis pathway that produces precursors for glycosylation of proteins. GFPT1 variants and defects in other enzymes of this pathway have previously been associated with congenital myasthenia. These findings identify leukoencephalopathy as a previously unrecognized phenotype in GFPT1-related disease and suggest that mitochondrial dysfunction could contribute to this disorder.

3.
Surg Neurol Int ; 9: 143, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-30105137

RESUMO

Background: Accessory breast, also known as supernumerary breasts, polymastia, or mammae erraticae, is a clinical condition of having an additional breast. Accessory breasts are usually seen along the embryonic milk line, with the majority located in the axilla. Polythelia is the presence of an additional nipple. We report a rare case of dorsal accessory ectopic breast with three nipples (two well formed and one rudimentary) occurring along with lipomeningomyelocele and diastematomyelia. Case Description: We report the case of an 18-year-old female who presented with chief complaints of swelling over the upper back since birth and spastic weakness of bilateral lower limbs with inability to walk since 2 years. Three-dimensional computed tomography scan of the dorsal spine was suggestive of a wide bony defect in the posterior spinal elements from D3 to D9 vertebrae. Diastematomyelia was also seen. Magnetic resonance imaging of the dorsal spine was suggestive of a complex spinal dysraphism with lipomeningomyelocele and diastematomyelia. During surgery, the patient's accessory breast was removed, lipomatous tissue and bony septum were excised, and dural repair was done. Histopathological examination was consistent with accessory ectopic breast with lipomeningomyelocele. Conclusion: Dorsal accessory breast, although a rare entity, whenever present should alert the clinician regarding the possibility of an underlying occult spinal dysraphism (OSD). Therefore, dorsal accessory breast can also be considered as a marker of OSD.

4.
World Neurosurg ; 117: 221-224, 2018 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-29929035

RESUMO

BACKGROUND: Anaplastic gangliogliomas at the cerebellopontine angle (CPA) are exceptionally rare with only a few reported cases in published literature. These are composed of atypical ganglion cells and astrocytes accounting for nearly 1% of all central nervous system tumors. The authors report the case of anaplastic ganglioglioma in an adult patient presenting as a CPA mass lesion. The presentation was bizarre, and the diagnosis was possible only after histopathologic examination of the resected mass. CASE DESCRIPTION: A 32-year-old lady presented with complaints of gradually progressive hearing loss and tinnitus in her right ear along with headache and ataxia. Neurologic evaluation revealed right-sided sensorineural hearing loss and truncal ataxia. Magnetic resonance imaging of the brain was suggestive of a well-defined, contrast-enhancing, solid-cystic mass at the CPA cistern that extended into the porus acusticus, causing enlargement of the internal acoustic meatus. The features were suggestive of vestibular schwannoma of the CPA. CONCLUSIONS: This unusual appearance of anaplastic ganglioglioma has not been previously described. Neurosurgeons need to keep in mind the possibility of anaplastic gangliogliomas in the differential diagnosis of CPA lesions.


Assuntos
Neoplasias Encefálicas/diagnóstico , Ganglioglioma/diagnóstico , Neuroma Acústico/diagnóstico , Adulto , Neoplasias Encefálicas/patologia , Neoplasias Encefálicas/cirurgia , Ângulo Cerebelopontino/diagnóstico por imagem , Ângulo Cerebelopontino/patologia , Ângulo Cerebelopontino/cirurgia , Diagnóstico Diferencial , Feminino , Ganglioglioma/patologia , Ganglioglioma/cirurgia , Humanos , Neuroma Acústico/patologia
5.
J Cytol ; 35(2): 83-89, 2018 Apr-Jun.
Artigo em Inglês | MEDLINE | ID: mdl-29643654

RESUMO

Objective: Endoscopic ultrasound-guided fine-needle aspiration cytology (EUS-FNAC) is a precise and safe technique that provides both radiological and pathological diagnosis with a better diagnostic yield and minimal adverse events. EUS-FNAC led to the remarkable increase in the detection rate of incidentaloma found during radiologic staging or follow-up in various malignancy or unrelated conditions. Aims: We did this preliminary study with an aim to evaluate the role of EUS-FNA in diagnosing and classifying adrenal lesions, clinical impact, and compare the outcome with the previously published literature. Materials and Methods: We included 32 consecutive cases (both retrospective and prospective) of EUS-guided adrenal aspirate performed over a period of 3.3 years. The indications for the aspirate in decreasing order were metastasis (most common carcinoma gall bladder) > primary adrenal mass > disseminated tuberculosis > pyrexia of unknown origin. On EUS, 28 cases revealed space occupying lesion or mass (two cases bilateral) and four cases revealed diffuse enlargement (two cases bilateral) with a mean size of 21 mm. Results: The cytology reports were benign adrenal aspirate (43.8%), metastatic adenocarcinoma (15.6%), histoplasmosis (9.4%), tuberculosis (9.4%), round cell tumor (6.2%), adrenocortical carcinoma (3.1%), and descriptive (3.1%). Three cases (9.4%) yielded inadequate sample. The TNM staging was altered in 22.23% of the cases by result of adrenal aspirate. Conclusions: EUS-FNA of the adrenal gland is a safe, quick, and sensitive and real-time diagnostic technique, which requires an integrated approach of clinician, endoscopist, and cytopathologist for high precision in diagnosis. Although the role of EUS-FNA for right adrenal is not much described, we found adequate sample yield in all the four patients that underwent the procedure.

7.
Surg Neurol Int ; 8: 83, 2017.
Artigo em Inglês | MEDLINE | ID: mdl-28607817

RESUMO

BACKGROUND: Olfactory neuroblastoma (ONB) is a rare malignant neuroectodermal tumor of the nasal cavity. Mixed olfactory neuroblastoma which contains areas of divergent differentiation is even rare. Till date, only 4 cases of mixed olfactory neuroblastomas have been reported. CASE DESCRIPTION: We report the case of a 46-year-old male who presented with the chief complaints of nasal bleeding and nasal obstruction since 4 months. Radiological imaging was suggestive of a large heterogeneous mass in the left superior nasal cavity with extensions into bilateral maxillary, ethmoidal, and sphenoidal sinuses, as well as into the anterior cranial fossa. Bifrontal osteoplastic craniotomy and excision of the intracranial part of the tumor from above and transnasal endoscopic removal of the mass in the nasal cavities and paranasal sinuses from below was done. Postoperative radiological imaging was suggestive of gross complete excision of the mass. Histopathological diagnosis was "mixed olfactory neuroblastoma-carcinoma (squamous and glandular differentiation) Hyams grade IV." On immunohistochemistry, the tumor cells were positive for neuron specific enolase (NSE), synaptophysin, chromogranin, and CD56 and peripherally for S100. Because of personal reasons, the patient did not take adjuvant radiotherapy. He presented again after 2 months with a full blown recurrence of esthesioneuroblastoma with similar extensions as before. The patient is now planned for salvage surgery followed by adjuvant chemoradiation. CONCLUSION: We report the 5th case in the world of mixed olfactory neuroblastoma-carcinoma with squamous and glandular differentiation. From an analysis of the findings in the 5 reported cases of mixed olfactory neuroblastomas, one might infer that a separate subcategory of ONB, i.e., mixed ONB, should be considered because mixed ONBs have an aggressive behavior, high rates of recurrence, and these tumors should be treated aggressively by multimodality treatment.

8.
Neuropathology ; 37(4): 358-364, 2017 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-28295636

RESUMO

We present a rare case of primary pituitary T cell lymphoma/leukemia (T-LBL) in association with adrenocorticotropic hormone (ACTH) and thyroid stimulating hormone (TSH) expressing pituitary adenoma in a 55-year-old woman highlighting the importance of intra-operative squash smears examination. The patient presented with complaints of headache, diminution of vision and recent onset altered sensorium. MRI revealed a mass lesion in the sellar-suprasellar region with non-visualization of pituitary gland separately, extending to involve adjacent structures diagnosed as invasive pituitary macroadenoma. Intra-operative tissue was sent for squash smear examination. The cytology showed a tumor comprising of sheets of immature lymphoid cells intermixed with clusters of pituitary acinar cells with many mitoses and tingible body macrophages. A diagnosis of presence of immature lymphoid cells within the pituitary was offered and differentials of infiltration by lymphoma cells from systemic disease versus primary central nervous lymphoma-like lymphoma arising in the pituitary adenoma were considered. Later paraffin section examination and immunohistochemistry corroborated with the squash findings and a final diagnosis of primary pituitary T cell lymphoma/leukemia in association with ACTH and TSH expressing pituitary adenoma was made. To date, only six cases of primary pituitary T cell lymphomas, including three T-LBL cases, have been reported. This is the seventh case and first one additionally describing cytohistological correlation and importance of intra-operative cytology.


Assuntos
Adenoma/diagnóstico , Citodiagnóstico/métodos , Neoplasias Primárias Múltiplas/diagnóstico , Neoplasias Hipofisárias/diagnóstico , Leucemia-Linfoma Linfoblástico de Células Precursoras/diagnóstico , Adenoma/patologia , Hormônio Adrenocorticotrópico/biossíntese , Feminino , Humanos , Pessoa de Meia-Idade , Neoplasias Primárias Múltiplas/patologia , Neoplasias Hipofisárias/patologia , Leucemia-Linfoma Linfoblástico de Células Precursoras/patologia , Tireotropina/biossíntese
9.
Neuropathology ; 37(2): 116-121, 2017 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-27645472

RESUMO

We describe a unique case of pleomorphic xanthoastrocytoma (PXA) in a 19-year-old male presenting with the chief complaint of seizures. On radiology, the tumor was located in the temporal lobe. It was cortically based and solid cystic in nature. Light microscopy showed pleomorphic large polygonal cells with inclusions, nuclear clustering, lipidization, and foamy cytoplasm intermingled with spindle cells arranged in sweeping pattern and focally containing cytoplasmic brownish black pigment. The pigment stained black with Fontana-Masson stain and bleached with potassium permanganate. Gomori silver stain showed reticulin fibers surrounding individual tumor cells as well as groups of cells. On immunohistochemistry, tumor cells were positive for GFAP, S-100 and focally for synaptophysin and CD34 but negative for HMB-45. CD34 revealed a specific membranous pattern around individual cells as well as groups of cells along the fibers replicating a reticulin pattern. The ultrastructural examination showed supporting melanosomes, thus confirming the melanin pigment. Sequencing for BRAF V600E showed a heterozygous mutation. To our knowledge only five cases of PXA with melanin pigment have been reported and none of which described BRAF V600E mutation analysis. This case provides further insight into the origin and pathogenesis of pigmented astrocytic tumor, additionally highlighting the characteristic CD34 staining pattern.


Assuntos
Astrocitoma/patologia , Neoplasias Encefálicas/patologia , Neuroglia/patologia , Neurônios/patologia , Adulto , Astrocitoma/genética , Astrocitoma/ultraestrutura , Neoplasias Encefálicas/genética , Neoplasias Encefálicas/ultraestrutura , Humanos , Masculino , Neuroglia/metabolismo , Neurônios/metabolismo , Proteínas Proto-Oncogênicas B-raf/genética , Adulto Jovem
10.
J Gastroenterol Hepatol ; 32(6): 1178-1182, 2017 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-27862319

RESUMO

BACKGROUND AND AIM: Helicobacter pylori (HP) infection can influence the inflammatory and immune responses in the gut and may therefore play a role in the development of gluten-related enteropathy in genetically susceptible individuals. Our objective was to assess the relationship between celiac disease and HP infection in children. METHODS: Children (1-18 years) diagnosed as celiac disease (CD) (n = 324) with submission of gastric and duodenal biopsies and duodenal histology having Marsh grade III features were eligible for the study. Non-celiac patients referred for endoscopy were selected as controls. We studied proportion of HP prevalence in children with confirmed CD as compared with HP prevalence in reference group comprising non-celiac children referred for endoscopy. We also evaluated predictors of HP infection in children with celiac disease. RESULTS: Of the 324 participants with CD, gastric HP was seen in 37 (11.4%) patients. The prevalence of HP in patients without CD (50%, P < 0.001) was significantly higher. Among patients with CD, HP infection was most frequent in patients with Marsh IIIa. In the stepwise regression analysis for risk factors of HP infection in CD patients: presence of gastritis, hemoglobin, and absence of scalloping were found to be independent predictors in a multivariate setup. CONCLUSION: Celiac disease and gastric HP infection have inverse relationship that raises the question whether development of HP infection confers protection against CD.


Assuntos
Doença Celíaca/epidemiologia , Doença Celíaca/etiologia , Gastrite/epidemiologia , Gastrite/microbiologia , Infecções por Helicobacter , Helicobacter pylori , Adolescente , Criança , Pré-Escolar , Estudos de Coortes , Estudos Transversais , Feminino , Humanos , Lactente , Masculino , Prevalência , Estudos Prospectivos , Índice de Gravidade de Doença
11.
Neurol India ; 64(6): 1376-1377, 2016 Nov-Dec.
Artigo em Inglês | MEDLINE | ID: mdl-27841236
12.
Neurol India ; 64(5): 988-94, 2016 Sep-Oct.
Artigo em Inglês | MEDLINE | ID: mdl-27625244

RESUMO

BACKGROUND: Subependymal giant cell astrocytomas (SEGA) are slow-growing benign intraventricular tumors, the pathogenesis of which is debated. Recent studies have shown that tuberous sclerosis complex (TSC) 1 and TSC2 genes are linked to the mammalian target of rapamycin (mTOR) cell signaling pathway. We aimed to analyze TSC1 and TSC2 gene mutation, hamartin and tuberin protein expression, and protein expression of mTOR signaling cascade in a series of SEGA to determine their role in pathogenesis. MATERIALS AND METHODS: Twenty-eight SEGA cases were retrieved from archival material. Immunohistochemistry was performed on formalin-fixed, paraffin-embedded tissue using antibodies against tuberin, hamartin, phospho-p70S6 kinase, S6 ribosomal protein, phospho-S6 ribosomal protein, phospho-4E-BP1, Stat3, and phospho-Stat3. Mutation analysis of TSC1 (exons 15 and 17) and TSC2 (exons 33, 39, and 40) was done by DNA sequencing. RESULTS: Loss of immunoexpression of either hamartin or tuberin was found in 19 cases (68%). Pathogenic point mutations in selected exons of TSC1 and TSC2 genes were present in 5 of 20 cases studied. Robust expression of mTOR downstream signaling molecules phospho-p70S6 kinase (100%), S6 ribosomal protein (82%), phospho-S6 ribosomal protein (64%), phospho-4E-BP1 (64%), and Stat3 (100%) was seen. Four cases (14%) showed immunopositivity for phospho-Stat3. There was no significant correlation of these markers with immunoloss of tuberin and hamartin. SIGNIFICANCE: There is a definite role for TSC1 and TSC2 genes in the pathogenesis of SEGA as evidenced by loss of protein expression and presence of mutations. Strong expression of mTOR downstream signaling proteins indicates activation of mTOR pathway in these tumors, suggesting that proteins in this pathway may have the potential to serve as therapeutic targets in these patients.


Assuntos
Astrocitoma/genética , Transdução de Sinais , Serina-Treonina Quinases TOR/fisiologia , Proteínas Supressoras de Tumor/fisiologia , Proteínas Adaptadoras de Transdução de Sinal , Astrocitoma/metabolismo , Análise Mutacional de DNA , Humanos , Imuno-Histoquímica , Fosfoproteínas , Proteína S6 Ribossômica , Esclerose Tuberosa , Proteína 1 do Complexo Esclerose Tuberosa , Proteína 2 do Complexo Esclerose Tuberosa
16.
J Indian Soc Periodontol ; 18(3): 379-84, 2014 May.
Artigo em Inglês | MEDLINE | ID: mdl-25024555

RESUMO

Plasma cell mucositis (PCM) is a very rare, chronic, multifocal, idiopathic, non-neoplastic plasma cell proliferative disorder of the upper aerodigestive tract. The classic clinical presentation is an intensely erythematous mucosa with surface changes described variously as papillomatous, cobblestone, nodular or velvety. It is a very rare condition <50 cases reported in literature. A 72-year-old male patient complained of sore throat, stomatodynia, dysphagia, multiple oral ulcers, enlarged swollen bleeding gums and mobile teeth. There was chronic inflammatory enlargement of the gingiva and palate with severe periodontitis. Histopathological examination revealed a hyperplastic epithelium with a dense infiltrate of mature polyclonal plasma cells in the superficial layer of the lamina propria. PCM is a diagnosis of exclusion, to be differentiated from other infective, reactive, autoimmune, allergic and neoplastic disorders with plasma cell infiltrates. Management with surgical and immunosuppressive therapy is mostly ineffective with short remissions and frequent relapses.

17.
J Heart Valve Dis ; 23(1): 55-65, 2014 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-24779329

RESUMO

BACKGROUND AND AIM OF THE STUDY: Fetuin-A is a circulating glycoprotein that inhibits ectopic calcification. The study aim was first, to assess serum fetuin-A level in patients with calcified rheumatic mitral valve disease (RMVD), and second, to demonstrate the presence of fetuin-A by immunohistochemistry (IHC) in calcified RMVD which, to date, has not been verified in other studies. METHODS: The study group comprised 68 adult patients with isolated RMVD and normal renal function. Of these patients, 34 (27 males, seven females; mean age 33.44 +/- 9.0 years) had severe calcification (Wilkins calcium score 3 or 4) and 34 (25 males, nine females; mean age 30.8 +/- 8.5 years) had mild calcification (Wilkins calcium score 1 or 2). A group of 32 age- and gender-matched healthy subjects (25 males, seven females; mean age 29.5 +/- 4.6 years) served as controls. Baseline serum fetuin-A levels were measured using an enzyme-linked immunosorbent assay (ELISA), while Wilkins calcium scores were assessed using either transthoracic or transesophageal echocardiography. Serum levels of calcium, phosphorus and alkaline phosphatase were assessed in all subjects. Histopathological examinations of ten severely calcific rheumatic mitral valves were made and compared with 10 non-calcified rheumatic mitral valves, all of which had undergone mitral valve replacement. RESULTS: Serum fetuin-A levels were significantly lower in RMVD patients than in controls (108.83 +/- 7.1 versus 114.46 +/- 3.32 ng/ml; p = 0.014). However, there was no significant difference in fetuin-A level between patients with severe (C3/C4) versus mild calcification (C1/C2) (108.84 +/- 7.82 versus 108.82 +/- 6.36 ng/ml; p = NS). No correlation of fetuin-A was seen with serum high-sensitivity C-reactive protein, calcium, phosphorus and alkaline phosphatase, or with Wilkins' calcium score. IHC analyses revealed the presence of fetuin-A in the mesenchymal matrix and calcified area of calcific valves, while minimal to absent fetuin-A deposition was detected in the mesenchymal matrix of non-calcified mitral valves. CONCLUSION: Serum fetuin-A levels were significantly decreased in patients with calcific RMVD. The present study was the first to demonstrate fetuin-A in the calcified mitral valve of rheumatic etiology, and suggests its possible role in the pathophysiology of calcific mitral valve disease. Further studies are required, however, to determine therapeutic implications.


Assuntos
Estenose da Valva Mitral/metabolismo , Valva Mitral/metabolismo , Cardiopatia Reumática/metabolismo , Calcificação Vascular/metabolismo , alfa-2-Glicoproteína-HS/metabolismo , Adulto , Proteína C-Reativa/análise , Estudos de Casos e Controles , Feminino , Próteses Valvulares Cardíacas , Humanos , Imuno-Histoquímica , Masculino , Pessoa de Meia-Idade , Valva Mitral/cirurgia , Estenose da Valva Mitral/cirurgia , Cardiopatia Reumática/cirurgia , Índice de Gravidade de Doença
18.
Brain Tumor Pathol ; 31(1): 62-7, 2014 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-23456087

RESUMO

Non-meningothelial mesenchymal tumors of the central nervous system (CNS), including those originating from the meninges, histologically correspond to tumors of soft tissue or bone. These individual entities arising from the meninges are rare, and probably have their origin in the multipotent primitive mesenchymal stem cells of the dura. Though it is a common bone tumor, the meningeal origin of osteochondroma has only very rarely been reported. We describe a case of a 35-year-old female with a well-demarcated, golf-ball-like osteochondroma of meningeal origin which was enucleated en bloc on craniotomy. Such a lesion can resemble a meningioma that exhibits metaplastic (osseous) change on imaging. However, provided that there is clinico-radiological awareness of such tumors, magnetic resonance imaging (MRI) can guide the way to this rare differential diagnosis, as it reflects the pathologic appearance of osteochondroma and allows the thickness of the cartilage cap to be estimated in order to check for rare malignant change. Complete excision along with the cartilage cap usually offers a favorable prognosis without recurrence.


Assuntos
Neoplasias Ósseas/diagnóstico , Neoplasias Ósseas/patologia , Transição Epitelial-Mesenquimal , Imagem por Ressonância Magnética , Meninges , Osteocondroma/diagnóstico , Osteocondroma/patologia , Adulto , Neoplasias Ósseas/cirurgia , Diagnóstico Diferencial , Feminino , Humanos , Neoplasias Meníngeas/patologia , Meningioma/patologia , Metaplasia , Osteocondroma/cirurgia , Resultado do Tratamento
19.
J Clin Neurosci ; 20(2): 331-2, 2013 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-23164826

RESUMO

Hepatitis B can have varied extrahepatic manifestations involving the skin, renal, haematological and nervous systems. Neurological manifestations in hepatitis B may take the form of Guillain-Barré syndrome and secondary systemic vasculitis-related mononeuritis multiplex. The clinical course of hepatitis B-related, vasculitis-related neuropathy is usually subacute to chronic and clinical evolution is relatively benign. To our knowledge, acute hepatitis B-associated vasculitis manifesting as acute distal symmetric polyneuropathy has not been reported. We report a 60-year-old man who presented with fever, mild hepatomegaly, skin lesions in the form of non-palpable purpura and acute onset distal symmetric sensorimotor polyneuropathy. Serum transaminase levels were raised and viral serological markers revealed acute hepatitis B. The patient remained anicteric throughout his clinical course. Nerve conduction studies showed severe axonal sensorimotor polyneuropathy and histopathological examination of sural nerve biopsy was suggestive of vasculitic neuropathy. The patient was first given a course of intravenous immunoglobulin with the antiviral drug entecavir. The fever subsided after 1 week of treatment. The patient was started on prednisolone in addition to the entecavir, and showed significant improvement in motor power and marked resolution in paresthesia after 2 weeks of treatment. Thus, acute onset distal symmetric sensorimotor polyneuropathy of vasculitic etiology can be a manifestation of acute hepatitis B.


Assuntos
Hepatite B/diagnóstico , Polineuropatias/diagnóstico , Vasculite/diagnóstico , Doença Aguda , Hepatite B/complicações , Humanos , Masculino , Pessoa de Meia-Idade , Polineuropatias/complicações , Polineuropatias/virologia , Vasculite/complicações , Vasculite/virologia
20.
Brain Tumor Pathol ; 30(1): 50-6, 2013 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-22615000

RESUMO

Medulloblastomas with myogenic differentiation, previously termed medullomyoblastomas, form rare variants of medulloblastomas. Occasional tumors showing combined myogenic differentiation and melanotic tubular structures have also been described. On studying the records of a tertiary-care super specialty hospital, of 80 cases of medulloblastoma in a 5-year period, 36 showed nodular islands of neuronal, three myogenic, two glial, and one melanotic differentiation. Of the three cases of medulloblastomas with myogenic differentiation, we came across an extremely rare variant of medulloblastoma showing, in addition to the predominant primitive neuroectodermal component, a single minute nodular focus of 0.8 cm diameter, with divergent differentiation into mainly myogenic, epithelial, cartilaginous, and osseous lineages. Two ependymoblastic rosettes were also identified at different foci. In conclusion, a medulloblastoma with a small nodular island of divergent differentiation into various lineages has been seldom documented in literature. Microscopic foci of multilineage differentiation may be explained by the teratoid potential of medulloblastoma, or pluripotent nature of the neural crest cells, and requires extensive sampling for detection. It remains to be confirmed through more reports whether medulloblastomas with differentiation respond to a treatment protocol similar to that of medulloblastomas.


Assuntos
Diferenciação Celular , Neoplasias Cerebelares/patologia , Meduloblastoma/patologia , Criança , Humanos , Imagem por Ressonância Magnética , Masculino , Neuroglia/patologia , Neurônios/patologia
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