[Brain dynamics according to Justo Gonzalo].
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History, 20th Century↗
Biomedical subjects
I Gonzalo
Publications and source records attributed to I Gonzalo.
From nearly tilted waves to cavity phase solitons in broad area lasers with squeezed vacuum.
Phase domains and phase solitons in two-level amplifying media damped by a squeezed vacuum are predicted for the first time. Two different types of pattern formation are found depending on the relative value of the cavity detuning to the squeezed parameter: the usual one in lasers via a supercritical Hopf bifurcation and a new one via pitchfork bifurcation.
Journal Article↗
[The neuroscientific work of Justo Gonzalo (1910-1986): the center syndrome and reversal metamorphopsia].
The Spanish neuroscientist Justo Gonzalo Rodriguez-Leal (Barcelona 1910, Madrid 1986) carried out different studies on cerebral functions, highlighting those made in patients with encephalic injuries suffered during the Spanish civil war. His book "Investigaciones sobre la nueva dinámica cerebral. La actividad cerebral en función de las condiciones de excitabilidad nerviosa", published in two volumes (the first one in 1945 and the second one five years later), gathers some of his fundamental contributions, among which the so-called central syndrome stands out. A dominant parietal lesion (central) equidistant from the visual, sensorial and auditory projection areas can lead to diverse perceptive dysfunctions, among them inversions in visual, tactile and acoustic perception. As the lesion becomes more peripheral, the resulting defect will be more unisensorial and crossed, while when it approaches the central region, the disorders will be bilateral and polysensorial. Justo Gonzalo explained all these phenomena later by a gradient system.
History, 20th Century↗
Clinical genetics of familial progressive supranuclear palsy.
Recent studies have shown that progressive supranuclear palsy (PSP) could be inherited, but the pattern of inheritance and the spectrum of the clinical findings in relatives are unknown. We here report 12 pedigrees, confirmed by pathology in four probands, with familial PSP. Pathological diagnosis was confirmed according to recently reported internationally agreed criteria. The spectrum of the clinical phenotypes in these families was variable including 34 typical cases of PSP (12 probands plus 22 secondary cases), three patients with postural tremor, three with dementia, one with parkinsonism, two with tremor, dystonia, gaze palsy and tics, and one with gait disturbance. The presence of affected members in at least two generations in eight of the families and the absence of consanguinity suggests autosomal dominant transmission with incomplete penetrance. We conclude that hereditary PSP is more frequent than previously thought and that the scarcity of familial cases may be related to a lack of recognition of the variable phenotypic expression of the disease.
Adult↗
[Diagnosis of transmissible spongiform encephalopathies in Spain. Population perspective].
OBJECTIVES: To describe specific aspects of the diagnostic process of transmissible spongiform encephalopathies in Spain and to evaluate the 14-3-3 protein test in cerebrospinal fluid. METHODS: The annual pattern of diagnostic certainty as well as those of demand and results of biochemical and genetic studies were studied using two sets of patients, those diagnosed for the 1993-1998 period, notified to a National Creutzfeldt-Jakob Disease Register (NCDJR), and those referred to the Tissue Bank for Neurological Research Laboratory (TBNRL). The 14-3-3 protein test was validated taking as a reference two clinical populations. RESULTS: Two-hundred and four Creutzfeldt-Jakob disease cases were registered at the NCDJR: 39 out of them and 28 other patients had been studied at the TBNRL. The proportion of definite Creutzfeldt-Jakob Disease cases decreased since 1996. Among those registered in 1997-1998, 35.5%, 36% and 20% had undergone 14-3-3 protein in LCR, histopathologic and genetic studies. The 14-3-3 test grave, for definite, sporadic Creutzfeldt-Jakob disease as compared for patients with other dementing disorders, the following data: 12/13 sensitivity; 33/35 specificity; and 12/14 and 33/34 predictive values of positive and negative test. Two familial cases were diagnosed by identification of mutations in the TBNRL. CONCLUSIONS: The results suggest that: a) the diagnostic certainty of Creutzfeldt-Jakob disease in Spain decreased due to a drop in autopsy rates; b) the 14-3-3 cerebrospinal fluid test has a high diagnostic value, and its use diffused rapid but incompletely; c) genetic studies are useful in some cases, and d) Creutzfeldt-Jakob disease undereporting may be considerable. Creutzfeldt-Jakob disease diagnosis and surveillance, are closely related and being consolidated in Spain.
Creutzfeldt-Jakob Syndrome↗
[Clinico-morphologic reconsideration of anaplastic lymphoma. Retrospective study of 60 patients with aggressive clinical course and multiple recurrences].
PURPOSE: To identify anaplastic large cell lymphoma Ki-1+ (ALCL-Ki-1+) among a group of patients with aggressive Hodgkin's disease (HD) and to know the biological behaviour of the neoplasia (ALCL-Ki-1+). PATIENTS AND METHODS: Biopsies and clinical data of sixty patients with previous morphological diagnosis of HD lymphocytic depletion (LD), syncytial variant of nodular esclerosis (NE-II) and other subtypes of HD with aggressive clinical features were reviewed. A morphological, immunohistochemical (IHQ), proliferative and flow cytometric (FCM) studies were performed in lymph node biopsies. RESULTS: Morphological study and IHQ identify three groups: 15 patients (25%) lymphocytic predominance (LP) HD, 36 (60%) ALCL-Ki-1+ and 9 (15%) of non-Hodgkin's lymphoma (NHL) and unclassifiable cases. Nine cases of LP show anaplastic and variable immunophenotype being the rest of B-cell nature. 75% of LP patients showed long survival and frequent second neoplasias (47%). ALCL-Ki-1+ group had good initial response to therapy (84%) and multiple relapses, 67% showed CD15 positive marker (the so-called ALCL-HD related). CONCLUSIONS: A retrospective study of a selected group of patients previously diagnosed of aggressive HD showed different pathological subtypes: LP, LP with anaplastic areas, ALCL-Ki-1+ Hr (Hodgkin's related) and ALCL-Ki-1+ (classical type), all of them were CD30+, which could represent different stages of the same neoplasia.
Adolescent↗
[Expression of latent membrane protein (LMP) in large-cell anaplastic lymphomas].
PURPOSE: The presence of Epstein-Barr virus (EBV)-encoded latent membrane protein (LMP) was investigated in 40 cases of lymphoproliferative diseases which include Hodgkin's disease (HD), anaplastic large cell lymphoma (ALCL) and non-Hodgkin's lymphoma (NHL) of B and T-cell nature. MATERIAL AND METHODS: All cases were immunophenotyped in paraffin-embedded lymph node tissues, with a complete panel of monoclonal antibodies against B-cells, T-cells, histiocytes, activation and proliferation markers and classified as: 24 anaplastic large cell lymphoma (ALCL, 8 classical type and 16 ALCL-HD related), 10 lymphocyte predominant HD (LP, 5 classical type and 5 with ALCL areas), 4 NHL (two T-Cell type and 2 T-cell rich B-cell NHL). Immunohistochemistry techniques were performed ABC-complex and phosphatase alkaline anti-phosphatase alkaline (APAAP). RESULTS: LMP was detected in 35% (14/40) of total cases. In LP group one third of cases were LMP+. In ALCL-HD related cases 44% were LMP+ versus 13% in ALCL group. All LMP cases were CD30+ except one NHL-T and a T-cell rich B-cell NHL. The predominant immunophenotype was LMP+/CD20+ (57%) versus LMP+/CD20-. Most cases were of B-cell (36%) lineage. Null ALCL cases were LMP-. CONCLUSIONS: LMP, the most oncogenic EBV protein could play a pathogenic role in lymphoproliferative processes. It is not exclusive of HD and appears in other NHL preferentially of B-cell nature, above all in ALCL cases relating the two neoplasias HD and ALCL, both CD30 positive.
Herpesvirus 4, Human↗
[Large-cell anaplastic lymphomas: a genetic and immunophenotypic study].
PURPOSE: To characterize from a genetic point of view a group of non-Hodgkin's anaplastic large-cell lymphomas (ALCL) by Southern blot and PCR methods with different probes (molecular study) and with direct or post 24-78 hours cultures with GTC banding techniques (cytogenetic). To correlate the results to the immunophenotype performed with a complete panel of monoclonal antibodies (MoAb) according to avidin-biotine and alkaline phosphatase (APAAP) methods. MATERIAL AND METHODS: Sixty cases of ALCL were reviewed and only 19 selected (with frozen or fresh material) because a complete immunohistologic and genotypic correlation had been done. According to CD15 expression two groups were considered, CD15+ (the so-called Hodgkin's related or Hodgkin's like) and CD15- or classical type. RESULTS: Only 26.5% of cases showed immuno -genotypic correlation. Immunohistochemistry is an accurate method for activation, proliferation and B-cell nature, but T-cell cases were not stained because T-cell paraffin markers are not completely specific. CD15 group had only 30% rearranged cases with scarce cytogenetic abnormalities, as it occurs in Hodgkin's disease (HD). ALCL classical type showed 66% rearranged cases, and one of the T-cell cases showed an incomplete t (2:5) translocation or polyploid cell lines. CONCLUSIONS: Both groups have different genetic and immunophenotypic behaviour which resembles HD or NHL. CD15 positive cases or ALCL HD-related constitute a borderline entity which has to be recognized because of the different therapy and clinical behaviour.
Hodgkin Disease↗
[T-cell-rich B-cell lymphoma: multifactorial study of 4 cases].
PURPOSE: With the correlational study of four cases in several areas (clinic, morphoimmunologycal, ultrastructural and genetic) we try to valorate the still controversial entity known as T-cell rich B-cell lymphoma (TRBL), and stablish some useful clues in order to settle down the differential diagnosis between TRBL, Hodgkin's disease (HD), and T-cell non-Hodgkin's lymphomas (TNHL). PATIENTS AND METHODS: Cases proceeded from Oncology Department, and had been firstly misdiagnosed either as HD (3 cases) or as TNHL (1 case). Biopsies were processed and stained in routine way, H&E, Giemsa and Wilder. Immunohystological study, using monoclonal antibodies against B-cells, T-cells, histiocytes, activation and proliferation markers, was also performed with avidin biotine peroxidase (ABC) method. Ultrastructural study was performed in three of the cases; two patients were studied by PCR and Southern blot. RESULTS: All of the cases showed a diffuse hystological pattern, with variable fibrosis, and proliferation of venules and capillaries. Small lymphoid cells, being positive for CD3, were dominant. Large blastic cells, positive for CD20, some of them with a Sternberg-like appearance, could be found, in a spitty pattern. Histiocytes were abundant and positive to CD68. Proliferation index (Ki-67) ranged between 13 and 24.5% being the stain mainly positive for B-cells and in a certain extent, also for T-cells. Ultrastructural features were closer to those of the NHL than to the ones found in HD. Molecular study failed to prove any rearrangement. CONCLUSIONS: TRBL is a rare entity between B-cell NHL group. Diagnosis and differential diagnosis (mostly with HD and T-cell NHL) have to be properly made, because of the very distinct prognosis and therapy.
Adult↗
[Biology of secondary nodal follicular b-lymphomas. A patho-dynamic study].
PURPOSE: To assess the aggressivity factors and tumour prognosis in a series of non-Hodgkin lymphomas by the use of computer-quantified specific antibodies and flow cytometry. PATIENTS AND METHODS: Sixty-one cases of follicular B-cell lymphoma: 34 of the germinal centre (24 centroblastic-centrocytic, CB-CC, and 10 centroblastic, CC) and 27 of the follicular cortex (FCL), were studied. All the cases had been diagnosed between 1971 and 1992 at the Pathology Department of the Fundación Jiménez Díaz. Morphologic and immunophenotype diagnosis was made on each case. Tumour proliferation studies were performed after labelling nuclei with proliferent nucleic acid-associated protein (PC 10); the positive nuclear areas were later quantified in a CAS-200 image analyser by means of proliferation programmes (PI) and nuclear receptors (ER). A flow cytometry study of ploidy was carried out on each case. The values attained were correlated with survival in months. The Wilcoxon's test for independent variables was used for the statistical study. RESULTS: The PI programme showed lowest proliferation for FCL (7.4%) followed by intermediate proliferation in CB-CC (16.9%) and high proliferation in CB (31.7%). The PC-10 results attained with both PI and ER programmes showed statistically significant correlation (p < 0.01). The correlation between ploidy, as quantified by flow cytometry, and survival showed statistically significant differences between diploid and aneuploid cases (p < 0.01); similar findings apply for diploids and diploids with high synthesis phase (p < 0.01). CONCLUSION: These findings support the usefulness of cell kinetics studies in lymphoma, and contribute to validate different evolutive patterns in accordance with the histologic subtype.
Biomarkers, Tumor↗
[A tissue bank for neurologic research, Madrid].
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Alzheimer Disease↗