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1.
Nature ; 506(7488): 339-42, 2014 Feb 20.
Artículo en Inglés | MEDLINE | ID: mdl-24553239

RESUMEN

Asymmetry is required by most numerical simulations of stellar core-collapse explosions, but the form it takes differs significantly among models. The spatial distribution of radioactive (44)Ti, synthesized in an exploding star near the boundary between material falling back onto the collapsing core and that ejected into the surrounding medium, directly probes the explosion asymmetries. Cassiopeia A is a young, nearby, core-collapse remnant from which (44)Ti emission has previously been detected but not imaged. Asymmetries in the explosion have been indirectly inferred from a high ratio of observed (44)Ti emission to estimated (56)Ni emission, from optical light echoes, and from jet-like features seen in the X-ray and optical ejecta. Here we report spatial maps and spectral properties of the (44)Ti in Cassiopeia A. This may explain the unexpected lack of correlation between the (44)Ti and iron X-ray emission, the latter being visible only in shock-heated material. The observed spatial distribution rules out symmetric explosions even with a high level of convective mixing, as well as highly asymmetric bipolar explosions resulting from a fast-rotating progenitor. Instead, these observations provide strong evidence for the development of low-mode convective instabilities in core-collapse supernovae.

2.
J Exp Med ; 169(6): 1895-901, 1989 Jun 01.
Artículo en Inglés | MEDLINE | ID: mdl-2659722

RESUMEN

A chemotactic protein for polymorphonuclear leukocytes (lung carcinoma-derived chemotaxin [LUCT]) was purified from culture fluid of the human lung giant cell carcinoma LU65C cells to electrophoretically homogeneous form through five sequential purification steps: DEAE-Sepharose, CM-Sepharose, HPLC on carboxyl-methylated-polyvinylalcohol resin, hydrophobic, and reversed-phase. The molecular mass was determined as approximately 10 kD by SDS-PAGE and isoelectric point was 10.7. The chemotactic activity (ED50 0.75 x 10(-9) M) was sevenfold more potent than that of FMLP (5 X 10(-9) M) and comparable with that of C5a (10(-9) M). NH2-terminal amino acid sequence and amino acid composition of LUCT strongly suggest that it may be closely related to the putative protein encoded by the cDNA clone (3-10C) and almost identical with a part of sequence of the chemotactic factor derived from stimulated human leukocytes in the 6th to 32nd, but not the NH2-terminal 5 amino acids. These results indicate that the carcinoma cells produce LUCT without any added stimulant and suggest that the previously isolated chemotactic monokines may correspond to des(1-5) of LUCT in the NH2-terminal region.


Asunto(s)
Carcinoma/análisis , Factores Quimiotácticos/aislamiento & purificación , Neoplasias Pulmonares/análisis , Proteínas de Neoplasias/aislamiento & purificación , Neutrófilos/fisiología , Secuencia de Aminoácidos , Aminoácidos/aislamiento & purificación , Carcinoma/patología , Línea Celular , Movimiento Celular , Factores Quimiotácticos/fisiología , Quimiotaxis de Leucocito , Humanos , Interleucina-8 , Neoplasias Pulmonares/patología , Datos de Secuencia Molecular , Homología de Secuencia de Ácido Nucleico
3.
J Cell Biol ; 121(2): 375-86, 1993 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-8468352

RESUMEN

We have analyzed the dynamics of neuronal intermediate filaments in living neurons by using the method of photobleaching of fluorescently-labeled neurofilament L protein and immunoelectron microscopy of incorporation sites of biotinylated neurofilament L protein. Low-light-level imaging and photobleaching of growing axons of mouse sensory neurons did not affect the rate of either axonal growth or the addition of intermediate filament structures at the axon terminal, suggesting that any perturbations caused by these optical methods would be minimal. After laser photobleaching, recovery of fluorescence did occur slowly with a recovery half-time of 40 min. Furthermore, we observed a more rapid fluorescence recovery in growing axons than in quiescent ones, indicating a growth-dependent regulation of the turnover rate. Incorporation sites of biotin-labeled neurofilament L protein were localized as numerous discrete sites along the axon, and they slowly elongated to become continuous arrays 24 h after injection. Collectively, these results indicate that neuronal intermediate filaments in growing axons turn over within the small area of the axoplasm possibly by the mechanism of lateral and segmental incorporation of new subunits.


Asunto(s)
Axones/metabolismo , Filamentos Intermedios/metabolismo , Proteínas de Neurofilamentos/metabolismo , Animales , Axones/ultraestructura , Biotina/metabolismo , Bovinos , Línea Celular/metabolismo , Línea Celular/ultraestructura , Filamentos Intermedios/ultraestructura , Ratones , Neuronas/metabolismo , Neuronas/ultraestructura
4.
Neuron ; 3(2): 229-38, 1989 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-2562784

RESUMEN

Two monoclonal antibodies, 5E6 and 1B6, were raised against microtubule-associated protein 1B (MAP1B), a major component of the neuronal cytoskeleton. 5E6 recognized the entire MAP1B population, while 1B6 detected only phosphorylated forms. Affinity-purified MAP1B appeared as a long, filamentous molecule (186 +/- 38 nm) with a small spherical portion at one end, forming long cross-bridges between microtubules in vitro. These results, together with in vivo data from immunogold methods, demonstrate that MAP1B is a component of cross-bridges between microtubules in neurons. By immunohistochemical analysis, phosphorylated forms were shown to exist mainly in axons, whereas unphosphorylated forms were limited to cell bodies and dendrites. Phosphorylated MAP1B was quite abundant in developing axons, suggesting its essential role in axonal elongation.


Asunto(s)
Proteínas Asociadas a Microtúbulos/metabolismo , Neuronas/metabolismo , Animales , Anticuerpos Monoclonales/inmunología , Cerebelo/citología , Cerebelo/crecimiento & desarrollo , Cerebelo/ultraestructura , Citoesqueleto/metabolismo , Citoesqueleto/ultraestructura , Técnica del Anticuerpo Fluorescente , Expresión Génica , Inmunohistoquímica/métodos , Microscopía Electrónica/métodos , Proteínas Asociadas a Microtúbulos/genética , Proteínas Asociadas a Microtúbulos/inmunología , Estructura Molecular , Sistema Nervioso/citología , Sistema Nervioso/metabolismo , Sistema Nervioso/ultraestructura , Neuronas/ultraestructura , Fosforilación , Ratas
5.
Science ; 358(6370): 1565-1570, 2017 12 22.
Artículo en Inglés | MEDLINE | ID: mdl-29038371

RESUMEN

With the first direct detection of merging black holes in 2015, the era of gravitational wave (GW) astrophysics began. A complete picture of compact object mergers, however, requires the detection of an electromagnetic (EM) counterpart. We report ultraviolet (UV) and x-ray observations by Swift and the Nuclear Spectroscopic Telescope Array of the EM counterpart of the binary neutron star merger GW170817. The bright, rapidly fading UV emission indicates a high mass (≈0.03 solar masses) wind-driven outflow with moderate electron fraction (Ye ≈ 0.27). Combined with the x-ray limits, we favor an observer viewing angle of ≈30° away from the orbital rotation axis, which avoids both obscuration from the heaviest elements in the orbital plane and a direct view of any ultrarelativistic, highly collimated ejecta (a γ-ray burst afterglow).

6.
Science ; 348(6235): 670-1, 2015 May 08.
Artículo en Inglés | MEDLINE | ID: mdl-25954004

RESUMEN

In core-collapse supernovae, titanium-44 ((44)Ti) is produced in the innermost ejecta, in the layer of material directly on top of the newly formed compact object. As such, it provides a direct probe of the supernova engine. Observations of supernova 1987A (SN1987A) have resolved the 67.87- and 78.32-kilo-electron volt emission lines from decay of (44)Ti produced in the supernova explosion. These lines are narrow and redshifted with a Doppler velocity of ~700 kilometers per second, direct evidence of large-scale asymmetry in the explosion.

7.
DNA Res ; 5(6): 327-34, 1998 Dec 31.
Artículo en Inglés | MEDLINE | ID: mdl-10048481

RESUMEN

We describe a novel mechanism of site-specific recombination in the unicellular marine cyanobacterium Synechococcus sp. PCC7002. The specific recombination sites on the smallest plasmid pAQ1 were localized by studying the properties of pAQ1-derived shuttle-vectors. We found that a palindromic element, the core sequence of which is G(G/A)CGATCGCC, functions as a resolution site for site-specific plasmid recombination. Furthermore, site-directed mutagenesis analysis of the element show that the site-specific recombination in the cyanobacterium requires sequence specificity, symmetry in the core sequence and, in part, the spacing between the elements. Interestingly, this element is over-represented not only in pAQ1 and in the genome of the cyanobacterium, but also in the accumulated cyanobacterial sequences from Synechococcus sp. PCC6301, PCC7942, vulcanus and Synechocystis sp. PCC6803 within GenBank and EMBL databases. Thus, these findings strongly suggest that the site-specific recombination mechanism based on the palindromic element should be common in these cyanobacteria.


Asunto(s)
Cianobacterias/genética , Plásmidos/genética , Recombinación Genética , Escherichia coli/genética , Eliminación de Gen , Vectores Genéticos , Modelos Genéticos , Señales de Clasificación de Proteína/análisis , Transformación Genética
8.
DNA Res ; 5(2): 127-9, 1998 Apr 30.
Artículo en Inglés | MEDLINE | ID: mdl-9679201

RESUMEN

We have determined the complete nucleotide sequence of pAQ1, the smallest plasmid of the unicellular marine cyanobacterium Synechococcus sp. PCC7002. The plasmid consists of 4,809 bp and has at least four open reading frames that potentially encode polypeptides of 50 or more amino acids. We found that a palindromic element, the core sequence of which is G(G/A)CGATCGCC, is over-represented not only in plasmid pAQ1 but also in the accumulated cyanobacterial genomic sequences from Synechococcus sp. PCC6301, PCC7002, PCC7942, vulcanus and Synechocystis sp. PCC6803 within GenBank and EMBL databases. It suggests that this sequence might mediate gene rearrangement, thus increasing genetic diversity, since recombination events are frequent in cyanobacteria.


Asunto(s)
Cianobacterias/genética , Plásmidos/genética , Secuencia de Aminoácidos , Proteínas Bacterianas/química , Proteínas Bacterianas/genética , Secuencia de Bases , Datos de Secuencia Molecular , Sistemas de Lectura Abierta , Plásmidos/química , Origen de Réplica
9.
Chem Phys Lipids ; 43(3): 161-77, 1987 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-3621383

RESUMEN

The effects of composition and molecular packing on the overall ionic property of mixed monolayer involving cationic, anionic and zwitterionic lipids were studied by measuring surface potential change when the concentration of sodium or calcium ions in the aqueous substrate was varied. Those lipids used were N,N-dimethyl-N-n-hexadecyl-n-octadecyl ammonium chloride (DDAC) as cationic lipid, stearic acid (StH) or sodium docosylsulfate (SDocS) as anionic lipid, and 1,2-dipalmitoyl-sn-glycero-3-phosphorylethanolamine (PE) and 1,2-distearoyl-sn-glycero-3-phosphorylcholine (PC) as ampholytic lipids. For the equimolar mixture of StH and DDAC, the reversal of apparent charge is observed when molecular packing exceeds 3.25 X 10(-10) M/cm2. The effect is rendered to the discreteness of cationic and anionic charges in the monolayer. It was also found that the addition of 30% of PC drastically changes the ionic property of PE closer to that of PC.


Asunto(s)
Liposomas , Aniones , Cationes , Ácidos Grasos , Potenciales de la Membrana , Modelos Biológicos , Conformación Molecular , Fosfolípidos , Compuestos de Amonio Cuaternario , Ácidos Esteáricos
10.
J Biosci Bioeng ; 92(1): 24-9, 2001.
Artículo en Inglés | MEDLINE | ID: mdl-16233052

RESUMEN

Synthesis of phytochelatins (PCs), heavy-metal-sequestering peptides, in the marine green alga, Dunaliella tertiolecta, was evaluated under various conditions of exposure to heavy metals. To investigate the effect of heavy metals on both PC synthesis and their upstream biosynthetic reactions, an ion-pair-HPLC system was developed in this study, by which PCs and their biosynthetic intermediates, cysteine (Cys), gamma-glutamylcysteine (gammaEC) and glutathione (GSH), could be determined simultaneously with high sensitivity. When the cells were exposed to Zn2+, the level of PCs was maximal at 200 microM and significantly higher than that obtained after exposure to 400 microM Cd2+, which is the strongest inducer of PC synthesis in higher plants in vivo and in vitro as well as in microalgae. The predominant PC subtype was PC4, followed by PC3 and PC5, whereas PC2, which is generally abundant in higher plants, has the lowest level among PC2 to PC5. These results suggest that the characteristics of PC synthase in D. tertiolecta including the requirement of heavy metals for its catalysis and substrate specificity towards GSH and PC(n) are considerably different from those in higher plants and other algae. While PC synthesis proceeded in the heavy-metal-treated cells, the level of GSH did not appreciably change. To maintain the same size of the GSH pool, GSH must be newly synthesized to balance the amount consumed for PC synthesis.

11.
Ann Otol Rhinol Laryngol ; 110(2): 122-6, 2001 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-11219518

RESUMEN

We produced drill-induced damage of the auditory ossicles of guinea pigs to study changes over time in the permeability of the blood vessels of the stria vascularis to horseradish peroxidase (HRP). In group A, the stimulus was applied for 10 seconds after intravenous injection of HRP. In group B, it was applied for 30 seconds, and in group C, for 60 seconds. The cochlea was fixed with 2% glutaraldehyde perfused through the round window, and the guinea pigs were then decapitated. The stria vascularis of the basal and third turns was examined. The leakage of HRP from the blood vessels of the stria vascularis significantly increased in relation to the duration of the stimulus in both the basal and third turns. The damage to intermediate cells also tended to be in relation to the duration of the stimulus. Extravascular permeation of HRP took place through the tubules in the endothelial cytoplasm. The vibratory stimulation presumably opened channels that are not normally open.


Asunto(s)
Permeabilidad Capilar/fisiología , Conducto Coclear/irrigación sanguínea , Conducto Coclear/ultraestructura , Modelos Animales de Enfermedad , Yunque/lesiones , Estría Vascular/ultraestructura , Vibración/efectos adversos , Animales , Oído Medio/cirugía , Endotelio Vascular/ultraestructura , Cobayas , Pérdida Auditiva Sensorineural/etiología , Peroxidasa de Rábano Silvestre/administración & dosificación , Peroxidasa de Rábano Silvestre/farmacocinética , Inyecciones Intravenosas , Complicaciones Posoperatorias/etiología , Factores de Tiempo
12.
DNA Seq ; 12(4): 281-4, 2001 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-11916262

RESUMEN

Recently, we have cloned and analyzed two polyhydroxyalkanoate (PHA) synthase genes (phaC1 and phaC2 in the pha cluster) from Pseudomonas aureofaciens. In this report, the deduced amino acid (AA) sequences of PHA synthase 1 and PHA synthase 2 from P. aureofaciens are compared with those from three other bacterial strains (Pseudomonas sp. 61-3, P. oleovorans and P. aeruginosa) containing the homologous pha cluster. The level of homology of either PHA synthase 1 or PHA synthase 2 was high with each enzyme from these three bacterial strains. Furthermore, multialignment of PHA synthase AA sequences implied that both enzymes of PHA synthase 1 and PHA synthase 2 were highly conserved in the four strains including P. aureofaciens.


Asunto(s)
Aciltransferasas/genética , Proteínas Bacterianas/genética , Pseudomonas/genética , Secuencia de Aminoácidos , Isoenzimas/genética , Datos de Secuencia Molecular , Alineación de Secuencia , Homología de Secuencia
13.
Jpn J Ophthalmol ; 43(6): 517-21, 1999.
Artículo en Inglés | MEDLINE | ID: mdl-10672882

RESUMEN

PURPOSE: To report and discuss cases of lamellar keratoplasty using corneas obtained during previous penetrating keratoplasty in keratoconus eyes. METHODS: Corneal buttons were obtained from 7 keratoconus patients and stored in a preserving solution for 7-60 days (average, 32.4 days) before use. The recipient eyes comprised recurrent pterygium 3 eyes, primary pterygium 1 eye, pseudopterygium 1 eye, corneal perforation with iris prolapse due to fungal corneal ulcer 1 eye, and limbal dermoid 1 eye. RESULTS: The recipient eyes ran favorable courses in general. Graft rejection developed in 2 eyes and was successfully treated with topical and systemic corticosteroid. CONCLUSIONS: Preserved corneas from keratoconus eyes were found useful in therapeutic lamellar keratoplasty. By this procedure, the current inadequate supply of donor corneas in eye banks in Japan can be augmented.


Asunto(s)
Córnea , Enfermedades de la Córnea/cirugía , Trasplante de Córnea/métodos , Criopreservación , Conservación de Tejido , Adolescente , Adulto , Anciano , Niño , Sulfatos de Condroitina , Mezclas Complejas , Medio de Cultivo Libre de Suero , Dextranos , Bancos de Ojos , Femenino , Gentamicinas , Humanos , Queratocono/cirugía , Masculino , Persona de Mediana Edad , Recurrencia , Donantes de Tejidos , Obtención de Tejidos y Órganos/métodos , Resultado del Tratamiento
14.
Nippon Ganka Gakkai Zasshi ; 99(6): 735-8, 1995 Jun.
Artículo en Japonés | MEDLINE | ID: mdl-7611011

RESUMEN

We treated sclero-corneomalacia in an 84-year-old woman, which occurred 7 months after cataract surgery on her left eye. Since medical therapy could not prevent progress of the sclero-corneal lesion and perforation of the eyeball was likely, we performed implantation of preservable sclera and keratoepithelioplasty. The patient has an uneventful postoperative course without recurrence and good visual acuity was preserved. To minimize the post-operative inflammation and early reform of the ocular surface, implantation of preservable sclera and keratoepithelioplasty is an effective procedure.


Asunto(s)
Extracción de Catarata/efectos adversos , Enfermedades de la Córnea/etiología , Enfermedades de la Esclerótica/etiología , Anciano , Anciano de 80 o más Años , Enfermedades de la Córnea/cirugía , Trasplante de Córnea , Femenino , Humanos , Complicaciones Posoperatorias , Esclerótica/trasplante , Enfermedades de la Esclerótica/cirugía
15.
Nippon Ganka Gakkai Zasshi ; 100(11): 905-10, 1996 Nov.
Artículo en Japonés | MEDLINE | ID: mdl-8953896

RESUMEN

Therapeutic lamellar keratoplasty was performed using corneas obtained from keratoconus patients undergoing penetrating keratoplasty. The corneas used in this series were stored in preservation solution for 7 to 59 days (average, 27.8 days) and submitted to surgery. The recipients were three patients with recurrent pterygium, one with primary pterygium, one with corneal perforation and iris prolapse due to fungal corneal ulcer, and one with limbal dermoid. Graft rejection developed in two cases postoperatively, but they were successfully treated with steroid therapy. During the entire period of clinical observation, there was no sign of recurrence of pterygium. In the case of the fungal corneal ulcer, the site of perforation healed quickly and the donor cornea maintained its transparency. A marked cosmetic improvement was achieved in the case of the limbal dermoid. Obtaining corneas from keratoconus patients and storing them for a short period is a potentially useful application for therapeutic lamellar keratoplasty.


Asunto(s)
Córnea , Trasplante de Córnea , Queratocono , Preservación de Órganos , Adulto , Anciano , Niño , Bancos de Ojos , Femenino , Humanos , Masculino , Persona de Mediana Edad
16.
Nippon Ganka Gakkai Zasshi ; 101(9): 711-7, 1997 Sep.
Artículo en Japonés | MEDLINE | ID: mdl-9311230

RESUMEN

We report 2 cases of multifocal choroiditis associated with subretinal fibrosis on whom the fluorescein fundus angiography (FAG) and the indocyanine green infrared angiography (IA) were performed. Case 1 was a moderately myopic 14-year-old girl who had no ocular symptoms. She had numerous small, round, discrete, partially conflued lesions with subretinal fibrosis scattered in the periphery and one discrete relatively large lesion along the superotemporal arcade in her left fundus. Subretinal fibrosis had progressed in the superior lesion. Some lesions had coalesced into a sword-like pattern over a period of 2 years. Case 2 was a high myopic 18-year-old man who had distorted vision in his right eye. He had some small whitish round lesions with one small choroidal neovascular tissue and subretinal fibrosis in the posterior pole and a sword-like lesion in the inferior periphery. Another choroidal neovascular tissue developed near the macula during the 6-month follow-up period. In FAG, the centers of the lesions hypofluoresced corresponding to the pigmentation and the edges hyperfluoresced. Some of the lesions showed window-defects and others tissue-staining. In IA, all the whole lesions hypofluoresced from an early stage of the angiography and some major choroidal vessels were visible through them. The hypofluorescent areas persisted into the late phase. The hypofluorescent areas of the IA were larger than those seen in FAG and in funduscopy. These findings indicate that the choriocapillaris was initially damaged and consequently the structures of the lesions partly disappeared at the level of the retinal pigment epithelium-choriocapillaris complex.


Asunto(s)
Coroiditis/patología , Adolescente , Coroides/patología , Femenino , Fibrosis , Angiografía con Fluoresceína , Fondo de Ojo , Humanos , Masculino , Neovascularización Patológica , Retina/patología
17.
Nippon Ganka Gakkai Zasshi ; 99(9): 1036-44, 1995 Sep.
Artículo en Japonés | MEDLINE | ID: mdl-7484505

RESUMEN

We report 2 unusual cases of congenital occipital hemianopsia associated with abnormal retinal correspondence (ARC)-exotropia. Two Japanese males, 25 and 28 years of age, visited our hospital for surgical correction of manifest exotropia with dissociated vertical deviation and overaction of the superior oblique muscle. Visual field examination demonstrated homonymous hemianopsia with approximately 5 to 10 degrees of macular sparing. Visually evoked potential examination showed small amplitude similar to dissociated vertical deviation patients in all half-field stimulation. Electrooculogram examination demonstrated defective pursuit to the ipsilateral side. Some investigators have speculated that progressive exotropia compensates for homonymous hemianopsia and is a rare contraindication for strabismus surgery. To confirm the deterioration of motor and visual functions before surgery in these patients, we tried injections of botulinum A type toxin into the lateral rectus muscle ipsilateral to the hemianopsia. Contrary to the hypothesis, our patients had no change in their binocular visual fields and visual function. Finally, we performed recession of the lateral rectus muscle, so that both patients were satisfied with their ocular alignment, with no marked change of visual behavior.


Asunto(s)
Toxinas Botulínicas/uso terapéutico , Exotropía/terapia , Hemianopsia/complicaciones , Retina/fisiopatología , Adulto , Potenciales Evocados Visuales , Exotropía/fisiopatología , Hemianopsia/congénito , Humanos , Masculino , Campos Visuales
18.
No To Shinkei ; 46(9): 873-8, 1994 Sep.
Artículo en Japonés | MEDLINE | ID: mdl-7999446

RESUMEN

Event-related brain potentials associated with both somatosensory and auditory detection tasks were recorded from the scalp in a 63-year-old man with finger agnosia and right-left disorientation resulted from cerebral infarction located around the region of the left angular gyrus. In a somatosensory oddball paradigm for discrimination between the right index finger (target, P = 0.2) and the left index finger (non-target, P = 0.8), early cognitive components P100, N140 and processing negativity were observed both in the target responses and non-target responses, however, no P300 component was recorded in both paradigms. In another paradigm to discriminate the index finger (target, P = 0.2) from small finger (non-target, P = 0.8) in the right hand, no P300 was evoked and only processing negativity was recorded both in the target responses and nontarget responses. These phenomena were obtained through characteristic behavior that the patient paid attention to the target stimuli as well as non-target stimuli and mentally counted them without discrimination. In contrast, in an auditory oddball paradigm P300 was clearly demonstrated to target stimuli that the patient correctly could count. The normal appearance of P300 in auditory detection tasks strongly suggests that the neural generator for the P300 was reserved intact. These results indicate that somatosensory information processing might be disturbed at the level of sensory discrimination needed for generation of P300 in our patient. It is also proposed that the neural events for somatosensory discrimination and auditory discrimination would take separate channels each other to reach the common generator of p300 in information processing.


Asunto(s)
Encéfalo/fisiopatología , Potenciales Evocados Auditivos , Potenciales Evocados Somatosensoriales , Síndrome de Gerstmann/fisiopatología , Infarto Cerebral/complicaciones , Infarto Cerebral/fisiopatología , Síndrome de Gerstmann/etiología , Humanos , Masculino , Persona de Mediana Edad
19.
No To Shinkei ; 48(5): 487-95, 1996 May.
Artículo en Japonés | MEDLINE | ID: mdl-8672309

RESUMEN

We report a right-handed 78-year-old man with early onset parkinsonism. The patient had an onset of micrographia at 23 years of the age in 1939. Seven years later he started to drag his right foot, and at 38 years of age, he walked with small steps with festination. Tremor was also present in his right hand. His daily life was independent as a otorhinolaryngologist. He visited our clinic on March 24, 1977 when he was mentally sound and showed mild parkinsonism consisting of masked face, stooped posture, small step gait, bradykinesia, and right side dominant rigidity and tremor. He showed good response to trihexyphenidyl and amantadine HCl. Two month later, he developed dyskinesia and some worsening of parkinsonism, and was admitted to our hospital for the first time. He was treated with 400 to 600 mg/day of levodopa/ carbidopa. He showed marked improvement, however, dyskinesia remained in his mouth. He was doing well until 77 years of age (June of 1993) when he developed hallucination and motor fluctuations. He was admitted again to our hospital on June 22, 1993. On admission, he was alert and appeared mentally sound. However, Hasegawa dementia scale was 18/30. Upward gaze was slightly restricted (3/5). Voice was somewhat small but no masking was noted. His posture was stooped and the gait was of small step. Dyskinesia was noted during walk. No rigidity nor tremor was noted. Deep tendon reflexes were lost but no sensory loss or motor weakness was noted. Routine laboratory studies were unremarkable. A cranial CT scan revealed only mild to moderate cortical atrophy. Motor and sensory conduction velocities were within normal limits, however, motor action potentials could not be obtained with stimulation to the right common peroneal nerve. He was treated with 600 mg/day of levodopa with carbidopa, 100 mg of amantadine HCl, 300 mg of Dops, and 25 mg of tiapride. He continued to show motor fluctuations, and was discharged on July 23, 1993. Since then his motor functions had become progressively worse with frequent falls, but he was still able to walk without support. On October 3 of 1994, he went to bed as usual. On the next morning, he was found dead in his bed at 9: 30. The patient was discussed in neurological CPC, and the chief discussant arrived at the conclusion that the patient had young-onset Parkinson's disease with Lewy bodies in the substantia nigra. Opinions were divided between Parkinson's disease and Lewy body negative young onset parkinsonism. Postmortem examination revealed obstruction of the trachea by aspirated foods, and the cause of death appeared to have been suffocation by the foods. Macroscopically, the external appearance of the brain was unremarkable except for slight frontal atrophy. The substantia nigra showed depigmentation in the lateral part, but the pigmentation of the medial part was well preserved. Upon histologic examination, the number of pigmented neurons in the dorsomedial part was well preserved. In the lateral part, pigmented neurons were well preserved in the dorsal area, however, in the ventral area, only non-pigmented neurons were seen; they appeared to be neurons in the pars reticulata. No gliosis was seen in any of the nigral areas. No Lewy bodies were seen in the remaining neurons. So-called immature neurons with rounded shape without neuromelanin could not be detected. The locus coeruleus neurons were well preserved. The putamen and the other basal ganglia structures were also intact. Slight myelin pallor was noted in the subcortical white matter, however, otherwise cerebral cortices were normal. The histology of this patient is unique in that only the ventrolateral part of the substantia nigra showed abnormal finding consisting of lack of pigmented neurons without gliosis. It is not clear whether the nigral change represents degeneration or a congenital "hypoplasia'. To our knowledge, such a unique pathology of the substantia nigra has not been reported in the literature. Our patient ma


Asunto(s)
Muerte Súbita/patología , Enfermedad de Parkinson/patología , Edad de Inicio , Anciano , Diagnóstico Diferencial , Humanos , Masculino , Neuronas/patología , Enfermedad de Parkinson/diagnóstico , Sustancia Negra/patología
20.
No To Shinkei ; 46(11): 1101-11, 1994 Nov.
Artículo en Japonés | MEDLINE | ID: mdl-7873285

RESUMEN

We report a 65-year-old woman with progressive multiple cranial neuropathy. She had been suffered from bronchial asthma since 1979 for which prednisolone had been prescribed. She noted an onset of pain around her nose in October, 1989, which extended into the periorbital regions bilaterally. In February, 1990, she was treated with stellate ganglion block and trigeminal nerve block; these treatments partially alleviated her pain. In May of 1991, she noted a difficulty in swallowing solid foods. In November of the same year, she developed right facial paresis; two weeks later, she noted numbness in her left face, and was hospitalized to our service on December 16, 1991. On admission, she was afebrile and general physical examination was unremarkable except for piping rales in her both lung fields. On neurologic examination, she was alert and oriented to all spheres; higher cerebral functions were intact. In the cranial nerves, her olfactory sense was lost bilaterally; her vision was markedly diminished bilaterally only to recognize hand movements; the optic fundi appeared normal; the pupils were isocoric and reacted to light promptly. The extraocular muscles were moderately weak to most of the directions more on the left; no nystagmus was present. Facial sensation was diminished bilaterally; the jaw deviated to right; right facial paresis of peripheral type was present; her hearing was diminished bilaterally more on the right. The movement of the soft palate was diminished on the right side; dysphagia was present; her voice was horse; the gag reflex was diminished. The sternocleidomastoid muscle was weak bilaterally; the tongue appeared normal. Examination of gait was differed because of headache, however, no apparent motor weakness was present. No ataxia or involuntary movement was noted. Deep reflexes were normally elicited and symmetric. Plantar response was flexor. Sensation in the extremities was intact. Kernig's sign was positive at 70 degree leg extension; eyeball tenderness was also present bilaterally, however, no nuchal stiffness was noted. Following abnormalities were present in the laboratory examination: WBC 11,400/microliters, ESR 50 mm/hr, CRP 6.1 mg/dl. The lumbar CSF was under a normal pressure containing 29 WBC/microliters (neutrophils 7, lymphocytes 20, others 2), 67 mg/dl of protein, and 53 mg/dl of sugar; cultures for acid-fast bacilli as well as for other bacteria were negative; no malignant cells were found. A cranial CT scan revealed an isodensity mass in the orbit and ill-defined low density areas in the white matters of the frontal lobes.(ABSTRACT TRUNCATED AT 400 WORDS)


Asunto(s)
Encéfalo/patología , Enfermedades de los Nervios Craneales/etiología , Dolor Facial/etiología , Cefalea/etiología , Anciano , Encéfalo/diagnóstico por imagen , Angiografía Cerebral , Femenino , Fibrosis , Humanos , Esclerosis , Tomografía Computarizada por Rayos X
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