ABSTRACT
This study examined the spatio-temporal variability in fish assemblage structure and composition following monthly sampling (August 2006 to July 2007). Three estuarine zones (upper, middle and lower) of the unvegetated intertidal and subtidal channel habitats located in the Marapanim Estuary were investigated. In each of these zones, salinity, organic matter and sediment types were measured to assess any correlation between habitat types and the fish fauna. A total of 41 496 fishes, belonging to 76 species and 29 families, was recorded. Recurring changes in both species composition and trophic structure were attributed to seasonal variations, while habitat type played a more permanent role in modifying the structure of fish assemblages. Zooplanktivores (e.g. Lycengraulis grossidens) and herbivores (e.g. Cetengraulis edentulus) used the intertidal habitat almost exclusively and were associated with salinity and substratum composition (gravel, silt and mud). In contrast, benthophages (e.g. Cathorops spixii) and benthophage-ichthyophages (e.g. Cynoscion leiarchus) were primarily associated with the subtidal habitat throughout the estuary and were highly related to the presence of sandy substrata. This study highlighted the intricate roles that local factors (such as habitat connectivity) may have on the distribution of fishes at the assemblage level. As such, incorporating habitat sharing or segregation between species should be viewed as essential for any comparisons of estuaries over large geographic scales, and in particular for conservation planning and management measures.
Subject(s)
Ecosystem , Estuaries , Fishes , Animals , Brazil , Perciformes , Salinity , SeasonsABSTRACT
The relationships between changes in habitat quality (disturbed and undisturbed sites) and the population parameters (density, size distribution, reproductive activity and diet) of a goby Microgobius meeki were investigated in a tropical estuary to assess its value as an indicator of anthropogenic changes, predominantly the effects of sedimentation and mangrove removal in shallow estuarine areas. Fish sampling surveys were conducted bimonthly between June 2009 and May 2010 over the entire estuarine gradient of the Paraguaçu River estuary, located on the central Brazilian coast. A predictive model was developed to assess the population changes of this species in 10 other tropical estuaries with different dimensions, basin features and distinct levels of anthropogenic disturbance. General linear models were used to relate the population structure of M. meeki to sediment type, habitat type resulting from mangrove conservation status, anthropogenic pressure and environmental characteristics such as salinity, dissolved oxygen concentrations and temperature. Sediment type and the presence of mangrove forests were the most effective predictors of local variability in the population structure of M. meeki. Individuals with mature gonads and high rates of feeding activity were associated predominantly with undisturbed habitats. Estuaries and estuarine sectors with high levels of sedimentation in shallow marginal areas, anthropogenic pressure from domestic and industrial effluents, and evidence of mangrove deforestation yielded the lowest capture rates of both juvenile and adult M. meeki. Based on these findings, M. meeki is identified as a potential indicator of the consequences of anthropogenic disturbance in transitional waters. A simple, but efficient collection protocol, in which overt signs of distress can be promptly observed is proposed. Testing the generality of this approach across different systems might prove useful in a broader conservation biology context.
Subject(s)
Conservation of Natural Resources , Environmental Monitoring/methods , Estuaries , Perciformes , Animals , Brazil , Ecosystem , Feeding Behavior , Fishes , Population Density , Rivers , SalinityABSTRACT
AIMS: To compare the sensitivity and specificity of new rabbit monoclonal antibody SP3 with those of mouse monoclonal and rabbit polyclonal antibodies using HER2 amplification defined by chromogenic in situ hybridisation (CISH) as the gold standard. METHODS: Serial sections from tissue microarrays (TMAs) containing 84 breast carcinomas were submitted to CISH (Zymed HER2 Spot-Light kit) and immunohistochemistry, using NeoMarkers SP3 (rabbit monoclonal), DAKO A0485 and DAKO HercepTest (polyclonal), Novocastra NCL-CB11, Cell Marque CM-CB11, and Genentech 4D5 (mouse monoclonal). RESULTS: The best antibody concordance was between SP3 and HercepTest (kappa = 0.74). SP3, A0485 and HercepTest detected all HER2 amplified tumours, but were less specific than mouse monoclonal antibodies. 3/38 (7.9%) and 8/38 (21.0%) non-amplified tumours were scored as 3+ using SP3 and A0485, respectively. 3/46 (6.5%) amplified tumours were negative for NCL-CB11. SP3, HercepTest and A0485 showed no gene amplification on 55%, 62.5% and 92.3% of the 2+ scored tumours, but most of the 2+ scored tumours using monoclonal antibodies were amplified by CISH (80-92.3%). CONCLUSIONS: SP3 is more sensitive than mouse monoclonal antibodies for Her2 assessment. However, HercepTest, CB11 and 4D5 show higher specificity than SP3 for the identification of HER2 gene amplification. Mouse monoclonal antibodies show less Her2 2+ tumours; most are amplified by CISH.
Subject(s)
Biomarkers, Tumor/immunology , Breast Neoplasms/immunology , Receptor, ErbB-2/immunology , Animals , Antibodies, Monoclonal/immunology , Biomarkers, Tumor/metabolism , Breast Neoplasms/metabolism , Breast Neoplasms/pathology , Chromogenic Compounds , Female , Humans , Immunoenzyme Techniques , In Situ Hybridization/methods , Mice , Neoplasm Invasiveness , Rabbits , Receptor, ErbB-2/metabolism , Reproducibility of Results , Sensitivity and SpecificityABSTRACT
Cerebrovascular diseases in patients between 15 and 40 years old are not a frequent subject in Latin-American literature, especially when focusing on neuropathology. We analyzed 47 brains from necropsies performed from 1987 to 1997 and selected on a basis of age and the presence of vascular pathology. From the 47 analyzed brains, 26 belonged to females (55.3%). When distributed among age groups, 12.8% (n=6) affected patients from 15 to 20 yo, 51,1% (n=24) from 21 to 30 yo, 36,2% (n=17) from 31 to 40 yo. The underlying diseases were: cardiac and haemathologic (19.2%), pregnancy complications (12.76%), infections, diseases of blood vessels and neurological (10.1% each) amongst others. The neuropathological abnormalities included cerebral and/or cerebellar herniation (16%), cerebral edema (13.8%), subarachnoid hemorrhage (10%), recent cerebral infarction (9%), intraparenchymatous hemorrhage (8.14%), hypoxic-ischemic encephalopathy (3%) and other events such as Sneddon syndrome and Lupus vasculitis. These findings express that the cerebrovascular phenomena in this age group are unique and closely related with the underlying disease.
Subject(s)
Cerebrovascular Disorders/pathology , Adolescent , Adult , Age Distribution , Age Factors , Cerebrovascular Disorders/etiology , Female , Heart Diseases/etiology , Heart Diseases/pathology , Hematologic Diseases/etiology , Hematologic Diseases/pathology , Humans , Male , Sex DistributionABSTRACT
The April Case of the Month (COM). The contributors report a case of a 70 year-old woman with recurrent meningiomas, one of which showed rhabdoid and lipomatous differentiation. Histopathological study of the first and second previous resections showed only typical meningothelial meningioma. On the third craniotomy, a new tumor specimen showed an admixture of classic meningothelial meningioma with lipomatous and rhabdoid foci. Immunohistochemical studies showed diffuse reactivity for epithelial membrane antigen and vimentin, as well as focal positivity for desmin and smooth muscle actin in the areas with rhabdoid features and S100 protein in the lipomatous foci. The presence of these three different and concomitant histological patterns only in the third surgical resection might support a metaplastic origin and, also, corroborates the concept that rhabdoid features are suggestive of an aggressive behavior.
Subject(s)
Meningioma/pathology , Neoplasm Recurrence, Local/pathology , Aged , Desmin/metabolism , Female , Frontal Lobe/pathology , Humans , Immunohistochemistry , Meningioma/classification , Meningioma/metabolism , Mucin-1/metabolism , Neoplasm Recurrence, Local/classification , Neoplasm Recurrence, Local/metabolism , S100 Proteins/metabolism , Vimentin/metabolismABSTRACT
An 8-year-old girl was seen in our outpatient clinic with a history of an angiomatous plaque on the chest since birth. She had complained of pain at the site of the lesion for 1 month. Histopathologic examination and immunohistochemical analysis led to the diagnosis of glomangioma, a rare congenital plaquelike glomus tumor. We review the literature and discuss the clinical presentation and evolution of this rare entity.
Subject(s)
Glomus Tumor/congenital , Skin Neoplasms/congenital , Actins/analysis , Child , Female , Glomus Tumor/chemistry , Glomus Tumor/pathology , Humans , Immunohistochemistry , Skin/pathology , Skin Neoplasms/chemistry , Skin Neoplasms/pathology , Thorax , Vimentin/analysisABSTRACT
BACKGROUND: Glassy cell carcinomas of the uterine cervix are poorly differentiated carcinomas composed of cells with a large, round to oval nucleus containing one or multiple prominent nucleoli, finely vacuolated eosinophilic to amphophilic cytoplasm and distinct cell borders. These cells occur in sheets and chords, with fibrovascular septae presenting a mixed inflammatory infiltrate. This neoplasm has a poor response to radiotherapy and a worse prognosis than the usual types of adenocarcinoma and squamous cell carcinoma. There are few reports on the cytologic and histopathologic features of this neoplasm. CASE: A 56-year-old woman presented with a large, exophytic cervical tumor. Exfoliative cytology showed clusters of cells and single cells with large, round to oval nuclei, with one or multiple nucleoli and moderate to large, finely granulated cytoplasm with distinct cell borders. The background of the smears had a polymorphous inflammatory infiltrate, necrotic debris and proteinaceous material. A high mitotic rate was observed, as were rare bizarre and atypical multinucleated cells. There was no evidence of koilocytes. These findings were highly suggestive of glassy cell carcinoma and were confirmed by the histologic and immunocytochemical findings, with positivity for cytokeratin (MNF116), vimentin and carcinoembryonic antigen and negativity for HMB-45. CONCLUSION: Glassy cell carcinoma of the cervix presents a cytologic picture that can be highly suggestive of the diagnosis in typical cases; however, in difficult cases ancillary techniques, such as immunocytochemistry, as well as histologic findings might confirm the diagnosis.
Subject(s)
Carcinoma, Adenosquamous/diagnosis , Uterine Cervical Neoplasms/diagnosis , Vaginal Smears , Carcinoma, Adenosquamous/chemistry , Carcinoma, Adenosquamous/pathology , Cell Nucleus/ultrastructure , Female , Humans , Immunohistochemistry , Middle Aged , Uterine Cervical Neoplasms/chemistry , Uterine Cervical Neoplasms/pathology , Vimentin/analysisABSTRACT
Invasive zygomycosis is a devastating fungal infection occurring as an opportunistic infection after bone marrow transplantation (BMT). Sinusitis can lead to fungal infection in immunosuppressed patients, and cavernous sinus thrombosis, an uncommon condition in immunocompetent patients, typically follows an infection involving the medial third of the face, nose, or paranasal sinuses. Patients undergoing unrelated-donor BMT (UD-BMT) are prone to develop life-threatening infections because of poor recovery of cellular immunity. Despite adequate clinical evaluation and treatment, the prognosis of patients with invasive fungal infections is dismal, especially when intracerebral structures are affected. We describe a case of a patient who underwent an UD-BMT and developed cavernous sinus thrombosis after sinusitis due to zygomycosis. Moreover, he also had disseminated fungal (Zygomycetes and Aspergillus) and viral (cytomegalovirus and adenovirus) infections.
Subject(s)
Bone Marrow Transplantation/adverse effects , Cavernous Sinus Thrombosis/microbiology , Opportunistic Infections/microbiology , Zygomycosis/etiology , Adult , Fatal Outcome , Humans , Immunocompromised Host , MaleABSTRACT
Thymomas are tumors characterized by a remarkable morphological heterogeneity and variable clinical behavior. This tumor has unique clinical associations, most notably with hematological abnormalities and myasthenia gravis. According with the Müller-Hermelink criteria, there are significant differences between the histological types of thymomas and the association with myasthenia gravis. Among the different histological types, medullary thymoma is the least frequent variant associated with this autoimmune disease. In this report we describe a case of medullary thymoma presenting in a 71-year- old woman with a myasthenic syndrome.
Subject(s)
Myasthenia Gravis/complications , Thymoma/complications , Thymus Neoplasms/complications , Aged , Female , Humans , Myasthenia Gravis/pathology , Thymoma/pathology , Thymus Neoplasms/pathologyABSTRACT
Neurocysticercosis is the most frequent and widespread neuroparasitosis of the human being. The development of brain and leptomeningeal lesions, with subsequent symptoms, are mainly related with the immune status of the host, and to the number and evolutional phase of the parasites. We present the pathological findings in 27 necropsies of patients with neurocysticercosis, which accounted for 3.1% of the necropsies. 77% of the patients were male and the age ranged from 18 to 85 years. In 26% there was previous history of alcoholism. Clinicopathological study showed that 50% of the cases were classified as asymptomatic form, 11% epileptic form, 11% intraventricular form and 11% combined form. 33% of the patients presented seizures as a factor of aggravation of the clinical picture. There was a single cysticercus in 60% of the cases, the cellulosae form present in 82% and the racemous form in 7% of the cases; the remaining 11% had both forms present. In 30% of the patients the cause of death was directly related with the presence of the cysticercus in the central nervous system. Our findings confirm the high morbidity of this disease.
Subject(s)
Neurocysticercosis/pathology , Adult , Aged , Aged, 80 and over , Autopsy , Female , Humans , Magnetic Resonance Imaging , Male , Middle AgedABSTRACT
We report the clinical, epidemiological and pathological findings of 14 patients with haemangioblastoma. Sixty-four percent occurred in males, with ages ranging from 16 to 60 years, with an average of 34, 4 years. Most of the tumours were confined to the cerebellum (n=9). The most frequent symptoms were headache (n=7) and dizziness (n=7), with a mean duration of 70 days. Von Hippel-Lindau syndrome (vHL) was diagnosed in 3 patients. Eleven patients were submitted to total surgical removal and in 3 the tumour was partially resected. A relapse rate of 28% in 3 years of follow-up was found. The patients with vHL showed recurrence in 66% of the cases. These findings corroborate those in current medical literature, showing an increased morbidity of this tumor when associated with vHL.
Subject(s)
Cerebellar Neoplasms/pathology , Hemangioblastoma/pathology , Spinal Cord Neoplasms/pathology , Adolescent , Adult , Aged , Cerebellar Neoplasms/surgery , Female , Hemangioblastoma/surgery , Humans , Male , Recurrence , Spinal Cord Neoplasms/surgery , Syndrome , von Hippel-Lindau Disease/complications , von Hippel-Lindau Disease/diagnosisABSTRACT
We report the clinical, epidemiological and pathological findings of 28 patients with medulloblastoma: 22 were male; age ranged from 1 to 50 years, with a mean of 15 years. The most frequent symptoms and signs were headache (64%) and vomiting (64%). Only one patient showed a desmoplastic medulloblastoma variant, the others showed classical medulloblastomas. Regarding treatment, most patients were submitted to total resection (n=10) or partial tumorectomy (n=7). A low rate of tumoral recurrence was observed, occurring in 21% of the patients (n=6) after 4 years of follow-up. Chemotherapy seemed to contribute to a lower recurrence rate amongst our patients. Our findings are similar to those reported in literature, thus helping to understand the biological behavior of this type of tumor.
Subject(s)
Cerebellar Neoplasms , Medulloblastoma , Adolescent , Adult , Age Distribution , Cerebellar Neoplasms/diagnosis , Cerebellar Neoplasms/epidemiology , Cerebellar Neoplasms/therapy , Child , Child, Preschool , Female , Humans , Infant , Male , Medulloblastoma/diagnosis , Medulloblastoma/epidemiology , Medulloblastoma/therapy , Middle Aged , Neoplasm Recurrence, Local , Prognosis , Sex DistributionABSTRACT
BACKGROUND: Prognostic factors in oligodendrogliomas are an area of controversy in neuropathology. Although grading and the study of some morphologic variables may be of value as prognostic parameters, the variability of postoperative disease free survival in patients with World Health Organization Grade 2 oligodendroglioma indicates that the biologic behavior of this entity remains unknown. The objective of the current study was to evaluate immunoexpression of the proliferation index (PI), epidermal growth factor receptor (EGFR), and bcl-2 as prognostic factors in patients with Grade 2 oligodendroglioma. METHODS: In a series of 19 cases of pure Grade 2 oligodendroglioma, we assessed the mitotic count, labeling index for MIB-1 and PCNA, and immunoreactivity for EGFR and bcl-2 with semiquantitative parameters and compared these with postoperative disease free survival. Statistical analyses using the Cox-Mantel nonparametric test and Spearman correlation coefficient were used to evaluate the data. RESULTS: Disease free survival was significantly shorter when the MIB-1 PI was > 5% (P = 0.0096) and the PCNA PI was > 9% (P = 0.00011) and when mitoses were observed (P = 0.00004). The paired variables also were found to correlate: MIB-1 versus PCNA (P = 0.04), MIB-1 versus mitotic count (P = 0.0184), and PCNA versus mitotic count (P = 0.0079). In all cases, there was immunoreactivity for EGFR; conversely, all cases were negative for bcl-2 in the cells with an oligodendrocyte phenotype. CONCLUSIONS: The PI was found to correlate with the postoperative disease free survival in patients with Grade 2 oligodendroglioma; therefore, the authors suggest a possible subdivision of Grade 2 oligodendrogliomas into two groups based on the mitotic count and PI.
Subject(s)
Biomarkers, Tumor/analysis , Brain Neoplasms/pathology , ErbB Receptors/analysis , Mitotic Index , Oligodendroglioma/pathology , Proto-Oncogene Proteins c-bcl-2/analysis , Adolescent , Adult , Aged , Brain Neoplasms/chemistry , Brain Neoplasms/mortality , Disease-Free Survival , Female , Humans , Immunohistochemistry , Ki-67 Antigen/analysis , Male , Middle Aged , Oligodendroglioma/chemistry , Oligodendroglioma/mortality , Prognosis , Proliferating Cell Nuclear Antigen/analysis , Survival RateABSTRACT
OBJECTIVE: Report a case of Krabbés disease with necropsy. METHODS: Review of medical and necropsy records. RESULTS: An 8 months-old male patient developed tremors, swallowing difficulty and excessive salivation for 4 months prior to admission, evolving with vomiting and fever. Physical examination showed microcephaly and diffuse pigmentation of the retinae. Neurological examination showed flexion of upper limbs with spastic hyperthony, symmetrical global hyperreflexia, nystagmus and spontaneous spasms. EEG showed multifocal irritative activity. There was increase in both CSF protein and gamaglobulin. The patient evolved with transitory hyperthermia, vomiting and pneumopathy, dying on the 23rd day after admission. Post mortem studies revealed microcephaly with widening of brain sulci. Histological examination revealed several globoid cells in the deep portion of the white matter, reactive gliosis and demyelination. CONCLUSIONS: These findings were similar to those in the world literature, indicating a poor prognosis due to substantial brain damage.
ABSTRACT
Oligodendrogliomas account for 4-5% of primary central nervous system tumours with a slow and infiltrative growth. We report the clinical and pathological findings of 15 cases of oligodendrogliomas. Eight patients were males and 7 were females. The ages ranged between 17 and 66 years, with a mean of 39.73 years. The symptoms reflected the growth and topography of the tumours; migraine (60%) and seizures (60%) were the most frequent symptoms. Frontal (n = 6), parietal (n = 2), temporal (n = 1) and occipital (n = 1) lobes were affected. Five patients undergone total resection of the tumor and 10 were submitted to partial resection, from which 3 received adjuvant radiotherapy, 1 adjuvant chemotherapy and 1 chemotherapy and radiotherapy. The overall recurrence rate was 60% for a 32 month follow up. Five recurrences were observed in patients submitted only to the surgical treatment and 4 in which adjuvant radio or chemotherapy were performed. These results are similar with the literature and may contribute to further understanding the biological behavior of these rare tumours.
Subject(s)
Brain Neoplasms/pathology , Oligodendroglioma/pathology , Adolescent , Adult , Age Distribution , Aged , Brain Neoplasms/epidemiology , Brain Neoplasms/therapy , Brazil/epidemiology , Central Nervous System Neoplasms/epidemiology , Central Nervous System Neoplasms/pathology , Female , Humans , Male , Middle Aged , Neoplasm Recurrence, Local , Oligodendroglioma/epidemiology , Oligodendroglioma/therapyABSTRACT
We report the clinical and pathological findings of 25 cases of craniopharyngiomas. Fourteen patients were males and 11 were females. The ages ranged between 3 and 64 years, with a mean of 30.52 years. The symptoms reflected the growth and topography of the tumours; visual disorders (72%), headache (68%), vomits (40%) and papilledema (24%) were the most frequent symptoms. Twelve cases were suprasellar; 10 tumours arose from sellar region, from which 8 presented suprasellar extension; frontal lobe (n = 2) and ponto cererebellar angle (n = 1) were also affected. Eleven patients undergone total resection of the tumor and 14 were submitted to partial resection, from which 1 received adjuvant chemotherapy. The overall recurrence rate was 48%. Eight recurrences were observed in the patients submitted to partial resection and 4 in which total resection were performed. These results are similar with the literature, corroborating to the extension of residual tumour after the surgical resection as the main prognostic factor for this neoplasm.
Subject(s)
Craniopharyngioma/pathology , Pituitary Neoplasms/pathology , Adult , Child , Child, Preschool , Craniopharyngioma/epidemiology , Craniopharyngioma/surgery , Female , Humans , Male , Middle Aged , Neoplasm Recurrence, Local , Pituitary Neoplasms/epidemiology , Pituitary Neoplasms/surgeryABSTRACT
Ependymomas are composed of neoplastic ependymal cells, affecting mainly children and young adults. We report the clinical and pathological findings of 22 cases of ependymomas. Fourteen patients were males and 8 were females. The ages ranged between 1 and 58 years, with a mean of 24.63 years. The symptoms reflected the growth and topography of the tumours; muscle weakness (59.1%), gait disorders (36.3%), sensitive disorders (36.3%), hyperreflexia and intracranial hypertension syndrome were the most frequent symptoms. Ten tumours affected the medulla, 7 the cerebral hemispheres, 2 the cerebral ventricles and 1 brain stem. Seven patients were submitted total resection of the tumor, from which one received adjuvant radiotherapy. 15 other patients were submitted to partial resection; from which 4 received adjuvant radiotherapy, 3 adjuvant chemotherapy and 1 chemotherapy and radiotherapy. The recurrence rate was 18.2%. These results are similar with the literature and may contribute to further understanding the biological behavior of these tumours.
Subject(s)
Brain Neoplasms/pathology , Ependymoma/pathology , Adolescent , Adult , Age Distribution , Brain Neoplasms/epidemiology , Brazil/epidemiology , Central Nervous System Neoplasms/epidemiology , Central Nervous System Neoplasms/pathology , Child , Child, Preschool , Ependymoma/epidemiology , Female , Humans , Infant , Male , Middle Aged , Neoplasm Recurrence, Local , Sex DistributionABSTRACT
OBJECTIVE: Describe the morbidity associated with a rare disease due to an embryological defect. METHODS: Retrospective revision of medical and necropsy reports. Bibliographic research using MEDLINE, LILACS and Index Medicus databases. RESULTS: 1 year-old male patient, admitted with generalized tonic-clonic seizures, evolving to deep coma and death in a few hours. Necropsy showed diffuse leptomeningeal malignant melanoma in brain stem, cerebellum, spinal cord and temporal lobe associated with a giant melanocytic nevus and satellite lesions. CONCLUSIONS: Neurocutaneous melanosis is a rare congenital syndrome characterized by the presence of large and/or multiple melanocytic nevi and pigmented tumors of the leptomeninges. It has a poor prognosis as demonstrated by the present report. It's physiopathology is believed to be due to a migration defect of the cells arising from the primitive neural crest. In these cases, an early diagnosis may improve the survival time.
ABSTRACT
We reviewed the cerebrospinal fluid (CSF) syndromes of 100 consecutive HIV-positive patients presenting acute consciousness compromise in emergency rooms, and correlated them with clinical data. The most frequent CSF syndromes were: absolute protein-cytological dissociation (21), viral (19), neurocryptococcosis (7), relative protein-cytological dissociation (6) and septic (4), moderate hypoglycorrachia (4), severe hypoglycorrachia (4) and hydroelectrolytic disturbance (3). One fifth of the patients had CSF syndromes considered sufficient for diagnosis or an immediate clinical decision. The most common clinical data were infective and neurological. There was little correlation between the clinical data and the CSF syndromes. We conclude that in HIV-positive individuals presenting acute consciousness disturbances there are frequently non-specific results in the CSF analysis that must be weighed against a detailed history and thorough physical examination. Taking this into account, in about one fifth of cases the CSF analysis can offer useful information for treatment.
Subject(s)
AIDS Dementia Complex/cerebrospinal fluid , AIDS Dementia Complex/diagnosis , HIV Seropositivity/cerebrospinal fluid , Adolescent , Adult , Aged , Female , Humans , Male , Middle AgedABSTRACT
Tuberous sclerosis complex is a group of autosomal disorders characterized by hamartomas and benign neoplastic lesions that invariably affect the central nervous system. We report a case of tuberous sclerosis that is the first presenting ultrastructural findings of this phacomatosis in the Latin American literature. The patient was a 2 year old girl presenting West syndrome non responsive to the clinical treatment with vigabatrin, trileptal and clonazepan, and undergoing left frontal lobectomy. The histopathological and ultrastructural findings were compatible with tuberous sclerosis. These results may help to further understand this controversial phacomatosis, warning to the clinical presentation as West syndrome.